Diagnosis For Physical Therapists - Davenport, Todd E. (SRG)

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1528_FM_i-xxxii 08/05/12 6:06 PM Page i

Diagnosis for
PHYSICAL
THERAPISTS
A Symptom-Based Approach
1528_FM_i-xxxii 08/05/12 6:06 PM Page ii

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1528_FM_i-xxxii 08/05/12 6:06 PM Page iii

Diagnosis for
PHYSICAL
THERAPISTS
A Symptom-Based Approach

Todd E. Davenport, PT, DPT, OCS James Gordon, PT, EdD, FAPTA
Assistant Professor Associate Dean and Chair
University of the Pacific University of Southern California
Department of Physical Therapy Division of Biokinesiology and Physical
Thomas J. Long School of Pharmacy and Therapy
Health Sciences Los Angeles, California
Stockton, California
Hugh G. Watts, MD
Kornelia Kulig, PT, PhD, FAPTA Adjunct Associate Professor
Associate Professor of Clinical Physical University of Southern California
Therapy Division of Biokinesiology and Physical
University of Southern California Therapy
Division of Biokinesiology and Physical Los Angeles, California
Therapy
Los Angeles, California

Chris A. Sebelski, PT, DPT, OCS, CSCS


Assistant Professor
Saint Louis University
Department of Physical Therapy & Athletic
Training
Doisy College of Health Sciences
Saint Louis, Missouri
1528_FM_i-xxxii 08/05/12 6:06 PM Page iv

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Library of Congress Cataloging-in-Publication Data

Diagnosis for physical therapists : a symptom-based approach / Todd E. Davenport ... [et al.].
p. ; cm.
Includes bibliographical references and index.
ISBN 978-0-8036-1528-1 — ISBN 0-8036-1528-0
I. Davenport, Todd E.
[DNLM: 1. Diagnostic Techniques and Procedures. 2. Physical Therapy Modalities. 3. Signs and Symptoms.
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1528_FM_i-xxxii 08/05/12 6:06 PM Page v

Preface

Direct access to physical therapy is a central The purpose of this book is to present a
tenet of the American Physical Therapy Asso- framework and body of content necessary to
ciation’s Vision 2020 statement. Vision 2020 support the acquisition and maintenance of
anticipates physical therapists being “recog- diagnostic reasoning skills for physical thera-
nized by consumers and other health care pro- pists. Special emphasis in this book is placed
fessionals as the practitioners of choice to on diagnostic reasoning at the level of pathol-
whom consumers have direct access for the ogy. This book proposes a detailed approach
diagnosis of, interventions for, and prevention for diagnosis at the level of pathology, based
of impairments, functional limitations, and on the notion that physical therapists must be-
disabilities related to movement, function, gin with the patient’s symptoms and signs as
and health.”1 One clear assumption of Vision the starting point for reasoning. The book’s
2020 – with its emphasis on consumer direct format supports the needs of both clinicians
access – is that physical therapists must func- who must make rapid decisions in the context
tion autonomously to determine whether of a busy clinic environment, as well as stu-
physical therapy is appropriate for their pa- dents who are in the initial stages of learning
tients. This function involves being able to de- and refining their diagnostic reasoning skills.
cide whether to begin physical therapy, refer
their patient to another health care provider For established clinicians:
for additional consultation or management, or ● The diagnostic reasoning framework pre-
both. If the decision to refer to another health sented in the book encourages a systematic
care provider is made, then the appropriate yet flexible approach to diagnostic reasoning
disposition and urgency must be established. ● Organization of the book into Adult Pain,
Determining the appropriateness of physical Adult Non-Pain, and Children sections en-
therapy for patients assumes physical therapists sure usefulness of the book across areas of
must engage in a process of diagnostic reasoning practice and the lifespan
as part of their overall evaluation. Diagnostic ● Symptoms of special diagnostic concern are
reasoning allows physical therapists to develop a presented at the beginning of each chapter,
well-reasoned plan for addressing the patient’s so this important information is available at
concerns, adequately communicate with other a glance
members of the health care team using the uni- ● Chapter previews organize possible conditions
versal language of the health care system, and by a pathologic category and the likelihood of
appropriately educate patients. In order to ade- causing the symptom of chief concern
quately determine the appropriateness of physi-
● Conditions are divided among remote and
cal therapy for patients, this diagnostic process
local pathologies that may manifest as the
must lead to a decision regarding the probable
symptom of chief concern
pathological or pathophysiological source of the
patient’s problem. It must be emphasized that ● Succinct descriptions of each condition in-
Vision 2020, with its emphasis on direct access, clude details regarding their clinical presenta-
presupposes that the physical therapist will be tion, contributing pathology/pathophysiology,
able to reach that decision without provision of confirmatory tests, and potential treatments
a diagnostic label beforehand by a physician or For physical therapy students and instructors:
other health care professional. However, to date,
no resource exists to support daily efforts of ● A series of foundational chapters provide
physical therapists to engage in diagnostic rea- the basic rationale for diagnosis by physical
soning and student physical therapists to learn therapists
diagnostic reasoning.
v
1528_FM_i-xxxii 08/05/12 6:06 PM Page vi

vi Preface

● The framework for diagnostic reasoning Many physical therapists have developed excel-
presented in this book will integrate seam- lent diagnostic skills, but these skills were hard-
lessly with existing coursework in pathology/ earned through independent study, on-the-job
pathophysiology and clinical management training, and trial and error. In order to reach
● Chapters are presented in a similar format the goal of direct access as described in Vision
that enunciates each step of the diagnostic 2020, the physical therapy profession must con-
reasoning process tinue to develop systematic, evidence-based
● Pathologic categories are presented using a approaches for diagnosis that are taught in en-
mnemonic ‘TIM VaDeTuCoNe’ in order to try-level curricula and refined in post-graduate
assist recall of categories of pathology that educational programs, so that all physical ther-
may contribute to the patient’s symptom of apists demonstrate a certain level of skill in
chief concern diagnosis. This book represents a first attempt
● Case chapters demonstrate the stepwise to describe and formalize diagnostic reasoning
application of the diagnostic reasoning by physical therapists at the level of pathology.
process presented in this book Much like the collective understanding that it
represents and to which it contributes, the ma-
Despite the overwhelming need for more sys- terial presented in this book remains an exciting
tematic approaches to diagnosis by physical and dynamic work in progress.
therapists, there remains a level of controversy
surrounding the topic of diagnosis by physical Reference
therapists, particularly as it relates to pathology. 1. www.apta.org/vision2020. Accessed 13 April 2012.
1528_FM_i-xxxii 08/05/12 6:06 PM Page vii

Contributors

Ragen L. Agler, PT, DPT, ATC Bernadette M. Currier, PT, DPT, MS, NCS
Physical Therapy Faculty Adjunct Instructor of Clinical Physical
USC Lung Transplant Program Therapy
Los Angeles, California University of Southern California
Division of Biokinesiology and Physical
Michael A. Andersen, PT, DPT, OCS
Therapy
Adjunct Instructor of Clinical Physical
Los Angeles, California
Therapy
University of Southern California Todd E. Davenport, PT, DPT, OCS
Division of Biokinesiology and Physical Assistant Professor
Therapy University of the Pacific
Los Angeles, California Department of Physical Therapy
Thomas J. Long School of Pharmacy and
Lucinda L. Baker, PT, PhD
Health Sciences
Associate Professor
Stockton, California
University of Southern California
Division of Biokinesiology and Physical Sharon K. DeMuth, PT, DPT, MS
Therapy Adjunct Assistant Professor of Clinical
Los Angeles, California Physical Therapy
University of Southern California
Kyle F. Baldwin, PT, DPT
Division of Biokinesiology and Physical
Adjunct Assistant Professor of Clinical
Therapy
Physical Therapy
Los Angeles, California
University of Southern California
Division of Biokinesiology and Physical Jesus F. Dominguez, PT, PhD
Therapy Assistant Professor of Clinical Physical
Los Angeles, California Therapy
University of Southern California
Robin I. Burks, PT, DPT, CHT*
Division of Biokinesiology and Physical
Former Instructor of Clinical Physical Therapy
Therapy
University of Southern California
Los Angeles, California
Division of Biokinesiology and Physical
Therapy Kathy Doubleday, PT, DPT, OCS
Los Angeles, California Physical Therapy Affiliate
Pacifica Spine
Julia L. Burlette, PT, DPT, OCS
Ojai, California
Assistant Professor of Clinical Physical Therapy
University of Southern California Elizabeth L. Ege, PT, DPT
Division of Biokinesiology and Physical Therapy Adjunct Instructor of Clinical Physical
Los Angeles, California Therapy
University of Southern California
Jason R. Cozby, PT, DPT, OCS
Division of Biokinesiology and Physical
Adjunct Instructor of Clinical Physical Therapy
Therapy
University of Southern California
Los Angeles, California
Division of Biokinesiology and Physical
Therapy
Los Angeles, California

*Deceased.
vii
1528_FM_i-xxxii 08/05/12 6:06 PM Page viii

viii Contributors

Daniel Farwell, PT, DPT Robbin Howard, PT, DPT, NCS


Adjunct Assistant Professor of Clinical Assistant Professor of Clinical Physical Therapy
Physical Therapy University of Southern California
University of Southern California Division of Biokinesiology and Physical
Division of Biokinesiology and Physical Therapy
Therapy Los Angeles, California
Los Angeles, California
John M. Itamura, MD
Beth E. Fisher, PT, PhD Associate Professor of Clinical Orthopaedic
Associate Professor of Clinical Physical Surgery
Therapy USC University Hospital
University of Southern California Los Angeles, California
Division of Biokinesiology and Physical
Stephanie A. Jones, PT, DPT, OCS, NCS
Therapy
Clinical Assistant Professor
Los Angeles, California
MGH Institute of Health Professions
James Gordon, PT, EdD, FAPTA School of Health and Rehabilitation Sciences
Associate Dean and Chair Department of Physical Therapy
University of Southern California Boston, Massachusetts
Division of Biokinesiology and Physical
Rob Landel, PT, DPT, OCS, CSCS, FAPTA
Therapy
Professor of Clinical Physical Therapy
Los Angeles, California
University of Southern California
Rose Hamm, PT, DPT, CWS, FACCWS Division of Biokinesiology and Physical
Assistant Professor of Clinical Physical Therapy
Therapy Los Angeles, California
University of Southern California
Covey J. Lazouras, PT, DPT, NCS
Division of Biokinesiology and Physical
Adjunct Instructor of Clinical Physical
Therapy
Therapy
Los Angeles, California
University of Southern California
Julie Hershberg, PT, DPT, NCS Division of Biokinesiology and Physical
Instructor of Clinical Therapy Therapy
University of Southern California Los Angeles, California
Division of Biokinesiology and Physical
Della Lee, PT, DPT, OCS, ATC
Therapy
Adjunct Instructor of Clinical Physical Therapy
Los Angeles, California
University of Southern California
Larry Ho, PT, DPT, OCS Division of Biokinesiology and Physical
Adjunct Assistant Professor of Clinical Therapy
Physical Therapy Los Angeles, California
University of Southern California
Kim Levenhagen, PT, DPT, WCC
Division of Biokinesiology and Physical
Instructor of Physical Therapy
Therapy
Saint Louis University
Los Angeles, California
Department of Physical Therapy & Athletic
Sally Ho, PT, DPT, OCS Training
Adjunct Assistant Professor of Clinical Doisy College of Health Sciences
Physical Therapy Saint Louis, Missouri
University of Southern California
J. Raul Lona, DPT, OCS, ATC
Division of Biokinesiology and Physical
Managing Partner & Co-owner
Therapy
Wilshire Linden Physical Therapy
Los Angeles, California
Beverly Hills, California
1528_FM_i-xxxii 08/05/12 6:06 PM Page ix

Contributors ix

Munesha Ramsinghani Lona, Phibun Ny, PT, DPT


PT, DPT, PCS Clinical Instructor of Physical Therapy
Pediatric Physical Therapist University of Southern California
California Children’s Services Division of Biokinesiology and Physical
Reseda, California Therapy
Los Angeles, California
Jennifer Lundberg, PT, DPT
Instructor of Clinical Physical Therapy Michael O’Donnell, PT, DPT, OCS,
University of Southern California FAAOMPT
Division of Biokinesiology and Physical Assistant Professor of Clinical Physical
Therapy Therapy
Los Angeles, California University of Southern California
Division of Biokinesiology and Physical
Bach T. Ly, PT, DPT
Therapy
Clinical Instructor of Physical Therapy
Los Angeles, California
University of Southern California
Division of Biokinesiology and Physical Shelly Olivadoti-Santoro, PT, DPT
Therapy Clinical Instructor of Physical Therapy
Los Angeles, California University of Southern California
Division of Biokinesiology and Physical
Jill S. Masutomi, PT, DPT, PCS
Therapy
Instructor of Clinical Physical Therapy
Los Angeles, California
University of Southern California
Division of Biokinesiology and Physical Marisa Perdomo, PT, DPT
Therapy Assistant Professor of Clinical Physical
Los Angeles, California Therapy
University of Southern California
Yogi Matharu, PT, DPT, OCS
Division of Biokinesiology and Physical
Director of Physical Therapy Clinical Services
Therapy
University of Southern California
Los Angeles, California
Division of Biokinesiology and Physical
Therapy Amy B. Pomrantz, PT, DPT, OCS, ATC
Los Angeles, California Assistant Professor of Clinical Physical
Therapy
Didi Matthews, PT, DPT, NCS
University of Southern California
Adjunct Assistant Professor of Clinical
Division of Biokinesiology and Physical
Physical Therapy
Therapy
University of Southern California
Los Angeles, California
Division of Biokinesiology and Physical
Therapy Elizabeth M. Poppert, PT, DPT, MS, OCS
Los Angeles, California Adjunct Instructor of Clinical Physical
Therapy
Wendi W. McKenna, PT, DPT, PCS
University of Southern California
Clinical Instructor of Physical Therapy
Division of Biokinesiology and Physical
University of Southern California
Therapy
Division of Biokinesiology and Physical
Los Angeles, California
Therapy
Los Angeles, California Michelle G. Prettyman, PT, DPT, MS
Assistant Professor
Lisa Meyer, PT, DPT, OCS
University of Maryland School of Medicine
Adjunct Instructor of Clinical Physical Therapy
Department of Physical Therapy and
University of Southern California
Rehabilitation Science
Division of Biokinesiology and Physical
Baltimore, Maryland
Therapy
Los Angeles, California
1528_FM_i-xxxii 08/05/12 6:06 PM Page x

x Contributors

Stephen F. Reischl, PT, DPT, OCS Christy L. Skura, PT, DPT, PCS
Adjunct Associate Professor Physical Therapist
University of Southern California Therapy West Inc. and Play Studio
Division of Biokinesiology and Physical Los Angeles, California
Therapy
Claire Smith, PT, DPT, NCS
Los Angeles, California
Adjunct Instructor of Clinical Physical
Jeffrey S. Rodrigues, PT, DPT, CCS Therapy
Instructor of Clinical Physical Therapy University of Southern California
University of Southern California Division of Biokinesiology and Physical
Division of Biokinesiology and Physical Therapy
Therapy Los Angeles, California
Los Angeles, California
Josiane Stickles, PT, DPT
Cassandra Sanders-Holly, PT, DPT, PCS Clinical Instructor of Physical Therapy
Clinical Instructor of Physical Therapy University of Southern California
University of Southern California Division of Biokinesiology and Physical
Division of Biokinesiology and Physical Therapy
Therapy Los Angeles, California
Los Angeles, California
Katherine J. Sullivan, PT, PhD, FAHA
Alison R. Scheid, PT, DPT, OCS, NCS Associate Professor of Clinical Physical
Staff, Financial District Clinic Therapy
San Francisco Sport and Spine Physical Therapy University of Southern California
San Francisco, California Division of Biokinesiology and Physical
Therapy
Stephen Schnall, MD
Los Angeles, California
Specialist, Orthopaedic Surgery — Hand
USC Center for Spinal Surgery Shirley Wachi-See, PT, DPT, OCS
Los Angeles, California Clinical Instructor of Physical Therapy
University of Southern California
Chris A. Sebelski, PT, DPT, OCS, CSCS
Division of Biokinesiology and Physical
Assistant Professor
Therapy
Saint Louis University
Los Angeles, California
Department of Physical Therapy & Athletic
Training Hugh G. Watts, MD
Doisy College of Health Sciences Adjunct Associate Professor
Saint Louis, Missouri University of Southern California
Division of Biokinesiology and Physical
Cheri Kay Sessions, PT, DPT, ATC
Therapy
Clinical Instructor of Physical Therapy
Los Angeles, California
University of Southern California
Division of Biokinesiology and Physical Katherine M. Weimer, PT, DPT, NCS
Therapy Adjunct Instructor of Clinical Physical
Los Angeles, California Therapy
University of Southern California
Robert C. Sieh, PT, DPT
Division of Biokinesiology and Physical
Pediatric Physical Therapist
Therapy
Mercy Riverside Rehabilitation
Los Angeles, California
Des Moines, Iowa
Michael S. Simpson, PT, DPT
Assistant Professor of Clinical Physical Therapy
University of Southern California
Division of Biokinesiology and Physical
Therapy
Los Angeles, California
1528_FM_i-xxxii 08/05/12 6:06 PM Page xi

Reviewers

Ronald P. Andrews, PT, PhD Donald L. Gabard, PT, PhD


Associate Professor Professor
University of New Mexico Chapman University
School of Medicine Department of Physical Therapy
Department of Orthopaedics/Physical Orange, California
Therapy Program
Judith Gale, PT, DPT, MPH, OCS
Albuquerque, New Mexico
Associate Professor
Theresa Geraldine Bernsen, PT, MA Creighton University
Assistant Professor School of Pharmacy and Health Professions
Saint Louis University Department of Physical Therapy
Doisy College of Health Sciences Omaha, Nebraska
Department of Physical Therapy
Charles J. Gulas, PT, PhD, GCS
Saint Louis, Missouri
Dean, Associate Professor
Mark W. Cornwall, PT, PhD, CPed Maryville University
Chair School of Health Professions
Northern Arizona University Saint Louis, Missouri
Department of Physical Therapy
Kevin Helgeson, PT, DHSc, SCS
Flagstaff, Arizona
Faculty
Janet P. Dolot, PT, DPT Rocky Mountain University of Health
Assistant Professor of Clinical Physical Professions
Therapy Doctor of Physical Therapy Program
New York Medical College Provo, Utah
School of Health Sciences and Practice
Eric Johnson, PT, DSc
Doctor of Physical Therapy Program
Professor
Valhalla, New York
Loma Linda University
Jacqueline S. Drouin, PT, PhD School of Allied Health Professions
Associate Professor Department of Physical Therapy
Oakland University Loma Linda, California
School of Health Sciences
David Krause, PT, MBA, DSc, OCS
Physical Therapy Program
Faculty
Rochester, Michigan
Mayo School of Health Sciences
Lisa L. Dutton, PT, PhD College of Medicine
Associate Professor Physical Therapy Doctoral Program
St. Catherine University Rochester, Minnesota
Henrietta Schmoll School of Health
David A. Lake, PT, PhD
Doctor of Physical Therapy Program
Interim Department Head / Professor
Minneapolis, Minnesota
Armstrong Atlantic State University
Staffan Elgelid, PT, PhD, MT, CFP College of Health Professions
Former Assistant Professor Department of Physical Therapy
Arkansas State University Savannah, Georgia
Department of Physical Therapy
Jonesboro, Arkansas

xi
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xii Reviewers

James J. Laskin, PT, PhD Andrew Priest, MPT, EdD


Executive Director, Associate Professor Chair and Associate Professor
The University of Montana Clarke College
The College of Health Professions and Physical Therapy Department
Biomedical Sciences Dubuque, Iowa
School of Physical Therapy & Rehabilitation
Pamela D. Ritzline, PT, EdD
Science
Associate Professor
Missoula, Montana
Director of Graduate Programs
Everett B. Lohman, III, PT, DSc, OCS The University of Tennessee Health Science
Professor and Assistant Dean of Graduate Center
Academic Affairs College of Allied Health Sciences
Loma Linda University Department of Physical Therapy
School of Allied Health Professions Memphis, Tennessee
Department of Physical Therapy
Joellen Roller, PT, DPT, EdD
Loma Linda, California
Chair, Division of Human Performance
Venita Lovelace-Chandler, PT, PhD, PCS Sciences
Professor and Associate Director Chair, Department of Physical Therapy
Texas Woman’s University University of Mary
School of Physical Therapy Bismarck, North Dakota
Dallas, Texas
Summer San Lucas, PT, DPT
John D. Lowman, PT, PhD, CCS Contract Instructor
Assistant Professor Loma Linda University
University of Alabama at Birmingham School of Allied Health Professions
School of Health Professions Department of Physical Therapy
Department of Physical Therapy Loma Linda, California
Birmingham, Alabama
David Scalzitti, PT, PhD, OCS
Susanne M. Morton, PT, PhD Associate Director - Research Department
Assistant Professor American Physical Therapy Association
University of Iowa Alexandria, Virginia
Graduate Program in Physical Therapy &
Frank B. Underwood, PT, PhD, ECS
Rehabilitation Science
Professor
Iowa City, Iowa
University of Evansville
Rose Marie Ortega, PT, DPT College of Education and Health Sciences
Former Professor and Academic Coordinator Department of Physical Therapy
of Clinical Education Evansville, Indiana
Stony Brook University
Shelly D. Weise, PT, EdD
Physical Therapy Program
Associate Professor
Stony Brook, New York
Angelo State University
Jaime C. Paz, PT, MS Department of Physical Therapy
Part Time Faculty San Angelo, Texas
Simmons College
School of Nursing and Health Sciences
Department of Physical Therapy
Boston, Massachusetts
1528_FM_i-xxxii 08/05/12 6:06 PM Page xiii

Manuscript Support

Shiren Assaly, PT, DPT Zachary J. Mertz, PT, DPT


Physical Therapist Physical Therapist
Physical Rehabilitation Network Lodi Physical Therapy
Sunnyvale, California Lodi, California
Emily Bannister, PT, DPT Sheila H. Mistry, PT, DPT
Physical Therapist Acute/ICU Physical Therapist
Santa Clara Valley Medical Center VA Palo Alto Health Care System
San Jose, California Palo Alto, California
Sara J. Belluomini, PT, DPT Jennifer L. Myers, PT, DPT
Physical Therapist Physical Therapist
Central Valley Physical Therapy ProActive Orthopedic and Sports Physical
Stockton, California Therapy, LLC
Vancouver, Washington
Jill Dietmeyer, PT, DPT, LMT
Physical Therapist Nella Tay, PT, DPT
REHAB at Nu’uanu Physical Therapist
Honolulu, Hawaii Marin General Hospital
Greenbrae, California
Kristina M. Gibson, PT, DPT
Physical Therapist Bryan Van Vliet, PT, DPT
Andersen Physical Therapy Physical Therapist
Modesto, California Tower Physical Therapy
Turlock, California
Kristen M. Judson, PT, DPT
Physical Therapist
Sutter Medical Foundation
Fairfield, California
Rachel Gail Lee, PT, DPT
Physical Therapist
Southland Physical Therapy
Irvine, California

xiii
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Acknowledgments and Dedication

This book owes its very existence to the Our families, close friends, and colleagues,
passion, dedication, and support of a host of whose encouragement sustained the process of
people: creating this book. Each page of this book is
The dedicated and expert physical thera- infused with their influences, ranging from the
pists who strive to provide efficient and high ideas that stemmed from countless hours of
quality healthcare for their patients every day, conversation to the inspiration of knowing
as well as the talented students who are train- that they may someday benefit from the appli-
ing to become physical therapists. Without cation of this book’s content by a physical
them, this book would not be necessary. therapist. Without them, this book would not
The many leaders and luminaries in the be meaningful.
physical therapy profession who have advo- We offer this book in memory of Robin I.
cated for the professional responsibility of Burks, PT, DPT, CHT, our friend and col-
physical therapists to determine the appropri- league, whose life was cut short by a brain tu-
ateness of physical therapy for their patients. mor during the creation of this book. His
Without them, this book would not be family, friends, colleagues, students, and pa-
relevant. tients miss his caring and gentle manner, his
The individuals who contributed to this sense of humor, and the simple personal wis-
textbook, including its authors, editors, illus- dom that complemented his expansive profes-
trators, and publishers. All their contributions sional knowledge. As Dr. Burks encouraged
are the result of their knowledge and hard the Doctor of Physical Therapy students at the
work; they have indelibly shaped our collective University of Southern California in his 2008
understanding of diagnostic reasoning by White Coat Ceremony address, we similarly
physical therapists. Without them, this book urge you to “…put on your coat, roll up your
would not be possible. sleeves, and make the world a better place.”

xv
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Contents in Brief

Section I Foundations 1
Chapter 1 Why Should Physical Therapists Know About Diagnosis? ________________ 1
James Gordon, PT, EdD, FAPTA • Hugh G. Watts, MD
Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology ______________ 8
Rob Landel, PT, DPT, OCS, CSCS, FAPTA
Chapter 3 Diagnostic Reasoning __________________________________________________________________ 16
Todd E. Davenport, PT, DPT, OCS
Chapter 4 How to Use This Book ________________________________________________________________ 22
Hugh G. Watts, MD
Chapter 5 Case Demonstration: Shoulder______________________________________________________ 31
Hugh G. Watts, MD
Acknowledgment: Susan Layfield, PT, DPT, OCS

Section II Adult Pain 41


Chapter 6 Diagnostic Considerations of Pain ________________________________________________ 41
Todd E. Davenport, PT, DPT, OCS • Lucinda L. Baker, PT, PhD
Chapter 7 Headaches ________________________________________________________________________________ 54
Michael O’Donnell, PT, DPT, OCS, FAAOMPT
Chapter 8 Temporomandibular Joint and Facial Pain______________________________________ 75
Sally Ho, PT, DPT, OCS
Chapter 9 Case Demonstration: Jaw Pain __________________________________________________ 100
Jesus F. Dominguez, PT, PhD • Michael S. Simpson, PT, DPT
Chapter 10 Neck Pain ________________________________________________________________________________ 108
Larry Ho, PT, DPT, OCS • Shirley Wachi-See, PT, DPT, OCS
Chapter 11 Shoulder Pain __________________________________________________________________________ 133
Chris A. Sebelski, PT, DPT, OCS, CSCS
Chapter 12 Elbow Pain ________________________________________________________________________________ 174
Julia L. Burlette, PT, DPT, OCS • John M. Itamura, MD
Chapter 13 Wrist and Hand Pain ________________________________________________________________ 201
Robin I. Burks, PT, DPT, CHT • Stephen Schnall, MD
Chapter 14 Anterior Thorax Pain ________________________________________________________________ 240
Yogi Matharu, PT, DPT, OCS
Chapter 15 Case Demonstration: Chest Pain ________________________________________________ 261
Amy B. Pomrantz, PT, DPT, OCS, ATC •
Chris A. Sebelski, PT, DPT, OCS, CSCS
Chapter 16 Posterior Thorax Pain ________________________________________________________________ 269
Kathy Doubleday, PT, DPT, OCS
xvii
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xviii Contents in Brief

Chapter 17 Case Demonstration: Infrascapular Pain ______________________________________ 287


Todd E. Davenport, PT, DPT, OCS • Hugh G. Watts, MD
Chapter 18 Lumbar Pain ______________________________________________________________________________ 293
Michael A. Andersen, PT, DPT, OCS • J. Raul Lona, DPT, OCS, ATC
Chapter 19 Hip Pain __________________________________________________________________________________ 316
Kyle F. Baldwin, PT, DPT • Todd E. Davenport, PT, DPT, OCS •
Michael A. Andersen, PT, DPT, OCS
Chapter 20 Case Demonstration: Hip Pain __________________________________________________ 353
Alison R. Scheid, PT, DPT, OCS, NCS •
Elizabeth M. Poppert, PT, DPT, MS, OCS
Chapter 21 Knee Pain ________________________________________________________________________________ 362
Della Lee, PT, DPT, OCS, ATC • Daniel Farwell, PT, DPT
Chapter 22 Lower Leg Pain ________________________________________________________________________ 392
Jason R. Cozby, PT, DPT, OCS
Chapter 23 Ankle Pain ________________________________________________________________________________ 417
Jason R. Cozby, PT, DPT, OCS • Lisa Meyer, PT, DPT, OCS •
Stephen F. Reischl, PT, DPT, OCS
Chapter 24 Foot Pain ________________________________________________________________________________ 444
Jason R. Cozby, PT, DPT, OCS • Lisa Meyer, PT, DPT, OCS •
Stephen F. Reischl, PT, DPT, OCS

Section III Adult Non-Pain 489


Chapter 25 Foundations of Neurological Differential Diagnosis ______________________ 489
Bernadette M. Currier, PT, DPT, MS, NCS • Beth E. Fisher, PT, PhD
Chapter 26 Cardiovascular and Pulmonary Clues from Examination ________________ 504
Jesus F. Dominguez, PT, PhD
Chapter 27 Dizziness ________________________________________________________________________________ 526
Rob Landel, PT, DPT, OCS, CSCS, FAPTA
Chapter 28 Loss of Balance and Falls __________________________________________________________ 550
Robbin Howard, PT, DPT, NCS • Didi Matthews, PT, DPT, NCS
Chapter 29 Sensory Abnormalities ______________________________________________________________ 571
Bernadette M. Currier, PT, DPT, MS, NCS •
Michelle G. Prettyman, PT, DPT, MS
Chapter 30 Abnormal Movement ________________________________________________________________ 608
Claire Smith, PT, DPT, NCS • Beth E. Fisher, PT, PhD
Chapter 31 Problems of Cognition, Communication, and Behavior ________________ 634
Julie Hershberg, PT, DPT, NCS
Chapter 32 Stiffness __________________________________________________________________________________ 665
Claire Smith, PT, DPT, NCS • Beth E. Fisher, PT, PhD
Chapter 33 Weakness ________________________________________________________________________________ 683
Katherine J. Sullivan, PT, PhD, FAHA • Katherine M. Weimer, PT, DPT, NCS
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Contents in Brief xix

Chapter 34 Case Demonstration: Inability to Stand ________________________________________ 716


Chris A. Sebelski, PT, DPT, OCS, CSCS
Chapter 35 Palpitations ______________________________________________________________________________ 721
Jesus F. Dominguez, PT, PhD
Chapter 36 Persistent Cough ______________________________________________________________________ 742
Jeffrey S. Rodrigues, PT, DPT, CCS
Chapter 37 Dyspnea __________________________________________________________________________________ 761
Ragen L. Agler, PT, DPT, ATC
Chapter 38 Edema ______________________________________________________________________________________ 779
Marisa Perdomo, PT, DPT
Chapter 39 Case Demonstration: Edema and Shoulder Pain __________________________ 798
Marisa Perdomo, PT, DPT • Chris A. Sebelski, PT, DPT, OCS, CSCS
Chapter 40 Failure of Wounds to Heal ________________________________________________________ 812
Rose Hamm, PT, DPT, CWS, FACCWS
Chapter 41 Case Demonstration: Fatigue ______________________________________________________ 833
Kim Levenhagen, PT, DPT, WCC • Chris A. Sebelski, PT, DPT, OCS, CSCS

Section IV Children 841


Chapter 42 Special Diagnostic Issues in Children __________________________________________ 841
Sharon K. DeMuth, PT, DPT, MS • Hugh G. Watts, MD
Chapter 43 Elbow Pain in a Child ________________________________________________________________ 851
Julia L. Burlette, PT, DPT, OCS • Jill S. Masutomi, PT, DPT, PCS
Chapter 44 Back Pain in a Child __________________________________________________________________ 873
Munesha Ramsinghani Lona, PT, DPT, PCS • Josiane Stickles, PT, DPT
Chapter 45 Hip Pain in a Child __________________________________________________________________ 898
Stephanie A. Jones, PT, DPT, OCS, NCS
Chapter 46 Case Demonstration: Hip Pain in a Child ______________________________________ 925
Hugh G. Watts, MD • Chris A. Sebelski, PT, DPT, OCS, CSCS
Chapter 47 Knee Pain in a Child __________________________________________________________________ 933
Jennifer Lundberg, PT, DPT • Cassandra Sanders-Holly, PT, DPT, PCS
Chapter 48 Case Demonstration: Knee Pain and Limping in a Child ________________ 959
Todd E. Davenport, PT, DPT, OCS • Sharon K. DeMuth, PT, DPT, MS
Chapter 49 Shin Pain in a Child __________________________________________________________________ 967
Covey J. Lazouras, PT, DPT, NCS
Chapter 50 Ankle and Foot Pain in a Child __________________________________________________ 982
Robert C. Sieh, PT, DPT
Chapter 51 Case Demonstration: Foot Pain in a Child __________________________________ 1007
Hugh G. Watts, MD
Chapter 52 Delayed Mobility (Crawling and Walking), Failure to
Thrive, and Psychomotor Delay in a Child __________________________________ 1013
Phibun Ny, PT, DPT • Shelly Olivadoti-Santoro, PT, DPT •
Cassandra Sanders-Holly, PT, DPT, PCS
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xx Contents in Brief

Chapter 53 The Child With a Painless Limp ________________________________________________ 1035


Hugh G. Watts, MD
Chapter 54 Tripping and Falling in a Child __________________________________________________ 1056
Christy L. Skura, PT, DPT, PCS • Bach T. Ly, PT, DPT
Chapter 55 Toe Walking in a Child ______________________________________________________________ 1072
Christy L. Skura, PT, DPT, PCS • Bach T. Ly, PT, DPT
Chapter 56 Joint Contractures in a Child ______________________________________________________ 1087
Cassandra Sanders-Holly, PT, DPT, PCS • Cheri Kay Sessions, PT, DPT, ATC
Chapter 57 Torticollis in a Child ________________________________________________________________ 1099
Hugh G. Watts, MD • Sharon K. DeMuth, PT, DPT, MS
Chapter 58 Case Demonstration: Torticollis in a Child __________________________________ 1108
Sharon K. DeMuth, PT, DPT, MS • Chris A. Sebelski, PT, DPT, OCS, CSCS
Chapter 59 Poor Posture and Scoliosis, Kyphosis, and Lordosis
in a Child ________________________________________________________________________________ 1113
Sharon K. DeMuth, PT, DPT, MS • Stephanie A. Jones, PT, DPT, OCS, NCS
Chapter 60 Bowed Legs and Knock Knees in a Child __________________________________ 1149
Wendi W. McKenna, PT, DPT, PCS
Chapter 61 In-Toeing and Out-Toeing in a Child __________________________________________ 1165
Wendi W. McKenna, PT, DPT, PCS
Chapter 62 Flat Feet in a Child __________________________________________________________________ 1180
Wendi W. McKenna, PT, DPT, PCS
Chapter 63 Weakness and Hypotonia in a Child __________________________________________ 1190
Elizabeth L. Ege, PT, DPT
Index ______________________________________________________________________________________ 1205
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Contents

Section I Foundations 1
Chapter 1 Why Should Physical Therapists Know About Diagnosis? ________________ 1
James Gordon, PT, EdD, FAPTA • Hugh G. Watts, MD
Introduction __________________________________________________________________________________ 1
Five Critical Issues Frame the Topic of Diagnosis by Physical Therapists ________________ 2
Critical Issue 1: What is a Diagnosis? __________________________________________________ 3
Critical Issue 2: Should Physical Therapists Diagnose Pathology? ______________________ 4
Critical Issue 3: Should Physical Therapists Adopt a Different
Definition for Diagnosis Than Other Health Professionals Use? __________________ 4
Critical Issue 4: Should Physical Therapists be Expected to
Diagnose Given the Current Legal Environment? ________________________________ 5
Critical Issue 5: What Information Should Physical Therapists
Use to Make Decisions About Treatment and Prognosis? ________________________ 6
Conclusion ________________________________________________________________ 6
Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology ______________ 8
Rob Landel, PT, DPT, OCS, CSCS, FAPTA
Introduction __________________________________________________________________________________ 8
Physical Therapists Use the Diagnostic Label ______________________________________________ 9
Physical Therapists Elaborate on the Diagnostic Label __________________________________ 10
The Diagnostic Label Alone is Insufficient to Guide Physical Therapists ______________ 11
A Process Guides Physical Therapists’ Search for an Underlying Etiology ______________ 12
Physical Therapists Identify the Functional Chief Concern ______________________________ 13
Physical Therapists Perform Task and Movement Analyses __________________________ 13
Physical Therapists Create and Test Hypothetical Relationships ____________________ 13
The Search for an Underlying Etiology is Dynamic and Ongoing ____________________ 14
Conclusion ______________________________________________________________________________________ 14
Chapter 3 Diagnostic Reasoning ____________________________________________________________________ 16
Todd E. Davenport, PT, DPT, OCS
Introduction __________________________________________________________________________________ 16
Both Diagnosis and Medical Screening Have Roles in Physical Therapy ____________ 16
Diagnosis is a Unique Combination of Reasoning Patterns __________________________ 17
Backward Reasoning Involves Creation and Testing of Clinical
Hypotheses ________________________________________________________________________ 17
Forward Reasoning Involves the Attempt to Recognize Patterns in
Clinical Presentation ________________________________________________________________ 18
Experts Create “Small Worlds” of Information to Use in
Diagnostic Reasoning ______________________________________________________________ 19
Preferred Diagnostic Reasoning Processes Depend on the Clinician
and the Case ____________________________________________________________________ 19
Conclusion ______________________________________________________________________________________ 20

xxi
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xxii Contents

Chapter 4 How to Use This Book ________________________________________________________________ 22


Hugh G. Watts, MD
Introduction __________________________________________________________________________________ 22
Principle of “Economy of Diagnoses” ______________________________________________________ 22
The Diagnostic Process ______________________________________________________________________ 23
Barriers to Communication ______________________________________________________________ 23
The Starting Point: Identify the Patient’s Chief Concern ______________________________ 23
Symptoms Versus Signs ________________________________________________________________ 24
Special Concerns ________________________________________________________________________ 24
TIM VaDeTuCoNe: A Database of Possible Causes ____________________________________ 25
Remote Versus Local Source of Pain __________________________________________________ 27
What Follows the Making of a TIM VaDeTuCoNe List? ________________________________ 28
Conclusion __________________________________________________________________________________ 30
Chapter 5 Case Demonstration: Shoulder ______________________________________________________ 31
Hugh G. Watts, MD
Acknowledgment: Susan Layfield, PT, DPT, OCS
Case Description ______________________________________________________________________________ 31
Case Outcome ____________________________________________________________________________ 40

Section II Adult Pain 41


Chapter 6 Diagnostic Considerations of Pain ________________________________________________ 41
Todd E. Davenport, PT, DPT, OCS • Lucinda L. Baker, PT, PhD
Introduction ____________________________________________________________________________________ 41
All People Share Common Nociceptive Processes ______________________________________ 42
Nociceptors Respond to Various Forms of Tissue Injury ______________________________ 42
Nociceptors Transmit Pain Signals Along Organized Neural Pathways ______________ 42
The Perception of Pain Severity Depends on Anatomical,
Physiological, and Cognitive Factors ________________________________________________ 43
A “Gate” Controls the Transmission of Pain Signals at the Spinal
Cord and Elsewhere ____________________________________________________________ 43
Input from the Cerebral Cortex and Midbrain Also Plays a Role in the
Gate’s Function __________________________________________________________________ 44
Emotional, Anticipatory, and Previously Learned Responses Also
Modulate Pain Severity __________________________________________________________ 46
Timing of Pain Suggests the Etiology of Underlying Pathology ________________________ 46
Factors in the History and Physical Examination Can Implicate
Affected Tissues __________________________________________________________________ 46
Descriptions of Pain May Relate to Either Injured Tissues or Emotional
Features __________________________________________________________________________ 47
Locations of Pain and Its Underlying Cause May Differ ________________________________ 47
Misinterpretation by the Central Nervous System Causes Referred Pain __________ 48
Long-Term Pain Causes Cyclic Anatomical, Physiological, and
Cognitive-Behavioral Changes ________________________________________________________ 49
Prolonged Activation of Nociceptive Pathways Causes Maladaptations
Throughout the Nervous System ________________________________________________ 50
The Sympathetic Nervous System Can Contribute to the Perception
of Pain ______________________________________________________________________________ 51
Psychological and Social Factors Perpetuate Pain Through
Behavior Reinforcement __________________________________________________________ 51
Conclusion __________________________________________________________________________________ 52
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Contents xxiii

Chapter 7 Headaches ________________________________________________________________________________ 54


Michael O’Donnell, PT, DPT, OCS, FAAOMPT
Description of the Symptom ________________________________________________________________ 54
Overview of Headache ______________________________________________________________________ 57
Description of Conditions That May Lead to Headache ________________________________ 57
Remote __________________________________________________________________________________ 57
LocaL ______________________________________________________________________________________ 60
Chapter 8 Temporomandibular Joint and Facial Pain ______________________________________ 75
Sally Ho, PT, DPT, OCS
Description of the Symptom ________________________________________________________________ 75
Overview of Temporomandibular Joint and Facial Pain ________________________________ 79
Description of Conditions That May Lead to Temporomandibular
Joint and Facial Pain __________________________________________________________________ 80
Remote __________________________________________________________________________________ 80
Local ______________________________________________________________________________________ 85
Chapter 9 Case Demonstration: Jaw Pain ____________________________________________________ 100
Jesus F. Dominguez, PT, PhD • Michael S. Simpson, PT, DPT
Case Description ________________________________________________________________________ 100
Case Outcome ________________________________________________________________________ 107
Chapter 10 Neck Pain ________________________________________________________________________________ 108
Larry Ho, PT, DPT, OCS • Shirley Wachi-See, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 108
Overview of Neck Pain ______________________________________________________________________ 112
Description of Conditions That May Lead to Neck Pain ________________________________ 113
Remote ________________________________________________________________________________ 113
Local ______________________________________________________________________________________ 117
Chapter 11 Shoulder Pain __________________________________________________________________________ 133
Chris A. Sebelski, PT, DPT, OCS, CSCS
Description of the Symptom ______________________________________________________________ 133
Overview of Shoulder Pain ________________________________________________________________ 142
Description of Conditions That May Lead to Shoulder Pain __________________________ 143
Remote ________________________________________________________________________________ 143
Local ______________________________________________________________________________________ 155
Chapter 12 Elbow Pain ________________________________________________________________________________ 174
Julia L. Burlette, PT, DPT, OCS • John M. Itamura, MD
Description of the Symptom______________________________________________________________ 174
Overview of Elbow Pain __________________________________________________________________ 180
Description of Conditions That May Lead to Elbow Pain __________________________ 182
Remote ________________________________________________________________________________ 182
Local ______________________________________________________________________________________ 182
Chapter 13 Wrist and Hand Pain __________________________________________________________________ 201
Robin I. Burks, PT, DPT, CHT • Stephen Schnall, MD
Description of the Symptom ______________________________________________________________ 201
Overview of Wrist and Hand Pain ________________________________________________________ 212
Description of Conditions That May Lead to Wrist and Hand Pain __________________ 212
Remote ________________________________________________________________________________ 212
Local ______________________________________________________________________________________ 218
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Chapter 14 Anterior Thorax Pain ________________________________________________________________ 240


Yogi Matharu, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 240
Overview of Anterior Thorax Pain ________________________________________________________ 243
Description of Conditions That May Lead to Anterior Thorax Pain __________________ 244
Remote ________________________________________________________________________________ 244
Local ____________________________________________________________________________________ 253
Chapter 15 Case Demonstration: Chest Pain ________________________________________________ 261
Amy B. Pomrantz, PT, DPT, OCS, ATC •
Chris A. Sebelski, PT, DPT, OCS, CSCS
Case Description __________________________________________________________________________ 261
Case Outcome ________________________________________________________________________ 268
Chapter 16 Posterior Thorax Pain ________________________________________________________________ 269
Kathy Doubleday, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 269
Overview of Posterior Thorax Pain ________________________________________________________ 272
Description of Conditions That May Lead to Posterior Thorax Pain __________________ 273
Remote ________________________________________________________________________________ 273
Local __________________________________________________________________________________ 277
Chapter 17 Case Demonstration: Infrascapular Pain ______________________________________ 287
Todd E. Davenport, PT, DPT, OCS • Hugh G. Watts, MD
Case Description ____________________________________________________________________________ 287
Case Outcome ________________________________________________________________________ 291
Chapter 18 Lumbar Pain ______________________________________________________________________________ 293
Michael A. Andersen, PT, DPT, OCS • J. Raul Lona, DPT, OCS, ATC
Description of the Symptom ______________________________________________________________ 293
Overview of Lumbar Pain __________________________________________________________________ 297
Description of Conditions That May Lead to Lumbar Pain ______________________________ 297
Remote ________________________________________________________________________________ 297
Local __________________________________________________________________________________ 303
Chapter 19 Hip Pain __________________________________________________________________________________ 316
Kyle F. Baldwin, PT, DPT • Todd E. Davenport, PT, DPT, OCS •
Michael A. Andersen, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 316
Overview of Hip Pain ______________________________________________________________________ 324
Description of Conditions That May Lead to Hip Pain ________________________________ 324
Remote ________________________________________________________________________________ 324
Local ______________________________________________________________ 336
Chapter 20 Case Demonstration: Hip Pain __________________________________________________ 353
Alison R. Scheid, PT, DPT, OCS, NCS •
Elizabeth M. Poppert, PT, DPT, MS, OCS
Case Description __________________________________________________________________________ 353
Case Outcome ________________________________________________________________________ 360
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Contents xxv

Chapter 21 Knee Pain ________________________________________________________________________________ 362


Della Lee, PT, DPT, OCS, ATC • Daniel Farwell, PT, DPT
Description of the Symptom ______________________________________________________________ 362
Overview of Knee Pain ______________________________________________________________________ 368
Description of Conditions That May Lead to Knee Pain ________________________________ 370
Remote ________________________________________________________________________________ 370
Local __________________________________________________________________________________ 372
Chapter 22 Lower Leg Pain ________________________________________________________________________ 392
Jason R. Cozby, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 392
Overview of Lower Leg Pain ______________________________________________________________ 398
Description of Conditions That May Lead to Lower Leg Pain ________________________ 399
Remote ________________________________________________________________________________ 399
Local __________________________________________________________________________________ 401
Chapter 23 Ankle Pain ________________________________________________________________________________ 417
Jason R. Cozby, PT, DPT, OCS • Lisa Meyer, PT, DPT, OCS •
Stephen F. Reischl, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 417
Overview of Ankle Pain ____________________________________________________________________ 424
Description of Conditions That May Lead to Ankle Pain ________________________________ 424
Remote ________________________________________________________________________________ 424
Local ____________________________________________________________________________________ 426
Chapter 24 Foot Pain ________________________________________________________________________________ 444
Jason R. Cozby, PT, DPT, OCS • Lisa Meyer, PT, DPT, OCS •
Stephen F. Reischl, PT, DPT, OCS
Description of the Symptom ______________________________________________________________ 444
Overview of Foot Pain ______________________________________________________________________ 454
Description of Conditions That May Lead to Foot Pain ________________________________ 454
Remote ________________________________________________________________________________ 454
Local __________________________________________________________________________________ 456

Section III Adult Non-Pain 489


Chapter 25 Foundations of Neurological Differential Diagnosis ______________________ 489
Bernadette M. Currier, PT, DPT, MS, NCS • Beth E. Fisher, PT, PhD
Introduction ________________________________________________________________________________ 489
Nervous System Pathology is Important to Consider in
Diagnostic Reasoning ________________________________________________________________ 489
Summary of Neurological Differential Diagnosis ________________________________________ 490
Chart Review __________________________________________________________________________ 491
History/Subjective Examination ________________________________________________________ 491
Objective Examination ________________________________________________________________ 491
Clinical Neurological Tests ______________________________________________________________ 492
Diagnostic Impression ________________________________________________________________ 493
Linking Examination Findings to Neuroanatomy ________________________________________ 493
Anatomy and Functions of the Nervous System ______________________________________ 493
Neurological Impairments and Associated Anatomical Regions____________________ 502
Conclusion____________________________________________________________________________________ 503
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination ______________ 504


Jesus F. Dominguez, PT, PhD
Introduction ________________________________________________________________________________ 504
General Observation is an Important Initial Basis for Diagnosing
Cardiovascular and Pulmonary Pathologies ________________________________________ 505
Assessment of Skin Provides Clues Regarding the Status of Cardiac
and Pulmonary Function ______________________________________________________________ 506
Color May Indicate the Presence of Impaired Blood Oxygenation __________________ 506
Surface Temperature Reflects the Rate of Underlying Tissue Metabolism ________ 508
Mobility and Turgor Suggest the Status of Nutrition, Hydration,
and Vascular Competence ______________________________________________________ 508
Lesions and Marks can Provide Clues to the Presence of Important
Medical Comorbidities __________________________________________________________ 508
Examination of the Thorax Provides Diagnostic Information Regarding
the Cardiovascular and Pulmonary Systems ________________________________________ 509
Configuration of the Chest Wall May Indicate the Presence of
Chronic Pulmonary Pathology____________________________________________________ 509
Chest Wall Excursion Reflects the Status of Ventilatory Mechanics ________________ 509
Palpation of the Thorax Provides Information Regarding the
Physical Composition and Orientation of Thoracic Organs ____________________ 510
Tonal Quality of Sound Transmitted Through the Thorax Provides
Information Regarding the Composition of the Lungs ________________________ 511
Vital Signs Provide Direct Information Regarding Various Aspects
of Cardiovascular and Pulmonary Functioning ______________________________________ 512
Heart Rate Indicates the Cardiovascular System’s Response to
Different Levels of Activity ________________________________________________________ 513
Pulse Rhythm Suggests the Status of Electrical Conduction
and Cardiac Output ______________________________________________________________ 513
Pulse Quality Provides Information Regarding the Status of
Contractile Force and Cardiac Output __________________________________________ 514
Blood Pressure Responds to Changes in Position and Activity in
a Predictable Fashion and Provides Important Clues to
Organ Perfusion __________________________________________________________________ 515
Respiratory Rate Suggests the Status of Ventilatory Mechanics
and Blood Oxygenation ____________________________________________________________ 518
Heart and Lung Sounds Reveal Clues About Cardiac and Pulmonary
Pathology ______________________________________________________________________________ 519
Cardiac Auscultation __________________________________________________________________ 519
Pulmonary Auscultation ________________________________________________________________ 522
Conclusion __________________________________________________________________________________ 524
Chapter 27 Dizziness ________________________________________________________________________________ 526
Rob Landel, PT, DPT, OCS, CSCS, FAPTA
Description of the Symptom ______________________________________________________________ 526
Overview of Dizziness ______________________________________________________________________ 529
Description of Conditions That May Lead to Dizziness ________________________________ 530
Chapter 28 Loss of Balance and Falls __________________________________________________________ 550
Robbin Howard, PT, DPT, NCS • Didi Matthews, PT, DPT, NCS
Description of the Symptom ______________________________________________________________ 550
Overview of Loss of Balance and Falls __________________________________________________ 552
Description of Conditions That May Lead to Loss of Balance and Falls ______________ 553
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Chapter 29 Sensory Abnormalities ______________________________________________________________ 571


Bernadette M. Currier, PT, DPT, MS, NCS •
Michelle G. Prettyman, PT, DPT, MS
Description of the Symptom ______________________________________________________________ 571
Overview of Sensory Abnormalities ______________________________________________________ 578
Auditory Abnormalities ________________________________________________________________ 578
Gustatory Abnormalities ______________________________________________________________ 578
Olfactory Abnormalities ________________________________________________________________ 579
Proprioceptive Abnormalities __________________________________________________________ 580
Tactile Abnormalities __________________________________________________________________ 580
Visual Abnormalities __________________________________________________________________ 581
Description of Conditions That May Lead to Sensory Abnormalities ________________ 581
Chapter 30 Abnormal Movement ________________________________________________________________ 608
Claire Smith, PT, DPT, NCS • Beth E. Fisher, PT, PhD
Description of the Symptom ______________________________________________________________ 608
Overview of Abnormal Movement ________________________________________________________ 611
Description of Conditions That May Lead to Abnormal Movement __________________ 612
Chapter 31 Problems of Cognition, Communication, and Behavior ________________ 634
Julie Hershberg, PT, DPT, NCS
Description of the Symptom ______________________________________________________________ 634
Overview of Problems with Cognition, Communication, and Behavior ______________ 638
Description of Conditions That May Lead to Problems with Cognition,
Communication, and Behavior ______________________________________________________ 638
Chapter 32 Stiffness __________________________________________________________________________________ 665
Claire Smith, PT, DPT, NCS • Beth E. Fisher, PT, PhD
Description of the Symptom ______________________________________________________________ 665
Overview of Stiffness ______________________________________________________________________ 668
Description of Conditions That May Lead to Stiffness __________________________________ 669
Chapter 33 Weakness ________________________________________________________________________________ 683
Katherine J. Sullivan, PT, PhD, FAHA • Katherine M. Weimer, PT, DPT, NCS
Description of the Symptom ______________________________________________________________ 683
Overview of Weakness ______________________________________________________________________ 687
Description of Conditions That May Lead to Weakness ________________________________ 687
Chapter 34 Case Demonstration: Inability to Stand ______________________________________ 716
Chris A. Sebelski, PT, DPT, OCS, CSCS
Case Description __________________________________________________________________________ 716
Case Outcome ________________________________________________________________________ 720
Chapter 35 Palpitations ______________________________________________________________________________ 721
Jesus F. Dominguez, PT, PhD
Description of the Symptom ______________________________________________________________ 721
Overview of Palpitations __________________________________________________________________ 721
Description of Conditions That May Lead to Palpitations ______________________________ 725
Chapter 36 Persistent Cough ______________________________________________________________________ 742
Jeffrey S. Rodrigues, PT, DPT, CCS
Description of the Symptom ______________________________________________________________ 742
Overview of Persistent Cough______________________________________________________________ 744
Description of Conditions That May Lead to Persistent Cough ________________________ 745
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Chapter 37 Dyspnea __________________________________________________________________________________ 761


Ragen L. Agler, PT, DPT, ATC
Description of the Symptom ______________________________________________________________ 761
Overview of Dyspnea ______________________________________________________________________ 764
Description of Conditions That May Lead to Dyspnea ________________________________ 764
Chapter 38 Edema ______________________________________________________________________________________ 779
Marisa Perdomo, PT, DPT
Description of the Symptom ______________________________________________________________ 779
Overview of Edema ________________________________________________________________________ 782
Description of Conditions That May Lead to Edema __________________________________ 783
Chapter 39 Case Demonstration: Edema and Shoulder Pain __________________________ 798
Marisa Perdomo, PT, DPT • Chris A. Sebelski, PT, DPT, OCS, CSCS
Case Description____________________________________________________________________________ 798
Case Outcome ________________________________________________________________________ 811
Chapter 40 Failure of Wounds to Heal ________________________________________________________ 812
Rose Hamm, PT, DPT, CWS, FACCWS
Description of the Symptom ______________________________________________________________ 812
Overview of Wounds That Fail to Heal __________________________________________________ 815
Description of Conditions That May Lead to Failure of Wounds to Heal ______________ 815
Chapter 41 Case Demonstration: Fatigue ______________________________________________________ 833
Kim Levenhagen, PT, DPT, WCC • Chris A. Sebelski, PT, DPT, OCS, CSCS
Case Description ____________________________________________________________________________ 833
Case Outcome ________________________________________________________________________ 839

Section IV Children 841


Chapter 42 Special Diagnostic Issues in Children ____________________________________________ 841
Sharon K. DeMuth, PT, DPT, MS • Hugh G. Watts, MD
Introduction ________________________________________________________________________________ 841
Special Features of History Taking in Children __________________________________________ 841
The Age of a Child is the Defining Feature in Differential Diagnosis ________________ 841
The History is Generally Obtained Secondhand Through the Parent ______________ 842
Obtaining Information About a Child’s Development ________________________________ 842
Some Pitfalls __________________________________________________________________________ 843
Never Forget the Possibility of Child Abuse ____________________________________________ 844
Special Features of the Physical Examination of a Child ______________________________ 844
Get to the Child’s Level ________________________________________________________________ 844
Watch While the Child is Waiting to Be Seen ________________________________________ 844
Potential Problems with Disrobing __________________________________________________ 844
Examine the Painful Area Last ________________________________________________________ 845
Confirmation of Discrepancies ________________________________________________________ 845
Examining the Child to Obtain Information About the Child’s Development ______ 845
Issues Related to Growth of Bones in Children ________________________________________ 846
Growth of the Long Bones ____________________________________________________________ 846
Growth Disturbances Due to Abnormal Muscle Activity ______________________________ 846
Leg-Length Discrepancy ______________________________________________________________ 846
Differences in Fractures Between Children and Adults ________________________________ 846
Epiphyseal Fractures __________________________________________________________________ 846
Physiological Differences ______________________________________________________________ 848
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Contents xxix

Mechanical Differences ________________________________________________________________ 848


Other Considerations __________________________________________________________________ 849
Conclusion __________________________________________________________________________________ 849
Chapter 43 Elbow Pain in a Child ________________________________________________________________ 851
Julia L. Burlette, PT, DPT, OCS • Jill S. Masutomi, PT, DPT, PCS
Description of the Symptom ______________________________________________________________ 851
Overview of Elbow Pain in a Child ________________________________________________________ 859
Description of Conditions That May Lead to Elbow Pain in a Child __________________ 859
Remote ________________________________________________________________________________ 859
Local __________________________________________________________________________________ 860
Chapter 44 Back Pain in a Child __________________________________________________________________ 873
Munesha Ramsinghani Lona, PT, DPT, PCS • Josiane Stickles, PT, DPT
Description of the Symptom ______________________________________________________________ 873
Overview of Back Pain in a Child ________________________________________________________ 878
Description of Conditions That May Lead to Back Pain in a Child __________________ 878
Remote ________________________________________________________________________________ 878
Local ____________________________________________________________________________________ 880
Chapter 45 Hip Pain in a Child ____________________________________________________________________ 898
Stephanie A. Jones, PT, DPT, OCS, NCS
Description of the Symptom ______________________________________________________________ 898
Overview of Hip Pain in a Child __________________________________________________________ 904
Description of Conditions That May Lead to Hip Pain in a Child ______________________ 904
Remote ________________________________________________________________________________ 904
Local __________________________________________________________________________________ 906
Chapter 46 Case Demonstration: Hip Pain in a Child ______________________________________ 925
Hugh G. Watts, MD • Chris A. Sebelski, PT, DPT, OCS, CSCS
Case Description __________________________________________________________________________ 925
Case Outcome ________________________________________________________________________ 932
Chapter 47 Knee Pain in a Child ____________________________________________________________________ 933
Jennifer Lundberg, PT, DPT • Cassandra Sanders-Holly, PT, DPT, PCS
Description of the Symptom ______________________________________________________________ 933
Overview of Knee Pain in a Child ________________________________________________________ 939
Description of Conditions That May Lead to Knee Pain in a Child __________________ 939
Remote ________________________________________________________________________________ 939
Local __________________________________________________________________________________ 942
Chapter 48 Case Demonstration: Knee Pain and Limping in a Child ________________ 959
Todd E. Davenport, PT, DPT, OCS • Sharon K. DeMuth, PT, DPT, MS
Case Description ____________________________________________________________________________ 959
Case Outcome ________________________________________________________________________ 966
Chapter 49 Shin Pain in a Child __________________________________________________________________ 967
Covey J. Lazouras, PT, DPT, NCS
Description of the Symptom ______________________________________________________________ 967
Overview of Shin Pain in a Child ________________________________________________________ 971
Description of Conditions That May Lead to Shin Pain in a Child ______________________ 971
Remote ________________________________________________________________________________ 971
Local __________________________________________________________________________________ 972
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xxx Contents

Chapter 50 Ankle and Foot Pain in a Child __________________________________________________ 982


Robert C. Sieh, PT, DPT
Description of the Symptom ______________________________________________________________ 982
Overview of Ankle and Foot Pain in a Child ______________________________________________ 988
Description of Conditions That May Lead to Ankle and Foot Pain in a Child ________ 988
Remote ________________________________________________________________________________ 988
Local __________________________________________________________________________________ 989
Chapter 51 Case Demonstration: Foot Pain in a Child __________________________________ 1007
Hugh G. Watts, MD
Case Description ____________________________________________________________________________ 1007
Case Outcome ________________________________________________________________________ 1012
Chapter 52 Delayed Mobility (Crawling and Walking), Failure to
Thrive, and Psychomotor Delay in a Child __________________________________ 1013
Phibun Ny, PT, DPT • Shelly Olivadoti-Santoro, PT, DPT •
Cassandra Sanders-Holly, PT, DPT, PCS
Description of the Symptom ____________________________________________________________ 1013
Overview of Delayed Mobility, Failure to Thrive, and Psychomotor
Delay in a Child ______________________________________________________________________ 1017
Description of Conditions That May Lead to Delayed Mobility,
Failure to Thrive, and Psychomotor Delay in a Child ______________________________ 1017
Chapter 53 The Child With a Painless Limp ________________________________________________ 1035
Hugh G. Watts, MD
Description of the Symptom ______________________________________________________________ 1035
Overview of a Painless Limp in a Child ________________________________________________ 1039
Causes of Limping ________________________________________________________________________ 1039
Reduce Pain __________________________________________________________________________ 1040
Compensate for Muscle Weakness ____________________________________________________ 1040
Accommodate a Deformity __________________________________________________________ 1041
Central Nervous System Dysfunction ________________________________________________ 1041
Descriptions of Conditions That May Lead to a Painless Limp in a Child __________ 1041
Chapter 54 Tripping and Falling in a Child ____________________________________________________ 1056
Christy L. Skura, PT, DPT, PCS • Bach T. Ly, PT, DPT
Description of the Symptom ______________________________________________________________ 1056
Overview of Tripping and Falling in a Child __________________________________________ 1060
Description of Conditions That May Lead to Tripping and Falling in a Child ________ 1061
Chapter 55 Toe Walking in a Child ______________________________________________________________ 1072
Christy L. Skura, PT, DPT, PCS • Bach T. Ly, PT, DPT
Description of the Symptom ______________________________________________________________ 1072
Overview of Toe Walking in a Child ______________________________________________________ 1076
Description of Conditions That May Lead to Toe Walking in a Child ________________ 1076
Chapter 56 Joint Contractures in a Child ______________________________________________________ 1087
Cassandra Sanders-Holly, PT, DPT, PCS •
Cheri Kay Sessions, PT, DPT, ATC
Description of the Symptom ______________________________________________________________ 1087
Overview of Joint Contractures in a Child ______________________________________________ 1090
Description of Conditions That May Lead to Joint Contractures in a Child ________ 1091
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Contents xxxi

Chapter 57 Torticollis in a Child ________________________________________________________________ 1099


Hugh G. Watts, MD • Sharon K. DeMuth, PT, DPT, MS
Description of the Symptom ______________________________________________________________ 1099
Overview of Torticollis in a Child ________________________________________________________ 1102
Description of Conditions That May Lead to Torticollis in a Child __________________ 1103
Chapter 58 Case Demonstration: Torticollis in a Child __________________________________ 1108
Sharon K. DeMuth, PT, DPT, MS • Chris A. Sebelski, PT, DPT, OCS, CSCS
Case Description ____________________________________________________________________________ 1108
Case Outcome ________________________________________________________________________ 1112
Chapter 59 Poor Posture and Scoliosis, Kyphosis, and Lordosis
in a Child ________________________________________________________________________________ 1113
Sharon K. DeMuth, PT, DPT, MS • Stephanie A. Jones, PT, DPT, OCS, NCS
Description of the Symptom ______________________________________________________________ 1113
Overview of Poor Posture and Scoliosis, Kyphosis, and Lordosis in a Child ________ 1126
Scoliosis ____________________________________________________________________________________ 1126
Structural Kyphosis __________________________________________________________________ 1127
Lordosis ________________________________________________________________________________ 1127
Description of Conditions That May Lead to Poor Posture and
Scoliosis, Kyphosis, and Lordosis in a Child ______________________________________ 1128
Chapter 60 Bowed Legs and Knock Knees in a Child __________________________________ 1149
Wendi W. McKenna, PT, DPT, PCS
Description of the Symptom ____________________________________________________________ 1149
Overview of Bowed Legs and Knock Knees in a Child ________________________________ 1153
Description of Conditions That May Lead to Bowed Legs in a Child ________________ 1153
Description of Conditions That May Lead to Knock Knees in a Child ______________ 1158
Chapter 61 In-Toeing and Out-Toeing in a Child ____________________________________________ 1165
Wendi W. McKenna, PT, DPT, PCS
Description of the Symptom ______________________________________________________________ 1165
Overview of In-Toeing and Out-Toeing in a Child ______________________________________ 1168
Description of Conditions That May Lead to In-Toeing in a Child __________________ 1169
Description of Conditions That May Lead to Out-Toeing in a Child ________________ 1173
Chapter 62 Flat Feet in a Child ____________________________________________________________________ 1180
Wendi W. McKenna, PT, DPT, PCS
Description of the Symptom ______________________________________________________________ 1180
Overview of Flat Feet in a Child ________________________________________________________ 1183
Description of Conditions That May Lead to Flat Feet in a Child ____________________ 1184
Chapter 63 Weakness and Hypotonia in a Child __________________________________________ 1190
Elizabeth L. Ege, PT, DPT
Description of the Symptom ______________________________________________________________ 1190
Overview of Weakness and Hypotonia in a Child ______________________________________ 1195
Description of Conditions That May Lead to Weakness and Hypotonia in
a Child ________________________________________________________________________________ 1196
Index ______________________________________________________________________________________ 1205
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1528_Ch01_001-007 07/05/12 12:52 PM Page 1

SECTION I
Foundations

CHAPTER1
Why Should Physical Therapists Know
About Diagnosis?
■ James Gordon, PT, EdD, FAPTA ■ Hugh G. Watts, MD

IN THIS CHAPTER: central tenet of the American Physical Therapy


■ Development of professional responsibility Association’s Vision 2020 statement, which
and education in physical therapy looks forward to physical therapists being “rec-
■ Role of diagnosis in the professional vision ognized by consumers and other health care
for the future of physical therapy professionals as the practitioners of choice to
■ Definition of diagnosis whom consumers have direct access for the di-
■ Conceptualization of diagnosis by physical agnosis of, interventions for, and prevention of
therapists impairments, functional limitations, and dis-
abilities related to movement, function, and
OUTLINE health.”1 One clear assumption of Vision 2020,
with its emphasis on direct access, is that phys-
Introduction 1 ical therapists must be able to engage in the
Five Critical Issues Frame the Topic of diagnostic process as part of their overall eval-
Diagnosis by Physical Therapists 2 uation, in order to develop a well-reasoned
Critical Issue 1: What is a Diagnosis? 3 intervention plan for addressing the patient’s
Critical Issue 2: Should Physical Therapists problems. This process must lead to a decision
Diagnose Pathology? 4 regarding the probable pathological or patho-
Critical Issue 3: Should Physical Therapists Adopt a physiological cause of the patient‘s problem,
Different Definition for Diagnosis Than Other followed by a determination of whether phys-
Health Professionals Use? 4 ical therapy intervention is the most appropri-
Critical Issue 4: Should Physical Therapists be ate to address the underlying pathology of the
Expected to Diagnose Given the Current Legal patient’s condition. Again, it must be empha-
Environment? 5 sized that Vision 2020, with its emphasis on
Critical Issue 5: What Information Should Physical direct access, presupposes that the physical
Therapists Use to Make Decisions About therapist will be able to reach that decision
Treatment and Prognosis? 6 without provision of a diagnosis beforehand
Conclusion 6 by a physician or other health care professional.
The development of diagnostic skills by
physical therapists is a critical step to estab-
Introduction lishing the autonomy necessary for universal
direct access to physical therapy. Since the
The close of physical therapy’s first 100 years introduction of a groundbreaking textbook on
marks a new direction in its history as a young medical screening in 1990,2 physical therapists
profession—establishing the autonomy neces- have made considerable progress toward the
sary to ensure patients can benefit from safe, development of systematic approaches to the
efficient, and universal direct access to physical consideration of pathology. Despite these ad-
therapy. Direct access to physical therapy is a vances, however, the profession lacks a strong

1
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2 Chapter 1 Why Should Physical Therapists Know About Diagnosis?

tradition of diagnosis.3 Many experienced Second, ongoing changes in the health care en-
physical therapists have developed excellent vironment shape the needs of patients and
diagnostic skills, but they have succeeded the clients and, as a result, the requisites of physi-
hard way—by independent study, on-the-job cal therapy as a profession. Therefore, our
training, and trial and error. To reach the goal discussion is a snapshot in time of current
of direct access, the physical therapy profes- thinking on this topic that continues to evolve.
sion must continue to develop systematic, In addressing the current controversy re-
evidence-based approaches for diagnosis that garding diagnosis by physical therapists, we
are taught in entry-level curricula, so that all believe five critical issues must be discussed:
physical therapists can be presumed to have
adequate skill in diagnosis. 1. What is a diagnosis? We assert that forming
This textbook adds to the effort to develop a diagnosis is a clinical reasoning process re-
physical therapists’ diagnostic skills for recog- lated to the patient’s underlying pathology that
nizing pathologies in a significant and unique concludes with a clinical label. The fundamen-
way. The purpose of this book is to present a tal relevance of the diagnostic label in main-
framework for physical therapists to become stream health care is as an identifier of the
more skilled diagnosticians of pathology and underlying pathology that is the cause of the
provide a ready resource to support the work patient’s condition. Physical therapists also
of physical therapists in direct access environ- use diagnostic reasoning to determine working
ments. It proposes a detailed symptom-based hypotheses regarding the physical impair-
approach for diagnosis based on the notion ments that may either result from, or con-
that, in starting the process of diagnostic in- tribute to, their patients’ presenting pathology.
vestigation, the physical therapist must begin 2. Should physical therapists diagnose pathol-
with the patient’s symptoms and signs. Much ogy? We propose that physical therapists are
like the collective understanding that it repre- profoundly involved in the diagnostic inves-
sents and to which it contributes, the material tigation of the patient’s pathology and that
presented in this textbook remains a work in they make critical decisions based on their
progress. conclusions about the nature, location, and
severity of the pathology. Even when making
Five Critical Issues Frame the most basic determination of whether a pa-
the Topic of Diagnosis by tient’s or client’s condition is amenable to
Physical Therapists physical therapy, the physical therapist makes
a diagnostic decision.
Despite the overwhelming recognition of the 3. Should physical therapists adopt a different
need for more systematic approaches to diagno- definition for diagnosis than other health pro-
sis by physical therapists—especially approaches fessionals? We contend that it is a mistake to
that can be taught to physical therapists during have a separate “physical therapy diagnosis,”
entry-level educational programs—considerable because the diagnostic label belongs to the pa-
controversy surrounds the topic of diagnosis by tient or client and not a specific profession.
physical therapists, particularly as it relates to We also propose that physical therapists’ use
pathology. of diagnostic reasoning to determine the un-
The rest of this chapter is devoted to dis- derlying mechanical etiology for the present-
cussing a conceptual perspective about diag- ing pathology is similar to the process by
nosis by physical therapists. In understanding which many other health care providers use
the physical therapist’s role as a diagnostician, their unique expertise to elaborate on the
we must agree to acknowledge two items. First, diagnostic label in order to determine a prog-
physical therapists fill similar professional nosis and appropriate interventions for a
roles, despite often substantial differences in patient or client.
practice area and physical proximity to refer- 4. Should physical therapists be expected to diag-
ring physicians. With this in mind, we will de- nose given the current legal environment? We
velop a unifying philosophy regarding the role propose that physical therapists have an eth-
of diagnostic reasoning in physical therapy. ical responsibility to use their skills to ensure
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Chapter 1 Why Should Physical Therapists Know About Diagnosis? 3

that patients receive the best possible care than consider it as a well-characterized, specific
and that this responsibility need not conflict label of pathology or pathophysiology. Sackett
with legal restrictions. defined the target disorder as:
5. What information should physical therapists
The anatomical, biochemical, physiologic,
use to make decisions about treatment and
or psychological derangement whose etiol-
prognosis? We suggest that detailed inves-
ogy (if known), maladaptive mechanisms,
tigations of the causes of movement dys-
presentation, prognosis, and management
function, and classification systems based
we read about in medical texts. Although
on clinical decision rules, are important
this element is usually called the disease,
evaluative processes conducted by physical
the usefulness of this ambiguous term is
therapists that characterize the search for
hampered by the inability of both patients
an underlying etiology. These approaches
and health scientists to agree on its appli-
will be strengthened by an association with
cation to specific situations. Accordingly,
the diagnosis of pathology made by phys-
we shall call this element of patient’s sick-
ical therapists, because they cannot be con-
ness the target disorder when it becomes
sidered conclusive without considering
the objective of the diagnostic process.4
pathology.
In this passage, Sackett and his colleagues4
Each of these critical issues is discussed in
are expressing what is generally accepted about
greater detail throughout the remainder of this
diagnostic labels: the diagnostic label frequently
chapter.
fails to precisely identify the disease or pathol-
Critical Issue 1: What is a Diagnosis? ogy. Nevertheless, a clinician’s application of a
diagnostic label represents the best attempt at a
As it is ordinarily understood in health care, di- shorthand categorization of the patient’s or
agnosis is defined as the process and product of client’s condition with a link to the underlying
a clinical investigation related to the pathology cause of the patient’s signs and symptoms.
underlying a patient’s or client’s symptoms and Physical therapists must make a decision
signs. The process of diagnosis involves a com- regarding the nature of the pathology and its
bination of inductive and deductive reasoning. severity in order to determine whether physi-
The product of the clinical reasoning process of cal therapy is appropriate to address the pa-
diagnosis is a label, which serves as shorthand tient’s condition. In Chapter 2, Dr. Rob Landel
communication between providers. This label discusses how physical therapists make deci-
is called a diagnosis. We put forward that the sions based on diagnostic information. For
term diagnosis should be used by physical ther- this discussion, deciding that the pathology
apists in the same manner as the rest of the causing a patient’s pain is not cancer or not
health care system. To be certain, all health care myocardial infarction or not an infection in
practitioners are constrained by their training, the kidney requires the physical therapist to
resources, practice-related legislation, and cur- undertake a diagnostic process in which the
rent knowledge. Therefore, there are many in- nature of the pathology is investigated, and
stances throughout the health care system in these pathologies are ruled as less likely
which the precise cause of the condition can- causes of the patient’s symptoms. Although
not be determined due to a lack of knowledge. the physical therapist does not always make
In these cases, the diagnostic label will neces- the definitive determination of the diagnostic
sarily be descriptive. Nevertheless, even in these label that will go in the patient’s medical
cases, the presumption is that the label would record, this does not mean that the therapist
identify the pathology if more information is not actively engaged in diagnosing pathol-
were available. ogy. Therefore, we propose that two interme-
Because the result of diagnostic reasoning diate stages of labeling are important for
is a label that may lack pathological specificity, physical therapists:
Sackett and colleagues4 proposed that we
might think of diagnostic labeling as an at- ● Physical therapists may form a diagnostic
tempt to identify the target disorder, rather impression, which is a working diagnosis
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4 Chapter 1 Why Should Physical Therapists Know About Diagnosis?

requiring further verification. In documen- physicians for patients presenting to outpatient


tation, the therapist may use the term im- orthopedic physical therapy clinics, Davenport
pression to put forward a possible diagnostic and colleagues5 found that less than a third of
label, or he or she may state that a patient’s referrals to physical therapy practices were
signs and symptoms “are consistent with” a specific as to the nature of the pathology and
certain diagnostic label. anatomical location. Many referral diagnoses
● An even more preliminary stage between were not much more than a restatement of the
the diagnostic process and a label is the di- patients’ symptoms. This suggests that at least
agnostic decision. This is the situation in some portion of physical therapists’ time in
which the physical therapist does not defin- the initial evaluative process is spent on two
itively label the condition, but makes a functions: (1) deciding whether the patient
decision based on a presumptive label. For presents with pathology that is amenable to
example, when a physical therapist decides physical therapy interventions and (2) how the
on a referral disposition, such as to send a nature and acuity of the pathology will shape
patient back to the referring physician in- the intervention plan. This makes the diagno-
stead of activating the emergency medical sis of pathology by physical therapists an im-
system, the therapist has made a diagnostic portant professional function.6
decision. This decision is based on conclu- First and foremost, it is the physical ther-
sions that the therapist has drawn about the apist’s responsibility to determine whether
pathology and its severity. the patient’s condition is appropriate for
physical therapy intervention. During the
Despite the less specific nature of diagnos- course of assessing and reassessing the pa-
tic labeling implied by diagnostic impression tient, information is often uncovered that
and diagnostic decision, a systematic and rigor- may necessitate modification of the original
ous diagnostic clinical reasoning process diagnosis. In this case, it is the legal and pro-
should inform physical therapists’ diagnostic fessional responsibility of the physical thera-
impressions and diagnostic decisions. Dr. Todd pist to bring this information to the attention
Davenport discusses the theory and applica- of the referring practitioner. The direct access
tion of cognitive science related to diagnostic environment places special importance on
judgments and decision making in Chapter 3. In this function, because physical therapists are
Chapter 4, Dr. Hugh Watts describes a process charged with making the initial observations
for arriving at a diagnostic label, diagnostic im- that suggest the nature of the underlying
pression, or diagnostic decision that describes pathology. This information is used to refer
the target disorder. Subsequently, Dr. Watts the patient to a physician or other health care
provides a case example to demonstrate the provider if further investigation or treatment
specific process for diagnosis around which is warranted, or if the treatment of the
this book is arranged. specific pathology is outside the scope of
physical therapy.
Critical Issue 2: Should Physical
Therapists Diagnose Pathology? Critical Issue 3: Should Physical
Therapists Adopt a Different
Physical therapists are often essential partici-
Definition for Diagnosis Than
pants in the process of identifying the pathology
Other Health Professionals Use?
that is causing a patient’s condition. In many in-
stances, physical therapists further specify the Physical therapists should use the common
nature of the pathology or its precise anatomical definition of diagnosis as a clinical reasoning
location when the diagnosis is within the physi- process and its resultant label for a variety of
cal therapist’s scope of practice. In some cases, reasons. Perhaps most importantly, a common
this means refining or correcting an initial diag- terminology is essential for physicians to un-
nosis by bringing new information to the atten- derstand which diagnoses are appropriate for
tion of the referring practitioner. referral to a physical therapist. As a practical
In a recent multicenter retrospective study matter, the reimbursement systems as well as
involving referral diagnoses provided by most large outcome databases are inextricably
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Chapter 1 Why Should Physical Therapists Know About Diagnosis? 5

linked to diagnoses. Finally, the database of physical therapist engages in the screening
clinical research evidence that physical thera- process, the patient is asked a series of general
pists both use and contribute to is primarily questions, often referred to as the systems
organized around the mainstream pathology- review.7 The goal of this part of the history
based diagnostic system. taking is not to define a diagnosis, but to make
Adoption of a differing definition for diag- sure that the patient has not neglected to men-
nosis may expose physical therapists and the tion an issue that may have an impact on
physical therapy profession to a communica- the patient’s overall well-being. By contrast,
tion disconnection from the mainstream the diagnostic interview uses directed ques-
health care community. One example of such tions and focused physical examination tests to
ambiguity arises in the term physical therapy answer the specific question “What is the dis-
diagnosis, which is often used in contradis- ease process that is causing my patient’s symp-
tinction to the term medical diagnosis. This toms?” Indeed, physical therapists should be
seems acceptable if these terms refer to the prepared to engage in both diagnosis and
processes of diagnosis in which physical thera- medical screening, because they serve different
pists and physicians engage, respectively, al- and important functions in practice.
though it would be more precise, then, to refer
to a physical therapist’s diagnosis and a physi-
Critical Issue 4: Should Physical
cian’s diagnosis. However, more often these
Therapists be Expected to
two terms appear to be used to distinguish the
Diagnose Given the Current
diagnostic labels that are assigned by physical
Legal Environment?
therapists and physicians. With this definition,
physical therapy diagnosis implies that when a Certainly, several legal and economic barriers
physical therapist engages in diagnosis, a dif- impede diagnosis by physical therapists who
ferent type of label results than when a physi- practice in many areas. These include state
cian engages in diagnosis. We assert that there practice act restrictions on diagnostic labeling
should not be a physical therapy diagnosis that and ordering of diagnostic tests, as well as the
is separate and distinct from the medical diag- lack of reimbursement by third-party payers
nosis. In the mainstream health care commu- for services related to diagnosis performed by
nity, there is simply the diagnosis and it belongs physical therapists. Any meaningful discussion
to the patient—not to a specific health care of the complexity of the laws, regulations, and
discipline. payment related to physical therapist practice
At best, introducing a separate category of is beyond the scope of this textbook. Neverthe-
diagnostic label for physical therapists invites less, we reiterate our assertion that the physical
confusion; at worst, this separate category will therapist has a professional and ethical respon-
invite derision and isolation from colleagues in sibility, regardless of how the state practice act
the health care community. The nursing pro- may read, to use his or her professional judg-
fession’s attempts to establish nursing diagnoses ment to make the most accurate determina-
were unsuccessful. If the physical therapy pro- tion of the underlying pathology, to inform
fession adopts a complementary and alterna- the physician of findings that may conflict
tive diagnostic language, it risks being viewed with the physician’s diagnosis, or to suggest
as providers of complementary and alternative tests that might clarify the diagnosis. Indeed,
treatments. Indeed, a private diagnostic system in the modern health care system, such a col-
in the physical therapy profession will not help laborative approach to diagnosis has become
it to foster optimal communication using a more the rule than the exception; physicians
common language. and health care professionals of different
It might seem politically advantageous to specialties all provide input to the diagnostic
avoid conflict with physicians by using the decision-making process.
term medical screening in place of diagnosis To summarize, physical therapists use
of pathology; however, this also leads to confu- diagnostic skills to first differentiate whether a
sion by redefining a word that has a common patient’s presenting pathology appears amenable
and consistent usage in medicine. When a to physical therapy intervention. In turn, this
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6 Chapter 1 Why Should Physical Therapists Know About Diagnosis?

information is used to determine whether the and different practice patterns within the
patient should receive physical therapy treat- physical therapy profession. It is critical for
ment, be referred to another health care physical therapists to elaborate on the referral
provider for additional testing or treatment, or diagnosis with information that further re-
both. Also, physical therapists should deter- fines the description of patients’ disablement,
mine the appropriate disposition and speed of or to further classify the patient within an ac-
referral based on their estimate of the nature cepted schema to define the appropriate treat-
and acuity of the underlying pathology. ment. Indeed, the first assumption in any of
Implicit in these decisions is a specific these approaches is that physical therapy is
consideration by physical therapists of how appropriate for the patient. Therefore, diag-
each form of pathology presents and con- nosis of pathology by physical therapists
tributes to the patient’s symptoms or disable- should play a prominent role in physical ther-
ment. As we discussed previously, the speci- apist practice, similar to the search for an un-
ficity of the diagnostic label that the physical derlying etiology that is represented by the
therapist provides reflects the training of classification of patient clinical presentation
physical therapists, constraints on ordering according to disablement models, impairment
tests, and the perceived acuity of the patient’s clusters, and clinical decision rules that pre-
condition. Physical therapists also use infor- dict response to treatment.
mation regarding the nature of pathology in We propose that, in the long run, linking
making treatment decisions when that pathol- classification-driven evaluative systems with
ogy has been determined to respond to physi- pathology-based diagnosis will strengthen the
cal therapy interventions (i.e., tendinitis physical therapy profession by establishing re-
versus tendinosis). If the diagnosis of pathol- lationships between our unique professional
ogy is excluded from physical therapist prac- reasoning and knowledge and the established
tice out of concern about inappropriate diagnostic language of health care.
consideration of the systemic disorders be-
yond the scope of physical therapy, this also Conclusion
would preclude the ability to diagnose muscu-
loskeletal and neuromuscular pathologies to The physical therapy profession has set as one
the extent that physical therapy treatment of its highest priorities the achievement of direct
decisions can be made. access. Refining the ability of all physical ther-
apists to carry out the process of diagnosis of
Critical Issue 5: What Information pathology is a necessary prerequisite for direct
Should Physical Therapists Use to access and, thus, is the focus of this textbook.
Make Decisions About Treatment We do not believe that accomplishment of this
and Prognosis? objective will result from downplaying the im-
Since the publication of several papers in the portance of diagnostic skills. Indeed, the major
1980s advocating for more detailed evaluative argument of organized opposition to direct
processes by physical therapists, several differ- access is that physical therapists are inade-
ent systems have developed. Some have sug- quately trained to recognize serious diseases.
gested that physical therapists should classify Over the long term, the physical therapy pro-
problems according to the constellations of fession will achieve direct access by continuing
impairments.3 Others have developed systems to demonstrate that physical therapists are well
that are intended to identify the relationships trained to systematically investigate the disease
between impairments and functional limita- processes that cause physical disablement with
tions, using disablement models.8 Still others sufficient specificity to assist in the achieve-
have argued for classification systems based ment of an accurate diagnosis within the con-
on clinical decision rules that identify the in- text of the health care team.
tervention of choice.9–11 In principle, none of
these approaches is mutually exclusive. In References
1. American Physical Therapy Association. Vision 2020.
fact, it appears that different approaches are http://www.apta.org/vision2020. Accessed June 20,
considered more useful for different patients 2009.
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Chapter 1 Why Should Physical Therapists Know About Diagnosis? 7

2. Goodman C, Snyder T. Differential Diagnosis in Physical 8. Sullivan KJ. Role of the physical therapist in neurologic
Therapy. Philadelphia: WB Saunders; 1990. differential diagnosis: a reality in neurologic physical
3. Sahrmann S. Are physical therapists fulfilling their therapist practice. J Neurol Phys Ther. 2007;31(4): 236–237.
responsibilities as diagnosticians? J Orthop Sports Phys 9. Fritz JM, Cleland JA, Childs JD. Subgrouping patients
Ther. 2005;35(9):556–558. with low back pain: evolution of a classification
4. Sackett DL, Straus SE, Richardson WS, Rosenberg W, approach to physical therapy. J Orthop Sports Phys Ther.
Haynes RB. Evidence-Based Medicine: How to Practice 2007;37(6):290–302.
and Teach EBM. 2nd ed. New York, NY: Churchill 10. Childs JD, Fritz JM, Piva SR, Whitman JM. Proposal
Livingstone; 2000. of a classification system for patients with neck pain.
5. Davenport TE, Watts HG, Kulig K, Resnik C. Current J Orthop Sports Phys Ther. 2004;34(11):686–696; dis-
status and correlates of physicians’ referral diagnoses cussion 697–700.
for physical therapy. J Orthop Sports Phys Ther. 11. Delitto A, Erhard RE, Bowling RW. A treatment-based
2005;35(9):572–579. classification approach to low back syndrome: identify-
6. Davenport TE, Kulig K, Resnik C. Diagnosing pathology ing and staging patients for conservative treatment. Phys
to decide the appropriateness of physical therapy: what’s Ther. 1995;75(6):470–485; discussion 485–489.
our role? J Orthop Sports Phys Ther. 2006;36(1):1–2.
7. American Physical Therapy Association. Guide to phys-
ical therapist practice, second edition. Phys Ther.
2001;81(1):9–746.
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CHAPTER2
Beyond the Diagnosis: The Search
for Underlying Etiology
■ Rob Landel, PT, DPT, OCS, CSCS, FAPTA

IN THIS CHAPTER: reached, for example, the cardiac surgeon


■ How physical therapists use and elaborate on
looks for the offending coronary artery, the in-
diagnostic labels ternist measures cholesterol and blood pres-
■ A process physical therapists use to search
sure, the dietician looks at eating habits, and
for underlying etiology the physical therapist examines exercise pat-
terns. Each health care provider goes beyond
OUTLINE the diagnosis in search of the underlying etiol-
ogy, in order to provide the optimal manage-
Introduction 8 ment plan.
Physical Therapists Use the Diagnostic The process of searching for an underlying
Label 9 etiology by a physician may be more familiar
Physical Therapists Elaborate on the to some than the search for an underlying
Diagnostic Label 10 etiology by a physical therapist. This chapter
The Diagnostic Label Alone is Insufficient provides an overview of the ways physical
to Guide Physical Therapists 11 therapists use diagnostic labels in making
A Process Guides Physical Therapists’ treatment and prognosis decisions, as well as
Search for an Underlying Etiology 12 how physical therapists search for the underly-
Physical Therapists Identify the Functional Chief ing etiology of a pathology.
Concern 13 Many health care providers wish to iden-
Physical Therapists Perform Task and Movement tify the tissue or pathology responsible for a
Analyses 13 patient’s symptoms, and they strive to under-
Physical Therapists Create and Test Hypothetical stand the root cause of the chief concern.
Relationships 13 Physical therapists use and elaborate on the
The Search for an Underlying Etiology is Dynamic diagnostic label for purposes of treatment.
and Ongoing 14 Perhaps the most intuitive manner in which
Conclusion 14 the physical therapist can elaborate on the di-
agnostic label is to revise it with their diagnos-
tic label, impression, or decision as described
Introduction in the previous chapter. For example, a patient
referred to a physical therapist with a diagno-
In the previous chapter, a process for symptom- sis of Back Pain may be referred back to the
based diagnosis was described. The outcome physician after a physical therapy evaluation
of that process is the diagnostic label, which with the diagnostic impression of Kidney Stone
is the pathology or the pathological process vs. Urinary Tract Infection. In this example, the
that is the root cause of the patient’s or client’s physical therapist is elaborating on the initial
chief concern.1–3 Once a diagnosis has been diagnostic label by advocating additional test-
established, the clinician’s evaluation must ing that may result in the label’s revision. Phys-
continue until the etiology of the pathology ical therapists, like other health care providers,
is uncovered. The focus of this investigation also use and elaborate on the diagnostic label to
is specific to the discipline of the searcher. create a plan for intervention and prognosis. This
Once a diagnosis of myocardial infarction is involves a search for the underlying etiology of

8
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Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology 9

the pathology denoted by the diagnostic label. might well be referred to physical therapy with
Diagnostic reasoning at the level of pathology a referral diagnosis of Shoulder Pain. Upon
is a similar process for both physicians and questioning, however, it becomes apparent
physical therapists and also for other health that the pain is aggravated by activities such as
care providers in allopathic settings. The walking or ascending stairs. A review of his
search for the underlying etiology, however, is medical history reveals he is a smoker, has high
specific to the discipline, and therefore physi- blood pressure and hypercholesteremia, is
cal therapists’ unique professional focus and sedentary, and had parents who died young
expertise characterize their search. of a stroke and a heart attack, respectively.
Another patient is referred for a diagnosis of
Physical Therapists Use Balance Retraining who in her history relates
the Diagnostic Label no imbalance but does have short-duration
dizzy spells that occur only when she rolls over
Given a particular patient problem, the first in bed onto her left side. Whereas the first
goal of the physical therapist’s evaluation is to patient requires an urgent referral to a cardiol-
determine if managing the causative pathol- ogist to investigate his angina, the second
ogy is within the scope of physical therapy. patient can be treated effectively for benign
This critical first step ensures the safety of the paroxysmal positional vertigo by the physical
patient, integrates the physical therapist into therapist in one or two visits and a physician
the health care system on a collegial basis, and consult is not needed.
results in an appropriate plan of care. This first In cases where caution is indicated as the
decision-making process leads to three possi- examination proceeds, the physical therapist
ble outcomes: must decide if the examination (and subse-
● Physical therapy is not indicated, quent intervention) can proceed without the
● Physical therapy is indicated but a consulta- need for further consultation with other
tion to another health care provider is health care providers. If so, in a patient with
required, or ankle pain who does not meet the criteria
necessitating radiographs,4,5 for example, or a
● Physical therapy can proceed independent
patient with transient dizziness whose clinical
of additional consultation.
examination reveals benign positional parox-
If physical therapy is not indicated, then ysmal vertigo,6–9 the diagnostic process con-
the physical therapist must decide how tinues until the underlying cause is found.
urgently the referral should be made. The Clearly, if the physical therapist’s evaluation
physical therapy episode of care ends when it does not reveal a specific pathology, then a
becomes apparent that referral to another consultation request made to a provider in the
health care provider for additional evaluation area of practice most likely to uncover the di-
and treatment will be more beneficial to the agnosis is warranted. It is likely that most cases
patient than either beginning or continuing will require this type of referral. The physical
physical therapy. This may be immediately therapist should proceed with the diagnostic
apparent, for example, in the patient referred process as far as is safely possible in order to
for shoulder pain who presents with shortness provide the consultant with as much informa-
of breath, bilateral extremity edema, general tion as possible.
pallor, and tachycardia. At the other end of the Even if physical therapy is deemed the
spectrum, it may not be until the patient has appropriate management strategy for the
had several treatment sessions but has failed to patient’s disablement, it remains essential to
meet expected goals for improvement that identify the presence of a medical condition
physical therapy is halted and referral to an- that will affect the outcome of any physical
other health care provider is made. Either way therapy intervention. Medical comorbidities
the diagnostic process should still be com- must be taken into consideration when de-
pleted beyond the simple identification of the signing a physical therapy intervention. For
presence of Red Flags. For example, a 67-year-old example, the presence of osteoporosis may
male patient’s report of left shoulder pain affect the nature of any intervention provided
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10 Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology

to a patient with a history of thoracic spine label to determine the most appropriate
pain, since the patient’s symptoms may be intervention and prognosis. Physicians can
related to compression fractures. Because often treat the pathology directly, whereas
confirmation and grading of osteoporosis physical therapists can do so only in some
requires access to imaging modalities outside cases (e.g., treating inflammation as noted
the scope of practice for physical therapists to above). Most interventions that a physical
order, referral to a physician for additional test- therapist can employ do not directly address
ing if a high index of clinical suspicion is present the pathological tissue. For example, physical
would be prudent, even though osteoporosis therapy interventions cannot directly address
itself is not a life-threatening condition and an intervertebral disk defect, heal a torn
would warrant a nonemergent referral. meniscus, or repair a sprained ankle. Rather,
Establishment of a tissue-specific diagnosis the role of the physical therapist is to provide
is important when the physical therapy inter- an optimal healing environment for the tissue
vention itself can directly influence the course that is injured, reduce abnormal mechanical
of the pathology. An example of this is the stresses placed on the injured tissue, and re-
presence of aseptic inflammation. Physical store optimal movement once the tissue is
therapy includes anti-inflammatory interven- healed. Ameliorating the contributing or asso-
tions that can directly treat the inflammation: ciated physical impairments can achieve these
ice, iontophoresis, and phonophoresis. Ion- goals. Therefore, even if the pathology is
tophoresis, in particular, needs precise place- known, the physical therapist must go beyond
ment in order to be effective, so knowledge of the diagnostic label and identify how it relates
the precise location of pathology is required. to the patient’s impairments, activity levels,
Shoulder impingement, for example, is a com- participation, and health. In addition, the
mon diagnosis in patients referred for physical physical therapist must determine which im-
therapy, yet the diagnosis is not specific enough pairments are causing the activity and partici-
to direct the application of iontophoresis. Fur- pation restrictions, and how they are doing so.
ther investigation must be done to identify the Accomplishing this provides the best chance at
exact location of the pathology (differentiating intervening with respect to the most relevant
between the supraspinatus or infraspinatus impairments.
tendon, for example). Physical therapists’ professional emphasis
Another specific tissue pathology directly on movement creates a unique and specialized
amenable to physical therapy intervention is goal in the search for underlying etiology, in
tendinopathy. Tendinopathy may present that physical therapists typically infer cause-
either as tendinitis or tendinosis, but the and-effect relationships between tissue pathol-
underlying pathologies are different and the ogy and a client’s movement patterns based on
interventions differ accordingly. Tendinitis im- skilled assessment. One of the major factors a
plies an inflammatory process, and appropri- physical therapist must determine is whether
ate anti-inflammatory measures should im- the abnormal movement resulted from the tis-
prove the condition. Tendinosis, however, is an sue pathology, or whether abnormal move-
alteration in the structure of the tendon and re- ment caused the tissue pathology. For example,
quires rest followed by progressive reloading of a patient with a chief concern of knee pain
the tendon through exercise.10 Providing an without a history of traumatic onset may re-
inappropriate treatment due to misdiagnosis will ceive a tissue-specific diagnosis from the
at best fail to improve the patient’s condition (eg, physician that is related to the root cause, such
using ice on a tendinopathy) and at worst exac- as a sprained medial collateral ligament of the
erbate it (eg, exercising an inflamed tendon). knee. One could argue that, upon having iden-
tified the pathology and arrived at the diagno-
Physical Therapists Elaborate sis of the sprained ligament, the differential
on the Diagnostic Label diagnostic process is completed. In this case,
however, the sprained ligament is not the re-
Physical therapists, like all other members sult of a single traumatic event, but due to
of the health care team, interpret the diagnostic overuse. Confronted with this situation, the
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Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology 11

physical therapist must now identify whether determine the stage of healing, so that appro-
any abnormal movement patterns led to the priate treatment is directed to the appropriate
overuse and overstress of the sprained liga- tissue at the appropriate time. In medicine,
ment, and then implement an intervention when a specific cause or source of the problem
strategy that will alleviate stress on the liga- is known and amenable to treatment, treat-
ment. Suppose movement analysis of the pa- ment of the cause or source is usually consid-
tient revealed a knee valgus thrust during each ered more effective than treating individual
weight acceptance phase of running, which signs and symptoms. Likewise, in physical
would repeatedly stress the medial collateral therapy, identifying specific patterns of move-
ligament. Knowing that the hip abductor mus- ment dysfunction (for example, aberrant
cles should control this aberrant movement, movement patterns14) may be a more effective
their strength is tested and they are found to be basis for determining interventions than man-
weak. The result of this search for the underly- aging individual impairments as isolated phe-
ing etiology of medial knee pain is a hip nomena. This concept has become known as
strengthening program. This case illustrates essentialism.15
the direction of investigation specific to physi- The essentialist approach to diagnostic
cal therapy that extends beyond the pathology classification states that an etiologic mecha-
identified by the medical practitioner. nism exists for every disease, whether or not
Physical therapists often attempt to isolate that mechanism has been discovered. This ap-
the patient’s or client’s movement dysfunction proach arose from the discovery of microor-
to the narrowest practical level in order to pro- ganisms as a causative agent for disease. The
vide treatment. Often, this involves a hierar- fact that a specific microorganism could be the
chical organization of the movement-related cause of a specific disease suggested that each
disablement using enablement/disablement and every disease must also have a singular
models. Physical therapists commonly con- cause. Furthermore, regardless of our current
sider the interrelationships among physical limitations in knowledge, it appears to be only
impairments, activity limitations, and partici- a matter of time before science identifies and
pation limitations.1,3,11–13 Physical therapists explains the diagnosis for every malady. Essen-
identify and manage movement dysfunctions tialism is the driving force behind science’s re-
as their unique elaboration on the diagnostic lentless pursuit to identify the origins for all
label, and this allows for the enhancement manner of disease causes. After the narrowest
of physical and functional abilities through possible level of cause has been identified, an
focused intervention. They restore, maintain, essentialist approach to treatment is to elimi-
and promote not only optimal physical func- nate or modify the cause.15
tion, but also optimal wellness and fitness Important limitations exist in the essen-
and optimal quality of life as it relates to tialist approach to diagnosis in consistently
movement and health. Finally, they prevent guiding physical therapists’ interventions and
the onset and progression of symptoms due prognoses. As mentioned earlier, the outcome
to impairments, functional limitations, and of the differential diagnostic process, the diag-
disabilities that may result from diseases, dis- nostic label, should provide clues as to the
orders, conditions, or injuries. Thus, the proper intervention and prognosis. In other
physical therapist must identify abnormal words, the diagnosis is just one driver of the
movement when it exists in a patient and choice of intervention. Unfortunately, the re-
restore optimal movement.1 ferral diagnosis provided to physical therapists
by a physician is tissue specific less than one-
The Diagnostic Label Alone is third of the time.16 Over two-thirds of referrals
Insufficient to Guide Physical to outpatient physical therapy clinics have as a
Therapists “diagnosis” terms like Shoulder Pain, Low Back
Pain, Knee Pain, and so on. Even a casual
A basic precept of patient management is look reveals that these are not diagnoses at
that it is important to determine the anatomi- all, but are simply restatements of a patient’s
cal or pathological source of symptoms and to or client’s symptoms. They are not specific
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12 Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology

regarding the pathology or tissue involved diagnostic system, some authors have gone so
and provide no indication of the appropriate far as to label them forms of physical therapy
intervention. Therefore, the ability for physi- diagnosis.23–25 Although relatively recently
cal therapists to establish a diagnosis and introduced into physical therapy practice,
search for underlying etiology even in the there is some support for the value of a specific
case of vague referral diagnoses becomes movement classification to help guide physical
critical to making treatment and prognosis therapy intervention.2,15,26–30 Impairment-
decisions. based classification systems are problematic,
however, when they fail to account for
A Process Guides Physical pathoanatomical and psychosocial factors and
Therapists’ Search for an the stage of the disorder.31 In addition,
Underlying Etiology although movement and motor control im-
pairments certainly manifest themselves in
So how do we proceed after a diagnosis is common ways for particular diagnoses, there
established? The foregoing situation reveals a is no certainty of cause and effect without the
significant shortcoming of the essentialist ap- relevance provided by the context of move-
proach. With the patient sitting in our clinic ment. For example, directing intervention at
we do not have time for science to “catch up” the movement impairments resulting from
and deliver us a clear treatment plan for each neuropathic or inflammatory pain would be
individual. We must act as best we can with the ineffective compared to treating the cause of
information currently available to develop a the pain directly. Similarly, psychological
plan of care. In contrast to essentialism, the processes such as depression, stress, and anxi-
nominalist approach to diagnostic classifica- ety can produce impairments that affect move-
tion uses groupings of signs and symptoms as ment. Identifying and treating impairments
the basis for establishing and labeling a dis- alone irrespective of other contributing factors
ease.15 A classic example of nominalism is the will result in physicians and physical therapists
management of a patient with the chief con- alike missing a significant aspect of the pa-
cern of low back pain. The literature has estab- tient’s disorder and thereby rendering care that
lished that lumbar pathoanatomy correlates results in poor outcomes.31
poorly with symptoms,17–21 leading to findings The health care community accepts nomi-
that 85% of the time the exact cause of low nalist diagnoses on the basis of consensus.
back pain remains undiagnosed.22 Of course, There is much research, debate, and revision
this does not mean low back pain cannot be that occurs prior to reaching a strong enough
treated effectively. The impairments relative to consensus that nominalist diagnoses should
the chief concern such as pain, or activity lim- become a part of the general standard of care.
itations such as the inability to stand, sit, or Along these lines, more research is necessary to
walk, or participation limitations such as the determine the validity and reliability of and
inability to work can still be identified and the clinical outcomes related to the proposed
guide formation of a plan of care. If specific nominalist approaches that involve classifica-
impairments can be identified that are directly tion according to impairment clusters before
related to the chief concern, and these impair- they can be universally accepted. Indeed, the
ments are addressed through an appropriate search appears to continue for a nominalist
intervention plan, then the patient stands the classification system for movement in health
best chance of realizing a positive change in and disease. In the meantime, considering the
their chief concern. patient as a whole—including hypothetical
Impairment-based classification systems cause-and-effect relationships among pathol-
have developed because of the physical thera- ogy, psychology, sociology, symptoms, and
pist’s emphasis on intervening at the level movement dysfunction—will remain the physi-
of physical impairments. Because the classifi- cal therapist’s challenge.
cation of disablement according to enablement/ Physical therapists’ search for underlying
disablement models and physical impairments causes requires impairments to be identified
takes on the characteristic of a nominalist and linked to function. Since the goal of physical
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Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology 13

therapy care is restoring function and healthy individual would accomplish this task.
reducing disability, and interventions may be The healthy individual possesses the resources
directed toward ameliorating impairments, it necessary for successful task completion: a
is imperative for the impairments being prerequisite amount of joint motion, strength,
treated to be linked to the patient’s functional motor control, proprioception, and so on.
losses and disabilities using a systematic ap- These resources are readily available to the
proach. This approach involves three steps: person who is able to rise from the chair with-
1. Careful identification of the functional chief
out difficulty. We can observe the patient’s
concern, performance of the task, compare it to the
2. Completion of a task and movement analy-
optimal performance, and hypothesize what
sis, and resources are lacking to interfere with task
3. Testing of hypothetical relationships between
accomplishment. This lack of resources is
physical impairments and functional limita- synonymous with impairments.
tions during physical examination. Physical Therapists Create and Test
This specialized approach is dynamic and Hypothetical Relationships
continues throughout an episode of care. The third step to linking impairment and func-
tion is testing the hypotheses via the physical
Physical Therapists Identify examination. To thoroughly test the hypotheses,
the Functional Chief Concern appropriate tests and measures must be chosen.
The first step to linking function and impair- Thus, for a patient having difficulty rising from
ments is to ask what impairments could be the a chair, we would ensure there is enough hip
cause of a given set of activity limitations or, flexion mobility,33 ensure there is sufficient hip
conversely, to ask “What activity limitations extensor strength,34 and so on. Any impairments
could ensue for a given set of impairments?” In found would very likely underlie the functional
most orthopedic settings, the patient’s chief limitation; amelioration of these specific im-
concern corresponds to an impairment. For pairments would stand an excellent chance of
example, a patient with a torn meniscus may decreasing the functional limitation.
report having pain. Given this impairment, the A very important by-product of this ap-
physical therapist must ask not only what proach is that the choice of tests and measures
pathology could be the source of the pain, but used in the physical examination is dictated by
also what activity and participation limitations the predictions made during the task analysis.
arise as a result of that pain. The key question For example, if strength is required to perform
for the patient is “What does this pain prevent the task optimally, then the clinician has no
you from doing?” Possible answers may be an choice but to measure strength to determine if
inability to squat, rise from a chair, or negoti- weakness prevents optimal performance. The
ate stairs. In the neurological setting, the clinician can choose how to determine if the
patient’s presenting concern commonly and impairment is present, for example, testing
conveniently identifies the functional limita- strength using manual muscle testing, hand-
tion, such as “I can’t walk” or “I can’t get up held dynamometry, or isokinetic dynamome-
from a chair.” try, but not whether it will be tested.
Several advantages emerge from this
process. First, the resulting physical examination
Physical Therapists Perform Task
is focused and efficient, because unnecessary
and Movement Analyses
tests are avoided. Second, the findings have
Once the activity limitation has been identi- meaning, because they relate to the patient’s
fied, the second step in linking impairment to concerns. Third, since the impairments stand a
function is to perform a task analysis, that is, to reasonable chance of being linked to activity and
compare how the patient does the activity and participation limitations, intervening in those
how someone in good health would optimally impairments will have the highest likelihood of
do it.32 For example, when a patient states she producing positive results in the activity and
cannot rise from a chair, one can ask how a participation limitations.
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14 Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology

The Search for an Underlying If the expected goals have been met, the out-
Etiology is Dynamic and Ongoing come is clear and the patient is discharged.
When the goals have not been met, however,
As noted previously, there are times when a the therapist must assess each step taken dur-
hypothesis is made about the pathology that ing the episode of care to determine the cause
causes a patient’s or client’s disablement. Based for lack of progress. The first step to review is
on this hypothesis, an intervention plan is de- the implementation of the intervention. If not
signed, complete with objectively measurable done well, treatment is repeated with im-
goals that have a time frame for success. At the proved implementation and the problem is re-
conclusion of this time frame, remeasurement assessed. If the treatment was implemented
is completed to determine whether the patient properly but the goals were still not met, the
or client has met the treatment goals. If the choice of treatment is reassessed. For example,
goals have been met, then not only was the when mobilizing a joint to gain range of mo-
intervention a success, but it is also possible tion fails to yield positive results, perhaps
that the initial diagnostic hypothesis was cor- stretching the muscles will. If the choice of in-
rect. For example, in the case of a patient with terventions was appropriate but the goals were
unilateral low back pain of nontraumatic still not met, then perhaps the overall strategy
onset, aggravated by extension and ipsilateral was incorrect and needs to be rethought. In
lateral flexion, one might surmise based on the this case, perhaps the problem was not lack of
history and physical examination that the range of motion, but lack of strength.
symptoms are due to a facet joint impinge-
ment. Following this biomechanically based Conclusion
hypothesis, a decision might be made to initi-
ate interventions that decompress the offend- Determining a tissue-specific diagnosis ini-
ing facet joint. If the patient improves, this tially drives decisions regarding intervention
might provide indirect evidence supporting and prognosis. Arriving at a diagnosis, how-
the initial treatment hypothesis. This type of ever, is not always the end of the process for
thinking is often used as a form of content va- determining the best possible intervention and
lidity for clinical reasoning; however, it neg- prognosis in physical therapy. In some cases, it
lects the possibility that many other structures is impossible to arrive at a diagnosis for lack of
could have been positively affected as the facet adequate differentiating tests or current med-
joint was decompressed (eg, the surrounding ical understanding. In some cases, although
musculature could have been stretched). It the offending tissue is identified, the interven-
would be better to know the exact underlying tions available to the physical therapist do not
pathology, of course, using tests and meas- allow direct treatment of the tissue itself.
ures that have criterion-related validity (in In all cases, the physical therapist must
this example, a diagnostic facet joint block), proceed beyond the diagnosis and determine
but this is not always feasible, practical, or the presence of movement abnormalities. Fur-
possible. As noted previously, a gold standard thermore, the therapist must determine
diagnostic criterion may not exist. Alterna- whether identified movement abnormalities are
tively, the patient may fail to improve within the cause or result of the pathology. The physi-
the specified time frame or even worsen, cal therapist must create hypothetical links
which may be interpreted as evidence against between function and individual physical im-
the initial hypothetical underlying etiology. pairments, and then test the hypotheses. This
Thus, the search for underlying etiology con- requires a clear understanding of the patient’s
siders not only information gathered from the or client’s functional chief concern, a thorough
history and examination, but also the response task and movement analysis, and testing hy-
to treatment. pothesized movement deficits during the phys-
Determining whether the response to ical examination. Finally, the patient’s or client’s
treatment was appropriate implies that response to treatment—or lack thereof—serves
progress is compared to an expected outcome. to verify or disconfirm the diagnosis.
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Chapter 2 Beyond the Diagnosis: The Search for Underlying Etiology 15

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a seven-year follow-up study. J Bone Joint Surg Am. chanical analyses of rising from a chair. J Biomech.
2001;83–A(9):1306–1311. 1992;25(12):1383–1391.
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CHAPTER3
Diagnostic Reasoning
■ Todd E. Davenport, PT, DPT, OCS

IN THIS CHAPTER: has dual meanings. It refers to the clinical rea-


■ The difference between diagnosis and med-
soning process of determining the sources of a
ical screening patient’s symptoms. This term is also used to
■ Cognitive models of diagnostic reasoning
describe the outcome of the clinical reasoning
process, which is a label that serves as short-
OUTLINE hand communication among different health
care providers. When the diagnostic label is
Introduction 16 coded, it can then be used to inform the
Both Diagnosis and Medical Screening processes of disease tracking and insurance re-
Have Roles in Physical Therapy 16 imbursement. The purpose of this chapter is to
Diagnosis is a Unique Combination of discuss a clinical reasoning process aimed at
Reasoning Patterns 17 determining the pathology responsible for a
Backward Reasoning Involves Creation and patient’s symptom(s), using the presenting
Testing of Clinical Hypotheses 17 symptom as the starting point.
Forward Reasoning Involves the Attempt to
Recognize Patterns in Clinical Presentation 18 Both Diagnosis and Medical
Experts Create “Small Worlds” of Information to Screening Have Roles in Physical
Use in Diagnostic Reasoning 19 Therapy
Preferred Diagnostic Reasoning Processes Depend
on the Clinician and the Case 19 Systems-based processes for medical screening
Conclusion 20 have been proposed for use by physical
therapists in order to move from diagnostic
uncertainty to a sufficient level of certainty to
determine if the patient/client requires referral
Introduction to another health care provider. However,
medical screening is different than diagnosis. As
In the previous chapter, the case was made for part of any effective assessment by a physical
why physical therapists need to know about di- therapist, the patient is asked a series of ques-
agnosis. The idea emphasized in that chapter tions, commonly called the review of systems.
was that physical therapists have the ethical The goal of the medical screening portion
obligation to engage in a systematic process of of the subjective examination is not to determine
diagnosing a disorder by differentiating be- the pathology or pathophysiology underlying
tween the many diseases that may cause the a patient/client’s clinical presentation, but to
symptom. Diagnostic reasoning is one compo- ensure that nothing that may impact the
nent of clinical reasoning by expert physical patient’s well-being has been overlooked. The
therapists. According to Edwards and col- organization of the review of systems is based
leagues, diagnostic reasoning includes the on anatomical structures or physiological sys-
“. . . formation of a diagnosis related to physical tems.2 Typically, these questions are integrated
disability and impairment with consideration into a thorough questionnaire format that is
of associated pain mechanisms, tissue pathol- designed to cover all areas and systems.
ogy. . . .”1 (p322) Indeed, determining the cause The clinical data acquired from the review
of a patient’s symptoms is the initial stage of of systems or medical screening is then typi-
deciding how to treat a patient. As previous cally examined by the physical therapist for the
chapters have pointed out, the term diagnosis presence of Red Flags. In 1994, the U.S. Agency

16
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Chapter 3 Diagnostic Reasoning 17

for Health Care Policy and Research3 released enough to allow physical therapists to consider
a brochure describing symptoms and signs how pathology may cause an individual pa-
that may indicate the presence of serious tient’s symptoms and signs given the patient’s
pathology in patients with low back pain, unique situation.
which have become known as Red Flags. Red
Flags are commonly interpreted to signal an Diagnosis is a Unique Combination
automatic need for patient/client referral to of Reasoning Patterns
another health care provider.4,5 However,
emerging literature indicates a lack of diagnos- The goal of the diagnostic process is to move
tic accuracy for individual Red Flags.6 Indeed, from uncertainty to a sufficient level of cer-
the diagnostic relevance of individual or clus- tainty regarding the cause of a patient’s pre-
ters of Red Flags must be interpreted for a senting symptoms or signs for referral and
given patient situation; a symptom or sign treatment decisions to be made. It is this cer-
considered a Red Flag for one patient/client tainty that allows for labeling of the pathology
may not necessarily be a Red Flag for another. hypothesized to be responsible for the patient’s
For example, a patient who received gastric by- clinical presentation. To achieve this goal,
pass and a patient with malignant cancer may physical therapists must answer a fundamental
both demonstrate significant weight change; question: “What disease is causing the patient’s
however, the etiology of weight change is far symptoms?” A formal process for finding the
less insidious in the patient who received a gas- answer to this question is necessary, because
tric bypass than in the one with metastatic the rate of errors in medical decision making
cancer. Also, a history of smoking may not be has been shown to decrease with implementa-
considered a Red Flag by itself, but it takes on tion of formal processes.
significant diagnostic relevance in a patient Several characteristics are important in a
with shoulder pain and upper extremity pares- well-designed diagnostic process. The process
thesias due to the elevated risk for a Pancoast should allow consideration of all the impor-
tumor. tant clinical data without missing any data that
Some authors advocate screening for mul- might be salient to the patient’s case. At the
tiple Red Flags in order to screen for certain same time, the process must be efficient and
forms of pathology.7 Scientific determination flexible to allow consistent application
of symptoms and signs that have the highest throughout the day and for different clinical
likelihood of indicating a disease is important. presentations and clinicians at various levels of
However, organizing this information around knowledge and skill. Efficiency and flexibility
the details of a specific patient’s case may be in a diagnostic process relates to the ability
more clinically meaningful than synthesizing a of clinicians to integrate several patterns of
collection of unrelated facts, as we discuss in a diagnostic thinking within the process. Each of
later section of this chapter. Organizing the these patterns is discussed in the following
search for underlying pathology around a sections.
process rather than fragments of information
Backward Reasoning Involves
enhances the search’s consistency and speed,
Creation and Testing of Clinical
because the clinician can recognize features of
Hypotheses
the case that point to a diagnosis rather than
needing to remember all of the Red Flags in a Backward reasoning involves the creation
cluster. In addition, the patient/client does not of hypotheses to be tested. The process of
always present with signs and symptoms that testing hypotheses has been referred to as the
fit dysfunction in a well-defined physiological hypothetico-deductive model. Pure use of this
system. Memorization of Red Flag clusters form of diagnostic reasoning would suggest
would impair perception of the necessary cues that a clinician should collect all the possible
to integrate diagnostic information from data by taking an absolutely complete history
across body systems. from the patient. This would be followed by an
These issues reinforce the importance of exhaustive physical examination and collation
establishing a diagnostic process that is flexible of an exhaustive set of tests. At that point, all
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18 Chapter 3 Diagnostic Reasoning

data would be sorted by the probabilities of a perceived frequency, variability, causation, and
specific disease being found in a patient or prototype of a disease.12
client based on relevant demographic charac- Clinicians may make rapid judgments
teristics and clinical test results until the most about the perceived frequency of a disease
likely diagnosis is reached. Although backward based on specific variables that are identified.
reasoning is a pattern that aims to carefully For example, you might consider a genetic
consider much information, this pattern is also basis to be more likely in children with motor
characterized by a slower processing time and delay and a family history of a heritable ge-
a higher potential for distractions that affect netic disease than in children with motor delay
decision accuracy.8 and no family history.
The exhaustive nature of pure backward Judgments regarding disease variability oc-
reasoning models makes them too time cur when a clinician makes inferences about
consuming for consistent use in the clinic, al- the entire population with a disease based on a
though their use has merit in computer mod- small subset. In this manner, you might infer
els. One example of backward reasoning com- that the entire population of patients with dif-
puter modeling is the use of expert systems or fuse leg pain has peripheral vascular disease
decision support systems. A decision support based on your experience with a few individu-
system is a computer system that is intended als with peripheral vascular disease.
to simulate the knowledge and expertise of a Clinicians may rapidly consider causation
human expert. The system is composed of a in determining whether a patient has a disease.
large computer database containing informa- In this scenario, you might consider the prob-
tion related to the diagnosis and treatment of ability of brain trauma to be most prominent
disease. The database can be searched using an in a patient with loss of consciousness follow-
inference engine, and the computer is pro- ing a motor vehicle accident.
grammed with software that provides a set of In making judgments about a prototype to
rules for basic deductions. After the patient’s determine if Mrs. Juarez has a certain disease,
presenting symptoms and signs have been en- you would compare Mrs. Juarez’s presentation
tered into the search engine, the computer to what you saw last week in Mrs. Smith. If
yields a likely diagnosis and treatment options. Mrs. Juarez and Mrs. Smith have the same
Of course, there are many advantages to this symptoms and signs, they must have the same
type of system, which are beyond the scope of pathology.
this chapter to discuss in detail.9,10 However, The pure use of forward reasoning has
computerized decision support systems have both advantages and disadvantages. One ad-
yet to be widely implemented into health care, vantage of forward reasoning is that decisions
emphasizing the need for clinicians to con- can be made faster than with backward rea-
tinue to develop hypothetico-deductive rea- soning.8 Problems with the use of forward
soning skills. reasoning alone relate to the fact that forward
reasoning is subject to errors,13,14 especially by
Forward Reasoning Involves
novice clinicians.12 These errors would be ad-
the Attempt to Recognize Patterns
dressed if a clinician has seen at least a repre-
in Clinical Presentation
sentative sample of all pathologies, which is
Forward reasoning (or forward-inductive rea- impractical and impossible. The need for ex-
soning) is the attempt to recognize patterns in perience with different patient presentations
clinical presentation that may indicate pathol- to engage in a forward reasoning process is
ogy. This approach also is called pattern recog- supported by studies that suggest expert clini-
nition. In this manner, hypothesis generation cians demonstrate behaviors consistent with
is triggered by the patient’s presentation.11 forward reasoning processes more frequently
Certain features of the patient’s clinical pres- than beginners,15–17 and also demonstrate
entation commonly facilitate hypothesis for- better recognition memory for salient and
mation. These features include clinicians’ nonsalient clinical information than begin-
judgments about how representative the pa- ners.18 However, our experience indicates that
tient’s clinical picture is with respect to the variations among patients can make it more
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Chapter 3 Diagnostic Reasoning 19

difficult to recognize underlying similarities. expertise and diagnostic reasoning processes.


This trend is particularly true for novice clini- The interaction between knowledge and
cians, although expert clinicians may be sub- process can be summarized in the following
ject to cue interference, in which conflicting example from Norman and Eva,21 involving
pieces of information are present in a patient’s three individuals who encounter a white dog
case. Perhaps for this reason, interfering pieces on the street:
of information may cause even clinical experts
The first ... says, “It looks like Lassie.
to participate in a more backward reasoning
It’s a dog.” The second ... says, “Well,
pattern.19
it has 4 legs, floppy ears and it
weighs about 20 kg. It barked a
Experts Create “Small Worlds”
minute ago. It’s furry. It must be a
of Information to Use in Diagnostic
dog.” His brother ... who recently
Reasoning
arrived from the Aleutians and has
Most recently, the small worlds hypothesis was only encountered husky dogs, says,
created to model diagnostic reasoning by ex- “It might be a dog because it has
perts.20 This hypothesis suggests that clinicians 4 legs, a tail and floppy ears. On
use both forward and backward reasoning the other hand, it could be a baby
through “chunking” clinical information into polar bear, because it’s white. And
manageable pieces that are used to establish I thought I heard it growl a minute
diagnostic certainty. Our experience indicates ago, so maybe it’s a wolf.”
the experienced clinician sorts through a series
The first individual used a pattern recogni-
of diagnostic possibilities by choosing those
tion approach to quickly arrive at the answer,
with the highest probabilities given the pa-
while the other two individuals appeared to
tient’s gender, age, locale of habitation, and
actively consider competing hypotheses in a
occupation. The questions then asked during
more hypothetico-deductive manner. The lat-
the subjective examination are specifically di-
ter two individuals differed in their experience
rected to eliminate as many causes as possible.
with similar animals, and the number of
Questions are chosen to be most differentiat-
competing hypotheses increases in parallel
ing; that is, the answer to the question should
with inexperience.22 Therefore, these less expe-
eliminate as many inappropriate diseases as
rienced individuals would need to adopt a
possible. At that point, a quick mental sorting
more hypothetico-deductive approach to de-
provides a more limited array of possibilities
termining the appropriate label for the animal,
that must be differentiated by selecting a nar-
by collecting additional data to assist in distin-
row, yet specific set of physical examination
guishing among the different possibilities.
tests.
This trend toward more forward reasoning
If final selection of a single diagnosis cannot
patterns by clinical experts is supported by
be made, referral for appropriate laboratory or
various scientific studies. For example, in one
imaging tests may be required. In the case of
study expert dentists ordered fewer diagnostic
most physical therapists, this latter step will re-
examinations than beginners, and tended to
quire referral to a physician. However, narrowing
make decisions based on prior patient experi-
down the group of likely causes and pathologies
ences rather than didactic coursework.16 In
will help the physical therapist to decide the
addition, analyses of self-reported thought
urgency and direction of the referral.
processes indicate that forward reasoning is
used more frequently by more experienced
Preferred Diagnostic Reasoning
medical residents in the critical care setting
Processes Depend on the Clinician
than novices.17 To say that the use of forward
and the Case
and backward reasoning is mutually exclusive,
Experienced clinicians tend to employ more however, may be an oversimplification; experts
inductive reasoning than less experienced and novices alike may use both patterns of rea-
clinicians to achieve diagnostic certainty, soning, but simply may display a predilection
bringing up an important relationship between toward one over the other.23,24 It seems necessary
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20 Chapter 3 Diagnostic Reasoning

then, for a symptom-based diagnostic process efficiency, as well as the flexibility needed for
to be flexible enough for clinicians to preferen- the various patient/client presentations and
tially use the pattern of reasoning that best types of clinical thinking displayed by clini-
fits the situation and the clinician’s level of cians at all levels of experience.
experience.
Characteristics of the case also influence References
the type of reasoning processes selected. Find- 1. Edwards I, Jones M, Carr J, Braunack-Mayer A, Jensen
ings from one study established that straight- GM. Clinical reasoning strategies in physical therapy.
Phys Ther. 2004;84(4):312–330; discussion 331–335.
forward cases were better processed by medical 2. Guide to Physical Therapist Practice. Second Edition.
residents than ambiguous ones, although the American Physical Therapy Association. Phys Ther.
memory for facts that characterized the cases 2001;81(1):9–746.
was higher in the ambiguous case than the 3. Clinical Practice Guideline, Acute Low Back Problems in
Adults. Silver Spring, MD: Agency for Health Care
simple case.25 In another study, the time spent Policy and Research Publications Clearinghouse;
on the diagnosis, the memory for case find- 1994.
ings, and the number of inferences from case 4. Boissonnault WG. Examination in Physical Therapy
findings were found to be significantly ele- Practice: Screening for Medical Disease. New York, NY:
Churchill-Livingstone; 1995.
vated in a problematic case compared to a case 5. Sizer PS, Jr., Brismee JM, Cook C. Medical screening for
that was seen as simple.26 Medical specialists red flags in the diagnosis and management of muscu-
also processed cases in their specialties faster loskeletal spine pain. Pain Pract. 2007;7(1):53–71.
and more accurately than cases outside their 6. Leerar PJ, Boissonnault W, Domholdt E, Roddey T.
Documentation of red flags by physical therapists for
expertise, although recall for findings within patients with low back pain. J Man Manip Ther.
the cases and pathophysiological explanations 2007;15(1):42–49.
were not significantly different.27 These find- 7. Henschke N, Maher CG, Refshauge KM. A systematic
ings indicate the importance of case complexity review identifies five “red flags” to screen for vertebral
fracture in patients with low back pain. J Clin Epidemiol.
or ambiguity to the pattern of clinical reason- 2008;61(2):110–118.
ing employed, in which cases that are per- 8. De Neys W. Automatic-heuristic and executive-analytic
ceived as complex are characterized by a more processing during reasoning: chronometric and
careful backward approach, and cases that are dual-task considerations. Q J Exp Psychol (Colchester).
2006;59(6):1070–1100.
perceived as simpler are characterized by a 9. Lobach DF, Hammond WE. Computerized decision
faster forward approach. Although the rate of support based on a clinical practice guideline im-
recall for findings between ambiguous and proves compliance with care standards. Am J Med.
simple cases may differ with clinical expertise, 1997;102(1):89–98.
10. Sucher JF, Moore FA, Todd SR, Sailors RM, McKinley
the goal of the diagnostic process remains BA. Computerized clinical decision support: a technol-
to identify underlying pathophysiological ogy to implement and validate evidence based guide-
explanations for patients’ symptoms and signs lines. J Trauma. 2008;64(2):520–537.
regardless of case complexity. 11. Crowley RS, Naus GJ, Friedman CP. Development of
visual diagnostic expertise in pathology. Proc AMIA
Symp. 2001:125–129.
Conclusion 12. Payne VL, Crowley R. Assessing the use of cognitive
heuristic representativeness in clinical reasoning. AMIA
Diagnostic reasoning involves a combination Annu Symp Proc. 2008:571–575.
13. Dawson NV. Physician judgment in clinical settings:
of pattern recognition and hypothetico- methodological influences and cognitive perform-
deductive reasoning. The type of reasoning ance. Clin Chem. 1993;39(7):1468–1478; discussion
that is involved with diagnosis depends on the 1478–1480.
expertise of the clinician. This highlights the 14. Elstein AS. Heuristics and biases: selected errors in clin-
ical reasoning. Acad Med. 1999;74(7):791–794.
need for a diagnostic process for physical ther- 15. Noll E, Key A, Jensen G. Clinical reasoning of an experi-
apists that is flexible yet thorough to accom- enced physiotherapist: insight into clinician decision-
modate for all levels of experience. Subsequent making regarding low back pain. Physiother Res Int.
chapters in this book are organized around a 2001;6(1):40–51.
16. Crespo KE, Torres JE, Recio ME. Reasoning process
process for symptom-based diagnosis, which is characteristics in the diagnostic skills of beginner,
described in the next chapter. This approach competent, and expert dentists. J Dent Educ.
supports the need for thoroughness and 2004;68(12):1235–1244.
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Chapter 3 Diagnostic Reasoning 21

17. Young JS, Smith RL, Guerlain S, Nolley B. How residents 23. Ferreira MB, Garcia-Marques L, Sherman SJ, Sherman
think and make medical decisions: implications JW. Automatic and controlled components of judgment
for education and patient safety. Am Surg. 2007; and decision making. J Pers Soc Psychol. 2006;
73(6):548–553; discussion 553–554. 91(5):797–813.
18. Norman GR, Brooks LR, Allen SW. Recall by expert 24. May S, Greasley A, Reeve S, Withers S. Expert therapists
medical practitioners and novices as a record of use specific clinical reasoning processes in the assess-
processing attention. J Exp Psychol Learn Mem Cogn. ment and management of patients with shoulder pain:
1989;15(6):1166–1174. a qualitative study. Aust J Physiother. 2008;54(4):
19. Patel VL, Groen GJ, Arocha JF. Medical expertise as 261–266.
a function of task difficulty. Mem Cognit. 1990; 25. Mamede S, Schmidt HG, Rikers R. Diagnostic errors
18(4):394–406. and reflective practice in medicine. J Eval Clin Pract.
20. Kushniruk AW, Patel VL, Marley AA. Small worlds and 2007;13(1):138–145.
medical expertise: implications for medical cognition 26. Mamede S, Schmidt HG, Rikers RM, Penaforte JC,
and knowledge engineering. Int J Med Inform. Coelho-Filho JM. Breaking down automaticity: case
1998;49(3):255–271. ambiguity and the shift to reflective approaches in clin-
21. Norman GR, Eva KW. Doggie diagnosis, diagnostic suc- ical reasoning. Med Educ. 2007;41(12):1185–1192.
cess and diagnostic reasoning strategies: an alternative 27. Rikers RM, Schmidt HG, Boshuizen HP, Linssen GC,
view. Med Educ. 2003;37(8):676–677. Wesseling G, Paas FG. The robustness of medical expert-
22. Joseph GM, Patel VL. Domain knowledge and hypothe- ise: clinical case processing by medical experts and subex-
sis generation in diagnostic reasoning. Med Decis perts. Am J Psychol. 2002;115(4):609–629.
Making. 1990;10(1):31–46.
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CHAPTER4
How to Use This Book
■ Hugh G. Watts, MD

IN THIS CHAPTER: One could explain the shoulder pain as due to


■ A detailed description of the process for
a rotator cuff tear, the cough due to a common
symptom-based diagnosis that is used cold, the hand paresthesias due to carpal tun-
throughout this book nel syndrome, and the pupillary constriction
to a congenital abnormality. Although it is
OUTLINE possible that the problems could have been
caused by four different diagnoses, health care
Introduction 22 thinking, and science in general, prefers the
Principle of “Economy of Diagnoses” 22 simplest hypothesis that can reasonably ex-
The Diagnostic Process 23 plain the multiple phenomena—in this case a
Barriers to Communication 23 Pancoast tumor of the upper lung invading
The Starting Point: Identify the Patient’s Chief into the region of the brachial plexus and
Concern 23 the sympathetic ganglia, resulting in Horner’s
Symptoms Versus Signs 24 syndrome.
Special Concerns 24 William of Occam, a 14th-century
TIM VaDeTuCoNe: A Database of Possible philosopher-theologian,1 is credited with
Causes 25 enunciating the principle that when having to
Remote Versus Local Source of Pain 27 choose among competing hypotheses, one
What Follows the Making of a TIM should favor the simplest one. Or stated differ-
VaDeTuCoNe List? 28 ently, when multiple explanations are available
Conclusion 30 for a phenomenon, the simplest version is pre-
ferred. This concept is commonly known as
“Occam’s razor” (where razor refers to a tool in
Introduction a logical argument used to cut absurdities
from philosophical discourse).
In the first chapter, two definitions of the term The concept of parsimony of diagnosis has
diagnosis were given. The term can refer to (1) not gone unchallenged. Saint’s triad, by
the process of deciding the cause of an illness as Hilliard and Weinberger, emphasizes the im-
well as to (2) the result of that process, which portance of considering the possibility of mul-
is a label that indicates the putative cause of the tiple separate diagnoses in a patient whenever
problem. The focus of this book is the first defi- his or her history and the results of the physical
nition, that is, the systematic technique of mak- examination are atypical of any single condi-
ing a diagnosis. This chapter provides a method tion.2 Although in the medical care of children,
by which a diagnosis can be made and is the the probability of several disease processes oc-
method that is used throughout this book. curring simultaneously is less likely, be aware
that, as the patient ages, there is an increasing
Principle of “Economy likelihood of disease developing in multiple
of Diagnoses” organ systems.
Was Occam wrong? No. Occam did not
If a patient has left shoulder pain and a cough, state that the simpler explanation is always
has reported paresthesias in the left hand, and right or that the more complex explanation is
on examination demonstrates a constricted always wrong. He emphasized that one should
pupil in the left eye, what is the likely cause? start from the simplest possible explanation and

22
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Chapter 4 How to Use This Book 23

only make it more complex when absolutely problem concerning them or a friend or rela-
necessary. This principle—the economy of di- tive. A coworker might come to you in a hall-
agnoses—has been a cornerstone of differential way and say, “I fell this weekend and now my
diagnosis and is still the best approach to mak- knee is swollen. Would you take a look and
ing a differential diagnosis. tell me if I need to see a physician?” In this in-
The process of making a diagnosis out- formal situation, the questions you ask of
lined in this chapter is modulated by the need “the patient” and the physical examination
for efficiency by the physical therapist. A phys- might not be as complete as in a formal situ-
ical therapist cannot spend several hours with ation. There will be no record of the visit, nor
each patient. It is important to develop a clin- will a follow-up visit be arranged. This poten-
ically viable process that gets to the diagnosis tially dangerous situation is compounded by
as efficiently as possible. the personal relationship, because we are un-
likely to think of some of the very serious dis-
The Diagnostic Process eases that might be involved, or that this
friend or relative is subject to socially stigma-
Barriers to Communication tized activity that might be the basis of a sex-
ually transmitted disease.
In order for a physical therapist to make a rea-
sonable diagnosis, information must flow
The Starting Point: Identify
freely between the patient and the therapist.
the Patient’s Chief Concern
Barriers to communication are those situa-
tions that may interfere with this proper flow The process of making a diagnosis starts with
of information. the patient’s presenting concern or symptom.
Why then does our discussion of the diag- Classically, the subject of differential diagnosis
nostic process begin by listing barriers to com- has been taught to physical therapy students
munication? Any information that is gathered using a body system approach. Here the term
is always filtered. Hence, the physical therapist system refers to the anatomical or physiologi-
should be aware of the presence of possible bi- cal system of the body involved in the disease
ases so that the information that is collected such as the cardiac or neurological system.
can be as free of distortions as possible. Early on, physical therapists learn about the
In trying to make a diagnosis, obtaining pain that can result from cardiac disease or the
accurate information is paramount. Anything particular limp that might result from a neu-
that interferes with the proper flow of infor- rological disease. Patients, however, do not ar-
mation needs to be identified and dealt with. rive at the clinic or office announcing that they
Barriers to communication warn the therapist have a cardiac or a neurological problem. In-
to recognize possible interactions between the stead, a patient presents, for example, with
therapist and the patient that may lead the shoulder pain. That shoulder pain could be
therapist astray when trying to make a diagno- caused by cardiac disease or gallbladder in-
sis. An obvious example would be a patient flammation irritating the underside of the di-
who does not speak the same language as the aphragm or cervical disk disease or a rotator
therapist. Even among patients who think they cuff tear. Another patient might be concerned
are speaking the same language, slang and about a feeling of generalized weakness that
other alternative word usages can impair com- could be secondary to a metabolic disorder, a
munication. Wide cultural differences between neurological disease, or psychological depres-
the patient and the local community, wide sion. Hence, the method taught here begins
generational gaps, or gender-sensitive issues with a patient who presents with a symptom,
may also impede the flow of information. the cause of which may originate in any of a
Another barrier to communication is the number of different body systems.
informal referral. Physical therapists are fre- Is a system approach the wrong way to ini-
quently subject to direct informal referrals. tially teach students differential diagnosis? We
These are the people, often fellow workers or believe not. The student first needs a ground-
relatives, who casually ask about a medical ing of information to work with. Ultimately,
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24 Chapter 4 How to Use This Book

however, the student needs a method for information that a 45-year-old male smokes
integrating this knowledge learned from the three packs of cigarettes a day would cer-
system texts into a useful clinical tool. tainly represent a cause for caution if he
were presenting with shoulder pain because
Symptoms Versus Signs of the possibility of a malignant lung tumor
as the cause, but not necessarily if he was
We have based this text on the symptoms that
concerned with foot pain that appears to be
a patient brings to our attention. Some of the
secondary to plantar fasciitis.
chapter titles suggest signs rather than symp-
Some situations that require special atten-
toms. Is there a significant difference between
tion are patients who arrive with:
terms symptom and sign? By some definitions2
● A nonspecific diagnosis from a referring
a symptom is “a subjective indication of a dis-
ease or change in condition as perceived by the physician. A patient referred with a diagno-
patient,” whereas a sign is “an objective finding sis of Shoulder Pain, Low Back Pain, or Neck
perceived by the examiner.”2,3 Other defini- Pain should be seen as one who arrived with
tions,4,5 however, do not make as clear a differ- no diagnosis. Pain is a symptom, not a
entiation. Instead, they define a symptom as “a pathology. The fact that a patient’s symp-
characteristic sign or indication of a disorder tom is translated into Latin (eg, cervicalgia
or disease.” In practice also, the distinction is for “neck pain”) does not change the situa-
not always clear. If a patient limps in to your tion that this is a patient who arrived with-
clinic and says, “I limp,” is that a sign or a out a diagnosis.
symptom or both? If the mother of a child ● Changes in symptoms since the last visit

says, “My child limps,” is the limp the mother or since the patient last saw the referring
has observed a sign or a symptom? We feel that physician.
this is not a productive debate and, therefore, ● Unusual pain patterns.

use the term chief concern as including both ● Night pain that is felt as a deep ache and not
signs and symptoms. affected by positional change. However,
note that the isolation and silence of dark-
Special Concerns ness often makes patients feel worse at
In this book, the term special concerns denotes nighttime.
situations in which the therapist needs to be ● Unrelenting pain.
especially wary. It does not mean that the ther- ● Migratory pains.
apist should stop thinking through the diag- ● Nonanatomical distribution of pains.
nostic possibilities and immediately refer the ● Acute changes:
patient elsewhere. ● with increased body temperature
We are all aware that some cardiac pathol- ● sudden weakness
ogy may be felt by the patient as pain in the ● sudden inability to bear weight.
medial aspect of the upper arm on the left.
● Signs of ischemia, for example:
Understandably, most therapists would be
● decreased pulses in the extremities
alarmed if a patient stated that the upper me-
● smooth, shiny skin
dial arm hurt, especially if the pain was wors-
● loss of hair over extremities.
ened by exertion. However, therapists are less
● Sensory changes:
likely to be aware that gallbladder inflamma-
● paresthesias
tion irritating the underside of the diaphragm
● sensory loss
may give right-sided shoulder pain, or that hip
● pain that radiates.
disease can be felt as pain in the anteromedial
aspect of the knee. Although the consequences ● Recent unintended weight loss.

of missing these connections are less grave ● Genitourinary problems, especially urinary

than with the cardiac disease, these cautions retention.


need to be heeded. ● Rapid onset of limited coordination, im-
Some special concerns need to be indi- paired special senses, or other neurological
vidualized to the presenting symptom. The signs.
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Chapter 4 How to Use This Book 25

When such conditions are noted, this does the use of the method. One convenient ap-
not mean that the patient needs immediate re- proach is to use a mnemonic. A mnemonic is
ferral back to the physician, because the condi- an aid to memory (from the Greek mnemos,
tions may well be part of the disease for which meaning “memory”). We have developed a
the patient was referred to the physical thera- mnemonic that we have found helpful in
pist to begin with. For example, a patient teaching the method of differential diagnosis
might be referred for a program to regain to physical therapists: TIM VaDeTuCoNe
range of motion of the hip following a hemor- (Box 4-2).
rhage in the psoas muscle resulting from anti- Who is TIM VaDeTuCoNe? TIM VaDeTu-
coagulant therapy for her atrial fibrillation CoNe is an artificial construct. It was de-
(Box 4-1). signed to allow the physical therapist to list
the potential diseases in an organized fashion.
TIM VaDeTuCoNe: A Database The order chosen for those items was one that
of Possible Causes would be relevant to a physical therapist. Why
are some letters in uppercase and some in
When using our method of making a diagno-
lowercase?—Simply to provide enough vow-
sis, one cannot overemphasize the impor-
els to make the word easier to sound out and
tance of making a list, mentally or written, of
to remember. To someone new to this
all possible causes. This is the database from
mnemonic, it may seem clumsy and no more
which the diagnosis is determined and forms
convenient than other mnemonics or than
the basis for future decisions concerning in-
not using a mnemonic at all. However, we
tervention. This list is a starting point and
have used this very effectively with both
needs to be sorted by the discriminating
entry-level doctoral students and post-
questions asked during the history taking
professional DPT students with many years
followed by the re-sorting done with the help
of clinical expertise. They have found it
of the appropriately discriminating physical
easy to remember and that it guides them
findings.
in making a list of possible diagnoses so
Such a list of possible causes of a symptom
that uncommon problems are not over-
can be very long, making it difficult to remem-
looked. Many students refer to it as the
ber them all. Lists of diagnoses, subdivided by
“TIM list.”
anatomical/physiological system, such as those
In this book, each chapter that describes
given in the American Physical Therapy Asso-
possible causes of a symptom will begin
ciation’s Guide to Physical Therapy Practice,6
with a Chapter Preview (see Chapter 7).
are not as useful to a physical therapist whose
The previews are sorted using TIM VaDeTu-
starting point is the patient’s presenting symp-
CoNe, and also by the estimates of the
tom. Just listing diseases as they come to mind,
condition’s prevalence in society.
however, also is not an efficient approach.
The physical therapist needs to organize
the disease processes in some way to facilitate
BOX 4-2 TIM VaDeTuCoNe—An
Artificial Construct
BOX 4-1 Getting Started with
the Diagnostic Process Trauma
Inflammation
● Barriers to communication are potential prob-
lems with any patient who arrives with any Metabolic
symptom; hence, we have not listed them in Vascular
each of the following chapters. Degenerative
● Special concerns may differ from one present-
Tumor
ing symptom to another. As a consequence,
we have listed them at the top of every diag- Congenital
nostic list in the following chapters. Neurogenic/Psychogenic
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26 Chapter 4 How to Use This Book

Into which category does a particular dis- Trauma (T)


ease fit? That can be a problem. There may be The Trauma category may include:
more than one process going on. Infection in a
bone may cause ischemia of the bone tissue, ● Rupture of a tendon or muscle.
which in turn will cause inflammation in the ● Strain: a tear of muscle tissue.
surrounding viable bone. However, for sim- ● Sprain: a tear of joint ligaments.

plicity, we have arbitrarily chosen the primary ● Fracture:


process as the one for categorizing. ● Cortical or metaphyseal
Sometimes it is difficult to choose between ● Stress
categories. For example should an arteriove- ● Avulsion: piece of bone to which muscle
nous (AV) malformation be considered a is attached is pulled off but the muscle is
“vascular disease” or a “tumor” or perhaps a intact
“congenital malformation”? All could fit. In ● Osteochondral
this situation, we have found that most physi- ● Physeal.
cal therapists think of an AV malformation as ● Dislocation.
a vascular problem, hence we have put it into ● Mechanical includes the obvious causes
that category. By that same thinking we have above but we have also included nerve en-
made several arbitrary decisions for consis- trapment and nerve instability.
tency across the many chapters of the book.
The choices we have made do not reflect Inflammation (I)
our concept of the pathophysiology. For exam- The inflammation category has been divided
ple, consider a prolapsed intervertebral disk. into “Aseptic” and “Septic.” The Aseptic sub-
Should it go under the “Trauma” or “Degener- category has been put first, because, again, this
ation” category? That might depend on is a category of illness that may be more likely
whether the prolapse occurred in a 15-year-old to be overlooked. Aseptic processes are those
as a result of a motor vehicle accident or in a that are not directly involved with microor-
45-year-old after lifting a heavy load. We also ganisms, whereas septic conditions are caused
recognize that ultimately, the prolapsed by microorganisms such as bacteria. Although
disk will irritate the nerve root through some aseptic conditions are the result of the
local inflammation. So should the disease be body responding to the antigens on the mi-
listed under “Inflammation”? We have made croorganism (eg, Lyme disease), the condition
an arbitrary decision to categorize all nerve might not be considered a septic process.
compression under Trauma. However, most physical therapists would put
Do “Neurogenic” and “Psychogenic” really it under Septic, so that is where we have
belong together? We still needed a category to placed it.
place items such as schizophrenia, anxiety, This category may include:
malingering, or, for example, dystonia muscu- ● Aseptic (ie, noninfectious):
lorum as a cause of torticollis. Ultimately, all ● Autoimmune diseases such as rheuma-
disease processes that occur in the brain toid arthritis, and associated rheumatoid
(whether neurogenic or psychogenic) have a diseases such as lupus and scleroderma,
common pathway of electrical impulses traveling arthritis associated with inflammatory
between cells, so the separation into a duality bowel disease, or psoriasis.
is artificial. Hence, we feel that these terms can ● Reactive arthritis (Reiter’s, hepatitis B, al-
be grouped together. lergic). Some would include all of the as-
We have studiously avoided a “Miscella- sociated rheumatoid diseases mentioned
neous” category. If such a category were used, above under reactive arthritis.
it could contain so many diagnoses that were ● Postsurgical capsulitis tendinitis when
difficult to remember that the process would tendon inflammation is truly present.
be counterproductive. Some pathologies are sometimes called
Let’s take a look now at what the individual tendinitis but are not associated with an
categories of TIM VaDeTuCoNe include. inflammatory response. These are now
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Chapter 4 How to Use This Book 27

more commonly termed tendinoses, or an osteosarcoma in the femur or a bron-


tendinopathies, such as rotator cuff, and are chogenic carcinoma in the lung.
listed under the “Degenerative” column. ● Malignant Metastatic: This type of tumor

● Septic (ie, infectious): is the result of spread of the primary tu-


● Acute: bacterial (eg, Staphylococcus), mor away from the organ of origin to a
fungal, or parasitic. new and distant site. In the past, the
● Chronic: bacterial (eg, tuberculosis), viral,
term secondary tumor was used synony-
fungal, or parasitic. mously with metastatic tumor. Now the
term secondary tumor refers to a new tu-
Metabolic (M) mor, of a different tissue origin than the
The Metabolic category may include: first, caused by the effects of the treatment
of the first tumor. An example would
● Diabetes, gout, hyperparathyroidism, en- be an osteosarcoma of the ileum resulting
docrine disorders, and issues related to from radiation treatment to a Ewing’s
pregnancy. sarcoma in the pelvis several decades
● Toxic (eg, lead poisoning).
earlier.
● Envenomation (eg, black widow spider bites).
● Benign: Benign tumors are ones that charac-
● Ethanol-induced polyneuropathy. teristically do not metastasize and usually
● Chronic drug use—either illegal drugs or are not a threat to life but can be locally
overuse of legal drugs—a problem that is aggressive and destructive (ie, pigmented
easily overlooked. villonodular synovitis).
● Pulmonary insufficiency caused, for exam- Congenital (Co)
ple, by asbestos inhalation. The Congenital category may include:
Vascular (Va)
● Congenital anomalies.
The Vascular category may include:
● Developmental conditions, such as develop-
● Cardiac. mental dislocation of the hip.
● Arterial: “too little” (eg, ischemia, acute [in- ● Hereditary diseases.
farction], chronic insufficiency, avascular
necrosis) or “too much” (eg, bleeding or Neurogenic/Psychogenic (Ne)
hematoma). The Neurogenic/Psychogenic category may
● Venous (eg, thrombosis).
include:
● Lymphatic (eg, lymphedema). ● Psychological diseases such as neuroses and
Degenerative (De) psychoses and malingering conditions such
as amyotrophic lateral sclerosis or spinal
The Degenerative category may include:
muscular atrophy.
● Tendinoses: As outlined above, some
pathologies that are sometimes called ten- Note that spinal cord injuries have been
dinitis but are not associated with an inflam- placed under the Trauma category.
matory response are now more commonly Does this system work for nonpain symp-
termed tendinoses, or tendinopathies, such toms such as weakness, tripping, palpitations,
as a rotator cuff tear. and joint contractures? We have found that it
does. However, the differentiation into remote
● Arthroses: Similar to conditions of the tendon,
versus local sources of the problem is not
joint pathologies formerly known as os- relevant to these diagnoses, so it has been
teoarthritis are now more commonly known as omitted.
osteoarthrosis and are considered degenerative.
Tumor (Tu) Remote Versus Local Source of Pain
The Tumor category may include: We all have a tendency to look for causes
● Malignant Primary: A malignant primary of pain that are found locally in the region
tumor is one at its site of origin, for example, where the patient complains of the symptom.
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28 Chapter 4 How to Use This Book

A moment’s reflection shows that such an ap- dealing with body organs that have no surface
proach is inappropriately limited. The source component, the brain has difficulty deciding
of such a pain could be remote instead of local. where the pain originates. It will assign the
By local pain we mean pain caused by a patho- source to the region on the surface that shares
logic process in the immediate region of the the same innervation as the structure deep in-
symptom, eg, muscle tissue rupture. By remote side. Hence pain from the heart may be misin-
pain we mean the pain that is caused at some terpreted as coming from the medial aspect of
other point than where the patient feels the the upper arm or axillary region. Pain in the
symptom, for example, pain felt in the foot as hip may be misinterpreted as coming from the
a result of a tumor at the fibular head pressing anteromedial aspect of the knee that shares
on the peroneal nerve or referred pain felt in the same innervation by the obturator nerve.
the anteromedial aspect of the knee due to a However, the pain experienced in the hand
pathologic process in the ipsilateral hip. Be- secondary to a thoracic outlet compression of
cause physical therapists are more likely to the nerves is not referred pain. The brain is not
overlook remote causes of pain, we give em- confused as it is when a patient feels pain in
phasis to these remote sources of pain. For this the axilla after injury to the heart muscle. Thus
same reason, in the diagnostic lists in the indi- we have two separate ways in which a patho-
vidual chapters of this book, the remote possi- logic process that takes place in one location is
bilities are always given first so they will be less felt in another. We have combined these two
likely to be overlooked. and use the term Remote Pain.
When dealing with pain in the knee the
difference between Remote and Local causes is What Follows the Making
reasonably clear. However, the closer the pain of a TIM VaDeTuCoNe List?
is to the trunk, the more difficult this differen- A diagnosis begins by gathering a complete list
tiation becomes. For example, when consider- of potential diagnoses that could result in the
ing neck pain-is a disk impingement on the patient’s symptoms. These are then sorted by the
nerve roots at the neck Remote or Local? Is likelihood of occurrence given the patient’s de-
mid-lumbar back pain that is the result of pan- mographics. Directed questions are then asked
creatitis Remote or Local? To answer these to eliminate as many inappropriate diagnoses as
questions, ponder how most physical thera- possible. Specific physical examination tests are
pists think about the particular clinical prob- selected to sort the remaining diagnoses to elim-
lem. In the neck, most physical therapists inate inappropriate ones.
would certainly think of cervical disk disease We have provided Case Demonstration
as a source of pain in the neck, hence we have chapters throughout this textbook to show the
included this under Local. However, with mid- application of the diagnostic process to exam-
lumbar pain, many physical therapists would ple patient cases. These chapters are structured
not think about pathology in an abdominal around the steps of the diagnostic process pre-
organ (eg, pancreatitis) or great vessel pathol- sented in Box 4-3, which are discussed in more
ogy (eg, an aortic aneurysm) as being a source detail next:
of the pain so could benefit from being re-
minded about the other possible sources of the 1. Identify the patient’s chief concern.
pain. Thus they are listed under Remote. 2. List barriers to communication.
Some readers may be unsure whether we 3. List special concerns.
consider systemic diseases, such as gout or 4. Draw a timeline and sketch the anatomy if
rheumatoid arthritis, Remote or Local. We con- needed. It is often useful to sketch a hori-
sider them as Local, in that the pathology, ie, zontal line, to an approximate scale, repre-
the swollen, hot joint is local. senting the time over which the patient’s
Is Remote pain the same as Referred pain? various symptoms and signs appeared,
Referred pain, as it is usually understood, marking significant events (Fig. 4-1).
comes from confusion on the part of the Drawing the anatomy can be especially
brain at deciphering the source of pain. When useful for remembering the location of the
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Chapter 4 How to Use This Book 29

BOX 4-3 Steps of the Diagnostic Process

Step 1 Identify the patient’s chief concern. Step 8 Re-sort the diagnostic hypothesis list
Step 2 Identify barriers to communication. based on the patient’s responses to
specific questioning.
Step 3 Identify special concerns.
Step 9 Perform tests to differentiate among
Step 4 Create a symptom timeline and the remaining diagnostic hypotheses.
sketch the anatomy (if needed).
Step 10 Re-sort the diagnostic hypothesis list
Step 5 Create a diagnostic hypothesis list based on the patient’s responses to
considering all possible forms of specific tests.
remote and local pathology that
could cause the patient’s chief Step 11 Decide on a diagnostic impression.
concern. Step 12 Determine the appropriate patient
Step 6 Sort the diagnostic hypothesis list by disposition.
epidemiology and specific case char-
acteristics.
Step 7 Ask specific questions to rule specific
conditions or pathological categories
less likely.

Date Date Date

Onset of Symptoms continue Initial physical


condition without change. therapy
Appointment with evaluation
appropriate doctor
FIGURE 4-1 Sample timeline.

internal organs in regard to remote causes 6. Sort all of the potential causes into clusters
of pain. This can be particularly useful if the based on the patient’s demographics:
reader’s knowledge of visceral anatomy is ● Reasonably likely

not strong. ● Possible

5. List possible remote and local diagnoses. ● Unlikely.

These are listed first since these are more 7. Take a history. Ask the most discriminating rel-
readily overlooked: evant questions to rule out as many options
● List the anatomic regions possibly in- as possible with the fewest questions. Ask
volved: thorax, abdomen, pelvis, etc. yourself, “Does this question asked exclude
● Within each anatomic region, list the or minimize the likelihood of one or more
possible organs that could cause the of the diagnoses on the TIM list?” If not,
symptom: abdomen, pancreas, gallblad- change or eliminate the question.
der, liver, spleen, great vessels, etc. 8. Based on the answers to the questions, re-sort
the potential causes into clusters as you did
For each of the organs, one could make a
in step 6:
TIM list of possible diagnoses, but that would
● Reasonably likely
be excessively tedious. If a reader’s knowledge
● Possible
of visceral anatomy is not strong, however,
● Unlikely.
then he or she should consider this step.
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30 Chapter 4 How to Use This Book

9. Conduct a physical examination. Look for raise special concerns. It continues with the
the most discriminating physical findings to formation of a diagnostic hypothesis list—a
rule out as many options as possible with database—consisting of all possible local and
the fewest tests. Again, ask yourself, “Does remote causes of the symptoms and signs. The
this physical test that I’m doing exclude or database is then sorted by probability on the
minimize the likelihood of one or more of basis of epidemiology, specific questions, and
the diagnoses on the TIM list?” specific physical examination findings. Now
10. Once again, re-sort the potential causes into turn to the next chapter for an example of how
clusters: one might go through the process.
● Reasonably likely

● Possible References
● Unlikely. 1. Drachman D. Occam’s razor, geriatric syndromes, and
the dizzy patient. Ann Intern Med. 2000;132:403–404.
11. Select the most probable diagnosis (the
2. Mosby’s Medical, Nursing, and Allied Health Dictionary.
diagnostic impression) for further evalua- 4th ed. St. Louis, MO: Mosby; 1994.
tion to establish a treatment plan. Remem- 3. Merriam-Webster’s Collegiate Dictionary. 11th ed. Spring-
ber the principle of economy of diagnoses field, MA: Merriam-Webster; 2003.
4. Stedman’s Medical Dictionary. 27th ed. Philadelphia, PA:
(Occam’s razor). Lippincott Williams & Wilkins; 2004.
12. Determine the appropriate patient disposition. 5. Oxford English Dictionary. 2nd ed. New York: Oxford
University Press; 1989.
6. Guide to Physical Therapist Practice. 2nd ed. Alexandria,
Conclusion VA: American Physical Therapy Association; 2003.
This chapter described the process for making
a diagnosis. The process begins with identifi-
cation of the patient’s chief concern, commu-
nication barriers, and features of the case that
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CHAPTER5
Case Demonstration: Shoulder
■ Hugh G. Watts, MD
Acknowledgment: Susan Layfield, PT, DPT, OCS

NOTE: This case demonstration was developed


Step 8 Re-sort the diagnostic hypothesis list
using the diagnostic process described in
based on the patient’s responses to specific
Chapter 4 and is designed to demonstrate that
questioning.
process. As Chapter 4 discussed, the diagnostic
Step 9 Perform tests to differentiate among
process begins with identification of the patient’s
the remaining diagnostic hypotheses.
chief concern. Communication barriers are then
Step 10 Re-sort the diagnostic hypothesis list
identified in order to establish features of the
based on the patient’s responses to specific
case that may influence the ability to gather
tests.
information during the diagnostic process.
Step 11 Decide on a diagnostic impression.
Features of the case that raise special concerns
Step 12 Determine the appropriate patient
are then established, in order to prioritize the
disposition.
additional information gathering required.
The process continues with the formation of a
diagnostic hypothesis list consisting of all possi-
ble local and remote causes of the symptom. The Case Description
hypothesis list is then sorted by probability on
the basis of epidemiology, specific questions, and Charles T. is a 58-year-old male with a referral
specific physical examination findings. The reader to physical therapy by an orthopedic surgeon
is encouraged to use this diagnostic process in with the diagnosis of Left Rotator Cuff Tendini-
order to ensure that thorough diagnostic clinical tis. His chief concern is an intermittent sharp
reasoning has been applied to all patient en- left anterior shoulder pain, the onset of which
counters. A brief list of the steps in the diagnos- was 2 weeks ago. He seems concerned because
tic process is given next. If more elaboration is he cannot recall a mechanism of injury nor
required on the information presented in this does he comment on any swelling in his shoul-
chapter, please consult Chapters 1 through 4. der or arm. The pain is aggravated by initial
exposure to a cold draft, for example, when
THE DIAGNOSTIC PROCESS taking out the trash, and when square dancing
with his wife during the first two to three
Step 1 Identify the patient’s chief concern. twirls with his left arm lifted up and out-
Step 2 Identify barriers to communication. stretched. Over the past 2 weeks, he believes
Step 3 Identify special concerns. his symptoms have not changed in frequency
Step 4 Create a symptom timeline and sketch or intensity. He denies any associated pain or
the anatomy (if needed). symptoms in his midback or upper extremity.
Step 5 Create a diagnostic hypothesis list Mr. T is an accountant. His recreation in-
considering all possible forms of remote and cludes square dancing two to three times a
local pathology that could cause the patient’s week. On the verbal confirmation of his intake
chief concern. form, Mr. T does not report any recent health
Step 6 Sort the diagnostic hypothesis issues or significant medical or surgical his-
list by epidemiology and specific case tory. Recently, he has been attempting to stay
characteristics. on a diet that his wife initiated for him. His
Step 7 Ask specific questions to rule specific weight loss is inconsistent per his report. He
conditions or pathological categories less believes he has lost 5 lbs in the past 4 weeks.
likely. Mr. T has a smoking history of 20 years and
31
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32 Chapter 5 Case Demonstration: Shoulder

quit 10 years ago. In his family history, his STEP #2: Identify barriers to communication.
father died of a heart attack at age 70 and his ● None
mother is 80 years old and healthy.
STEP #3: Identify special concerns.
Several special concerns were identified during
the initial intake and questioning that raised
concern regarding potential underlying etiolo-
gies of the patient’s chief concern. These
include:
● Age of the patient
● History of smoking
● Obesity
● Sedentary profession.

Teaching Comments: One body of liter-


ature that is helpful is the data collected for
the Framingham Heart Study. The Framing-
ham study is a long-term project that origi-
nally began in 1948 and has since been ex-
panded to a third generation with the
objective of identifying common factors or
characteristics that contribute to cardiovascu-
Anterior Posterior
lar disease (CVD) by following its develop-
ment over a long period of time in a large
group of participants who had not yet devel-
Teaching Comments: Clinicians need
oped overt symptoms of CVD or experience a
to view the anatomical representation of the
heart attack or stroke.
region where the pain is located with care.
Reliance on the accuracy of the patient’s in-
terpretation of the location of pain should not
supersede the clinical reasoning process STEP #4: Create a symptom timeline
nor prohibit the therapist from examining the and sketch the anatomy (if needed).
patient for other sources of pain. Instead it
should serve as a potential starting point.

STEP #1: Identify the patient’s chief


concern.
● Intermittent sharp left shoulder pain
that interferes with recreational activities

July 5 July 19 July 21

Onset of L Symptoms continue Initial physical


shoulder without change. therapy
symptoms Appointment with evaluation
orthopedic surgeon
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Chapter 5 Case Demonstration: Shoulder 33

Teaching Comments: In the case of Mr.


T, this step may seem irrelevant because his
timeline is simple. However, it does illus-
trate the lack of change during the 2-week
period of aggravation. It also allows you to
appreciate the temporal features of com-
peting diagnoses. The length of his symp-
toms illustrates that it is unlikely that any
acute perforations or ruptures are the
cause of his pain. This temporal feature
combined with the intensity of his symptoms
(he continues to work, perform household
chores, and participate in square dancing)
furthers the unlikelihood that he has experi-
enced any acute perforations or rupture as
the cause of his pain.
Example of PT’s sketch
Teaching Comments: Clinicians need to
remind themselves of the proximity of various
organs to the region of the symptoms their
patients are reporting. Perhaps even more
important is the review of the neurotube
development of various organs, which is the
theory behind referred pain.

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.

Teaching Comments: As recommended Teaching Comments: Conditions that


in Chapter 4, it may be helpful for the are shown with a strikethrough are those
clinician to break this step down even fur- that are determined to be less likely based on
ther to reduce the risk of “missed” potential the reasons that follow the hypothesis. It is
hypotheses. understood that some may return to the list
following questioning.

Remote Remote
T Trauma T Trauma
Cervical spine disk herniation Cervical spine disk herniation
Internal organ injuries: Internal organ injuries (very unlikely due to
lack of trauma to the trunk)
• Diaphragm • Diaphragm
• Liver • Liver
• Lung • Lung
• Spleen • Spleen
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34 Chapter 5 Case Demonstration: Shoulder

Post–laparoscopic procedure Post–laparoscopic procedure (no surgical


history)
Thoracic outlet syndrome Thoracic outlet syndrome
I Inflammation I Inflammation
Aseptic Aseptic
Acute cholecystitis Acute cholecystitis (presentation, no
scapular pain)
Costochondritis Costochondritis (presentation, no scapular
pain)
Gaseous distention of the stomach Gaseous distention of the stomach
(presentation)
Inflammatory bowel disease Inflammatory bowel disease (absent
medical history)
Rheumatoid arthritis–like diseases of the Rheumatoid arthritis–like diseases of the
cervical spine: cervical spine:
• Inflammatory muscle disease • Inflammatory muscle disease
• Psoriatic arthritis • Psoriatic arthritis
• Scleroderma • Scleroderma (presentation, patient sex)
• Systemic lupus erythematosus • Systemic lupus erythematosus
(presentation, patient sex)
Septic Septic
Acute viral/idiopathic pericarditis Acute viral/idiopathic pericarditis
(presentation, time course)
Cat-scratch disease Cat-scratch disease (presentation)
Cervical epidural abscess Cervical epidural abscess
Cervical lymphadenitis Cervical lymphadenitis (presentation)
Gastric ulcer Gastric ulcer (presentation)
Hepatitis Hepatitis (presentation)
Perihepatitis Perihepatitis (presentation)
Pleuritis Pleuritis (presentation)
Pneumonia Pneumonia (presentation)
Subphrenic abscess Subphrenic abscess (presentation)
M Metabolic M Metabolic
Osteomalacia Osteomalacia
Va Vascular Va Vascular
Aneurysm Aneurysm
Coronary artery insufficiency Coronary artery insufficiency
Myocardial infarction Myocardial infarction (presentation, time
course)
Pulmonary embolism Pulmonary embolism (presentation, time
course)
Sickle cell pain crisis Sickle cell pain crisis (presentation, time
course)
De Degenerative De Degenerative
Osteoarthritis of the cervical spine Osteoarthritis of the cervical spine
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Breast tumor • Breast tumor
• Pancoast tumor • Pancoast tumor
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Chapter 5 Case Demonstration: Shoulder 35

Malignant Metastatic: Malignant Metastatic:


Not applicable Not applicable
Benign: Benign:
Not applicable Not applicable
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Not applicable Not applicable

Teaching Comments: “Not applicable”


means that the author of that chapter or case
was unable to locate a reasonable diagnosis
to place under that diagnostic category.
The user of the textbook may be able to
discover a diagnosis that would be appropri-
ate or may choose a different category for
a previously mentioned diagnoses. Each of
these discrepancies would be considered
acceptable. The algorithmic steps are de-
signed to enhance the clinical reasoning, not
stifle it.

Local Local
T Trauma T Trauma
Dislocation such as acromioclavicular, Dislocation such as acromioclavicular,
glenohumeral, or sternoclavicular glenohumeral, or sternoclavicular joints
joints (presentation)
Fractures Fractures (presentation)
Glenohumeral joint sprain/subluxation Glenohumeral joint sprain/subluxation
Glenoid labrum tear Glenoid labrum tear
Muscle strain, such as pectoralis major, Muscle strain, such as pectoralis major,
rotator cuff, upper trapezius, levator rotator cuff, upper trapezius, levator
scapulae scapulae
Subacromial impingement syndrome Subacromial impingement syndrome
I Inflammation I Inflammation
Aseptic Aseptic
Adhesive capsulitis Adhesive capsulitis (presentation)
Biceps tendinitis Biceps tendinitis
Bursitis Bursitis
Complex regional pain syndrome Complex regional pain syndrome
(presentation)
Fibromyalgia Fibromyalgia (patient sex, age,
presentation)
Myofascial pain syndrome Myofascial pain syndrome (presentation,
time course)
Neuralgic amyotrophy Neuralgic amyotrophy (initial presentation)
Polymyalgia rheumatica Polymyalgia rheumatica (initial presentation
with activity)
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36 Chapter 5 Case Demonstration: Shoulder

Reiter’s syndrome Reiter’s syndrome (presentation, time


course)
Rheumatoid arthritis Rheumatoid arthritis (patient age,
presentation, medical history)
Rheumatoid arthritis–like diseases of Rheumatoid arthritis–like diseases of
shoulder: shoulder:
• Inflammatory muscle disease • Inflammatory muscle disease (patient sex,
age, presentation)
• Psoriatic arthritis • Psoriatic arthritis (patient sex, age,
presentation)
• Scleroderma • Scleroderma (patient sex, age,
presentation)
• Systemic lupus erythematosus • Systemic lupus erythematosus (patient
sex, age, presentation)
Rotator cuff tendinitis Rotator cuff tendinitis
Septic Septic
Osteomyelitis Osteomyelitis (presentation, medical
history)
Septic arthritis Septic arthritis (presentation)
Skeletal tuberculosis Skeletal tuberculosis (presentation, medical
history)
M Metabolic M Metabolic
Amyloid arthropathy Amyloid arthropathy (presentation)
Gout Gout
Hereditary neuralgic amyotrophy Hereditary neuralgic amyotrophy
(presentation, medical history)
Heterotopic ossification Heterotopic ossification (medical history)
Pseudogout Pseudogout (patient sex)
Va Vascular Va Vascular
Aneurysm Aneurysm
Avascular necrosis of the humeral head Avascular necrosis of the humeral head
Compartment syndrome Compartment syndrome
Deep venous thrombosis, upper extremity Deep venous thrombosis, upper extremity
Quadrilateral space syndrome Quadrilateral space syndrome (patient age,
presentation)
De Degenerative De Degenerative
Biceps tendinopathy Biceps tendinopathy
Osteoarthritis/osteoarthrosis Osteoarthritis/osteoarthrosis
Rotator cuff tear/tendinopathy Rotator cuff tear/tendinopathy
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Chondrosarcoma • Chondrosarcoma
• Lymphoma • Lymphoma
• Osteosarcoma • Osteosarcoma
• Synovial sarcoma • Synovial sarcoma
Malignant Metastatic, such as: Malignant Metastatic, such as:
• Metastases, including from primary • Metastases, including from primary
breast, kidney, lung, prostate, and thyroid breast, kidney, lung, prostate, and thyroid
disease disease
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Chapter 5 Case Demonstration: Shoulder 37

Benign, such as: Benign, such as:


• Enchondroma • Enchondroma (patient age, presentation)
• Lipoma • Lipoma (patient age, presentation)
• Osteoblastoma • Osteoblastoma
• Osteochondroma • Osteochondroma (patient age, presentation)
• Osteoid osteoma • Osteoid osteoma
• Unicameral bone cyst • Unicameral bone cyst
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Erb’s palsy Erb’s palsy (patient age, presentation)
Neuropathic arthropathy Neuropathic arthropathy (patient age,
presentation, family history)

STEP #7: Ask specific questions to rule ● Do you have stiffness or pain in other
specific conditions or pathological joints of your body? Answer: No. Makes less
categories less likely. likely gout of the shoulder, osteomalacia,
and pathologies of the cervical spine.
Teaching Comments: The therapist ● Has there been any swelling, redness, or
should strive to ask the most discriminating warmth in area? Answer: No. Makes less
relevant questions to rule less likely as many likely deep venous thrombosis such as the
options as possible with the least number of subclavian, gout within the shoulder, septic
questions to the patient. The phrase “makes arthritis of glenohumeral joint, osteoid
less likely” is used rather than “rules out” be- osteoma, and unicameral bone cyst.
cause there are enough exceptions to the
usual behavior of diseases that the less con-
crete term is less liable to lead one astray. Teaching Comments: The user should
note that the majority of questions were
asked specifically to rule less likely a number
● Do you have neck pain? Answer: No. of the diagnoses or diagnostic categories that
● Does your shoulder hurt if you move your were remaining on the potential list for Mr. T’s
neck? Answer: No. Makes less likely cervical chief concern. This is an efficient method
spine disk herniation, levator scapula and of questioning—not only questioning the
upper trapezius strain, rheumatoid arthritis– patient for the answer but asking questions
like disease of the cervical spine, and specifically toward a particular diagnoses or
osteoarthritis/osteoarthrosis of the cervical diagnostic category.
spine.
● Do you have any restriction in your range
of shoulder motion? Answer: No. Makes less STEP #8: Re-sort the diagnostic
likely avascular necrosis of humeral head, hypothesis list based on the patient’s
tendinitis, osteomyelitis of shoulder, os- responses to specific questioning.
teoarthritis/osteoarthrosis of the shoulder Remote
complex joints, and unicameral bone cyst.
T Trauma
● Is the pain constantly present? Answer: No.
Makes less likely avascular necrosis/ Cervical spine disk herniation (absent neck
osteonecrosis of humeral head and unicam- pain, shoulder pain with neck movement)
eral bone cyst. Thoracic outlet syndrome
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38 Chapter 5 Case Demonstration: Shoulder

I Inflammation Rotator cuff tendinitis (no restriction in


Aseptic shoulder range of motion)
Rheumatoid arthritis–like diseases of the Septic
cervical spine: Not applicable
• Inflammatory muscle disease (absent M Metabolic
neck pain, shoulder pain with neck
Gout (no stiffness/pain in other joints, no
movement)
swelling/redness/warmth)
• Psoriatic arthritis (absent neck pain,
shoulder pain with neck movement) Va Vascular
Septic Aneurysm
Not applicable Avascular necrosis of the humeral head (no
restriction in shoulder range of motion,
M Metabolic
no constant pain)
Osteomalacia (no pain in other joints) Compartment syndrome
Va Vascular Deep venous thrombosis, upper extremity
Aneurysm De Degenerative
Coronary artery insufficiency Biceps tendinopathy
De Degenerative Osteoarthritis/osteoarthrosis (no restriction
Osteoarthritis of the cervical spine (absent in shoulder range of motion)
neck pain, shoulder pain with neck Rotator cuff tear/tendinopathy
movement) Tu Tumor
Tu Tumor Malignant Primary, such as:
Malignant Primary, such as: • Chondrosarcoma
• Breast tumor • Lymphoma
• Pancoast tumor • Osteosarcoma
Malignant Metastatic: • Synovial sarcoma
Not applicable Malignant Metastatic, such as:
Benign: • Metastases, including from primary
Not applicable breast, kidney, lung, prostate, and
thyroid disease
Co Congenital
Benign, such as:
Not applicable • Osteoblastoma
Ne Neurogenic/Psychogenic • Osteoid osteoma (no
Not applicable swelling/redness/warmth)
• Unicameral bone cyst (no restriction in
Local
shoulder range of motion, no constant
T Trauma pain, no swelling/redness/warmth)
Glenohumeral joint sprain/subluxation Co Congenital
Glenoid labrum tear
Not applicable
Muscle strain, such as pectoralis major,
rotator cuff, upper trapezius, levator Ne Neurogenic/Psychogenic
scapulae (absent neck pain, shoulder Not applicable
pain with neck movement)
STEP #9: Perform tests to differentiate
Subacromial impingement syndrome
among the remaining diagnostic
I Inflammation hypotheses.
Aseptic
Biceps tendinitis (no restriction in shoulder
range of motion)
Bursitis
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Chapter 5 Case Demonstration: Shoulder 39

Benign:
Teaching Comments: Clinicians should
Not applicable
look for the most discriminating physical
findings to rule out as many options as Co Congenital
possible with the fewest tests. Not applicable
Ne Neurogenic/Psychogenic
● Shoulder active range of motion. Full and Not applicable
pain free. Makes less likely biceps and rota- Local
tor cuff tendinopathy, chondrosarcoma,
T Trauma
bursitis, and osteoblastoma.
● Palpate for mass; listen for bruit over Glenohumeral joint sprain/subluxation
region including auscultation over lungs. (pain-free shoulder range of motion)
Negative. Makes less likely breast tumor. Glenoid labrum tear (pain-free shoulder
range of motion)
Subacromial impingement syndrome
Teaching Comments: Breast cancer in (pain-free shoulder range of motion)
males exhibits similar signs as in females with I Inflammation
a lump or swelling in the breast tissue,
Aseptic
skin dimpling, nipple retraction or discharge
Bursitis (pain-free shoulder range of
from the nipple, and/or redness of the nipple/
motion)
breast skin.
Septic
Not applicable
STEP #10: Re-sort the diagnostic
hypothesis list based on the patient’s M Metabolic
responses to specific tests. Not applicable
Remote Va Vascular
T Trauma Aneurysm (auscultation negative for
Thoracic outlet syndrome (pain-free bruits)
shoulder range of motion) Compartment syndrome
Deep venous thrombosis, upper extremity
I Inflammation
De Degenerative
Aseptic Biceps tendinopathy (pain-free shoulder
Not applicable range of motion)
Septic Rotator cuff tear/tendinopathy (pain-free
Not applicable shoulder range of motion)
M Metabolic Tu Tumor
Not applicable Malignant Primary, such as:
Va Vascular • Chondrosarcoma (pain-free shoulder
Aneurysm (no bruits on auscultation) range of motion)
Coronary artery insufficiency • Lymphoma
• Osteosarcoma
De Degenerative • Synovial sarcoma
Not applicable Malignant Metastatic, such as:
Tu Tumor • Metastases, including from primary
Malignant Primary, such as: breast, kidney, lung, prostate, and
• Breast tumor (negative palpation) thyroid disease
• Pancoast tumor Benign, such as:
Malignant Metastatic: • Osteoblastoma (pain-free shoulder range
Not applicable of motion)
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40 Chapter 5 Case Demonstration: Shoulder

Co Congenital physician should be the immediate next


Not applicable step.
Ne Neurogenic/Psychogenic
Case Outcome
Not applicable
At this point, Mr. T.’s treating physical thera-
STEP #11: Decide on a diagnostic pist was unsure as to a specific diagnosis, but
impression. was certain that his symptoms warranted fur-
● Remaining conditions include coronary ar- ther medical evaluation. Mr. T. was referred to
tery insufficiency, Pancoast tumor, com- his family physician after first telephoning
partment syndrome, upper extremity deep him. Mr. T. later called to report that his physi-
venous thrombosis, as well as local malig- cian wasted no time in performing cardiac
nant primary and metastatic tumors. function tests and cardiac bypass surgery was
STEP #12: Determine the appropriate performed successfully within a week. Note
patient disposition. that this patient had gone to the physical ther-
apist and had filled in a “screening form”
● Refer the patient to a physician for additional
where the patient’s answers were interpreted as
testing and treatment, because none of the
indicating that his symptom was due to a
remaining health conditions is amenable to
process that could be construed as appropriate
physical therapy independently.
for treatment by a physical therapist.
● Clear communication with the referral
source and potentially the patient’s family
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SECTION II
Adult Pain

CHAPTER6
Diagnostic Considerations of Pain
■ Todd E. Davenport, PT, DPT, OCS ■ Lucinda L. Baker, PT, PhD

IN THIS CHAPTER: Psychological and Social Factors Perpetuate Pain


■ Anatomy and physiology of pain Through Behavior Reinforcement 51
■ Pain perception and clinical presentation Conclusion 52

OUTLINE
Introduction 41 Introduction
All People Share Common Nociceptive
Processes 42 Disability and distress related to pain are among
Nociceptors Respond to Various Forms of Tissue the most common reasons that lead people to
Injury 42 seek rehabilitation. Pain is a somatosensory
Nociceptors Transmit Pain Signals Along Organized modality—along with thermoreception, touch,
Neural Pathways 42 and proprioception—that is defined as the
The Perception of Pain Severity Depends unpleasant sensory experience associated with
on Anatomical, Physiological, and actual or potential tissue damage.1 Pain serves
Cognitive Factors 43 sensory, emotional, and cognitive functions.2,3
A “Gate” Controls the Transmission of Pain Signals Pain’s sensory-discriminative function allows for
at the Spinal Cord and Elsewhere 43 self-preservation. When a hand is placed on a
Input from the Cerebral Cortex and Midbrain Also hot stove, the sensory-discriminative func-
Plays a Role in the Gate’s Function 44 tion of pain compels the withdrawing of the
Emotional, Anticipatory, and Previously Learned hand and inspecting for tissue damage. Tissue
Responses Also Modulate Pain Severity 46 damage results when the sensory-discriminative
Timing of Pain Suggests the Etiology of function of pain is impaired. One example of
Underlying Pathology 46 this phenomenon is the skin ulceration often
Factors in the History and Physical Examination seen on the plantar surfaces of the feet in peo-
Can Implicate Affected Tissues 46 ple with diabetic polyneuropathy. The affective
Descriptions of Pain May Relate to Either Injured function of pain provides emotional unpleas-
Tissues or Emotional Features 47 antness to pain sensations. This causes people
Locations of Pain and Its Underlying to avoid additional pain and the tissue damage
Cause May Differ 47 that pain represents. The cognitive-evaluative
Misinterpretation by the Central Nervous System function serves to encourage learning and
Causes Referred Pain 48 foster behavioral adaptation. Disorder involv-
Long-Term Pain Causes Cyclic Anatomical, ing the affective and cognitive-evaluative
Physiological, and Cognitive-Behavioral functions of pain may result in maladaptive
Changes 49 behavioral responses to pain, such as a
Prolonged Activation of Nociceptive Pathways disabling avoidance of work, family, and
Causes Maladaptations Throughout the recreational activities.
Nervous System 50 The same basic anatomical and physiolog-
The Sympathetic Nervous System Can Contribute ical processes that generate and conduct pain
to the Perception of Pain 51 sensations are found in all people. However,
41
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42 Chapter 6 Diagnostic Considerations of Pain

there are vast differences in how people are most likely to transmit information at low
respond to pain sensations. This causes a great levels of an adequate stimulus, whereas silent
variation in how people will demonstrate pain nociceptors are characterized by such high
in the clinic. Individual differences in how activation thresholds that they must be sensi-
pain sensation is processed often make it tized by inflammatory chemicals to facilitate
difficult to interpret for both patients and their function (Box 6-1).7
clinicians in the diagnostic process. This chapter
discusses pain as it relates to the diagnostic Nociceptors Transmit Pain Signals
process. The chapter first describes the processes Along Organized Neural Pathways
that all people have in common, and then
Axons transmitting nociceptive information
describes anatomical and physiological reasons
from the periphery project onto the dorsal
for individual differences in pain perception.
horn of the spinal cord. Six subdivisions of
dorsal root ganglion receive input from axons
All People Share Common transmitting information about one nocicep-
Nociceptive Processes tive modality each.8 The A␦ axons of mechan-
ical and thermal nociceptors make unisynaptic
The association between pain and actual tissue
connections with neurons that transmit pain
damage suggests that all people share common
signals to the central nervous system, referred
anatomy and physiology that mediate pain
to as projection neurons. In addition, A␦ axons
perception. Nociception occurs when pain is
synapse with wide dynamic range neurons that
perceived through the activation of peripheral
integrate pain signals from multiple regions of
pain receptors due to tissue damage.
the body. The C axons of polymodal nocicep-
tors make multisynaptic connections with
Nociceptors Respond to Various
projection neurons by way of interneurons in
Forms of Tissue Injury
the substantia gelatinosa of the spinal cord.
Pain receptors are free nerve endings called The functional relevance of these local neural
nociceptors. Nociceptors are broadly categorized circuits is discussed in a later section.
by their location. Nociceptors located through-
out the skin and musculoskeletal structures are
called somatic nociceptors. Somatic nociceptors BOX 6-1 A “Toxic Soup” Sensitizes
are primarily classified by their adequate Nociceptors
stimuli—stimuli to which they respond. Me- Among their many functions, inflammatory sub-
chanical nociceptors are activated by intense stances released during tissue injury sensitize
pressure.4 Thermal nociceptors respond to nociceptors, enhancing the primary hyperal-
temperatures less than 5°C and greater than gesia that results from mechanical stimulation
45°C. Mechanical and thermal nociceptors of nociceptors.2 Histamine, the first chemical
transmit action potentials along A␦ axons released in the inflammatory process through
when activated. The A␦ axons are thinly myeli- mast cell degranulation, activates polymodal
nated and conduct at velocities of 5 to 30 m/s.4 nociceptors. Bradykinins are potent pain-producing
substances that result from conversion of a
Polymodal nociceptors are activated by mechan- plasma protein in the blood clotting cascade.
ical, thermal, and chemical stimuli, sending Prostaglandins and leukotrienes are derivatives
action potentials along C fibers to the spinal of arachidonic acid formed after damage to the
cord when activated.4 The C fibers are small, cell membranes that sensitize nociceptors. The
unmyelinated axons that conduct at velocities neuropeptide substance P is released from
less than 1 m/s. activated nociceptors, leading to additional
Visceral nociceptors transmit information nociceptor sensitization. Other inflammatory
about distention, ischemia, and chemical chemicals increase nociception by promoting
irritation of the deep organs along C fibers.5 tissue damage adjacent to the initial area of
injury, including cytokines and oxygen free radicals.
Visceral nociceptors are further classified by These substances serve to sensitize surrounding
their activation thresholds into low, high, and nociceptors, producing a secondary hyperalgesia.
silent nociceptors.6 Low-threshold nociceptors
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Chapter 6 Diagnostic Considerations of Pain 43

Projection neurons transmit action poten-


BOX 6-2 Pitfalls of Measuring Pain
tials along five organized tracts within the
Severity
ventral and lateral regions of the spinal cord.4,9
Their axons are called the anterolateral system Change in pain severity is commonly considered
of the spinal cord because of their location. useful to estimate treatment efficacy. Unfortunately,
Axons of the anterolateral system cross sides health care providers are poor at estimating
within one to two spinal levels rostral to the patients’ levels of pain.49 Accurate estimation
of pain severity is crucial—overestimation may
input level before ascending to target nuclei in
lead to overtreatment and vice versa. The
the brainstem, hypothalamus, and thalamus. visual/verbal analog scale (V/VAS), in which the
The spinothalamic tract is the most promi- patient rates pain on a scale from 0 (lowest) to
nent in the anterolateral system, transmit- 10 (greatest), is widely used because of its intu-
ting nociceptive signals to cortical processing itive appeal and convenience. However, V/VAS
centers by way of thalamic interneurons. The ratings do not predict the need for analgesic
spinohypothalamic tract transmits nociceptive medication,50 and the magnitude of clinically
signals to supraspinal autonomic centers that meaningful change for V/VAS ratings has yet to
mediate neuroendocrine and cardiovascular be determined. Measurements of pain-related
function, anxiety level, or disability beliefs may
control. Many spinohypothalamic axons do
serve as more effective indicators of treatment
not cross sides. efficacy.
The spinoreticular tract projects onto the
reticular formation and thalamus for control
of consciousness and relay to cortical centers,
respectively. Neurons composing the spinomes- Melzack and Wall10 first described the gate
encephalic tract project onto the periaqueduc- control hypothesis to explain how the anatomy
tal gray matter in the midbrain that inhibits and physiology of the central nervous system
the transmission of pain signals from the control the transmission of information about
periphery. In addition, neurons from the spin- pain all along the neuraxis. This theory has
omesencephalic tract project onto the insula, been greatly elaborated since it was first pro-
which may also mediate the autonomic control posed. The mechanisms responsible for the
of pain perception. The cervicothalamic tract gate include the morphology of axons carrying
originates in the lateral cervical nucleus and somatosensory information, the structure of
ascends along the dorsal column–medial local neural circuits, and the nature of inter-
lemniscal pathway. This tract may be signifi- connections between local neural circuits and
cant for people with neck pain. supraspinal centers. Interactions among differ-
ent regions of the central nervous system are
also responsible for creating thoughts, feelings,
The Perception of Pain Severity learning, and behaviors related to pain. These
Depends on Anatomical, cognitive processes may also modulate the
Physiological, and Cognitive Factors severity of a person’s pain sensations.
One would think that the most severe injuries
A “Gate” Controls the Transmission
would cause the most incapacitating pain.
of Pain Signals at the Spinal Cord
However, some people may report incapaci-
and Elsewhere
tating pain levels due to an undetectable
tissue injury. There are also numerous sto- Neural networks that transmit sensory infor-
ries of people reacting meaningfully to their mation about light cutaneous pressure from
environment despite sustaining severe in- the periphery compete with the transmission
juries in combat. Clearly, the perception of of pain signals within the spinal cord. Touch
pain severity is modulated by a variety of receptors transmit along large-diameter A␤
anatomical, physiological, and cognitive axons to the dorsal horn of the spinal cord.4,9
processes. This challenges attempts at repro- This arrangement allows integration of touch,
ducible and meaningful measurement of temperature, and pain signals before transmis-
pain severity (Box 6-2). sion to the thalamus. The larger diameter and
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44 Chapter 6 Diagnostic Considerations of Pain

thicker myelination of the A␤ axons also pro- the inhibitory interneuron is itself inhibited,
vide faster conduction speeds than the A␦ and causing an increase in activation of the wide
C axons that carry pain signals. Because light- dynamic range neuron and perception of
touch sensation signals reach the wide dynamic pain (Fig. 6-1C). When both the A␤ and C
range neuron faster than pain signals, light- axons provide input, pain signal transmis-
touch sensation is perceived first. sion by the wide dynamic range neuron is
Interaction among A␤, C, and inhibitory modulated (Fig. 6-1D).
interneurons also modulates the transmission
of pain signals at the level of the spinal cord Input from the Cerebral Cortex
(Fig. 6-1A). In addition to their synaptic con- and Midbrain Also Plays a Role
nection to a wide dynamic range neuron, A␤ in the Gate’s Function
axons also make an excitatory synapse onto
inhibitory interneurons that project onto the Descending inputs to the dorsal horn of the
wide dynamic range neuron. Normally, the spinal cord from the midbrain and cerebral
inhibitory interneuron prevents the activa- cortex modulate pain perception by way of
tion of the wide dynamic range neuron. Con- two interrelated neurotransmitter systems:
sequently, the inhibitory interneuron normally serotonin and enkephalin. Neurons in the pe-
inhibits the perception of pain. Touch input riaqueductal gray matter of the midbrain
increases the level of inhibition of the wide synapse onto serotonergic projection neurons
dynamic range neuron and decreases the per- in the raphe nuclei of the medulla. These sero-
ception of pain (Fig. 6-1B). The C axon makes tonergic projection neurons descend to the
an inhibitory synapse with the inhibitory dorsal horn of the spinal cord, reducing pain
interneuron. When C axonal input is greatest, perception by two mechanisms (Fig. 6-1E).9

Pain Pain
afferent afferent

Wide Wide
dynamic dynamic
Cutaneous range cell Cutaneous range cell
afferent afferent
A B
FIGURE 6-1 (A) Somatosensory afferents also synapse with two different types of neurons—one excitatory synapse
with an excitatory interneuron (1) and one excitatory synapse with the wide dynamic range cell. The excitatory
interneuron makes an excitatory synapse with the inhibitory interneuron and the wide dynamic range cell. Noci-
ceptive afferent makes two synapses—one inhibitory synapse with an inhibitory interneuron (2) and one excita-
tory synapse directly with the wide dynamic range cell. The inhibitory interneuron synapses with the wide dynamic
range cell. (B) When somatosensory input is greatest, touch signals are propagated in two ways. The cutaneous
afferent excites the wide dynamic range cell directly and indirectly through activating the excitatory interneuron.
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Chapter 6 Diagnostic Considerations of Pain 45

Pain Pain
afferent afferent

1 YIELD

Wide Wide
dynamic dynamic
Cutaneous range cell Cutaneous range cell
afferent afferent
C D

Descending
inhibitory pathways

Pain
afferent

Wide
dynamic
Cutaneous range cell
E afferent
FIGURE 6-1—cont’d (C) When nociceptive input is greatest, pain signals are propagated in two ways.
The pain afferent excites the wide dynamic range cell directly and indirectly by inhibiting the inhibitory
interneuron. (D) When somatosensory and nociceptive input are present, input from the somatosensory
afferent mitigates the transmission of pain signals by activating the inhibitory interneuron. (E) Descend-
ing inhibitory input from serotonergic projection neurons in the raphe nucleus of the medulla reduces the
transmission of nociceptive signals directly by inhibiting the wide dynamic range cell and indirectly by
exciting the inhibitory interneuron.
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46 Chapter 6 Diagnostic Considerations of Pain

Serotonin inhibits the wide dynamic range indicator of rehabilitation outcomes. Bandura16
neuron that transmits pain signals to the cere- broadly describes efficacy expectations as an
bral cortex. In addition, serotonin excites individual’s task- and situation-specific estimate
enkephalinergic inhibitory interneurons in the of personal mastery. Bandura16 suggested that
dorsal horn of the spinal cord, which further individuals would avoid environments and
reduces the transmission of pain signals. The activities that seemed to exceed their own esti-
primary somatosensory cortex also gives rise mate for coping. In addition, self-efficacy is
to a network of projection neurons that inhibit positively associated with the magnitude and
perception of somatic sensation.9 The targets persistence of coping behaviors once they are
of these neurons include the thalamus and initiated.17–19 Therefore, pain-related self-efficacy
dorsal horn of the spinal cord. influences an individual’s choice of environ-
ment and activities in response to anticipated
Emotional, Anticipatory, and symptom coping. These ideas appear to explain
Previously Learned Responses Also the significant association between self-efficacy
Modulate Pain Severity and pain-related activity avoidance, in that
Axons connecting the frontal cortex, hypo- patients with low self-efficacy more frequently
thalamus, thalamus, and amygdala to the tended toward increased pain-related activity
periaqueductal gray matter of the midbrain avoidance beliefs12,20–22 and poorer functional
may provide the neuroanatomical basis for outcomes.23 Perhaps unsurprisingly then, patient
integrating pain perception, cognitive- education with the intent of modifying patient
emotional state, autonomic regulation, and self-efficacy is an area of developing interest and
motor activity.11 This neural circuit appears research in physical therapy.24
at least partially responsible for the unique
psychological features of people that power- Timing of Pain Suggests the
fully influence how pain is perceived. Emo- Etiology of Underlying Pathology
tional and cognitive aspects of pain both
positively and negatively affect coping. Cog- Temporal aspects of pain provide important
nitive factors that negatively influence pain clues about affected tissues and the nature of
coping include increased fear of pain and pain-producing pathology. Short-term pain is
catastrophizing about pain. Fear of pain is more often the result of an active pathology.
defined as “a highly specific negative emo- Long-term pain is more likely to be the result
tional reaction to pain involving a high of neuroplastic changes within the central
degree of mobilization for escape/avoidance nervous system. The nature of these changes
behavior,”12 which relates to excessive anxiety are discussed in a later section of this chapter.
about pain. Otherwise healthy people with a
high fear of pain report a higher severity of
Factors in the History and Physical
experimentally induced acute pain than peo-
Examination Can Implicate Affected
ple with a low fear of pain. Catastrophizing is
Tissues
defined as “an exaggerated negative mental Differentiation among pain-producing patholo-
act brought to bear during an actual or antic- gies depends on their dissimilar patterns of
ipated painful experience,”13 reflecting “a factors that aggravate, alleviate, and associate
tendency to focus on pain and negatively with the perception of pain. Aggravating factors
evaluate one’s own ability to cope with are those activities of daily living, exercise, work,
pain.”14 Although fear of pain and catastro- or recreation; active, passive, or resisted move-
phizing are related, catastrophizing behavior ments; or palpation that worsen pain. Conversely,
more strongly predicts the self-reported alleviating factors relieve pain. Associated factors
severity of experimentally induced acute pain include a wide variety of constitutional, derma-
than fear of pain itself.15 tological, mental, and neurological signs and
The importance of metacognitive processes symptoms that may appear simultaneously
on pain perception has led some authors to with pain. To be certain, clinicians should also be
hypothesize that self-efficacy is an important alert for aggravating, alleviating, and associated
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Chapter 6 Diagnostic Considerations of Pain 47

factors that indicate an immediate need to


BOX 6-3 How “Good” Are History and
refer for additional medical treatment. These
Physical Examination Findings?
are listed at the beginning of each chapter
discussing pain in each body region. Various statistical properties are used to represent
Three sources of lower extremity pain— the effectiveness of questions and tests for pur-
lumbar herniated intervertebral disk, lumbar poses of diagnosis.51 Sensitivity is the proportion
spinal stenosis, and peripheral vascular disease— of people who give a positive result for the disorder
a test is intended to reveal. Specificity is the prob-
may be distinguished on the basis of different
ability that a test will not give a positive result for
movements that aggravate or alleviate pain. people in whom the target disorder is absent.
People with a displaced lumbar disk classically Positive predictive value is the ratio of people
report pain that worsens during positions of with the disease that test positive to the entire
lumbar flexion that include bending, kneeling, population of people with the disorder regardless
sitting, and squatting. Conversely, symptoms of test result. Negative predictive value is the
associated with herniated lumbar disk are most ratio of people without the disease that test
likely alleviated in positions of extension, such as negative to the entire population of people
standing. People with lumbar spinal stenosis without the disorder regardless of test result. Use
of several questions and tests in clusters is com-
classically report pain that worsens with the
mon to help compensate for the low sensitivity,
positions of lumbar extension, and pain relief specificity, and predictive values of many questions
during positions of flexion.25 People with and physical examination tests.
peripheral vascular disease of the lower extremi-
ties also report pain during standing and walk-
ing—which involves lumbar extension—and an individual’s description and corresponding
relief with sitting—which involves lumbar flexion. pain-generating structure is known to exist,
The stoop test,26 in which an individual such as cardiac (“crushing”), neuropathic
assumes a squat position, places the lumbar (“shooting and stinging”), muscular (“sharp”),
spine in a position of relative flexion. Repro- vascular (“cramping”), and visceral (“dull and
duction of symptoms with the stoop test may diffuse”). Although the relationship between
rule out lumbar spinal stenosis and confirm description and pathology can vary, it provides
the presence of a lumbar herniated disk. Alle- one shorthand approach to ruling out pathol-
viation of symptoms with this test may rule ogy in the differential diagnostic process.
out the herniated lumbar disk as a likely cause Words such as “punishing,” “sickening,”
of symptoms and confirm the presence of and “excruciating” suggest that an individual
either lumbar spinal stenosis or peripheral attributes a strong emotional meaning to pain.
vascular disease. The bicycle test,27 in which an The McGill Pain Questionnaire28,29 is a
individual performs aerobic exercise on a psychometric test that measures the frequency
bicycle, maintains the spine in a relatively of emotionally charged terms that individuals
flexed position while challenging the cardio- use to describe their perception of pain,
vascular system. Reproduction of symptoms reflecting the state of individuals’ emotional
with the bicycle test implicates peripheral vas- reasoning about their pain experience. An
cular disease as a cause of the individual’s lower increased frequency of affective descriptions of
extremity pain. The effectiveness of all history pain may relate to symptoms of depression
and physical examination tests may be repre- and anxiety. In turn, depression and anxiety
sented by their statistical properties (Box 6-3). may lead to difficulty soliciting necessary
information during the history and lead to
Descriptions of Pain May Relate to
poor treatment outcomes.
Either Injured Tissues or Emotional
Features
Locations of Pain and Its
Words selected to describe the internal and Underlying Cause May Differ
deeply personal phenomenon of pain may pro-
vide useful information regarding its underlying “Where does it hurt?” is one of the first ques-
pathology. An anecdotal relationship between tions a clinician often asks a patient who
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48 Chapter 6 Diagnostic Considerations of Pain

presents with pain. The answer may be mis- pain is a specific type of neuropathic pain,
leading. Pathology that is remote or local to which originates from a lesion of the nervous
the painful area may be responsible for the system. Projected pain is another type of
patient’s symptoms. Local pain is perceived at neuropathic pain that occurs when injury to a
the location of tissue damage (Fig. 6-2A). peripheral nerve occurs along its course
Remote pain is perceived at a distance from the (Fig. 6-2C). One example of projected pain is
location of the responsible pathology.30 medial forearm pain due to an ulnar nerve
Pathology causing remote pain is often confused entrapment at the cubital tunnel. Referred pain
with pathology causing local pain, making it is perceived at a distance from the site of
important to consider remote causes of pain pathology, though not secondary to irritation
first in the diagnostic process. of a spinal or peripheral nerve. Pain may be
Remote pain may be neuropathic or referred from one somatic structure to another,
referred. Neuropathic pain occurs with damage a visceral structure to a somatic structure, or
to the nociceptive system itself. Examples of one visceral structure to another (Fig. 6-2D).31
neuropathic pain syndromes include diabetic Pain referred from one somatic structure to
polyneuropathy, post-herpetic neuralgia, and another often follows predictable, irregular
traumatic nerve injury (formerly referred to as soft tissue patterns called sclerotomes.32 Pain
complex regional pain syndrome type II). referred from a visceral structure to a somatic
Radicular pain is a type of neuropathic pain structure may also follow a dermatomal
that occurs when mechanical impingement of distribution.33
a spinal nerve root causes the sensation of pain
in a dermatome, or peripheral distribution of a Misinterpretation by the Central
spinal nerve root (Fig. 6-2B). One example of Nervous System Causes Referred Pain
radicular pain is medial forearm pain due to
C7–T1 herniated nucleus pulposus with While direct activation of the nociceptive
impingement of the C8 nerve root. Radicular pathway causes local pain, referred pain results

A B
FIGURE 6-2 Possible causes of medial elbow pain (red ellipse) include (A) local pain secondary to medial
epicondylosis; (B) radicular pain secondary to C7–T1 herniated nucleus pulposus (arrow);
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Chapter 6 Diagnostic Considerations of Pain 49

C D

FIGURE 6-2—cont’d (C) projected pain due to cubital tunnel syndrome (arrow); and (D) referred pain
secondary to myocardial infarction (arrow).

from a misinterpretation of the origin of pain ated with heart pathology is often erroneously
signals by the central nervous system. As attributed to the jaw and shoulder.
sensory information is passed rostrally along
the neuraxis, sensory information from somatic Long-Term Pain Causes Cyclic
and visceral structures converges. Convergence Anatomical, Physiological, and
of afferent inputs in spinal and supraspinal Cognitive-Behavioral Changes
centers, such as the thalamus and cerebral
cortex, results in the loss of region specificity Long-term pain is a prevalent and disabling
in processing of pain signals. Convergent chronic health issue. Chronic pain is defined
processing of several body regions leads to the as persisting beyond 3 months, which has
perception of poorly localized pain that exists been established as a reasonable average tissue
beyond the area of tissue injury. In addition, healing time. In addition, chronic pain has
excitability of the dorsal horn neurons leads to no biological value because it provides no
an expansion of their receptive fields, also useful sensory-discrimination information.1
leading to an overlap in processing of nocicep- Chronic pain is a negative result of learning,
tive inputs from various somatic and visceral characterized by structure-function mal-
structures.34,35 Parallel processing of informa- adaptations throughout the nervous system
tion carried by a segmental innervation shared due to psychosocial pain behavior reinforcement
by somatic and visceral structures also causes and psychological predisposition. Although
referral from a visceral structure to a somatic chronic pain is an accepted term in the neuro-
structure. For example, sensory innervation of physiology literature, persistent pain may be
the heart, shoulder, and jaw overlap.33 Because a more acceptable term for use in the clinic
experience with shoulder and jaw pain from because of the negative connotation of chronic
local pathology is more common than pain pain. Persistent pain has also been adopted by
originating from heart pathology, pain associ- some authors. In general, authors may also
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50 Chapter 6 Diagnostic Considerations of Pain

differentiate between chronic pain due to neurotransmitter, neuropeptide, or other


malignant disease and chronic pain not caused molecule in the presence of a sufficient
by malignant disease. membrane voltage. These are also commonly
located on the soma. A crucial ligand- and
Prolonged Activation of Nociceptive voltage-gated ion channel to long-term
Pathways Causes Maladaptations potentiation is the NMDA receptor. NMDA
Throughout the Nervous System receptors allow the influx of calcium ions
across the neuronal membrane. NMDA
Nociceptive pathways constantly adapt to opti- receptors are responsive to binding of the
mally transmit and respond to information neurotransmitter glutamate (the ligand
about noxious stimuli. Prolonged activation of gate), although the ion channel associated
nociceptive pathways results in a whole host of with it has a “cap” of magnesium ions that
structural and functional maladaptations at must be removed by a sufficiently high
various levels of the central nervous system. membrane potential (the voltage gate) to
These maladaptations may lead to the percep- allow the influx of calcium ions.
tion of chronic pain.
Receptors located on neuronal cell mem- Long-term potentiation in nociceptive wide
branes provide the cellular basis for learning dynamic range neurons depends on NMDA
and memory at all levels of the central nervous receptor activity.40 Substance P41 and calcitonin
system, called long-term potentiation. Certain gene-related peptide bind to ligand-gated ion
neuronal membrane receptors, called ion chan- channels on wide dynamic range nociceptive
nels, respond to binding of specific substances neurons, causing a change in membrane poten-
by permitting an influx of ions from the tial that removes the “cap” of magnesium ions
extracellular space.36 This causes a change in from the NMDA receptor. If glutamate also
voltage across the neuronal cell membrane.37 binds to the NMDA receptor at this time, the
If enough ion channels respond simultane- calcium ion channel associated with the NMDA
ously to depolarize the cell membrane at the receptor opens. The resulting influx of calcium
soma, a critical voltage threshold is reached ions initiates a cascade of intracellular processes
that allows generation of an action potential.38 that causes structural changes to the wide
In the dorsal horn of the spinal cord, wide dynamic range neuron to facilitate its transmis-
dynamic range neurons accepting input from sion of pain signals. Long-term potentiation of
nociceptive axons have three classes of mem- nociceptive pathways is also referred to as
brane receptors39: central sensitization or wind-up.
Neuroanatomical changes eventually result
● Ligand-gated ion channels that open in from maladaptive learning by the central nerv-
response to binding of a neurotransmitter, ous system. Hypothesized changes in the spinal
neuropeptide, or other molecules. These are dorsal root ganglion include disinhibition of
common at the soma, providing for the spinal cord neurons,42 axonal sprouting by local
small changes in membrane voltage that spinal interneurons outside their normal distri-
may culminate in an action potential. Ligand- bution,16,43 and formation of noradrenergic
gated ion channels on wide dynamic range axons where they did not previously exist.44
nociceptive neurons are responsive to sub- Neuroanatomical changes are also present in
stance P, calcitonin gene-related peptide, supraspinal structures. Changes in cerebral
and other molecules. cortex activation also contribute to persistent
● Voltage-gated ion channels that open in pain, paralleling cognitive and behavior changes.
response to changes in voltage across the cell People with persistent lower back pain demon-
membrane. These are common along the strate a larger representation of the lower back in
axon, allowing for propagation of an action the primary sensory cortex than healthy people.17
potential to the axon terminal.38 In addition, experimental induction of prolonged
● Ligand- and voltage-gated ion channels cutaneous and muscle pain reduces primary
that open in response to binding of a motor cortex excitability.18–21
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Chapter 6 Diagnostic Considerations of Pain 51

The Sympathetic Nervous System coupled outside the spinal cord, where noci-
Can Contribute to the Perception ceptors become abnormally sensitized to
of Pain sympathetic autonomic signaling. Coupling of
nociceptive and sympathetic autonomic nerv-
Abundant research and clinical evidence sug- ous systems may be direct or indirect.45 Direct
gest that the sympathetic autonomic nervous coupling occurs after complete or partial nerve
system can contribute to chronic pain disorders injury, when nociceptors become sensitized
involving neuropathic pain. Complex regional to autonomic signal molecules called cate-
pain syndrome (CRPS) refers to a group of cholamines. Catecholamines may also cause
chronic neuropathic pain disorders. CRPS is indirect coupling by triggering the formation
divided into two types based on etiology22: of prostaglandins during tissue inflammation
● CRPS I refers to chronic neuropathic that, in turn, sensitize nociceptors.
pain without identifiable nerve trauma
(formerly known as reflex sympathetic Psychological and Social Factors
dystrophy). Perpetuate Pain Through Behavior
● CRPS II refers to chronic neuropathic pain Reinforcement
that corresponds with previous nerve Continual activation of nociceptive pathways
trauma (formerly known as causalgia). may cause cyclic disorders in thoughts, beliefs,
emotions, and consequent interaction with the
CRPS I and II share a common pathologi- environment. Fear and avoidance of pain after
cal progression, which has been divided into tissue injury initially serve an adaptive role by
three hypothetical stages23: allowing damaged tissues to heal. In some
● Acute/warm phase—The first phase of CRPS people, initial pain avoidance behavior causes
occurs immediately after the precipitating a maladaptive anxiety involving movements
event, if one is present. The affected limb is and activities that may cause pain. Increased
warm and swollen. There may be increased fear and anxiety regarding pain result in the
hair and nail growth, spontaneous burning formation of fear avoidance beliefs. Correspond-
pain, and pain with normal non-noxious ingly, fear avoidance beliefs cause initially
sensory stimulation (allodynia). adaptive pain avoidance behaviors—intended
● Instability/dystrophic phase—This second to avoid pain—to become maladaptive fear
phase of CRPS typically begins 3 months avoidance behaviors—intended to avoid anxi-
after the precipitating event, and is charac- ety related to movements and activities that
terized by pain in a more extended area, cause pain. Fear avoidance beliefs and behav-
limitation of movement, joint stiffness. iors are maintained and amplified through
Osteoporosis and muscle wasting are typically social and environmental reinforcements, such
evident in the second stage of CRPS. as rest, medication, attention, or compensation.2
Eventually, fear avoidance beliefs and behav-
● Atrophic phase—This third phase of CRPS
iors may be more disabling than pain severity
generally presents 6 months after the initia-
itself.46 Fear avoidance beliefs and behaviors
tion of symptoms. In this stage, pain may
may become magnified by a spiraling cycle
actually decrease, although the skin over the
of decline, in which progressive disuse and
affected area becomes cool, cyanotic,
deconditioning contribute to additional fear
smooth, and glossy.
avoidance.
Surgical ablation of the sympathetic chain Cognitive factors that influence the percep-
has long been known to partially or completely tion of acute pain severity also play important
resolve CRPS in some patients, even though roles in the perception of chronic pain. Cata-
the sympathetic autonomic nervous system strophizing is associated with limited confi-
does not directly participate in nociception. dence in one’s own ability to cope with symp-
This suggests that the nociceptive afferent toms, leading to the perception of pain as
and autonomic nervous systems may become inescapable and unpredictable. This perception
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52 Chapter 6 Diagnostic Considerations of Pain

of pain reinforces fear avoidance beliefs and 9. Martin J. Neuroanatomy: Text and Atlas. 3rd ed. New
contributes to learned helplessness. Learned York, NY: McGraw-Hill; 2003.
10. Melzack R, Wall P. Pain mechanisms: a new theory.
helplessness relates to an individual’s belief Science. 1965;150(699):971–979.
that negative outcomes due to pain are in- 11. Bandler R, Shipley MT. Columnar organization in the
evitable.15,47 Helplessness interferes with learning midbrain periaqueductal gray: modules for emotional
of new coping strategies. Learned helplessness expression? Trends Neurosci. 1994;17(9):379–389.
12. McNeil D, Au A, Zvolensky M, McKee D, Klineberg
eventually leads to hopelessness, when all efforts I, Ho C. Fear of pain in orofacial pain patients. Pain.
to improve pain are perceived as futile.15 Help- 2001;89(2–3):245–252.
lessness and hopelessness result in cessation of 13. Sullivan M, Thorn B, Haythornthwaite J, et al. Theoret-
an individual’s efforts to self-manage symp- ical perspectives on the relation between catastrophiz-
ing and pain. Clin J Pain. 2001;17(1):52–64.
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psychological antecedents to pain experience: experi-
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16. Bandura A. Self-efficacy: toward a unifying theory of
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set of stimulus-specific anatomical structures mechanisms governing the motivational aspects of goal
systems. J Pers Soc Psychol. 1983;45:1017–1028.
and physiological processes are present in 19. Bandura A, Cervone D. Differential engagement of
almost all people, and a growing body of clin- self-reactive influences in cognitive motivation. Organ
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are substantial differences in how patients 20. Le Pera D, Graven-Nielsen T, Valeriani M, et al. Inhibi-
tion of motor system excitability at cortical and
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the diagnosis of pain’s underlying causation 2001;112(9):1633–1641.
requires a thorough understanding of pain’s 21. Valeriani M, Restuccia D, Di Lazzaro V, et al. Inhibi-
anatomy, physiology, and psychology. tion of biceps brachii muscle motor area by painful
heat stimulation of the skin. Exp Brain Res.
2001;139(2):168–172.
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CHAPTER7
Headaches
n Michael O’Donnell, PT, DPT, OCS, FAAOMPT

Description of the Symptom n Neurological symptoms, such as dizziness,


paresthesias, or weakness
This chapter describes pathology that may lead n New onset after age 50
to headaches. Local causes are defined as n New onset of headache in patient with risk
pathology occurring within the cranial factors for cancer or HIV infection
vault, as well as the temporomandibular joint n Progressing intensity or frequency
(Fig. 7-1). Remote causes are defined as occur- n Signs of illness, such as fever, nuchal rigid-
ring outside this region. Each symptom de- ity, or rash
scription in this chapter is followed by a brief n Signs of papilledema
summary of clinical presentation. n Sudden onset of headache
n Worsening with exertion or Valsalva
Special Concerns maneuver
Headache with:
n Awakening from sleep
n History of head trauma inside of 2 weeks

A Anterior lateral view B Posterior lateral view


FIGURE 7-1 Local causes of headache include cranium, contents of the cranial vault, articular and periartic-
ular structures of the temporomandibular joint, and nasal sinuses. (A) Anterior lateral view; (B) posterior
lateral view.

54
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Chapter 7 Headaches 55

CHAPTER PREVIEW: Conditions That May Lead to Headache

HEADACHES
T Trauma
REMOTE LOCAL
COMMON
Cervicogenic headache 57 Post-traumatic headache 67
UNCOMMON
Not applicable Temporomandibular dysfunction 71
RARE
Not applicable Not applicable

I Inflammation
REMOTE LOCAL
COMMON
Not applicable Aseptic
Not applicable
Septic
Fever-induced headache 63
Meningitis 66
Sinusitis 68
UNCOMMON
Not applicable Aseptic
Encephalitis 62
Temporal arteritis 70
Septic
Not applicable
RARE
Not applicable Not applicable

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Hypoglycemia 63
Medication overuse headache 65
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Benign exertional headache 60
Benign sexual headache 61
Hypoxia 64
(continued)
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56 Chapter 7 Headaches
HEADACHES

Va Vascular
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Arterial dissection (carotid and vertebral) 60
Carotid artery dissection 60
Idiopathic intracranial hypertension
(pseudotumor cerebri) 64
Idiopathic intracranial hypotension 65
Stroke or transient ischemic attack 68
Subdural hematoma 69
RARE
Not applicable Arterial dissection (carotid and vertebral) 60
Subarachnoid hemorrhage 69
Vertebral artery dissection 60

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Not applicable

UNCOMMON
Not applicable Not applicable

RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Tumors (brain) 72
Tumors leading to headaches:
• Acoustic neuroma 72
• Central nervous system 72
• Leptomeningeal metastasis 72
• Meningeal tumors 72
• Primary brain tumors 72
• Skull base tumors 72
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Chapter 7 Cervicogenic Headache 57

HEADACHES
Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Migraine 66
Tension-type headache 71
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Cluster headaches 61

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Headache Description of Conditions That


May Lead to Headache
The International Headache Society (IHS) rec-
ognizes 14 broad categories and 300 different Remote
types of headaches.1,2 Headaches are divided
among primary and secondary headaches. Pri- n Cervicogenic Headache
mary headaches are unrelated to a specific Chief Clinical Characteristics
causative pathology (eg, migraine), whereas This presentation typically is characterized by
secondary headaches occur due to an underly- unilateral head pain associated with neck move-
ing pathological process (eg, brain tumor). ment; sustained or awkward cervical posture;
Each headache is characterized by a hallmark restricted cervical range of motion; and
frequency, duration, and time course, which ipsilateral neck, shoulder, or arm pain.4 This con-
may be helpful to distinguish among the dif- dition is characterized by moderate to severe
ferent types of headaches (Table 7-1). While it episodic pain that originates in the neck or
is true that headaches could be the harbinger suboccipital region and spreads to the head.
of grave pathological conditions, only 2% of Pain attacks last from 3 weeks to 3 months, and
all headache complaints are caused by serious may vary in frequency from occurring every 2
disease or pathological conditions.3 Most days to 2 months. People with cervicogenic
pathology that necessitates a patient’s or headache describe symptoms that do not change
client’s referral to a physician for consultation sides during an attack,5 and may also present
can be ruled less likely with a history and a with nausea, dizziness, and phonophobia or
clinical examination, whereas tests such as photophobia that is unresolved with migraine
neuroimaging and electroencephalograms medications.
rarely contribute to the diagnosis of headache.
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58 Chapter 7 Cervicogenic Headache


HEADACHES

TABLE 7-1 n Diagnosis of Headache Based on Features of the Symptom Timeline


RELATIVE DURATION OF SYMPTOMS
Hours Days Weeks Months Constant
Frequency of Symptoms
Hourly
• Cluster
headaches
Daily
• Chronic • Cervicogenic • Acute post- • Cervicogenic • Medication
migraine headache traumatic headache overuse
• Cluster • Chronic headache • Chronic post- headache
headaches migraine • Cervicogenic traumatic
• Hypoxia • Hypoxia headache headache
• Idiopathic • Hypoxia • Idiopathic
intracranial • Idiopathic intracranial
hypotension intracranial hypotension
(lumbar hypotension (fistula)
puncture) (lumbar
puncture)
• Idiopathic
intracranial
hypotension
(fistula)
Weekly
• Chronic • Cervicogenic • Cervicogenic • Cervicogenic
migraine headache headache headache
• Cluster • Chronic • Migraine
headaches migraine
Monthly
• Chronic • Chronic
migraine migraine
Yearly
• Chronic • Chronic
migraine migraine
Constant
• Temporal
arteritis
Variable
• Benign • Arterial • Arterial • Arterial • Fever-
exertional dissection dissection dissection induced
headache (carotid) (carotid) (vertebral) headache
• Benign sexual • Benign • Arterial • Encephalitis
headache exertional dissection • Intracranial
• Hypoglycemia headache (vertebral) hypotension
• Subarachnoid • Benign sexual • Benign sexual (fistula)
hemorrhage headache headache • Meningitis
• Tension-type • Meningitis • Encephalitis • Sinusitis
headache • Sinusitis • Intracranial • Stroke or
• Stroke or hypotension transient
transient (lumbar ischemic
ischemic attack puncture) attack
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Chapter 7 Cervicogenic Headache 59

HEADACHES
TABLE 7-1 n Diagnosis of Headache Based on Features of the Symptom Timeline—cont’d
RELATIVE DURATION OF SYMPTOMS
Hours Days Weeks Months Constant

Variable
• Subarachnoid • Intracranial • Subdural
hemorrhage hypotension hematoma
• Subdural (fistula) • Temporo-
hematoma • Meningitis mandibular
• Temporo- • Sinusitis dysfunction
mandibular • Stroke or • Tension-type
dysfunction transient headache
• Tension-type ischemic attack • Tumor
headache • Subarachnoid
hemorrhage
• Subdural
hematoma
• Temporo-
mandibular
dysfunction
• Tension-type
headache

Background Information such as painful restricted


Initial management includes amelioration of cervical mobility, crepita-
cervical spine musculoskeletal impairments. tions6 with movement, uni-
Nerve blockade may be effective in recalcitrant lateral feeling of tension,
cases. A favorable response to this intervention pain aggravated by head
also is pathognomic.6 Typical management of movements, pressure on
cervicogenic headache involves rehabilitative ipsilateral trigger points.
interventions. Onset Acute (trauma) or pro-
Clinical Features of Cervicogenic longed insidious onsets
Headache (degenerative joint disease
of upper cervical vertebrae).
Pain Character Dull, nonthrobbing, and
Duration 3 weeks to 3 months.
nonlancinating. Intensity
Frequency Every 2 days to every 2
can be moderate to severe.
months.
Location Side locked unilaterally. Lo-
Time Course May be present when awak-
calized to upper cervical or
ening and worsen as day
occipital region. Pain may ra-
goes on; activity dependent.
diate to frontal or orbital re-
Associated Phonophobia, dizziness,
gion, vertex, temples, or ears.
Symptoms/ ipsilateral eyelid edema,
Precipitants Trauma, sustained neck
Signs ipsilateral blurred vision.
postures, mechanical faults
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60 Chapter 7 Arterial Dissection (Carotid and Vertebral)

Local Vertebral artery—bilateral


HEADACHES

occipital, unilateral
n Arterial Dissection (Carotid frontal.
and Vertebral) Precipitants Sustained exertion, trauma,
Chief Clinical Characteristics or cervical manipulation.
This presentation often involves steady pain in a Onset 85% of cases of headache
pattern that loosely follows the distribution of are characterized by grad-
the affected artery, associated with Horner’s ual onset. On occasion,
syndrome, ipsilateral tongue weakness, diplopia, onset can be sudden with
syncope, tinnitus, amaurosis fugax, or dysgeusia. “thunderclap” qualities.
Background Information Duration Carotid artery-related
Dissections are typically seen in people ages 30 headaches resolve within
to 39 as compared to cerebral ischemia. The 1 week. Vertebral artery-
most common symptoms of internal carotid related headaches may last
artery dissection (ICAD) are headache and/or 5 weeks.
cervical pain.7 Zetterling reports the character- Frequency Related to the precipitating
istic pain to have a sudden onset,7 whereas incident.
Biousse reports that 85% of patients with Time Course Headache may appear
ICAD have a gradual onset of pain.8 The 1 hour to 90 days prior to
headache pain is severe and is located in the dissection.
ipsilateral periorbital, periauricular, or upper Associated Horner’s syndrome, hy-
cervical region. Dodick states that components Symptoms/ poglossal nerve palsy
of the clinical presentation greatly assist in Signs (ipsilateral tongue weak-
making the diagnosis. He states that the onset ness), diplopia, syncope,
of pain is frequently temporally related to cer- tinnitus, amaurosis fugax,
vical manipulation, sustained exertion, or dysgeusia.
trauma. Horner’s syndrome accompanies the n Benign Exertional Headache
headache and there may be ipsilateral tongue
weakness.3 Vertebral artery dissection is less Chief Clinical Characteristics
common than ICAD and may go unrecog- This presentation involves head pain that is
nized until an ischemic event occurs.3 Pain is associated with exertion or a Valsalva-type
localized to the occiput or posterior neck bilat- maneuver.
erally or the frontal region unilaterally. Verte- Background Information
bral artery dissection (VAD) can produce When the headache occurs with a sudden on-
brainstem or cerebellar ischemia. This can set, it is thought to be due to acute venous dis-
result in Wallenberg’s syndrome. A young person tention.9 In cases with a more gradual onset, it
with occipital or posterior neck pain presenting has been shown to be due to arterial spasm.10
with Wallenberg’s syndrome is highly likely to Despite having a vascular nature, Sjaastad and
have a VAD.8 Individuals suspected of this condi- others have demonstrated there is no convinc-
tion require emergent medical attention. ing association between this condition and
Clinical Features of Headache migraines.4,10 Sentinel headache associated
Secondary to Arterial Dissection with subarachnoid hemorrhage or subdural
Pain Character Severity ranges from mild to hematoma must be ruled less likely in individ-
excruciating. uals suspected of benign exertional headache.
Carotid artery—constant, A combination of avoidance or modification
steady ache. of aggravating activities and nonsteroidal anti-
Vertebral artery—steady inflammatory or beta-blocker medications is
pressure or throbbing. typically considered in order to manage exer-
Location Carotid artery—neck pain tional headaches.
or headache that radiates
to eye, ear, or face.
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Chapter 7 Cluster Headaches 61

Clinical Features of Benign Exertional Duration May last up to 48 hours fol-

HEADACHES
Headache lowing sexual activity.
Pain Character Throbbing or pulsatile. Frequency Related to precipitating
Location Bilateral. activity.
Precipitants Physical exertion, particu- Time Course Preorgasmic type—occurs
larly in hot weather, with sexual activity and
high humidity, or high intensifies with sexual
altitudes. excitement.
Onset Rapid. Orgasmic type—very sud-
Duration 5 minutes to 24 hours. den onset occurs with
Frequency Related to precipitating orgasm.
activity. Associated Neck and jaw pain.
Time Course Begins quickly following Symptoms/
activity. Signs
Associated May be characterized by
n Cluster Headaches
Symptoms/ migraine-like symptoms
Signs in those who are suscep- Chief Clinical Characteristics
tible to migraines. This presentation can include an excruciating
unilateral headache in the trigeminal distri-
n Benign Sexual Headache bution, lasting a short period of time but occur-
ring relatively frequently, and associated with
Chief Clinical Characteristics
ipsilateral facial autonomic signs (Fig. 7-2A).
This presentation may include variable head and
This condition generally comes in bouts of sev-
facial symptoms related to sexual activity. Three
eral headaches of short duration that occur sev-
different subtypes of headache related to sexual
eral times a day over a period of several weeks.
activity have been identified. The first, regarded
It is rare and occurs more commonly in males
as the dull type, comes on as the sexual excite-
than females.11,12 The pain associated with clus-
ment increases.
ter headaches is very severe and is often de-
Background Information scribed as “boring, stabbing, or a hot poker in
Sentinel headache associated with subarach- the eye.”13 This condition is localized to the or-
noid hemorrhage or subdural hematoma bital and temporal region. Attacks of pain are
must be ruled less likely in individuals sus- accompanied by one or more autonomic features
pected of benign sexual headache. A ipsilateral to the pain. These signs include pto-
combination of avoidance or modification of sis, miosis, lacrimation, nasal congestion, and
aggravating activities and nonsteroidal anti- rhinorrhea or conjunctiva injection. Each attack
inflammatory or beta-blocker medications is may last anywhere from 15 minutes to 3 hours
typically considered in order to manage exer- and the attacks come in clusters of one per day
tional headaches. to as many as eight per day. Cluster periods can
Clinical Features of Benign Sexual last from 7 days to 1 year. In the episodic state
Headache there are periods of remission that last from 6
to 12 months.
Pain Character Dull, throbbing, or explosive.
Location Bilateral occipital, temporal, Background Information
or facial. This condition is triggered by alcohol con-
Precipitants Sexual activity or posturally sumption, although it does not appear to pre-
related. cipitate headaches during periods of remis-
Onset Depending on type, may be sion.12 Other vasodilators have also been
gradual onset, which in- shown to induce attacks, such as nitroglycerin
creases as sexual excitement tablets and histamine.14 Altitude hypoxemia
increases, or may have sud- and sleep apnea–induced hypoxemia also
den onset in the case of have been documented to induce cluster
orgasmic headaches. headaches during cluster periods.15 A seasonal
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62 Chapter 7 Encephalitis
HEADACHES

A Cluster B Migraine

C Sinus D Tension
FIGURE 7-2 Locations for common types of headaches: (A) cluster; (B) migraine; (C) sinus; (D) tension.

preponderance of cluster attacks occurs in the Frequency Range from one per week to
spring or fall. There is also a clock-like regular- eight or more per day.
ity with the onset of individual attacks.12 The Time Course One to 4 months separated
pathophysiology of cluster headaches remains by remission that can last
unclear, but the trigeminovascular system, hy- 6 to 24 months. Chronic
pothalamus, and autonomic nervous system cluster headaches con-
appear to be important components. Lifestyle tinue for 1 year without
modification to reduce risk factors, abortive remission.
medication, and prophylactic medication may Associated Parasympathetic overactiv-
be considered in the management of cluster Symptoms/ ity causes lacrimation,
headaches. Signs nasal stuffiness, runny
Clinical Features of Cluster Headaches nose, forehead and facial
sweating. Partial sympa-
Pain Character Excruciating intensity, bor-
thetic paralysis causes
ing or tearing, described
ptosis, miosis (Horner’s
as “hot poker” or “eye
syndrome).
being pushed out.”
Location Almost always unilateral in n Encephalitis
a trigeminal distribution,
typically orbital, retro- Chief Clinical Characteristics
orbital, temporal, supra- This presentation often involves an abrupt on-
orbital, and infraorbital. set of severe bifrontal headache associated with
Precipitants Smoking, ethanol consump- fever, confusion, altered level of consciousness,
tion, other vasodilators focal neurological deficits, and seizures.
such as nitroglycerin Background Information
tablets, histamine, altitude Several viral sources can lead to encephalitis
hypoxemia, sleep apnea. (eg, arbovirus, mumps, herpes simplex and
Onset Rapid onset reaching its herpes zoster, Epstein-Barr, measles, and
peak in 10 to 15 minutes. chickenpox), but the specific virus often can-
Duration 15 minutes to 3 hours. not be identified.16 Sudden and severe
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Chapter 7 Hypoglycemia 63

headaches may occur early and may be the appear to be related to more chronic presenta-

HEADACHES
only manifestation of encephalitis.3 Magnetic tions in which headache occurs after fever
resonance imaging is the imaging procedure of subsides. In these cases the headache may be
choice to confirm the diagnosis.3 Individuals caused by meningeal involvement. Manage-
suspected of this condition require emergent ment of fever-induced headaches involves
medical attention. treatment of the underlying bacterial infection
Clinical Features of Headache or supportive intervention for viral causes.
Secondary to Encephalitis Clinical Features of Fever-Induced
Pain Character Abrupt and severe. Headache
Location Bifrontal. Pain Character Variable intensity.
Precipitants Viral or bacterial infection, Location Bilateral and diffuse; occipi-
such as herpes simplex, tal, frontal, or retro-orbital.
Coxiella burnetii, and Precipitants Viral or bacterial infection.
Listeria monocytogenes. Onset May occur concurrently
Onset Rapid. with or following onset of
Duration Several days to 4 to 5 months fever.
in some chronic forms. Duration Related to acute phase of
Frequency Incidental to underlying illness. Resolves in less than
disease. 1 month after successful
Time Course Headache occurs with en- treatment or spontaneous
cephalitis. Resolves within remission of infection.
3 months after infection is Frequency Occurs with frequency of
treated effectively with an- underlying disease.
tibiotics or spontaneously Time Course Variable.
ends. Associated Fever, nausea, vomiting,
Associated Fever, confusion, altered Symptoms/ malaise, chills, photopho-
Symptoms/ level of consciousness, Signs bia, skin rash, myalgia.
Signs focal neurological deficits,
seizures. n Hypoglycemia
Chief Clinical Characteristics
n Fever-Induced Headache This presentation includes a pressing or
Chief Clinical Characteristics pounding bifrontal headache occurring within
This presentation may be characterized by a 16 hours after fasting or 30 minutes of ingest-
bilaterally distributed diffuse headache that is ing food. Headaches due to hypoglycemia are
associated with fever, nausea, vomiting, more common in individuals with diabetes
malaise, chills, photophobia, skin rash, and mellitus and those who skip meals. Headaches
myalgias. due to this condition also may be associated
with nausea, light-headedness, confusion, and
Background Information
lethargy.
Noncephalic causes may be viral in nature
such as influenza or adenovirus. Bacterial Background Information
causes include Rocky Mountain spotted fever Headaches associated with fasting may result
and Rickettsia. The cause of headache in these from metabolic changes such as hypoglycemia
acute cases is unclear but may be related to the or the accumulation of certain metabolites.17
interaction of inflammatory mechanisms with In those individuals who suffer from more
pain transmission. Some infective agents have chronic types of headache such as migraine or
a predilection for the central nervous system. tension-type headaches, hypoglycemia may
They may invade the brainstem nuclei where be a precipitating factor.18 A small percentage
the release of toxins activates headache mech- of individuals have the onset of headache
anisms. In more chronic situations, headache within 30 minutes of breaking their fast.
may be caused by delayed septicemia. Viral ill- This could be due to postprandial hypo-
nesses such as herpes simplex or Epstein-Barr glycemia. Management of headaches secondary
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64 Chapter 7 Hypoxia

to hypoglycemia includes lifestyle and suspected of headaches due to hypoxia should


HEADACHES

dietary modifications. Individuals with be referred for evaluation.


diabetes mellitus who are suspected of having Clinical Features of Headache
hypoglycemia may require consultation regard- Secondary to Hypoxia
ing their current regimen of insulin therapy.
Pain Character Pounding.
Clinical Features of Headache Location Frontal; 25% are unilateral.
Secondary to Hypoglycemia Precipitants Pulmonary disease and other
Pain Character Pressing or pounding. disorders of oxygen deliv-
Location Bitemporal or bifrontal. ery (eg, anemia, carbon
Precipitants Diabetes mellitus, fasting, or monoxide intoxication),
skipping meals. sudden ascent to high alti-
Onset Within 16 hours after fasting. tude (10,000 ft), or exer-
Duration 15 to 30 minutes following tion at high altitude.
the ingestion of food. Onset Within 24 hours of hypoxic
Frequency Incidental to individual’s incident.
diet and activity level. Duration Up to 48 hours.
Time Course Resolves within 15 to Frequency Incidental to individual’s
30 minutes of nutrition diet and activity level.
intake. Time Course Occurs within 24 hours of
Associated Nausea, light-headedness, being exposed to hypoxic
Symptoms/ confusion, lethargy. state and resolves within
Signs 48 hours of returning
to normal atmospheric
n Hypoxia conditions.
Chief Clinical Characteristics Associated Nausea, vomiting, vertigo,
This presentation involves a pounding frontal Symptoms/ palpitations, impaired
headache within 24 hours of ascent to a high Signs vision.
altitude, which may be associated with nausea,
vomiting, vertigo, palpitations, and impaired n Idiopathic Intracranial
vision. Hypertension (Pseudotumor
Cerebri)
Background Information
Chief Clinical Characteristics
Situations that can produce hypoxia may be en-
This presentation can include a gradual onset
vironmental with reduced ambient oxygen, such
of throbbing, pressing headache that involves the
as high altitude or normal altitude with low O2
entire cranium.
conditions. This condition also may occur in
disease conditions such as pulmonary disease or Background Information
disorders of oxygen delivery such as anemia, car- This condition exists when an increase in
diac failure, or carbon monoxide poisoning. The intracranial pressure occurs that is caused by
etiology of headaches associated with this con- hydrocephalus or intracranial mass. Along with
dition is unclear, although the increase in cere- headaches the condition is characterized by
bral blood flow associated with hypoxia may be papilledema, but there are no abnormalities in
the underlying cause. The increase in cerebral the composition of the cerebrospinal fluid and
blood flow is caused by vasodilation, which can there are no localizing neurological signs. The
produce pain by stretching of the vessel exciting exact cause is unknown but several hypotheses
trigeminal sensory afferents, which in turn have been suggested. The most commonly
innervate the large pain-producing cranial accepted hypotheses are an increase in the
vessels.18 Headaches secondary to hypoxia are brain water content, increased resistance to
managed with supplemental oxygen. In particu- cerebrospinal fluid outflow, or a hypothalamic-
lar, individuals with cardiopulmonary disease pituitary disturbance. Management of this
or other disorders of oxygen transport who are health condition may range from medications
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Chapter 7 Medication Overuse Headaches 65

to lower intracranial pressure to emergency patching, percutaneous fibrin sealant place-

HEADACHES
surgical intervention in patients with deterio- ment, and surgical leak repair.20
rating visual field impairments.19 Clinical Features of Headache
Clinical Features of Headache Secondary to Idiopathic Intracranial
Secondary to Idiopathic Intracranial Hypotension
Hypertension (Pseudotumor Cerebri) Pain Character Aching, dull, deep, con-
Pain Character Throbbing or pressing. stricting, or throbbing.
Location Can be the entire cranium Location Bilateral, frontal, occipital,
or may be unilateral. or generalized.
Precipitants Obstruction of the cere- Precipitants Lumbar puncture or devel-
brospinal fluid flow at any opment of fistula in which
of several sites. upright position produces
Onset Acute or gradual. headache rapidly.
Duration Typically 1 week (in the case of Onset Rapid—within 20 seconds of
lumbar puncture), or may assuming upright posture.
be several weeks or months Duration Typically 1 week (in the case of
(in the case of fistula). lumbar puncture), or may
Frequency Daily. be several weeks or months
Time Course Prolonged. The condition is (in the case of fistula).
not always self-limiting. Frequency Incidental.
Associated Papilledema, visual field de- Time Course Initially headache occurs
Symptoms/ fects, loss of visual acuity, rapidly when assuming
Signs nausea, and to a lesser upright position. In the
degree vomiting. Occa- case of lumbar puncture
sionally hearing difficul- headache comes on more
ties and tinnitus. gradually over the course
of 1 week.
n Idiopathic Intracranial Associated Dizziness, nausea, vomiting.
Hypotension Symptoms/ Occasionally hearing diffi-
Chief Clinical Characteristics Signs culties and tinnitus.
This presentation typically involves a deep,
aching headache that occurs rapidly after assum- n Medication Overuse Headache
ing an upright posture. This condition most Chief Clinical Characteristics
commonly is caused by a lumbar puncture. In This presentation may involve progressively
the case of post–lumbar puncture headache, worsening daily headaches present greater than
onset is within 7 days and resolves within 14 days. 15 days per month in the presence of regular over-
Headaches are worse when standing and use of a medication for greater than 3 months.21
decrease when lying down. Those that do not Headaches due to medication overuse resolve or
resolve within 14 days may be due to a persist- revert to their previous pattern within 2 months
ent fistula. of ceasing the overused medication. Headache
intensity may be widely variable from day to day.
Background Information
In either case the underlying problem is a leak Background Information
of cerebrospinal fluid. Major trauma, neuro- Inappropriate use of headache medications—
surgery, or erosive lesions also may cause such as ergotamine, triptans, opioids, and sim-
cerebrospinal fluid leaks. With a decrease of ple or combination analgesics—may con-
cerebrospinal fluid, there is a decrease in pres- tribute to the development of chronic daily
sure and volume that causes cerebral veins to headaches.22 This condition also is known as
dilate. The brain then loses its cerebrospinal analgesic rebound headache, chronic daily
fluid cushion. Management may include op- headache, chronic migraine, medication-
tions ranging from bed rest, epidural blood induced migraine, or transformed migraine.
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66 Chapter 7 Meningitis

In addition, cardiovascular drugs (eg, calcium into the upper thoracic region and even the
HEADACHES

channel blockers, antiarrhythmics, phospho- extremities.16


diesterase inhibitors, alpha-1 agonists and
antagonists, beta-adrenergic antagonists, an- Background Information
giotensin converting enzyme inhibitors, an- The headache associated with this condition
giotensin II receptor antagonists, and organic may be caused by meningeal irritation with di-
nitrates), anti-infective agents (eg, various an- rect stimulation of the sensory terminal in the
tibacterial, antifungal, and antiretroviral meninges.16 It may also be due to increased in-
drugs), immunomodulatory drugs, anti- tracranial pressure caused by an accumulation
inflammatory drugs, antihistamines, medica- of exudates, which obstructs the circulation of
tions used to treat asthma, and tricyclic antide- cerebrospinal fluid.3 In addition, this headache
pressants are most commonly implicated may be caused by a distant infection.26 To
in promoting headaches.23–25 A retrospective properly identify the infective organism for the
diagnosis is made when the offending agent is most appropriate treatment, a lumbar punc-
withdrawn and the headache pattern ceases to ture must be performed.16 Identification of the
be daily. Although discontinued use of these infective organism guides management of this
medications can relieve the undesirable symp- health condition.
toms, discontinuation should occur with Clinical Features of Headache
appropriate medical consultation including Secondary to Meningitis
appropriate substitution as needed.
Pain Character Moderate or severe.
Clinical Features of Headache Location Frontal or generalized.
Secondary to Medication Overuse Precipitants Viral or bacterial infection.
Pain Character Dull. Onset Rapid.
Location Variable. Duration Several days to 3 months.
Precipitants Ingestion of antipyretic or Frequency Incidental to underlying
anti-inflammatory anal- disease.
gesics, triptans, ergota- Time Course Typically has a relatively rapid
mine, or opioids. onset. Resolves within 3
Onset Weeks or months after initi- months after infection is
ating use of offending treated effectively with
medications. antibiotics or sponta-
Duration Constant. neously ends.
Frequency Daily. Associated Fever, cough, skin rash,
Time Course Typically begins after a Symptoms/ lethargy, altered level of
3-month or more period Signs consciousness, and me-
of taking offending med- ningism as demonstrated
ications. Resolves within a by nuchal rigidity and
few days after discontinu- photophobia.
ing the medication.
n Migraine
Associated Typically absent of migraine-
Symptoms/ like symptoms. Chief Clinical Characteristics
Signs This presentation often includes a severe pul-
sating and throbbing unilateral headache that
n Meningitis may switch sides, associated with nausea, vom-
Chief Clinical Characteristics iting, diarrhea, abdominal cramps, polyuria,
This presentation can involve a rapid onset of sweating, facial pallor, photophobia, or phono-
progressive frontal or generalized headache that phobia (see Fig. 7-2B). This condition is a
is associated with fever, cough, skin rash, chronic condition of recurring attacks of tran-
lethargy, altered level of consciousness, nuchal sient focal neurological symptoms, headaches,
rigidity, or photophobia. Headache secondary or both.27 Not only is this process responsible for
to meningitis may last several days to 3 months. producing headaches, the condition can
Symptoms may radiate to the cervical region and also interfere with the function of other body
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Chapter 7 Post-Traumatic Headache 67

systems.28 Migraines are preceded by aura in 20% Onset Males—10 to 13 years.

HEADACHES
of cases.29 Even in individuals who commonly Females—menarche.
experience them, auras may not accompany Duration 4 to 72 hours.
every headache. Auras can be visual, somatosen- Frequency At least five attacks per year,
sory, olfactory, or involve speech disturbances. but chronic migraines
The location of headache may switch sides from may occur daily.
episode to episode. Time Course Typically upon awakening
in the morning and takes
Background Information
several hours to build to
The pathophysiology of this condition is very
maximum intensity.
complex. Current theories postulate that a cas-
Associated Nausea, vomiting, diarrhea,
cade of events occurs that involves vasodilata-
Symptoms/ anorexia, abdominal
tion of meningeal blood vessels, irritation of
Signs cramps, polyuria, sweat-
perivascular sensory nerves, and stimulation of
ing, pallor of the face,
brainstem nuclei. Additionally, extrinsic factors
photophobia, or phono-
such as hormonal fluctuations, fatigue, or anxi-
phobia. Impairment of
ety may be triggers that initiate the pathophysi-
concentration, memory
ological cascade. This condition also has a
impairment, depression,
strong familial tendency and begins at a young
fatigue, anxiety, irritabil-
age, suggesting that genetic factors may predis-
ity, and light-headedness.
pose individuals to migraine attacks. The use of
neuroimaging is not indicated in individuals n Post-Traumatic Headache
with migraine symptoms and a normal neuro-
logical exam. Certain pharmaceutical agents, Chief Clinical Characteristics
such as triptans and ergots, are effective in pre- This presentation may be characterized by a
venting migraine attacks from becoming too vertex or band-like headache that develops
severe.30 Other medications can be considered within 7 days after head trauma or regaining
to prevent migraine attacks. consciousness following head trauma and
persists for 3 months or greater.21 Acute post-
Clinical Features of Migraine traumatic headaches may last 2 to 8 weeks
Pain Character Very severe and is typically after trauma, and chronic post-traumatic
described as pulsating and headaches may last up to 6 months to 4 years.
throbbing. The headache Post-traumatic headache may be associated
may start out as a dull with somatic symptoms (eg, dizziness, photopho-
steady ache and progress bia, phonophobia, tinnitus, blurring of vision,
to the more characteristic and rapid fatigue) and psychological symptoms
features. (eg, depression, anxiety, apathy, insomnia,
Location Typically unilateral (but can decreased libido, irritability, and frequent mood
switch sides). Localized to swings).
temple, forehead or eye, or
back of head. Background Information
Precipitants Stress, relaxation after stress, Rebound headache must be ruled out before
fatigue, too little or too the diagnosis of chronic post-traumatic
much sleep, skipping meals, headache is reached.31 The severity of the head
menstruation, weather injury does not correlate to the duration or in-
changes, high altitudes, ex- tensity of this headache. This condition occurs
posure to glare or flickering in 30% to 90% of people with concussion, and
lights, loud noises, physical it was the most common symptom reported in
activity, food triggers such professional football players who sustained a
as red wine, food additives mild traumatic brain injury.32 Patients sus-
such as MSG, nitrates or as- pected of post-traumatic headaches should be
partate, caffeine or caffeine monitored for signs of skull fracture, subarach-
withdrawal. noid hemorrhage, and subdural hematoma.
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68 Chapter 7 Sinusitis

Post-traumatic headaches are commonly produces inflammation of the nasal mucous


HEADACHES

managed with rest and nonsteroidal anti- membrane. The inflammation causes an
inflammatory medication. obstruction of the orifices of the nasal sinuses.
Clinical Features of Post-Traumatic Management of headache secondary to sinusi-
Headache tis centers on appropriate treatment of the
underlying infection.
Pain Character Mild to moderate intensity,
steady ache. Clinical Features of Headache
Location May be a vertex or may be Secondary to Sinusitis
band-like around the Pain Character Strong pressure sensation.
head. Location Frontal—frontal region,
Precipitants Trauma and, if present vertex, behind eyes.
prior to trauma, concomi- Maxillary—maxillary region,
tant primary form of upper teeth forehead.
headache. Ethmoid—behind eyes,
Onset Acute form—within 2 weeks temporal region.
of trauma. Sphenoid—occipital region,
Chronic form—begins when vertex, frontal region be-
amnesia resolves. hind eyes.
Duration Acute form—2 to 8 weeks Precipitants Viral or bacterial infection
following trauma. of the upper respiratory
Chronic form—may last up tract.
to 6 months and in about Onset Gradual.
25% of cases can persist Duration Days to months.
for up to 4 years. Frequency Variable.
Frequency Daily. Time Course Strongest after waking.
Time Course Develops within 7 days fol- Associated Purulent discharge, swelling
lowing trauma and in the Symptoms/ of eyelids, and lacrimation.
chronic form headache Signs
can last 3 months or
longer. n Stroke or Transient Ischemic
Associated Somatic symptoms include Attack
Symptoms/ dizziness, photophobia, Chief Clinical Characteristics
Signs phonophobia, tinnitus, This presentation may include a sudden onset
blurring of vision, easily of atypical hemicranial pain ipsilateral to the
fatigable. Psychological affected cerebral hemisphere, associated with
symptoms include depres- hemiplegia, aphasia, excessive salivation,
sion, anxiety, apathy, in- facial symmetry, and altered level of conscious-
somnia, decreased libido, ness. The presentation of headache due to a
irritability, frequent mood cerebrovascular event is similar to migraine
swings. and tension-type headaches except for the
associated neurological signs.
n Sinusitis
Background Information
Chief Clinical Characteristics
Headache occurs in about one-third of cere-
This presentation typically includes a strong
bral infarcts and in 10% to 15% of patients
pressure sensation associated with facial
with transient ischemic attacks (TIAs).3
headache and upper respiratory infection (see
Headaches associated with stroke or TIA pres-
Fig. 7-2C).
ent as a new symptom or for people
Background Information with chronic headache as a different presenta-
This condition results from inflammation of tion of headache.33 A sentinel or premonitory
the nasal membranes and the nasal sinuses. It headache may occur in approximately one-
is caused when an upper respiratory infection third of cases.3 According to the IHS, focal
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Chapter 7 Subdural Hematoma 69

nervous system signs or symptoms should be- of blood in the subarachnoid space that sur-

HEADACHES
gin within 48 hours after onset of the rounds the central nervous system.35 Ruptured
headache.21 The severity, quality, and duration aneurysms and arteriovenous malformations
of headache pain associated with cerebrovas- can lead to this condition. Hypertension and di-
cular accident (CVA) or TIA is variable as is abetes mellitus are two significant risk factors,35
the location of the pain. Dodick3 reports that and polycystic kidney disease, Ehlers-Danlos
the headache symptoms more closely resemble syndrome, systemic lupus erythematosus, and
tension or migraine headaches than the pregnancy are also known risk factors.36
headaches of hemorrhagic stroke. According Polmear37 reported that between 50% and 60%
to Jousilahti and colleagues,34 chronic of patients with this condition described a pre-
headache is an independent predictor of vious history of an atypical headache days to
stroke, especially during a short follow-up. weeks before the event. This warning sign is
Therefore, chronic headache may be a marker known as a sentinel headache.37 The headache
of an underlying disease process that leads to may be accompanied by signs of meningeal
acute stroke.34 Individuals suspected of this irritation (which produces nuchal rigidity),
condition require emergent medical attention. along with varying degrees of neurological
Clinical Features of Headache dysfunction such as seizures, hemiparesis, and
Secondary to Stroke or Transient dysphasia.36 The diagnosis is confirmed with a
Ischemic Attack CT scan without contrast along with lumbar
puncture.36 Emergent medical attention is neces-
Pain Character Variable, resembling mi-
sary for individuals suspected of this condition.
graine or tension-type
headache. Clinical Features of Headache
Location Hemicranial, ipsilateral to Secondary to Subarachnoid
ischemic hemisphere. Hemorrhage
Precipitants Risk factors for stroke or Pain Character Severe.
transient ischemic attack, Location Hemifacial with possible
including hypertension radiation to the cervical,
and smoking. thoracic, or lumbar regions.
Onset Abrupt. Precipitants Physical exertion, such as ex-
Duration Hours to 2 to 3 weeks. ercise or sexual intercourse.
Frequency Related to the precipitating Onset Abrupt.
incident. Duration Days or weeks prior to sub-
Time Course Within 48 hours preceding arachnoid hemorrhage.
stroke or transient ischemic Frequency Related to the precipitating
attack. incident.
Associated Hemiplegia, aphasia, drool- Time Course Abrupt onset occurring
Symptoms/ ing, facial symmetry, and al- within seconds of hemor-
Signs tered level of consciousness. rhage and can continue
for several hours. The
n Subarachnoid Hemorrhage headache typically re-
Chief Clinical Characteristics solves within 1 to 3 weeks.
This presentation can involve a sudden onset of Associated Slight change in level of
severe and abrupt hemifacial pain that is asso- Symptoms/ consciousness, blurred
ciated with a slight change in level of conscious- Signs vision, diplopia, asym-
ness, blurred vision, diplopia, asymmetrical metric pupil response, and
pupil response, and nuchal rigidity. Headaches nuchal rigidity.
due to this condition may radiate to the cervi- n Subdural Hematoma
cal, thoracic, and lumbar spine.
Chief Clinical Characteristics
Background Information This presentation involves a new onset of
Subarachnoid hemorrhage accounts for 3% to unilateral or occipital headache associated
9% of all strokes and is defined as the presence with a decline in level of consciousness and
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70 Chapter 7 Temporal Arteritis

focal neurological deficits. Secondary symp- n Temporal Arteritis


HEADACHES

toms include autonomic signs, vomiting, som- Chief Clinical Characteristics


nolence, or signs of personality change.38 This presentation includes an acute onset of
Background Information severe temporal pain in an individual over
The location of headache depends on the loca- 50 years of age, associated with symptoms of jaw
tion of hematoma. Somewhere between 62% or tongue claudication, scalp tenderness, tem-
and 80% of patients with chronic hematomas poral artery swelling and pulselessness, diplopia,
report headaches.33 Symptoms develop within elevated erythrocyte sedimentation rate, al-
hours to weeks after the precipitating event. tered level of consciousness, fever, weight loss,
This condition may be caused by severe sneez- malaise, and myalgias.
ing, coughing, strain from heavy lifting, and Background Information
whiplash injury.39 It is also a rare though well- This condition, also known as giant cell arteri-
documented sequel of lumbar punctures.40 Pre- tis, is the most common form of vasculitis in
disposing factors include advanced age, alco- adults.41 In individuals over 50 years old,
holism, and coagulation disorders.38 Subdural 94.8% sensitivity and 100% sensitivity was ob-
hematomas form when bridging veins rupture tained if a symptom cluster consisting of jaw
and blood accumulates in the space between the claudication, new-onset headache, and abnor-
arachnoid and the dura. Individuals suspected mal temporal arteries on examination was
of a subdural hematoma should be referred for used to diagnose this condition compared
CT scan without contrast on an emergent basis. with temporal arterial biopsy.42 The clinical
Clinical Features of Headache manifestations of this condition are caused
Secondary to Subdural Hematoma by local and systemic inflammatory disease.
Pain Character Mild or moderate. Localized arterial inflammation causes en-
Location Unilateral side ipsilateral to dovascular damage, vessel stenosis and occlu-
hematoma with the spe- sion, ultimately leading to tissue ischemia or
cific region depending on necrosis.43 This process leads to the clinical
site of hematoma. manifestations of jaw claudication and visual
Precipitants 60% to 70% of time sub- disturbances or loss. This condition can lead to
dural hematoma is pre- permanent blindness if left untreated. It can be
ceded by trauma such as a treated successfully with administration of
fall, severe coughing or proper steroidal medication. Even though
sneezing, strain from headaches and other clinical symptoms may
heavy lifting, or whiplash. subside within a few days of initiating this
Onset May occur within hours to form of treatment, it should continue for 1 to
weeks after precipitating 2 years due to the continued risk of blindness.
event. Clinical Features of Headache
Duration Hours or weeks. Secondary to Temporal Arteritis
Frequency Related to the precipitating Pain Character Severe and throbbing.
incident. Location May be diffuse or localized
Time Course May be steadily progressive to temporal region. Bitem-
or may recur after pain- poral in half of reported
free interval. cases.
Associated Changes in personality or Precipitants Exposure to cold.
Symptoms/ cognitive abilities, neuro- Onset Acute onset of new headache
Signs logical signs such as weak- in a patient/client age
ness, sensory changes or 50 years or older.
seizures, lethargy, drowsi- Duration Typically 2 to 3 months
ness, or decreased level of before seeking medical
consciousness. Also may care.
be accompanied by auto- Frequency Related to the precipitating
nomic symptoms. incident.
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Chapter 7 Tension-Type Headache 71

Time Course Tends to be worse at night. n Tension-Type Headache

HEADACHES
Associated Jaw or tongue claudication, Chief Clinical Characteristics
Symptoms/ temporal artery swelling This presentation can involve a band-like
Signs and pulselessness, diplopia, distribution of nonthrobbing headache radiat-
elevated erythrocyte sedi- ing from frontal region to occiput, associated
mentation rate, altered with stress, anxiety, and neck tenderness (see
level of consciousness, Fig. 7-2D).
fever, weight loss, malaise,
or myalgias. Background Information
This condition is the most common headache
n Temporomandibular Dysfunction presentation, accounting for more than two-
Chief Clinical Characteristics thirds of all headache episodes (Rasmussen)
This presentation may be characterized by and affecting 33% to 80% of the popula-
headache radiating from the muscles of masti- tion.45,46 Women are affected slightly more
cation, the periauricular region, or the tem- often than men.45 Prevalence peaks in the
poromandibular joint associated with abnor- 30- to 39-year-old age group45 and is found to
mal jaw function, asymmetric chewing, increase in incidence with increasing educa-
bruxism, neck pain, tinnitus, and vertigo. tional levels.45 It is important to differentiate
Background Information between the episodic type of tension headache
Headache pain related to this condition may be and the more chronic form because the man-
caused by pathology of the temporomandibu- agement of these two variations differs.46 This
lar joint or may be an associated symptom of differentiation is based entirely on the fre-
another form of headache. This condition is quency and duration of occurrence. This
believed to be an aggravating factor in condition lacks the IHS migraine-defining fea-
headaches and only the cause if clearly related tures of nausea, phonophobia, or photopho-
to the clinical signs and symptoms involving bia.45 Increased muscle hardness has been
the masticatory system.44 Typical management demonstrated in patients who suffer from
involves a combination of rehabilitative and chronic tension headaches, whether or not a
pharmacological interventions for headaches headache is currently present.47 Rehabilitative
related to temporomandibular dysfunction. interventions, non-narcotic analgesics, and
triptans may be used to manage this health
Clinical Features of Headache condition.
Secondary to Temporomandibular
Dysfunction Clinical Features of Tension-Type
Headache
Pain Character Dull ache.
Location Muscles of mastication, periau- Pain Character Mild to moderate tightness,
ricular region, or the tem- pressure, or dull ache48
poromandibular joint. Most that is nonthrobbing.49
often headache is unilateral Location Usually bilateral (but may be
but may be bilateral. unilateral in 10% to 15%
Precipitants Abnormal jaw function, asym- of cases), radiating from
metrical chewing, bruxism. forehead to occiput. Ten-
Onset Within minutes to hours of derness can radiate into
precipitating activity. the posterior cervical mus-
Duration Hours or weeks. cles. In more involved cases
Frequency Incidental to precipitating the pain distribution is
incident. “cape-like,” radiating along
Time Course Variable, depending on under- the upper trapezius mus-
lying cause of temporo- cles covering the shoul-
mandibular dysfunction. ders, scapula, and inter-
Associated Neck pain, tinnitus, and scapular areas.50 Also
Symptoms/ vertigo. may be described as
Signs “band-like.”
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72 Chapter 7 Tumors (Brain)

Precipitants Stress or anxiety,51 not eat- neoplasm. Individuals suspected of headaches


HEADACHES

ing on time, fatigue, and related to tumor should be referred for appro-
lack of sleep are common priate evaluation including neuroimaging.
precipitants in those expe- Headache features that are most suggestive
riencing this condition.52 of a space-occupying lesion include3:
Onset Variable.
l Headache that is subacute and progressive
Duration Can last from 30 minutes to
in nature
several days.
l New onset of headache after age 40 or
Frequency Infrequent episodic—less
significant change in an existing headache
than 10 times per year. Fre-
pattern
quent episodic—at least 10
l Headache associated with:
episodes occurring more
l Nausea or vomiting not due to migraine
than one time but less than
or illness
15 times per month.
l Abnormal neurological signs
Chronic—occurring greater
l Altered level of consciousness or seizures
than 15 times per month
l Night pain.
for more than 3 months.1
Time Course Variable. Tumors leading to headaches include:
Associated Tenderness is the most com-
l Malignant primary, such as:
Symptoms/ mon physical finding in
lMeningeal tumors
Signs patients with this condi-
l Primary brain tumors.
tion.53 Palpation may re-
l Malignant metastatic, such as:
veal tenderness in pericra-
l Central nervous system parenchymal
nial muscles and tension
metastasis
in the nuchal musculature
l Leptomeningeal metastasis
or trapezius.48 This condi-
l Skull base metastasis.
tion does not worsen with
l Benign, such as:
exercise.45
l Acoustic neuroma.

n Tumors (Brain) Clinical Features of Headache


Chief Clinical Characteristics Secondary to Tumor
This presentation typically includes a headache Pain Character Mild, moderate, or severe
of variable intensity and location that is intensity.
relieved with bending forward and associated Location Dependent on location of tu-
with papilledema, nausea and vomiting, focal mor. Frontal or frontotem-
neurological changes, and seizures. poral is most common.
Background Information Precipitants Bending forward.
It is uncommon for headache to be the only Onset May be gradual until tumor
clinical manifestation of a brain tumor in a expands to a critical vol-
patient with a normal neurological exam. ume, then may be very
Between 50% and 60% of patients with brain rapid.
tumors report having headaches and many Duration Typically 2 to 3 months
who have them do not report severe pain.54 before seeking medical
Tumors most likely to be detected early are care.
those that are rapidly growing, supratentorial Frequency Incidental.
(such as glioblastoma), or obstructing the out- Time Course Few brain tumor headaches
flow of cerebrospinal fluid (such as an intra- last more than 10 weeks
ventricular or posterior fossa tumor), because without other symptoms
they are more likely to produce abnormal developing.
neurological signs and symptoms.3 Headaches Associated Papilledema, nausea, vomit-
from tumors are often nondescript and are not Symptoms/ ing, focal neurological
themselves pathognomic of a diagnosis of Signs changes, and seizures.
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Chapter 7 References 73

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graine, tension-type and cervicogenic headache. 27. Spierings ELH. Mechanisms of migraine and actions of
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CHAPTER8
Temporomandibular Joint and Facial Pain
n Sally Ho, PT, DPT, OCS

Description of the Symptom Special Concerns


n Fever with jaw and tooth pain
This chapter describes pathology that may lead n Pain in masseter area with changes in
to temporomandibular joint and facial pain. salivation
Local causes of temporomandibular and facial n Progressive limitation of mandibular ROM
symptoms include pathology of the temporo- and increasing pain
mandibular joint, associated articular and pe- n Severe facial pain with hypersensitivity to
riarticular structures (shading), and the facial touch
bones including the sinuses, excluding the n Sudden onset of severe headache
frontal bones and pharynx. Remote causes are n Stiff neck associated with fever
defined as occurring outside these areas.

75
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76 Chapter 8 Temporomandibular Joint and Facial Pain

CHAPTER PREVIEW: Conditions That May Lead to Temporomandibular Joint


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

and Facial Pain

T Trauma
REMOTE LOCAL
COMMON
Headaches: Anteriorly displaced disk with or without reduction 86
• Cervicogenic headache 81 Dental trauma:
• Edentulism and denture wear 87
• Malocclusion 87
• Tooth injury 87
Dislocations:
• Dislocation of the mandibular condyle into the middle
cranial fossa 89
Internal derangement of the temporomandibular joint 92
Trismus 95
UNCOMMON
Headaches: Dislocations:
• Post-traumatic headache 82 • Dislocation of the mandibular condyle 89
Fractures:
• Mandible 90
• Maxilla 90
• Orbit 90
• Temporal bone 91
• Zygomatic arch 91
RARE
Not applicable Dislocations:
• Botulinum toxin–induced dislocation 89

I Inflammation
REMOTE LOCAL
COMMON
Aseptic Aseptic
Systemic lupus erythematosus 84 Adhesion of the temporomandibular joint 85
Ankylosing spondylitis 85
Septic Capsulitis of the temporomandibular joint 86
Otitis media 84 Herpes zoster 91
Myofascial pain disorder syndrome 92
Retrodiscitis of the temporomandibular joint 94
Rheumatoid arthritis of the temporomandibular
joint 94
Synovitis of the temporomandibular joint 94
Temporal arteritis 95
Septic
Acute viral parotitis 85
Dental infection:
• Tooth abscess 87
Osteomyelitis of the mandible 93
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Chapter 8 Temporomandibular Joint and Facial Pain 77

Inflammation (continued)

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


REMOTE LOCAL
Periodontal disease:
• Alveolar osteitis 93
• Gingivitis 93
• Periodontitis 93
Sinusitis 94
UNCOMMON
Aseptic Aseptic
Multiple sclerosis 82 Facial nerve palsy 90
Septic Septic
Not applicable Not applicable
RARE
Not applicable Not applicable

M Metabolic
REMOTE LOCAL
COMMON
Chronic fatigue syndrome 80 Gout of the temporomandibular joint 91
Fibromyalgia 81 Pseudogout of the temporomandibular joint 93
UNCOMMON
Adverse effects of medication 80 Effects of radiation/chemotherapy 90
RARE
Not applicable Not applicable

Va Vascular
REMOTE LOCAL
COMMON
Angina pectoris 80 Avascular necrosis 86
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

De Degenerative
REMOTE LOCAL
COMMON
Osteoarthrosis/osteoarthritis Ankylosis of the temporomandibular
of the cervical spine 83 joint 86
Osteoarthrosis/osteoarthritis of the temporomandibular
joint 92
(continued)
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78 Chapter 8 Temporomandibular Joint and Facial Pain

Degenerative (continued)
TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

REMOTE LOCAL
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Malignant Primary, such as: Malignant Primary:
• Brain tumor 84 Not applicable
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign: Benign, such as:
Not applicable • Acoustic neuroma 96
• Ganglion cyst of the temporomandibular joint 96
UNCOMMON
Not applicable Malignant Primary, such as:
• External auditory canal tumor 96
• Neoplasm in the temporomandibular region 97
• Parotid gland tumor 97
Malignant Metastatic:
Not applicable
Benign, such as:
• Osteochondroma of the mandibular condyle 97
• Parotid gland tumor 97
• Synovial cyst of the temporomandibular joint 97
RARE
Malignant Primary, such as: Malignant Primary:
• Nasopharyngeal carcinoma 84 Not applicable
• Oropharyngeal carcinoma 84 Malignant Metastatic, such as:
Malignant Metastatic: • Metastases from adenocarcinoma of the colon 96
Not applicable • Metastases from intracapsular stomach tumor 96
Benign: Benign, such as:
Not applicable • Synovial chondromatosis of the temporomandibular
joint 97
• Temporal bone chondroblastoma 98

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Developmental defects of the temporomandibular
joint 88
Mandibular hypoplasia 92
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Chapter 8 Temporomandibular Joint and Facial Pain 79

Congenital (continued)

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


REMOTE LOCAL
RARE
Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Anxiety 80 Atypical facial pain 86
Depression 81 Trigeminal neuralgia 95
Headaches:
• Migraine 82
• Tension-type headache 82
UNCOMMON
Neuralgias involving the cranial Dislocations:
nerves: • Recurrent neurological dislocations of the
• Glossopharyngeal nerve 83 temporomandibular joint 89
• Vagus nerve 83
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Temporomandibular with TMD related to daily microtrauma often


Joint and Facial Pain demonstrate a greater forward head position
than individuals without TMD. The increased
Pain in the temporomandibular joint (TMJ) and tension in the posterior cervical musculature
facial area can be caused by various structures in caused by the forward-headed posture can set
the head, neck, and oral cavity. The proximity of off a pathomechanical cycle of masticatory
these structures and their common neuron pool muscle imbalance, pain, disk displacement,
in the trigeminocervical nucleus (where spinal and altered kinematics of the temporo-
nerves C1, C2, and C3 and cranial nerves V, VII, mandibular joint.1 This cycle is exacerbated by
IX, and XI converge) play a responsible role in parafunctional habits, such as bruxing, clench-
the multifaceted symptoms of this area.1 The ing, gum chewing, and fingernail biting.
condition may present with numerous associated Bruxing (grinding teeth) is primarily a
symptoms such as headache, tinnitus, altered nocturnal behavior, whereas clenching may
mandibular kinematics, TMJ noises, limited jaw occur during the day or at night. Both of these
opening, swallowing difficulty, toothache, dizzi- behaviors are involuntary. In severe cases,
ness, and vertigo. All of these symptoms that re- internal derangement and osteoarthritis may
late to the TMJ and facial area are defined as result. The microtrauma caused by daily
temporomandibular dysfunction (TMD). clenching and bruxing will produce mechani-
TMJ and facial pain affects individuals of cal stress, which further leads to the release of
all ages. This symptom often is influenced by free radicals. Research has suggested that the
posture, oral habit, and stress level. General- free radicals produced by mechanical stresses
ized poor body habit may lead to microtrau- may cause oxidative stress, which in turns
matic pathology in the TMJ and facial area, causes collapse of the lubrication system. This
headaches, earaches, and tinnitus. Individuals is considered a major initiator of internal
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80 Chapter 8 Adverse Effects of Medication

derangement and osteoarthrosis/osteoarthritis n Anxiety


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

of the temporomandibular joint.2 After it has Chief Clinical Characteristics


been determined that physical therapy is indi- This presentation is associated with the
cated, physical therapist management of TMD unpleasant experience of fear in the absence of
should address these underlying issues. an object or activity that would justify this
response. The Diagnostic and Statistical Manual
Description of Conditions That of Mental Disorders,6 text revision (DSM-IV-TR)
May Lead to Temporomandibular classifies several anxiety disorders that preclude
Joint and Facial Pain normal activities of daily living, each with its own
unique symptom clusters.
Remote
Background Information
n Adverse Effects of Medication Anxiety and fear may occur either as a cause or
Chief Clinical Characteristics consequence of temporomandibular and facial
This presentation typically includes spasm of the pain. Anxiety and fear occur as a typical
masseter muscle, or trismus, associated with response to injury, but they should be addressed
use of medication. when they appear to occur out of scale with
symptoms, or if anxiety causes significant dis-
Background Information
ablement. Patients with muscle pain related to
Cardiovascular drugs (eg, calcium channel
acute TMD are more likely to have personality
blockers, antiarrhythmics, phosphodiesterase
characteristics of anxiety.7 Patients with chronic
inhibitors, alpha-1 agonists and antagonists,
TMD report more stress-related impairment of
beta-adrenergic antagonists, angiotensin con-
daily activities than patients with acute TMD.8
verting enzyme inhibitors, angiotensin II
Therefore, a comprehensive team approach that
receptor antagonists, and organic nitrates),
combines medicine, physical therapy, psycho-
anti-infective agents (eg, various antibacterial,
logical counseling, behavioral modification,
antifungal, and antiretroviral drugs), im-
and stress management may ensure optimal
munomodulatory drugs, anti-inflammatory
outcome of chronic pain control.9,10
drugs, antihistamines, medications used to
treat asthma, and tricyclic antidepressants are n Chronic Fatigue Syndrome
most commonly implicated in promoting Chief Clinical Characteristics
headaches that may involve temporomandibu- This presentation is defined as a new onset of un-
lar joint and facial pain.3–5 Discontinued use of explained or persistent or recurrent physical or
these medications can relieve the undesirable mental fatigue that substantially reduces
symptoms, but this should occur with appro- activity level, postexertional malaise, and
priate medical consultation including appro- exclusion of other potentially explanatory med-
priate substitution as needed. ical or psychiatric conditions; also requires at
least one symptom from each of the two of the fol-
n Angina Pectoris lowing categories: autonomic manifestations,
Chief Clinical Characteristics neuroendocrine manifestations, and immune
This presentation involves paroxysmal thoracic manifestations.11
pain, often radiating to the left arm. Neck and
Background Information
jaw pain also may be associated with pallor,
Other possible clinical features include pain,
sweating, nausea, and weakness.
which may serve as the chief symptom that
Background Information directs patients toward physical therapists for
This condition is most often caused by referred management, and sleep dysfunction. This
pain from ischemia of the myocardium and health condition is diagnosed on the basis of
precipitated by effort or excitement. If symp- clinical examination. There is significant
toms are increased with physical exertion or diagnostic overlap with major depression,
cannot be reproduced with TMJ and cervical fibromyalgia, and systemic lupus erythemato-
spine assessment, an emergent medical referral sus. Optimal treatment includes activity mod-
to assess for this condition is indicated. ification and stress management, anaerobic
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Chapter 8 Cervicogenic Headache 81

reconditioning, and medication for relief of substance P in the cerebrospinal fluid. Pre-

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


associated symptomatology. ferred management of fibromyalgia typically
occurs through a comprehensive team approach
n Depression including medication, physical therapy, psy-
Chief Clinical Characteristics chotherapy, and aerobic reconditioning.
This presentation usually involves sadness or grief
in combination with loss of interest in daily HEADACHES
activities. Major depressive disorder is one of n Cervicogenic Headache
several mood disorders categorized by the Chief Clinical Characteristics
DSM-IV-TR.6 This presentation typically includes unilateral
Background Information head pain associated with neck movement; sus-
Depression may occur either as a cause or con- tained or awkward cervical posture; restricted
sequence of TMJ and facial pain. Depressed cervical range of motion; and ipsilateral neck,
mood may occur as a typical response to injury, shoulder, or arm pain.15 This condition is
but it should be addressed when it appears to characterized by moderate to severe episodic
occur out of scale with symptoms or if depres- pain that originates in the neck or suboccip-
sion causes significant disablement. Patients ital region and spreads to the head (Fig. 8-1).
with chronic muscular TMD demonstrate in- Pain attacks last from 3 weeks to 3 months, and
creased depression scores compared to pain-free may vary in frequency from occurring every
controls and patients diagnosed with displace- 2 days to 2 months. People with cervicogenic
ment of the temporomandibular articular headache describe symptoms that do not
disk.12,13 Patients’ psychological state may influ- change sides during an attack,16 and may also
ence the outcome of treatment for patients with present with nausea, dizziness, and phonopho-
TMD. Therefore, a comprehensive team ap- bia or photophobia that is unresolved with
proach that combines medicine, physical ther- migraine medications.
apy, psychological counseling, behavioral modi- Background Information
fication, and stress management may ensure Initial management includes amelioration of
optimal outcome of chronic pain control.9,10 cervical spine musculoskeletal impairments.
Nerve blockade may be effective in recalci-
n Fibromyalgia
trant cases. A favorable response to this
Chief Clinical Characteristics intervention also is pathognomic.17 Typical
This presentation is defined as chronic (greater management of cervicogenic headache
than 3 months) widespread pain throughout the involves rehabilitative interventions.
body, tender to 4 kg of palpation pressure at 11
out of 18 specific anatomically defined sites.
Patients with this syndrome often experience
associated symptoms of morning stiffness, sleep
disturbance, generalized fatigue, depression,
C0-4
headaches, and temporomandibular pain/ C2-4 C0-3
dysfunction. C3-5 C4-6
Background Information C5-7
Reported estimates regarding the prevalence of
fibromyalgia range from 0.7% to 10.5% in
various countries worldwide.14 It primarily
occurs in women of childbearing age, but men
and patients across the life span also can be
affected. In addition to the clinical signs and
symptoms, lab tests are used to confirm this FIGURE 8-1 Referral patterns of the cervical
diagnosis; individuals with fibromyalgia have a facet joints. Cervical facet joints may refer pain to
low serotonin level in the blood and cere- the jaw region, and these symptoms may be associ-
brospinal fluid, and show elevated levels of ated with suboccipital headaches.
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82 Chapter 8 Migraine

n Migraine Background Information


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

Chief Clinical Characteristics Computed tomography scan, magnetic reso-


This presentation typically includes a severe pul- nance imaging, along with clinical assess-
sating and throbbing unilateral headache with ment can confirm the diagnosis. Appropriate
possible jaw pain that may switch sides, asso- management of the head injury through
ciated with nausea, vomiting, diarrhea, surgical and medical intervention can
abdominal cramps, polyuria, sweating, facial resolve secondary temporomandibular joint
pallor, photophobia, or phonophobia. This and facial symptoms.
condition is a chronic condition of recurring
attacks of transient focal neurological symptoms, n Tension-Type Headache
headaches, or both.18 This condition can also Chief Clinical Characteristics
interfere with the function of other body This presentation typically includes a band-
systems, resulting in the characteristic associ- like distribution of nonthrobbing headache ra-
ated symptoms such as nausea, vomiting, diating from frontal region to occiput, asso-
pallor, photophobia, and phonophobia.19 ciated with jaw pain, stress, anxiety, and neck
Migraines are preceded by aura in 20% of tenderness.
cases.20 Even in individuals who commonly Background Information
experience them, auras may not accompany This condition is the most common
every headache. Auras can be visual, somato- headache presentation, accounting for more
sensory, olfactory, or involve speech distur- than two-thirds of all headache episodes
bances. The location of headache may switch and affecting 33% to 80% of the popula-
sides from episode to episode. tion.22,23 Women are affected slightly more
Background Information often than men.22 Prevalence peaks in the
The pathophysiology of this condition is very 30- to 39-year-old age group22 and is found
complex. Current theories postulate that a to increase in incidence with increasing
cascade of events occurs that involve vasodi- educational levels.22 It is important to differ-
latation of meningeal blood vessels, irritation entiate between the episodic type of tension
of perivascular sensory nerves, and stimula- headache and the more chronic form because
tion of brainstem nuclei. Additionally, extrin- the management of these two variations
sic factors such as hormonal fluctuations, differs.23 This differentiation is based entirely
fatigue, or anxiety may be triggers that initiate on the frequency and duration of occur-
the pathophysiological cascade. This condi- rence. This condition lacks the IHS migraine-
tion also has a strong familial tendency defining features of nausea, phonophobia, or
and begins at a young age, suggesting that photophobia.22 Increased muscle hardness has
genetic factors may predispose individuals to been demonstrated in patients who experi-
migraine attacks. The use of neuroimaging is ence chronic tension headaches, whether
not indicated in individuals with migraine or not a headache is currently present.24
symptoms and a normal neurological exam. Rehabilitative interventions, non-narcotic
Certain pharmaceutical agents, such as trip- analgesics, and triptans may be used to
tans and ergots, are effective in preventing manage this health condition.
migraine attacks from becoming too severe.21 n Multiple Sclerosis
Other medications may be used to reduce the
frequency of attacks. Chief Clinical Characteristics
This presentation typically includes weakness,
n Post-Traumatic Headache incoordination, paresthesias, speech disturbance,
visual complaints, and trigeminal neuralgia.
Chief Clinical Characteristics
Trigeminal nerve involvement in patients with
This presentation typically includes pain and
multiple sclerosis is a distinct condition.
dysfunction of the face and jaw. Concussion or
vestibular system compromise may be caused Background Information
by acute head injury, which also may clinically The incidence of trigeminal neuralgia in
manifest in temporomandibular or facial pain. individuals with this condition is higher
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Chapter 8 Osteoarthrosis/Osteoarthritis of the Cervical Spine 83

than that in the general population. The dysphonia, vocal fatigue, and effortful

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


differences in the two groups are younger phonation.27
onset of age and a preponderance of patients
reporting bilateral symptoms in patients Background Information
with multiple sclerosis.25 The mechanism The level of lesion may be determined based
is possibly related to demyelination of the on the patient’s symptoms and signs. The
trigeminal entry root in the pons. Magnetic cause is usually idiopathic; however, tumors,
resonance imaging and computed tomogra- trauma, and viruses may be contributory.
phy confirm the diagnosis. Typical manage- Lesions affecting the proximal segment of the
ment of trigeminal neuralgia related to multiple vagus nerve include oropharyngeal signs, such
sclerosis involves options ranging from as absent gag reflex, uvular deviation, nasopha-
medication to surgery. ryngeal reflux, and absent cough reflex. Lesions
affecting the distal segment will result in an
NEURALGIAS INVOLVING THE unremarkable oropharyngeal examination.28
CRANIAL NERVES A comprehensive cranial evaluation through
clinical examination, brain scan, and magnetic
n Glossopharyngeal Nerve resonance imaging confirms the diagnosis and
Chief Clinical Characteristics directs the most appropriate medical interven-
This presentation usually includes painful tion. Treatment typically includes antiepileptic
attacks on the tonsillar region and pharynx and antispasticity medications.26
with possible radiation to the posterior auric-
ular and jaw areas. Symptoms consist of n Osteoarthrosis/Osteoarthritis of
painful attacks that are provoked with the Cervical Spine
normal activities of speaking, swallowing, Chief Clinical Characteristics
coughing, sneezing, or head rotation. This presentation typically includes neck pain,
Background Information stiffness of the affected cervical joints, and
In patients who report attacks during chew- tightness of the adjacent musculature. One
ing and swallowing, it is not uncommon to resultant effect may be referred pain to the jaw
observe striking weight loss. The cause is and facial area. In addition, the cervical
idiopathic in a majority of cases; potential positioning that may result from altered
causes include tumors of the brainstem cervical spine position in osteoarthrosis
region, morphological abnormalities of the may cause primary local pathology of the tem-
stylohyoid process, and multiple sclerosis. poromandibular joint.
Attacks are mainly unilateral; multiple scle- Background Information
rosis should be considered in the event of Biochemical processes adversely affect the bone,
bilateral attacks. A comprehensive cranial synovium, and articular cartilage. Over time,
evaluation through clinical examination, these processes affect the structure of all these
brain scan, and magnetic resonance imaging tissues in concert, causing pain and the charac-
confirms the diagnosis and directs the most teristic morphological changes of synovitis,
appropriate medical intervention. The diag- subchondral sclerosis, bone marrow changes,
nosis is confirmed with favorable response and osteophyte formation.29 Joint space nar-
of lidocaine injections to trigger areas. rowing, subchondral sclerosis, and osteophytes
Laryngeal electromyography also may be apparent on plain radiographs along with clini-
considered. In addition to lidocaine injec- cal examination maneuvers that result in
tions, treatment typically includes antiepilep- downgliding or compression of the affected cer-
tic and antispasticity medications.26 vical functional spinal units confirm the diag-
nosis. Rehabilitative interventions and nons-
n Vagus Nerve teroidal anti-inflammatory medications can
Chief Clinical Characteristics alleviate local cervical spine pain. Surgical de-
This presentation typically includes pain compression of the affected neural foramen
that may radiate to the auricular or jaw may be indicated for individuals in whom neu-
regions, combined with hoarseness, breathy rologic involvement is clinically significant.
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84 Chapter 8 Otitis Media

n Otitis Media changes, clonus, spasticity, weakness, and in-


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

Chief Clinical Characteristics coordination is indicative of brain malignancy.


This presentation typically includes fever, pain Background Information
in the ear and preaurical area, tinnitus, hear- Brain tumors account for 2% of all cancers an-
ing loss, and vertigo. nually. Brain tumors are classified according to
Background Information their cellular origin and histological appear-
The most common cause of otitis is bacterial ance, which helps to guide treatment, making
infection of the ear, including various strains. a comprehensive neurosurgical evaluation po-
Physical examination confirms the diagnosis, tentially necessary. The most common brain
including inspection of the acoustic canal for primary metastatic tumor in adults is glioblas-
signs of infection, including redness and effu- toma multiforme. Magnetic resonance imag-
sion. The natural history of most cases of oti- ing and brain scan confirm the diagnosis.
tis media without intervention is favorable; Treatment may include surgical resection of
however, use of medications directed at the the tumor, radiation, and chemotherapy.33
appropriate infective agent may be helpful to
n Nasopharyngeal Carcinoma
reduce symptoms and the potential for adverse
effects of prolonged illness. In some cases, Chief Clinical Characteristics
tympanocentesis may be considered as a diag- This presentation may include facial pain
nostic and therapeutic procedure.30 combined with headaches, palpable mass in
the head or neck, bloody nasal discharge,
n Systemic Lupus Erythematosus chronic unilateral nasal congestion, unilateral
Chief Clinical Characteristics hearing loss or frequent ear infections, or
This presentation typically includes a charac- cranial nerve signs.
teristic “butterfly” skin rash over the maxillae, Background Information
generalized body ache, fatigue, heart/lung/ Nasopharyngeal tumors usually develop in
kidney involvement, and joint pain or swelling. the wall of the nasopharynx. The relatively
Background Information large amount of space that can be occupied
This is a chronic, idiopathic autoimmune disor- in this region of the body results in a late
der that can affect virtually any organ in the onset of presenting symptoms and diagnostic
body. The TMJ is often involved in this health delay. This may result in a poorer prognosis
condition. This health condition most often related to advanced tumor development and
affects women of childbearing age. However, increased potential for metastatic spread. The
men also can be affected less frequently. Over diagnosis is confirmed with fused positron
20% of individuals with this condition may de- emission tomography/computed tomogra-
velop fibromyalgia during the course of their phy and head/neck magnetic resonance
illness.31 Blood test, urine test, and skin and kid- imaging. Treatment typically depends on
ney biopsies confirm the diagnosis. Treatment cancer staging, ranging from surgical resec-
includes medication, physical therapy, and tion and radiation therapy to chemotherapy.
management of associated complications of the
n Oropharyngeal Carcinoma
skin, lungs, kidneys, joints, and nervous system.
Chief Clinical Characteristics
TUMORS This presentation may involve facial pain
n Brain Tumor combined with hoarseness, throat pain,
change in the tongue, pain in the tongue, and
Chief Clinical Characteristics
lump in the neck region, dysphagia, dyspnea,
This presentation typically includes headaches,
coughing, or hemoptysis.
facial pain, and possible loss of motor function
of one or more extremities.32 A history of gen- Background Information
eral malaise, nausea, vomiting, weight loss, Up to 90% of oropharyngeal carcinomas are
fever, and headache that worsens with position squamous cell carcinomas, or abnormal
changes with upper motor neuron signs such collections of squamous cells on histological
as seizures, personality changes, cognitive observation. Lower patient socioeconomic
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Chapter 8 Ankylosing Spondylitis 85

status, patient and clinician delay in recog-

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


nizing the health condition, lack of indirect
laryngoscopy performed on physical exami-
nation, failure to inspect the site of the
tumor, and clinician failure to consider tu-
mor or infection as potential causes of symp-
toms are associated with overlooking this
disease process. In turn, overlooking this dis-
ease process results in reduced prognosis.34
Computed tomography and magnetic reso-
nance imaging can further confirm the diag-
nosis. Treatment typically depends on cancer
staging, ranging from surgical resection and
radiation therapy to chemotherapy.35

Local
n Acute Viral Parotitis
Chief Clinical Characteristics FIGURE 8-2 Deflection of the mandible with mouth
This presentation typically includes low-grade opening.
fever, malaise, and pain and swelling in the
affected side of the face. The pain and swelling
may last 3 to 10 days and subside gradually is termed the anchored disk phenomenon.
without sequelae. Magnetic resonance imaging that reveals the
characteristic adherence of the disk to the gle-
Background Information noid fossa during the opening phase confirms
Inflammation of the parotid gland may be the diagnosis. Treatment ranges from rehabilita-
caused by viral infections, such as mumps, tive interventions to arthroscopic release.
Epstein-Barr, and influenza A. Analgesics and
cold compresses are helpful in relieving pain n Ankylosing Spondylitis
and swelling during the early stage of the con- Chief Clinical Characteristics
dition. Supportive treatment of the underlying This presentation typically includes temporo-
viral infection also may be indicated. mandibular joint (TMJ) and facial pain and lim-
ited TMJ range of motion, associated with a
n Adhesion of the slowly progressive and significant loss of general
Temporomandibular Joint spinal mobility. Symptoms may be worse in the
Chief Clinical Characteristics morning and improve with light exercise.
This presentation typically includes a sudden
onset of extremely limited mouth opening Background Information
(between 10 and 30 mm), with deflection of the This condition is a progressive disease that
mandible toward the ipsilateral side (Fig. 8-2). affects primarily the axial joints of the spine and,
There usually is no pain in the involved joint or eventually, the TMJ. It is more common in
the adjacent muscles. males, as well as people of American indigenous
descent, less than 40 years of age, or who carry
Background Information the human leukocyte antigen B27. It also may
Temporomandibular joint overloading caused be associated with fever, malaise, and inflamma-
by macrotrauma (eg, injury) or microtrauma tory bowel disease. The diagnosis is confirmed
(eg, bruxing) may lead to damage of the joint lu- with plain radiographs of the sacroiliac joints
brication system. The decrease in lubrication and lumbar spine, which reveal characteristic
may cause increased friction between the articu- findings of sacroiliitis and “bamboo spine.” The
lar disk and glenoid fossa. An adhesive force TMJ dysfunction with this condition may be
created by this condition may keep the disk from associated with TMJ ankylosis, which may be
sliding down the slope of the articular eminence apparent on plain radiographs or computed to-
during normal month opening. This condition mography. Blood panels including erythrocyte
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86 Chapter 8 Ankylosis of the Temporomandibular Joint

sedimentation rate are useful to track disease n Atypical Facial Pain


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

activity. Treatment includes various classes of Chief Clinical Characteristics


nonsteroidal and biological anti-inflammatory This presentation typically includes constant,
medications and exercise. Surgery to correct boring pain primarily in the lower facial area.
joint deformities also may be indicated.
Background Information
n Ankylosis of the This is an idiopathic disorder. It is different
Temporomandibular Joint from trigeminal neuralgia due to its atypical
Chief Clinical Characteristics distribution, and its symptoms are not parox-
This presentation typically includes temporo- ysmal in nature. However, its pain pattern is
mandibular and facial pain, limited opening, similar to that caused by nasopharyngeal carci-
and loss of vertical height of the involved ramus. noma, oropharyngeal carcinoma, or infection
caused by tooth distraction. There is a high
Background Information comorbidity between post-traumatic disorder
Temporomandibular joint (TMJ) ankylosis syndrome and chronic orofacial pain. Psycho-
can result from trauma, infection, growth metric questionnaires may be beneficial for
defect, ankylosing spondylitis,36 and inade- assessment given the potential effect of affec-
quate surgical treatment of the condylar area. tive causes or consequences of this health con-
Myositis ossificans of the medial pterygoid dition on treatment. Physical examination,
muscle may also lead to ankylosis of the plain radiographs, magnetic resonance imag-
temporomandibular joint. It is important to ing, and computed tomography confirm the
determine the exact cause of the ankylosis for diagnosis. Various classes of medications (eg,
a successful treatment outcome.37 Treatment antidepressants and anti-seizure and narcotic
depends on the underlying cause of ankylo- agents) in combination with a biopsychosocial
sis, and the main intervention is surgical team approach to pain management are among
reconstruction of the TMJ if rehabilitative usual interventions.
measures are inadequate to restore functional
opening. n Avascular Necrosis
Chief Clinical Characteristics
n Anteriorly Displaced Disk With or This presentation typically includes temporo-
Without Reduction mandibular joint arthropathy, headache,
Chief Clinical Characteristics facial pain, and occlusal disturbance.
This presentation typically includes pain, joint
Background Information
noises, altered mandibular kinematics where the
Inflammatory internal derangement of the
mandible deflects to the affected side during
temporomandibular joint, sickle cell disease,
mouth opening (see Fig. 8-2), and limited
and bisphosphonate medications may lead to
mouth opening.
avascular necrosis of the mandibular condyle.
Background Information Magnetic resonance imaging is useful to con-
When an anteriorly displaced disk is reduced firm the diagnosis and monitor the condition’s
during mouth closing, an opening click and a progression.38 Intervention is directed at the
closing click will be present (reciprocal click- underlying etiology. Reconstructive surgery
ing). If the disk is completely displaced anteri- for the affected portion of the mandible
orly without reduction, joint clicking is usually and teeth may be indicated depending on the
absent. This condition may be caused by direct extent of involvement.
macrotrauma to the joint or by microtrauma
from daily clenching and bruxing. However, n Capsulitis of the
the patient may experience crepitus due to Temporomandibular Joint
arthritic changes.1 Clinical examination and Chief Clinical Characteristics
dynamic magnetic resonance imaging of the This presentation typically includes pain in the
temporomandibular joint confirm the diagno- temporomandibular joint and its immediate
sis. Treatment typically involves rehabilitative vicinity, limited opening, and other myofascial
interventions and splinting. symptoms.
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Chapter 8 Tooth Injury 87

Background Information unilateral chewing patterns that result in

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


Inflammation of the capsule of the temporo- overuse of the ipsilateral masticatory muscu-
mandibular joint results in capsulitis of the lature and overload the contralateral TMJ.
temporomandibular joint (TMJ). The cause of Positive correlations between poor denture
the inflammation can be either macrotrauma fit and daytime clenching have also been
such as motor vehicle accident, or micro- reported. Clinical examination confirms the
trauma, such as clenching and bruxing. diagnosis. Adjustment of denture base to
Capsulitis may also be secondary to synovitis provide proper stability and retention can
or retrodiscitis. Medical/dental history and correct symptoms, and proper denture
physical examination can confirm the diagno- support is important in preventing further
sis. Treatment includes medication, modali- arthritic changes in the TMJ.39
ties, and reeducation regarding proper oral
habit. Symptoms usually improve when the n Malocclusion
inflammation is resolved, so nonsurgical inter- Chief Clinical Characteristics
ventions are directed at reducing the underly- This presentation typically includes pain in the
ing inflammation of the TMJ capsule. facial area and dysfunction of the temporo-
mandibular joint (TMJ).
DENTAL INFECTION
Background Information
n Tooth Abscess
This condition encompasses several bite
Chief Clinical Characteristics abnormalities, as shown in Figure 8-3: (A)
This presentation typically includes sharp crossbite, in which the lower tooth has a
pain in the local area and often radiates to the more buccal position than its opposing
jaw, cheek, eye, and ear of the side ipsilateral upper tooth; (B) open bite, in which the up-
to the infection. Ingestion of cold drink or per and lower incisors and canines are forced
food usually worsens symptoms. outward; (C) over bite, in which the upper
Background Information incisors and canines protrude anteriorly ex-
If left untreated, usually uncomplicated gum cessively over their lower antagonist teeth;
boils and tooth decay may lead to a tooth (D) under bite, in which the lower incisors
abscess. In severe cases, soft tissue swelling of and canines protrude anteriorly beyond
the involved jaw may be apparent on obser- their upper antagonist teeth; and other types
vation. Patients suspected of a dental abscess of bite malalignments.
should be referred to a dentist for additional Clinical examination and dental x-ray
evaluation and management. Dental exami- confirm the diagnosis. Correction of the
nation and radiographs can identify abscess. contributing malocclusion potentially can
Treatment includes antimicrobial and anal- alleviate TMJ symptoms. However, inade-
gesic medication.39 quate orthodontic adjustment often causes
increased symptoms in the TMJ region.
DENTAL TRAUMA
n Edentulism and Denture Wear n Tooth Injury
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation typically includes temporo- This presentation typically includes pain in the
mandibular joint (TMJ) and facial pain, jaw, face, and cheek area potentially associated
altered mandibular dynamics, and crepitus. with low-grade fever and general malaise.
Background Information Background Information
It has been reported that approximately 15% This condition can be external or iatrogenic.
to 20% of patients who wear complete Tooth injuries that can cause facial pain
dentures may present with this condition. include fractured teeth, a newly placed
Individuals with edentulism who do not crown, impacted wisdom teeth, status
wear dentures also may present with this post–endodontic surgery, and toothbrush-
condition, but to a much lesser degree. related tooth erosion are included in this cat-
However, loss of posterior teeth may result in egory. These factors make dental history
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88 Chapter 8 Developmental Defects of the Temporomandibular Joint


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

Midline
top

Midline
bottom
A B

C D
FIGURE 8-3 Bite abnormalities in malocclusion: (A) crossbite; (B) open bite; (C) over bite;
(D) under bite.

important to solicit in assessing a patient’s fa- Background Information


cial and TMJ pain. Clinical examination con- Congenital fusion of gums with ankylosis of
firms the specific diagnosis. Referral to the the TMJ, flattening of the condylar head,
dentist to correct the specific health condition bilateral zygomatic-mandibular fusion with
can help relieve symptoms in a timely manner. mandibular hypoplasia, and hemimandibular
hyperplasia are a few examples of congenital
n Developmental Defects of the conditions. Clinical examination and diagnos-
Temporomandibular Joint tic imaging modalities may be used to clarify
Chief Clinical Characteristics the specific nature of developmental defect of
This presentation may include pain in the the TMJ, and this directs appropriate surgical
facial and temporomandibular joint (TMJ) and nonsurgical management of specific
area, along with facial asymmetry. defects.
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Chapter 8 Recurrent Neurological Dislocations of the Temporomandibular Joint 89

DISLOCATIONS n Dislocation of the Mandibular

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


n Botulinum Toxin–Induced Condyle into the Middle Cranial
Dislocation Fossa
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation can involve chewing diffi- This presentation may include pain on
culty, dysphasia, and recurrent temporo- mandibular movement, pain on preauricu-
mandibular joint dislocations following bot- lar palpation, preauricular depression,
ulinum toxin injections. shortening of height of mandibular ramus,
deviation of mandible to opposite site, and
Background Information premature occlusion with open bite.
Botulinum toxin has been proven to be suc- Background Information
cessful in treating various movement disor- This condition is rare, occurring when the
ders and spastic masticatory musculature mandibular condyle dislocates into the
of patients with cerebral palsy. Recently, it middle cranial fossa. The most common
has also been shown to be effective in treat- mechanism of injury is a direct blow to the
ing excessive salivation in patients with mandible from a frontal or a lateral direction
Parkinson’s disease, stroke, and amyotrophic with an open-mouth position. The result
lateral sclerosis. This health condition occurs is fracture of the condylar neck and possi-
when botulinum toxin diffuses into the sur- ble neurological signs. Computed tomog-
rounding masseter muscle, leading to jaw raphy confirms the diagnosis. Treatment
dislocation secondary to excessive muscle involves reduction of the dislocation with
weakness. Medical history, clinical signs, and surgical stabilization of the affected tem-
electromyography can confirm the diagno- poromandibular joint. Long-term follow-
sis. Electrical activity of the masseter muscle up in these individuals is important due to
typically returns to normal within 4 months the potential development of craniofacial
of botulinum toxin injection.40 asymmetries and temporomandibular dys-
n Dislocation of the Mandibular function.41 Recurrent dislocations may be
Condyle treated by the injection of sclerosing agents
or autologous blood, intermaxillary fixation,
Chief Clinical Characteristics or various surgical stabilization procedures.
This presentation typically includes pain in the
jaw area, excessive translation, deviation n Recurrent Neurological
upon opening, and joint noises. Dislocations of the
Temporomandibular Joint
Background Information
This condition occurs when the condylar Chief Clinical Characteristics
head is displaced beyond the mandibular This presentation typically includes pain and
tubercle (out of the glenoid fossa). Fre- spasm in the temporomandibular joint
quently, the condylar head remains within area with involuntary open-lock of the jaw
the joint capsule. Acute dislocation of the characteristic of dislocation.
mandible may lead to secondary osteoarthri- Background Information
tis of the temporomandibular joint due Nontraumatic dislocation of the temporo-
to fibrous adhesion and synovial inflamma- mandibular joint has been described as a
tion. Early investigation of intra-articular complication in a number of neurological
injuries is important. Computed tomography diseases such as parkinsonism, multiple scle-
and dynamic magnetic resonance imaging rosis, and amyotrophic lateral sclerosis. It is
confirm the diagnosis and direct medical believed that increased tone in the lateral
stabilization. Treatment includes reduction pterygoid muscles causes the recurrence
of the dislocation. Recurrent dislocations of involuntary dislocation of the temporo-
may be treated by the injection of sclerosing mandibular joint. The injection of botu-
agents or autologous blood, intermaxillary linum toxin to the lateral pterygoid muscle
fixation, or various surgical stabilization can inhibit the release of acetylcholine at the
procedures. neuromuscular junction, which results in its
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90 Chapter 8 Effects of Radiation/Chemotherapy

paresis and the decrease of mouth opening. associated with shortening of the affected
TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

This method is the treatment of choice for mandibular ramus, deviation of mandible to
recurrent neurological dislocation.10,42–44 opposite site, and premature occlusion with
Clinical examination confirms the diagnosis. open bite.
n Effects of Radiation/ Background Information
Chemotherapy Mandibular condylar fractures usually occur
after trauma, and most often present in
Chief Clinical Characteristics
either condylar neck fracture or dislocation
This presentation involves severe pain and
of the temporomandibular joint. Condylar
discomfort in the face and neck area, trismus,
fractures account for approximately 25% to
and difficulty swallowing.
35% of all mandibular fractures. This diag-
Background Information nosis is confirmed with computed tomogra-
Radiation therapy and chemotherapy for car- phy. In children with this condition, the use
cinoma of the head and neck and regional of a functional appliance immediately after
lymphoma may lead to pain, mucositis, stom- the trauma is recommended. It has been doc-
atitis, avascular necrosis, change in taste umented that the functional activation of the
sensation, dry mouth, tooth decay, and gum masticatory muscles can prevent mandibular
disease. The soft tissue effects of radiation and asymmetries and facial malformation.45
chemotherapy can alter the normal tissue ex-
tensibility and flexibility that permits usual n Maxilla
facial and jaw function. Rehabilitative inter- Chief Clinical Characteristics
ventions such as physical therapy modalities This presentation can involve pain and edema
and exercise may help relieve symptoms and of the lips, midface, and eyes, possibly associ-
improve function. ated with sunken deformity of the involved side,
diplopia, malocclusion, anterior open bite,
n Facial Nerve Palsy
and upper airway compromise.
Chief Clinical Characteristics
This presentation typically includes unilateral Background Information
motor deficits of the muscles of facial This condition may include compound frac-
expression that are innervated by the seventh ture of the articular eminence and fracture
cranial nerve, demonstrated by paralysis of of the glenoid fossa. This health condition
ipsilateral facial muscles, drooping of eyelid, may be caused by blunt trauma involving a
inability to close eyes tightly, deviation of direct blow to the maxilla, such as a motor
mouth angle, and flaccid facial expression. vehicle accident. It may be associated with
Individuals with this condition often report injuries to the orbit. Computed tomography
pain in the facial area of the involved side. confirms the diagnosis. Surgical repair of the
fracture and correction of perifracture
Background Information trauma to the upper airway, overlying soft
Changes to the muscles of facial expression that tissues, and associated trauma to the orbit
are responsible for these symptoms and signs may be indicated in complicated cases.
often occur rapidly, such as over the course of
1 day. This condition, also called Bell’s palsy, is n Orbit
caused by a viral infection. Clinical signs and Chief Clinical Characteristics
symptoms confirm the diagnosis. Treatment This presentation typically involves orbital
includes steroid dose pack and pain medication. and cheek pain, vertical diplopia, orbital
edema, enophthalmos, ecchymosis, tender-
FRACTURES ness of the orbital margin, and reduced cheek
n Mandible and upper gum sensation.
Chief Clinical Characteristics Background Information
This presentation may include pain on A direct blow to the eye such as might occur
mandibular movement, preauricular pal- in a motor vehicle accident or boxing match
pation, preauricular depression, possibly or with a ball may cause fracture of the orbit.
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Chapter 8 Herpes Zoster 91

Enophthalmos may suggest herniation of the in the case of differing intensity of color

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


orbital contents into the maxillary sinus in perception between eyes, beginning with red.
large orbital fractures. Vertical diplopia may Computed tomography of the head and face
suggest entrapment of the optic musculature confirms the diagnosis. Treatment usually
in the fracture site, and severe pain with eye involves a combination of surgical and non-
movements might suggest significant orbital surgical stabilization of the fracture, as well as
hemorrhage or edema. Computed tomogra- management of associated conditions.48
phy of the face and head confirms the diagno-
sis and guides appropriate management, n Gout of the Temporomandibular
which can include surgical repair of the frac- Joint
ture and correction of perifracture trauma to Chief Clinical Characteristics
the orbital contents in complicated cases.46 This presentation involves abrupt, acute pain
in the temporomandibular joint (TMJ), facial,
n Temporal Bone and ear area. TMJ range of motion also may be
Chief Clinical Characteristics limited and chewing usually aggravates pain.
This presentation is characterized by pain
and swelling of the cheek area and temporo- Background Information
mandibular joint, fluid or bleeding from the This health condition affects males predomi-
ear on the affected side, unilateral facial paral- nantly. In addition, certain lifestyle factors
ysis, vertigo, or hearing changes. predispose to the development of gout, such
as a diet high in purines and protein and
Background Information alcohol consumption. Gout is an acutely
Temporal bone fractures are caused by sig- benign crystal deposition disease that rarely
nificant blunt trauma to the skull base. A affects the temporomandibular joint alone.
lateral blow to the temporal bone causes a However, if left untreated, crystal deposition
longitudinal fracture pattern, which ac- (tophi) can cause chronic disruption of
counts for 80% of fractures. Significant joint morphology. Plain radiographs of the
blunt trauma to the frontal or occipital first metatarsophalangeal joint usually con-
region causes a transverse fracture pattern firm the diagnosis. Management of the un-
that typically is more serious or fatal. Com- derlying etiology can resolve symptoms, in-
puted tomography confirms the diagnosis. cluding administration of medication and
Treatment usually involves a combination of modification of potentially contributing
surgical and nonsurgical stabilization of the lifestyle factors.
fracture, as well as management of associ-
ated conditions.47 n Herpes Zoster
Chief Clinical Characteristics
n Zygomatic Arch This presentation typically includes an
Chief Clinical Characteristics exquisitely painful rash or blisters along the
This presentation typically involves pain and first division of the trigeminal nerve, poten-
swelling of the cheek area, with possible inden- tially accompanied by flu-like symptoms. Indi-
tation of the cheek on the affected side, numb- viduals with this condition also demonstrate a
ness of the cheek and upper gum, and visual field previous history of varicella exposure or infec-
disturbances. Painful temporomandibular joint tion. Pain associated with this condition may
movement, lower eyelid deformity, and pares- be disproportionate to the extent of skin irrita-
thesia of the upper lip also may be present. tion. The presence of the rash, extreme pain,
Background Information general malaise, and unclear association with
Blunt trauma to the cheek is the main cause of neck or face movement aids in the differential
this condition. Often soft tissue swelling diagnosis.
can obscure deformities related to this injury. Background Information
Visual field deficits may suggest traumatic The virus may remain dormant in the cranial
optic neuropathy or concomitant orbital floor nerve nuclei until its reactivation during
injury. Optic neuropathy should be suspected a period of stress, infection, or physical
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92 Chapter 8 Internal Derangement of the Temporomandibular Joint

exhaustion. Treatment includes the administra- surgeon for diagnostic workup of the specific
TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

tion of antiviral agents as soon as the zoster deformity, because this will guide manage-
eruption is noted, ideally within 48 to 72 hours. ment. In general, several different surgical
If timing is greater than 3 days, treatment is procedures ordered in stages may be necessary
aimed at controlling pain and pruritus and to optimally address mandibular hypoplasia
minimizing the risk of secondary infection.49 depending on the extent of involvement and
functional compromise.52
n Internal Derangement of the
Temporomandibular Joint n Myofascial Pain Disorder
Syndrome
Chief Clinical Characteristics
This presentation is characterized by joint pain, Chief Clinical Characteristics
headache and facial pain, joint noises, closed lock, This presentation involves diffuse muscle pain
and occlusal disturbance.50 with exquisite trigger points in the head, face,
and neck area. Symptoms may also manifest in
Background Information headaches, earaches, and tinnitus.
This condition involves disharmony of the
disc–condyle relationship during mouth open- Background Information
ing and closing. Disk displacement with or This condition is the most prevalent cause of
without reduction, masticatory muscle dysfunc- chronic orofacial pain and temporomandibu-
tion, synovitis, and malocclusion are among lar joint dysfunction. The etiology of this syn-
causes of this condition. Advanced cases involve drome is multifactorial and includes chronic
bone marrow edema, osteochondritis dissecans, overuse, poor postural habits, and internal
and avascular necrosis. The microtrauma caused derangement of the temporomandibular joint.
by functional and parafunctional activities leads Individuals with chronic rheumatological con-
to the release of free radicals that cause oxidative ditions such as lupus, fibromyalgia, chronic
stress, which in turn reduces the effectiveness of fatigue syndrome, and irritable bowel syn-
synovial fluid. Over time, this process leads to drome have a higher incidence of this health
osteoarthrosis/osteoarthritis of the temporo- condition. Brass and woodwind musicians,
mandibular joint.2 Magnetic resonance imaging voice-over artists, and professional public
and laboratory findings of joint effusion, speakers may also experience overuse syn-
synovial hypertrophy, adhesion, and pathologi- drome due to the exaggeration of mandibular
cal synovial fluid confirm the diagnosis. Nons- movements required by their professions. This
teroidal anti-inflammatory medications and diagnosis is confirmed by clinical examination
physical therapy modality treatment are benefi- primarily. To relieve symptoms, patients must
cial for this condition.51 be instructed in proper head and neck posture,
frequent practice breaks, diaphragmatic
n Mandibular Hypoplasia breathing, relaxation, and stretching exercises
Chief Clinical Characteristics for oral balance.
This presentation may include facial and
temporomandibular joint (TMJ) pain associ- n Osteoarthrosis/Osteoarthritis of
ated with uneven growth of the mandibular the Temporomandibular Joint
rami, rotated facial appearance, overgrowth of Chief Clinical Characteristics
mandibular alveolar bone, or malocclusion. This presentation typically includes pain in the
temporomandibular joint (TMJ) with crepitus
Background Information
during mouth opening and closing.
This condition is commonly thought of as a
congenital malformation of the maxillofacial Background Information
structure, but it is possible blunt trauma also This condition may be caused by acute, direct
may result in this condition on an acquired trauma to the jaw or chronic microtrauma from
basis. Often, congenital mandibular hypopla- bruxism, tooth loss, and malocclusion.39
sia presents early in life. It may be present Biochemical processes related to chronic trauma
at birth or present during development. The adversely affect the bone, synovium, and articu-
patient should be referred to a craniofacial lar cartilage. Over time, these processes affect
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Chapter 8 Pseudogout of the Temporomandibular Joint 93

the structure of all these tissues in concert, caus- intervention. Treatment may include irriga-

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


ing pain and characteristic morphological tion with warm saline or aqueous chlorhexi-
changes of synovitis, subchondral sclerosis, bone dine, as well as administration of analgesic
marrow changes, and osteophyte formation.29 and antimicrobial agents.54
Joint space narrowing, subchondral sclerosis,
and osteophytes may be apparent on plain n Gingivitis
radiographs of the TMJ, which confirms the Chief Clinical Characteristics
diagnosis. Treatment can be accomplished by This presentation is characterized by gum
nonsteroidal anti-inflammatory medications, swelling, bleeding, and halitosis potentially as-
physical therapy modalities, and splinting. If sociated with jaw or facial tenderness.
malocclusion is involved in the etiology of this
Background Information
health condition, orthodontic management
This health condition is present when bacterial
and/or orthognathic surgery may be indicated.53
adhesion occurs and coaggregation (plaque)
n Osteomyelitis of the Mandible becomes calcified (tartar), creating inflamma-
tion confined to the gums. This process creates
Chief Clinical Characteristics a pocket around the tooth that becomes home
This presentation can involve severe pain in the to a variety of organisms that eventually may
jaw and facial area, along with tinnitus, disk lead to periodontitis. Regular dental hygiene
displacement, and limited mouth opening. including checkup and cleaning are useful to
Background Information prevent and treat this condition.55
This condition may be caused by tooth infec-
n Periodontitis
tion, iatrogenic trauma with dental procedure
such as tooth extraction, and radiation expo- Chief Clinical Characteristics
sure. Magnetic resonance imaging and com- This presentation may include by gum
puted tomography confirm the diagnosis; swelling, bleeding, and halitosis potentially
bone biopsy and culture may be used to differ- associated with jaw or facial tenderness and
entiate this health condition from primary tooth loosening or loss.
and metastatic tumors of the mandible and to
Background Information
determine the type of infective organism. High-
This condition is considered a progression
dose antibiotic medication is indicated for this
of gingivitis, in which the organisms that
condition. Physical therapy modality treatment
occupy the gum sulcus created by gum in-
can provide palliative support. Hyperbaric
flammation cause additional deepening of the
chamber treatment can be beneficial in more
sulcus and eventual destruction of periodon-
serious cases, and surgical resection with recon-
tal tissue. Treatment of periodontitis involves
struction may be indicated.
resolution of the underlying gum infection
and gum sulcus by a dental professional.
PERIODONTAL DISEASE
n Alveolar Osteitis n Pseudogout of the
Chief Clinical Characteristics Temporomandibular Joint
This presentation typically involves a sharp Chief Clinical Characteristics
increase in pain 2 to 5 days following tooth This presentation typically includes a sudden on-
extraction, accompanied by facial or jaw pain set of deep stabbing hip pain, worsened with
with halitosis, unpleasant taste, an empty weight bearing and hip passive range of motion,
socket, and tenderness. and associated with tenderness, warmth, and
redness of overlying soft tissues.
Background Information
This health condition is most common Background Information
after excision of the lower molars than other This condition is more common in older
teeth. Individuals suspected of this health males. It is less common in the temporo-
condition should be referred to a dental mandibular joint. This health condition’s
professional for additional evaluation and presentation mimics gout; however, calcium
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94 Chapter 8 Retrodiscitis Arthritis of the Temporomandibular Joint

pyrophosphate dihydrate crystal deposits a significant impact on daily function.


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

mediate its characteristic joint pain and Younger age of onset is associated with a
articular cartilage destruction. Blood tests and greater extent of disability later. Temporo-
microscopic examination of aspirated synovial mandibular joint inflammation was found in
fluid confirm the diagnosis. 87% of the juvenile rheumatoid arthritis pa-
tients through the use of contrast-enhanced
n Retrodiscitis of the MRI.57 The diagnosis is confirmed with
Temporomandibular Joint the presence of rheumatoid factor in blood
Chief Clinical Characteristics tests. A regimen of nonsteroidal, steroidal, or
This presentation may include preaurical pain, biological anti-inflammatory agents may be
palpable tenderness in the external auditory used to manage this health condition.
meatus with the examiner’s fifth finger, tinni-
tus, and altered mandibular dynamics during n Sinusitis
mouth opening and closing. Chief Clinical Characteristics
This presentation involves swollen maxillary,
Background Information
nasal, or paranasal sinuses, along with
Inflammation of the retrodiscal pad results
tenderness and pain in the paranasal and
in retrodiscitis of the temporomandibular
anterior facial area with possible fever. This
joint. The inflammation may be caused by
condition also may be associated with sinus
local trauma, overuse, or disk displacement.
headaches, nasal congestion, facial pressure,
In addition, it also may occur secondary to sys-
nasal discharge, discolored postnasal drainage,
temic connective diseases. Biting down on a
fatigue, and tooth pain.
cotton roll with the back molars on the ipsilat-
eral side can relieve pain because of the Background Information
distraction of the temporomandibular joint. Sinusitis is caused by infection of the sinuses.
This maneuver can be used to confirm the di- Important differential diagnosis includes mi-
agnosis. Undiagnosed systemic disease or local graine in individuals presenting with headache
infection can be further worked up by appro- and tumor in individuals with chronic unilateral
priate imaging and laboratory studies. Treat- congestion. Differentiation between this condi-
ment of retrodiscitis is based on the etiology. tion and migraine without aura occurs based
Anti-inflammatory medication, modalities, a on lateralization and quality of symptoms.
soft diet, and an intraoral appliance are usually Computed tomography or nasal endoscopic
effective. examination is necessary to make an accurate
diagnosis. Treatment includes antimicrobial
n Rheumatoid Arthritis of the agents directed at the specific infective agent.
Temporomandibular Joint
Chief Clinical Characteristics n Synovitis of the
This presentation typically is characterized by Temporomandibular Joint
morning stiffness and generalized pain through- Chief Clinical Characteristics
out multiple joints in a symmetric distribution, This presentation typically includes tenderness
with possible tenderness and swelling of af- and pain in the temporomandibular joint and
fected joints, as well as temporomandibular its immediate vicinity, possibly associated with
joint crepitus and limited mouth opening. headache, crepitus, clicking, or locking.
Background Information Background Information
Women are twice as likely as men to be This inflammatory temporomandibular joint
affected. Symptoms associated with this pro- arthropathy is defined by the inflammation
gressive inflammatory joint disease are of the synovium. If left untreated, this joint
caused by synovial membrane thickening and pathology may result in malocclusion, degener-
cytokine production in synovial fluid. Articu- ation, osteochondritis dissecans, and avascular
lar cartilage erosion, synovial hypertrophy, necrosis of the mandibular condyle. The char-
and constant joint effusion56 eventually cause acteristic joint effusion may be demonstrated
bony erosions and joint deformities that have on magnetic resonance imaging. Treatment
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Chapter 8 Trismus 95

typically includes anti-inflammatory medica- spontaneously. Excessive sensitivity to touch,

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


tion and rehabilitative interventions to restore cold, wind, talking, or chewing also may be
range of motion and movement quality and to present.
address pain. Background Information
This condition most commonly affects
n Temporal Arteritis
middle-aged and elderly individuals, females
Chief Clinical Characteristics slightly more than males. The cause of trigem-
This presentation typically includes severe inal neuralgia is unknown, but it has been
headache, unilateral or bilateral, over postulated that trigeminal nerve focal de-
the scalp–temporal artery region. Jaw claudi- myelination, tumor (eg, acoustic neuroma), or
cation presenting as pain or stiffness during vascular compression may be the etiology. If
chewing is highly suggestive of this condition upper motor neuron signs are present, the
due to ischemia of the muscles of mastication. possibilities of multiple sclerosis and brain-
Other nonspecific signs and symptoms of stem tumor should be considered. This is a
this disorder include malaise, myalgia, condition that requires immediate referral for
weight loss, fever, arthralgia, and possible medical assessment. Magnetic resonance im-
blindness. aging and computed tomography are the
Background Information methods of choice to confirm the diagnosis.
This condition is caused by a subacute inflam- Pharmacologic therapy is extremely important
mation of the external carotid arterial system, in this condition. In severe cases, surgical
affecting especially the superficial temporal ar- decompression and/or rhizotomy may be
tery and the vertebral artery. It appears indicated.
either unilaterally or bilaterally. This condition n Trismus
rarely occurs before age 50 and it is twice as
prevalent in women. The diagnosis is con- Chief Clinical Characteristics
firmed with biopsy of the temporal arteries. This presentation typically includes severe
In individuals over 50 years old, 94.8% sensi- spasm and pain in the masseter muscle, limited
tivity and 100% sensitivity was obtained if a opening or closed lock, and earache with
symptom cluster consisting of jaw claudica- tinnitus.
tion, new-onset headache and abnormal Background Information
temporal arteries on examination was used Trismus is defined as spasm of the masseter
to diagnose this condition compared with muscles due to the motor disturbance of
temporal arterial biopsy.58 Ophthalmologic the trigeminal nerve. Trismus can be caused
evaluation is vital because loss of vision is by a variety of etiologies, such as dental
associated with this condition. Vision loss usu- infection, dental procedure (with superior/
ally is characterized by sudden onset, and per- inferior nerve anesthesia), trauma to the jaw,
manent blindness may result if left untreated. nasopharyngeal/oropharyngeal tumors, adverse
The therapy of choice for this condition is oral effect of medication (eg, tricyclic antidepres-
prednisone. Even though headaches and other sant, succinylcholine, phenothiazines), and
clinical symptoms may subside within a few radiotherapy/chemotherapy. Commonly, tris-
days of initiating treatment, prednisone mus is caused by overstretching of the masseter
should continue for 1 to 2 years due to the muscle while an individual’s mouth is kept
continued risk of blindness. open during dental procedures over an
extended period of time. Functional sequelae
n Trigeminal Neuralgia of this health condition may involve poor nu-
Chief Clinical Characteristics trition and oral hygiene. Clinical examination
This presentation typically includes lightning- including limited mouth opening confirms the
like momentary jabs of excruciating facial pain diagnosis in the absence of other contributing
along the distribution of the second and third pathologies. Rehabilitative interventions are
division of the trigeminal nerve. Pain may per- the treatment of choice for this health condi-
sist for a few minutes to weeks and then abate tion, including range-of-motion exercises and
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96 Chapter 8 Acoustic Neuroma

splinting. Treatment should begin as soon as Background Information


TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

possible to address functional impairment This condition involves pseudocysts formed


related to trismus. by connective tissue with a fibrous lining
and filled with viscous fluid, which arises
TUMORS
from the joint capsule. Palpation usually
n Acoustic Neuroma reveals a smooth, firm, and tender mass in
Chief Clinical Characteristics the preaurical area. Computed tomography
This presentation typically includes facial and magnetic resonance imaging confirm
pain or numbness, headache, hearing loss, the diagnosis. Treatment may include surgi-
tinnitus, and gait and balance disturbance. cal resection in recalcitrant cases that are
characterized by significant symptoms or
Background Information functional impairment.
This condition is a benign tumor that grows
on the vestibular division of the eighth cranial n Metastases from
nerve in the internal auditory canal. Brain- Adenocarcinoma of the Colon
stem responsive audiometry is the main diag- Chief Clinical Characteristics
nostic test. The specificity is approximately This presentation typically includes pain,
97%. Magnetic resonance imaging can fur- swelling, trismus, dysphagia, paresthesia of the
ther confirm the diagnosis. Commonly, surgi- facial area, and progressive limitation of
cal intervention is recommended. Surgical temporomandibular joint mobility. A thorough
removal of the acoustic neuroma can some- subjective assessment should include ques-
times cause delayed hearing loss. Postopera- tioning about weight loss, night pain/sweats,
tive medical treatment includes the use of fever and chills, bowel habits, blood in stools,
vasoactive medication such as hydroxyethyl and general malaise.
starch and nimodipine; these medications are
effective to preserve hearing.59 Background Information
This rare condition is the most common
n External Auditory Canal Tumor metastatic tumor to the temporomandibu-
Chief Clinical Characteristics lar joint. For individuals with a previous
This presentation typically includes pain, history of colon cancer, this metastatic
swelling, limited temporomandibular joint process must be considered. The diagnosis
mobility, and hearing loss. Physical examina- is confirmed with biopsy, computed tomog-
tion can usually confirm the diagnosis, raphy, and magnetic resonance imaging.
including bloody ear discharge. Needle biopsy has been advocated to be
considered with all surgical procedures,
Background Information even simple tooth extractions.61 Treatment
Lack of response to topical or systemic med- involves appropriate management of the
ications to address more benign conditions underlying malignancy with a combination
that mimic this health condition may raise of chemotherapy, radiation therapy, and
clinical suspicion and reduce diagnostic de- surgical resection of the primary or relevant
lay. The most common of this type of tumor metastatic tumors.
is squamous cell carcinoma. A combination
of preoperative radiation therapy and surgi- n Metastases from Intracapsular
cal resection of the tumor is effective and has Stomach Tumor
fewer side effects than other interventions.60 Chief Clinical Characteristics
n Ganglion Cyst of the This presentation typically includes progres-
Temporomandibular Joint sive limitation of the temporomandibular
joint function.
Chief Clinical Characteristics
This presentation typically includes articular Background Information
pain, preauricular swelling, limited temporo- Adenocarcinoma of the gastric cardia may
mandibular joint mobility, and joint noises. metastasize to the temporomandibular joint.
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Chapter 8 Synovial Cyst of the Temporomandibular Joint 97

This is a very rare condition. One case report n Parotid Gland Tumor

TEMPOROMANDIBULAR JOINT AND FACIAL PAIN


showed that the condyle and disk were ante- Chief Clinical Characteristics
riorly displaced by the tumor, resulting in This presentation typically includes pain and
progressive crossbite. However, no destruc- swelling in the masseter area, and facial nerve
tive changes were noted on the radiography. palsy ipsilateral to the side of symptoms.
Tumor staging indicated that this lesion was
the only distant metastasis.62 Treatment Background Information
involves appropriate management of the This health condition is usually benign. In
underlying malignancy with a combination rare cases, parotid gland tumor can be
of chemotherapy, radiation therapy, and malignant in nature or referred from an-
surgical resection of the primary or relevant other organ such as the kidney.64 Associated
metastatic tumors. facial weakness increases the likelihood
of malignancy. This diagnosis is confirmed
n Neoplasm in the with histopathological and immunohisto-
Temporomandibular Region chemical findings from parotid gland biopsy.
Chief Clinical Characteristics Based on these findings, treatment may
This presentation typically includes pain in range from surgical resection of the tumor
the epipharyngeal region, parotid gland, or with or without parotidectomy, chemother-
the temporomandibular joint. Progressive apy, or radiation therapy.65
worsening of limitation in mandibular move-
ment and increasing pain suggest this health n Synovial Chondromatosis of the
condition. Temporomandibular Joint
Background Information Chief Clinical Characteristics
Neoplasm of the oral cavity accounts for This presentation typically includes articular
approximately 5% of all malignancies in the pain, preauricular swelling, occasional snap-
body.61 Metastatic tumors of the oral cavity ping with temporomandibular joint move-
comprise 1% of all neoplasms. Confirmation ment, and restricted joint movement.
by clinical signs and imaging studies is nec-
Background Information
essary for making a differential diagnosis.63
This is an uncommon disease of cartilagi-
Treatment depends on tumor staging, and
nous transformation of synovial membrane
may include surgical resection, chemother-
with formation of loose bodies in the joint
apy, and radiation therapy.
space. The cause of this condition is un-
n Osteochondroma of the known. Plain radiographs confirm the diag-
Mandibular Condyle nosis when they reveal multiple loose bodies
Chief Clinical Characteristics in the joint. Surgical resection of joint loose
This presentation typically includes pain, lim- bodies and joint reconstructions are poten-
ited function of the temporomandibular joint, tial treatments for this health condition.
and crepitus. n Synovial Cyst of the
Background Information Temporomandibular Joint
This condition is a benign tumor consisting
Chief Clinical Characteristics
of projecting adult bone capped by cartilage
This presentation typically includes articular
projecting from the lateral contours of
pain, preauricular tenderness and swelling,
endochondral bones. This is an uncommon
limited temporomandibular joint mobility,
health condition. Panoramic radiograph,
and joint noises. The clinical presentation of
magnetic resonance imaging, computed
synovial cysts resembles those characteristics
tomography, and arthroscopy confirm the
of the ganglion cysts.
diagnosis. Condylectomy with temporo-
mandibular joint reconstruction may be Background Information
considered as a form of clinical management This health condition involves cysts lined by
for this health condition. synovial cells that contain gelatinous fluid. It
1528_Ch08_075-099 15/05/12 12:25 PM Page 98

98 Chapter 8 Temporal Bone Chondroblastoma

may be caused by displacement of the syn- 9. Glaros AG. Emotional factors in temporomandibular
TEMPOROMANDIBULAR JOINT AND FACIAL PAIN

ovial tissue during embryogenesis or hernia- joint disorders. J Indiana Dent Assoc. 2000;79(4):20–23.
10. Korszun A. Facial pain, depression and stress—connec-
tion of the synovium into underlying bone tions and directions. J Oral Pathol Med. Nov 2002;
due to trauma. Clinical imaging and biopsy 31(10):615–619.
confirm the diagnosis. Surgical resection may 11. Carruthers BM, Jain AK, DeMeirleir KL, et al. Myalgic
be considered for patients with significant encephalomyelitis/chronic fatigue syndrome: clinical
working case definition, diagnostic and treatment pro-
functional compromise and symptoms. tocols (a consensus document). J Chronic Fatigue Syndr.
2003;11(1):7–115.
n Temporal Bone 12. Selaimen CM, Jeronymo JC, Brilhante DP, Grossi ML.
Chondroblastoma Sleep and depression as risk indicators for temporo-
mandibular disorders in a cross-cultural perspective:
Chief Clinical Characteristics a case-control study. Int J Prosthodont. Mar–Apr
This presentation typically includes hearing 2006;19(2):154–161.
loss, otalgia, otorrhea, and may be pain in 13. Yap AU, Tan KB, Chua EK, Tan HH. Depression and
the facial area referred from tumor mass in the somatization in patients with temporomandibular dis-
orders. J Prosthet Dent. Nov 2002;88(5):479–484.
external auditory canal. 14. McBeth J, Jones K. Epidemiology of chronic muscu-
Background Information loskeletal pain. Best Pract Res Clin Rheumatol. Jun
2007;21(3):403–425.
This condition usually is a benign tumor 15. Sjaastad O. Benign exertional headache. Headache.
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netic resonance imaging, and biopsy confirm 17. Sjaastad O, Pfaffenrath V. Cervicogenic headache: diag-
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resection of the tumor and reconstruction of 18. Spierings ELH. Mechanisms of migraine and actions
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poromandibular joint (TMJ) may need to be 19. Ferrari MD. Migraine. Lancet. Apr 4 1998;351(9108):
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Neurol. 1997;17(4):303–306.
21. Maizels M. Headache evaluation and treatment by
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CHAPTER9
Case Demonstration: Jaw Pain
■ Jesus F. Dominguez, PT, PhD ■ Michael S. Simpson, PT, DPT

NOTE: This case demonstration was devel- discomfort began about 8 months earlier after
oped using the diagnostic process described eating popcorn. There were several unpopped
in Chapter 4 and demonstrated in Chapter 5. kernels in the bag and he felt the pain in his left
The reader is encouraged to use this diagnostic lower jaw immediately after biting down on
process in order to ensure thorough clinical one. The discomfort occurred only with
reasoning. chewing for the next few days and he went to
see his primary care physician who prescribed
THE DIAGNOSTIC PROCESS ibuprofen and a soft diet for 1 week. Mr. K.D.
states that over the course of the next several
Step 1 Identify the patient’s chief concern.
weeks, the discomfort resolved completely.
Step 2 Identify barriers to communication.
Approximately 6 months ago, he again felt
Step 3 Identify special concerns.
left lower jaw discomfort after helping his
Step 4 Create a symptom timeline and sketch
brother move heavy furniture. At the time, he
the anatomy (if needed).
thought that he had “strained some jaw mus-
Step 5 Create a diagnostic hypothesis list
cles” because he remembers clenching his teeth
considering all possible forms of remote
during the strenuous lifting. He mentioned
and local pathology that could cause the
this in passing to his primary care physician
patient’s chief concern.
who advised him to begin taking ibuprofen
Step 6 Sort the diagnostic hypothesis list
again and return in 2 weeks if the jaw discom-
by epidemiology and specific case
fort did not resolve. Mr. K.D. stated that he did
characteristics.
not find the ibuprofen helpful this time, but he
Step 7 Ask specific questions to rule specific
did not return for a follow-up visit.
conditions or pathological categories less
Over the course of the next 6 months, he
likely.
noted that his jaw discomfort would come on
Step 8 Re-sort the diagnostic hypothesis list
when he would push his lawnmower or push
based on the patient’s responses to specific
trashcans to the curb, though these activities
questioning.
would not always provoke his symptoms. He
Step 9 Perform tests to differentiate among
described the severity of the symptoms as 4/10
the remaining diagnostic hypotheses.
on a visual analog scale (VAS), with 10 being the
Step 10 Re-sort the diagnostic hypothesis list
most unbearable discomfort that he could
based on the patient’s responses to specific
imagine. The symptom required 3 to 5 minutes
tests.
to dissipate after stopping the activity, but he
Step 11 Decide on a diagnostic impression.
would be left with a dull ache that persisted for
Step 12 Determine the appropriate patient
10 minutes. At a yearly dental cleaning visit
disposition.
2 months ago, he mentioned the symptoms to
his dentist who noted that the left temporo-
mandibular joint was mildly tender to palpa-
tion. Dental x-rays at the time did not reveal any
Case Description evidence of dental abnormalities. The dentist
then recommended the patient be evaluated
Mr. K.D. is a 57-year-old male accountant re- by a physical therapist with a provisional diag-
ferred to physical therapy with a diagnosis of nosis of temporomandibular dysfunction and
“jaw pain secondary to left temporomandibular instructed the patient to make an appointment
joint dysfunction.” He stated that the jaw within a week.

100
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Chapter 9 Case Demonstration: Jaw Pain 101

Beginning 3 weeks prior to his physical referred by his dentist. This pattern of
therapy evaluation, Mr. K.D. also began to behavior could most likely present a
notice shortness of breath associated with the barrier to a timely diagnosis and effec-
jaw pain. He attributed this to a recent increase tive treatment. He also demonstrated a
in smoking because of job-related stressors. limited understanding of the need to
The severity of the symptoms was now 6/10 inform his physician of the change in
on the VAS and would persist for more than the quality of the jaw discomfort, the
15 minutes after he sat down to “catch his associated symptoms, and the new pat-
breath.” He also reported that the “quality” of tern of provocation. Failure to inform a
the jaw discomfort was different than what health care provider of new symptom
he had felt 8 months prior. Although the jaw patterns could significantly confound
discomfort and the shortness of breath would the diagnostic process and could lead to
resolve simultaneously, he was now experi- the development of ineffective treat-
encing these symptoms daily. He denied any ment strategies.
concomitant nausea, vomiting, dizziness, or
STEP #3: Identify special concerns.
diaphoresis.
Past medical history was significant for ● Unusual symptoms occurring with
hypertension, for which he took metoprolol, physical exertion. Raises the index of
100 mg daily, and smoking (64 pack-years). clinical suspicion for contributory car-
There was no known family history of coro- diovascular or pulmonary pathology.
nary artery/peripheral vascular disease or dia- ● Patient’s age and a diagnosis of hyper-
betes. Mr. K.D. said that he did not engage in tension. Raises the index of clinical sus-
any regular exercise. He weighed 90 kg and was picion for contributory cardiovascular
168 cm tall. His body mass index (BMI) was or pulmonary pathology.
calculated to be 31.9 kg/m2, placing him in the ● Smoking history, including a recent in-
“obese” category. crease in smoking. Raises the index of
clinical suspicion for contributory car-
STEP #1: Identify the patient’s chief diovascular or pulmonary pathology.
concern.
● Unresponsive to previously effective
Mr. K.D. reports his chief concern as left treatment. Raises the possibility of a
jaw pain. different pathology presenting to create
STEP #2: Identify barriers to a similar previous chief concern.
communication. STEP #4: Create a symptom timeline
● History of limited communication and sketch the anatomy (if needed).
with health care professionals. Mr.
K.D. did not return to his primary care
physician for a reassessment and waited
2 months to make an appointment with
the physical therapist after being

8 months 6 months
1992 ago ago Today

Diagnosed Diagnosed Onset of “new” Onset of


with essential with TMJ symptoms associated
hypertension Dysfunction in the jaw symptoms
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102 Chapter 9 Case Demonstration: Jaw Pain

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.
Remote Remote
T Trauma T Trauma
Poor posture Poor posture
Whiplash Whiplash (absence of whiplash mechanism)
I Inflammation I Inflammation
Aseptic Aseptic
Chronic fatigue syndrome Chronic fatigue syndrome
Dental caries Dental caries (absence of positive findings
on recent dental examination)
Fibromyalgia Fibromyalgia
Sjögren’s syndrome Sjögren’s syndrome (gender not typical)
Subacute granulomatous thyroiditis Subacute granulomatous thyroiditis
Systemic lupus erythematosus Systemic lupus erythematosus
Septic Septic
Dental abscess Dental abscess (absence of positive
findings on recent dental examination)
Otitis media Otitis media (absence of ear pain or other
associated symptoms)
Post-herpetic neuralgia Post-herpetic neuralgia
Rheumatoid arthritis Rheumatoid arthritis
Septic arthritis Septic arthritis
Sinus abscess Sinus abscess
Sinus infection Sinus infection
Suppurative parotitis Suppurative parotitis
Tetanus Tetanus (time course)
M Metabolic M Metabolic
Carnitine deficiency Carnitine deficiency (age of onset not
typical)
Forbes’ disease Forbes’ disease (age of onset not typical)
Hypocalcemic tetany Hypocalcemic tetany (age of onset not
typical)
McArdle’s disease McArdle’s disease (age of onset not typical)
Medication-induced dyskinesia Medication-induced dyskinesia (no history
of Parkinson’s medications)
Mitochondrial myopathy Mitochondrial myopathy (age of onset not
typical)
Paget’s disease Paget’s disease (age of onset not typical)
Pompe’s disease Pompe’s disease (age of onset not typical)
Tarui’s disease Tarui’s disease (age of onset not typical)
Va Vascular Va Vascular
Angina pectoris Angina pectoris
Coronary insufficiency Coronary insufficiency
Cranial/temporal arteritis Cranial/temporal arteritis (symptoms are
intermittent, worse with exertion)
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Chapter 9 Case Demonstration: Jaw Pain 103

Dissecting aneurysm Dissecting aneurysm (time course, no


associated abdominal or back pain)
Myocardial infarction Myocardial infarction (time course)
Sickle cell crisis pain Sickle cell crisis pain (age not typical)
De Degenerative De Degenerative
Osteoarthritis of cervical spine Osteoarthritis of cervical spine (absence of
neck pain not typical)
Tu Tumor Tu Tumor
Acoustic neuroma Acoustic neuroma
Co Congenital Co Congenital
Hyperostosis cortical infantile Hyperostosis cortical infantile (age not
typical)
Paroxysmal extreme pain disorder Paroxysmal extreme pain disorder (age not
typical)
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Depression Depression
Gastroesophageal reflux disease Gastroesophageal reflux disease
Hiatal hernia Hiatal hernia
Multiple sclerosis Multiple sclerosis
Stress/anxiety Stress/anxiety
Local Local
T Trauma T Trauma
Barotrauma Barotrauma (no associated ear pain)
Bruxism Bruxism
Dental trauma Dental trauma (absence of positive findings
on recent dental examination)
Edentulism and denture wear Edentulism and denture wear (absence of
positive findings on recent dental
examination)
Ernest syndrome Ernest syndrome (absence of jaw trauma)
Malocclusion Malocclusion (absence of positive findings
on recent dental examination)
Mandibular atrophy Mandibular atrophy (absence of positive
findings on recent dental examination)
Onychophagia Onychophagia (worsened with prolonged
physical exertion)
Pathological fracture Pathological fracture (absence of positive
findings on recent dental examination)
Temporal tendinitis Temporal tendinitis
Temporomandibular ankylosis Temporomandibular ankylosis
Temporomandibular dysfunction Temporomandibular dysfunction
Trismus Trismus (able to open mouth to speak and
give history)
I Inflammation I Inflammation
Aseptic Aseptic
Capsulitis or synovitis Capsulitis or synovitis
Dental ulcer Dental ulcer (absence of positive findings
on recent dental examination)
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104 Chapter 9 Case Demonstration: Jaw Pain

Gingivitis Gingivitis (absence of positive findings on


recent dental examination)
Impacted wisdom teeth Impacted wisdom teeth (absence of
positive findings on recent dental
examination)
Jaw cyst Jaw cyst (absence of positive findings on
recent dental examination)
Rhabdomyolysis Rhabdomyolysis (no recent changes in
physical training regimen)
Septic Septic
Acute pulpitis or pulpal abscess Acute pulpitis or pulpal abscess (absence of
positive findings on recent dental
examination)
Acute suppurative sinusitis Acute suppurative sinusitis
Gout Gout (unlikely as only joint affected)
Herpes zoster Herpes zoster (unlikely with absence of
history of vesicular eruptions in the
painful zone)
Lyme disease Lyme disease
Osteomyelitis Osteomyelitis
Pericoronitis Pericoronitis (age of onset not typical)
Pseudogout Pseudogout (unlikely as only joint affected)
M Metabolic M Metabolic
Sialolithiasis Sialolithiasis
Va Vascular Va Vascular
Avascular necrosis Avascular necrosis (absence of positive
findings on recent dental examination)
Microvascular compression Microvascular compression (absence of
positive findings on recent dental
examination)
De Degenerative De Degenerative
Felty’s syndrome Felty’s syndrome (no long-standing history
of rheumatoid arthritis)
Fibrous dysplasia Fibrous dysplasia (age not typical)
Osteoarthritis/osteoarthrosis of the Osteoarthritis/osteoarthrosis of the
temporomandibular joint temporomandibular joint
Tu Tumor Tu Tumor
Ameloblastoma Ameloblastoma
Ewing’s sarcoma Ewing’s sarcoma (age not typical)
Giant cell tumor Giant cell tumor
Multiple myeloma Multiple myeloma
Neoplasm/tumors of the mandible and Neoplasm/tumors of the mandible and
maxilla maxilla
Odontoma Odontoma
Oropharyngeal cancer Oropharyngeal cancer
Osteosarcoma Osteosarcoma
Parotid (Warthin’s) tumor Parotid (Warthin’s) tumor
Pathological fracture secondary to Pathological fracture secondary to
metastases metastases
Squamous cell carcinoma Squamous cell carcinoma
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Chapter 9 Case Demonstration: Jaw Pain 105

Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Trigeminal neuralgia Trigeminal neuralgia

STEP #7: Ask specific questions to rule Systemic lupus erythematosus (no fatigue
specific conditions or pathological or malaise)
categories less likely. Septic
● Have you had any associated numb- Post-herpetic neuralgia (no change in facial
ness or tingling in your face? No, sensation)
making less likely forms of pathology Rheumatoid arthritis (no fatigue)
involving neurogenic pain. Septic arthritis (no fatigue)
● Have you had congestion in the ear or Sinus abscess (no nasal congestion,
nose? No, ruling less likely sinus or ear fatigue)
pathology (ie, infection and acoustic Sinus infection (no nasal congestion,
neuroma). Fever? No, would also rule fatigue)
out (r/o) infection. Suppurative parotitis (symptoms not worse
● Have you experienced any uninten- with eating)
tional weight gain or loss? No, ruling M Metabolic
less likely cancer as a contributory cause
Not applicable
of the patient’s symptoms.
● Are your symptoms worsened or alle-
Va Vascular
viated by eating? No, ruling less likely Angina pectoris
upper gastrointestinal pathology as a Coronary insufficiency
cause of this patient’s symptoms. De Degenerative
● Do you feel fatigued or “out of it”? No, Not applicable
ruling less likely systemic forms of
Tu Tumor
aseptic inflammation (ie, rheumatoid
arthritis and chronic fatigue syndrome) Acoustic neuroma (no aural congestion)
and neurogenic problems (ie, multiple Co Congenital
sclerosis), which are associated with Not applicable
fatigue.
Ne Neurogenic/Psychogenic
STEP #8: Re-sort the diagnostic hypothesis Depression
list based on the patient’s responses to Gastroesophageal reflux disease (no
specific questioning. change in symptoms with eating)
Remote Hiatal hernia (no change in symptoms with
T Trauma eating)
Multiple sclerosis (no fatigue or malaise)
Poor posture
Stress/anxiety
I Inflammation
Local
Aseptic
T Trauma
Chronic fatigue syndrome (no fatigue or
malaise) Bruxism
Fibromyalgia (no fatigue or malaise) Temporal tendinitis
Sjögren’s syndrome (no fatigue or malaise) Temporomandibular ankylosis
Subacute granulomatous thyroiditis (no Temporomandibular dysfunction
fatigue or malaise)
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106 Chapter 9 Case Demonstration: Jaw Pain

I Inflammation Co Congenital
Aseptic Not applicable
Capsulitis or synovitis Ne Neurogenic/Psychogenic
Septic Trigeminal neuralgia (no change in facial
Acute suppurative sinusitis (no nasal sensation)
congestion)
STEP #9: Perform tests to differentiate
Lyme disease (no fatigue or malaise)
among the remaining diagnostic
Osteomyelitis (no fever)
hypotheses.
M Metabolic ● Cardiac and pulmonary auscultation.
Sialolithiasis (no change in perceived facial Normal first (S1) and second (S2) heart
status) sounds without murmurs. A fourth
Va Vascular heart sound (S4, often associated with
Not applicable long-standing hypertension) was also
noted. Auscultation of the lungs
De Degenerative revealed mild inspiratory crackles
Osteoarthritis/osteoarthrosis throughout both lower lung fields.
of the temporomandibular joint ● Temporomandibular examination.
Tu Tumor Localized tenderness to palpation of
Ameloblastoma (no change in perceived the masseter and temporalis muscles.
facial status, fatigue/malaise, weight Mr. K.D. stated these symptoms were
change) unlike his current chief concern. Mild
Ewing’s sarcoma (no change in perceived and painless reciprocal click was noted.
facial status, fatigue/malaise, weight STEP #10: Re-sort the diagnostic
change) hypothesis list based on the patient’s
Giant cell tumor (no change in perceived responses to specific tests.
facial status, fatigue/malaise, weight
Remote
change)
Multiple myeloma (no change in perceived T Trauma
facial status, fatigue/malaise, weight Not applicable
change) I Inflammation
Neoplasm/tumors of the mandible and
maxilla (no change in perceived facial Aseptic
status, fatigue/malaise, weight change) Not applicable
Odontoma (no change in perceived facial Septic
status, fatigue/malaise, weight change) Not applicable
Oropharyngeal cancer (no change in M Metabolic
perceived facial status, fatigue/malaise,
Not applicable
weight change)
Osteosarcoma (no change in perceived Va Vascular
facial status, fatigue/malaise, weight Angina pectoris
change) Coronary insufficiency
Parotid (Warthin’s) tumor (no change in De Degenerative
perceived facial status, fatigue/malaise,
Not applicable
weight change)
Pathological fracture secondary to Tu Tumor
metastases (no change in perceived facial Not applicable
status, fatigue/malaise, weight change) Co Congenital
Squamous cell carcinoma (no change in
Not applicable
perceived facial status, fatigue/malaise,
weight change)
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Chapter 9 Case Demonstration: Jaw Pain 107

Ne Neurogenic/Psychogenic STEP #12: Determine the appropriate


Depression patient disposition.
Stress/anxiety ● Refer the patient to a cardiologist by

Local telephone for additional evaluation and


treatment.
T Trauma
● Educate the patient about the urgency
Bruxism (temporomandibular joint of the need for a cardiology appoint-
examination revealed symptoms that ment to take place.
were inconsistent with chief concern) ● Inform the patient of symptoms and
Temporal tendinitis (temporomandibular signs of an acute myocardial infarction,
joint examination revealed symptoms with instructions to activate the emer-
that were inconsistent with chief concern) gency medical system if these symp-
Temporomandibular ankylosis toms and signs take place.
(temporomandibular joint examination
revealed symptoms that were Case Outcome
inconsistent with chief concern)
Temporomandibular dysfunction At the follow-up visit, the cardiologist ordered
(temporomandibular joint examination blood laboratory tests including a cardiac en-
revealed symptoms that were zyme panel, a resting electrocardiogram, and
inconsistent with chief concern) an exercise tolerance test to aid in ruling car-
I Inflammation diovascular disease less likely. The results of
the blood work indicated that the patient had
Aseptic significantly elevated total and low-density
Capsulitis or synovitis (temporomandibular lipoprotein cholesterol levels. The resting
joint examination revealed symptoms electrocardiogram was normal, but the exer-
that were inconsistent with chief concern) cise tolerance test demonstrated electrocardio-
Septic graphic changes consistent with myocardial
Not applicable ischemia. During the exercise tolerance test,
M Metabolic Mr. K.D. also experienced his typical dull left
Not applicable jaw pain with shortness of breath and both
Va Vascular resolved within 10 minutes of rest. He subse-
quently underwent a coronary angiogram that
Not applicable revealed 75% stenosis of the proximal left
De Degenerative anterior descending artery. A balloon angio-
Osteoarthritis/osteoarthrosis of the plasty with stent placement was performed
temporomandibular joint and the patient was discharged 1 day after the
(temporomandibular joint examination procedure. Mr. K.D. subsequently returned
revealed symptoms that were 3 weeks later to the physical therapy clinic
inconsistent with chief concern) for treatment of his temporomandibular
Tu Tumor dysfunction.
Not applicable
Co Congenital
Not applicable
Ne Neurogenic/Psychogenic
Not applicable
STEP #11: Decide on a diagnostic
impression.
● Rule out angina pectoris secondary
to coronary insufficiency.
1528_Ch10_108-132 11/05/12 3:54 PM Page 108

CHAPTER10
Neck Pain
■ Larry Ho, PT, DPT, OCS ■ Shirley Wachi-See, PT, DPT, OCS

Description of the Symptom ■ Horner’s sign (ptosis, enophthalmos, an-


hidrosis, miosis, and facial flushing)
This chapter describes pathology that may ■ Pain and stiffness associated with fever or
lead to neck pain. Local causes of neck pain recent illness
are defined as pathology occurring within the ■ Positive alar ligament test, Sharp-Purser
vertebral column from the occipital condyle test
to the seventh cervical vertebra, along with ■ Radicular symptoms into bilateral upper
associated joint and soft tissue structures. extremities accompanied by lower extrem-
Remote causes are defined as occurring out- ity symptoms (eg, bilateral or quadrilateral
side this region. The chapter is divided be- paresthesias)
tween anterior and posterior/posterolateral ■ Signs and symptoms of vertebral artery in-
neck pain. sufficiency including dizziness, dysphagia,
dysarthria, dystonia, and disorientation
Special Concerns ■ Signs of upper cervical instability (eg, un-
■ Ataxia or other gait disturbances willingness to actively move head and neck,
■ Clonus, positive Hoffman’s and Babinski
reports of dizziness, nausea, or paresthesias
tests, or hyperreflexia with neck flexion)
■ Drop attacks
■ Syncope
■ Facial paresthesias or muscle paresis

Poste
Posterior
erior

A Anterior B Posterolateral

108
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Chapter 10 Neck Pain 109

CHAPTER PREVIEW: Conditions That May Lead to Neck Pain

NECK PAIN
T Trauma
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Shoulder pathology 115 Blunt trauma to larynx or Brachial plexus/nerve root lesion 118
Thoracic outlet trachea 118 Cervical disk herniation 120
syndrome 116 Cervical dystonia 120
Cervical facet dysfunction 120
Cervicogenic headache 121
Coital neck pain 122
Fractures:
• Lower cervical spine (C3–C7) 124
• Upper cervical spine (C0–C2) 124
Muscle strain 125
UNCOMMON
Internal organ injuries: Digastric and stylohyoid Entrapments:
• Diaphragm muscle strain 122 • Greater occipital nerve (occipital
• Liver neuralgia) 123
• Lung • Spinal accessory nerve 123
• Spleen
Spontaneous
pneumomediastinum 115
T4 syndrome 116
RARE
Stylohyoid syndrome Not applicable Subluxations/dislocations:
(Eagle’s syndrome) 116 • Congenital 128
• Degenerative 128
• Traumatic 128

I Inflammation
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Not applicable Aseptic Aseptic
Not applicable Cervical lymphadenitis 121
Fibromyalgia 123
Septic Rheumatoid arthritis of the cervical
Cervical lymphadenitis spine 127
121
Septic
Not applicable
UNCOMMON
Aseptic Aseptic Aseptic
Not applicable Complex regional pain Ankylosing spondylitis 117
syndrome 122 Chronic fatigue syndrome 122
(continued)
1528_Ch10_108-132 11/05/12 3:54 PM Page 110

110 Chapter 10 Neck Pain

Inflammation (continued)
NECK PAIN

LOCAL LOCAL POSTERIOR AND


REMOTE ANTERIOR POSTEROLATERAL
Septic Septic Complex regional pain syndrome 122
Pericarditis 114 Abscess or cyst Polymyalgia rheumatica 127
Pleurisy 115 infection 117 Systemic lupus erythematosus 129
Septic
Bacterial meningitis 117
RARE
Aseptic Aseptic Aseptic
Not applicable Not applicable Fibromyositis 124
Lyme disease 125
Septic Septic Meningismus (aseptic meningitis) 125
Submandibular space Pharyngoesophageal Pachymeningitis cervicalis
infection (Ludwig’s diverticulum 127 hypertrophica 126
angina) 116 Thyroiditis 129 Transverse myelitis 129
Septic
Cervical osteomyelitis 121

M Metabolic
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Not applicable Not applicable Osteomalacia 126
Osteoporosis 126
UNCOMMON
Medication overuse Not applicable Crowned dens syndrome 122
headache 114 Paget’s disease 126
RARE
Not applicable Not applicable Retropharyngeal calcific
tendinitis 127

Va Vascular
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Acute myocardial Not applicable Not applicable
infarction 113
UNCOMMON
Acute coronary Arteritis 117 Not applicable
insufficiency 113 Hemorrhage 124
Pericarditis 114
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Chapter 10 Neck Pain 111

Vascular (continued)

NECK PAIN
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
RARE
Dissecting aneurysm Dissection of the Arteritis 117
of the thoracic aorta 113 internal carotid artery Epidural hematoma 123
123 Internal carotid arteritis 125
Subarachnoid hemorrhage 128
Subdural hematoma 128
Vertebral artery dissection 130

De Degenerative
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Temporomandibular Not applicable Cervical degenerative disk
dysfunction 129 disease 118
Cervical degenerative joint
disease 118
Cervical stenosis (bilateral and
unilateral) 121
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Riedel’s struma 115 Not applicable Subluxations/dislocations:
• Congenital 128
• Degenerative 128
• Traumatic 128

Tu Tumor
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Malignant Primary, such as: Malignant Primary, Not applicable
• Breast tumor 116 such as:
• Pancoast tumor 117 • Anaplastic carcinoma 129
Malignant Metastatic: • Carcinoma of the
Not applicable esophagus 130
Benign: Malignant Metastatic:
Not applicable Not applicable
Benign:
Not applicable
(continued)
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112 Chapter 10 Neck Pain

Tumor (continued)
NECK PAIN

LOCAL LOCAL POSTERIOR AND


REMOTE ANTERIOR POSTEROLATERAL
RARE
Not applicable Malignant Primary: Not applicable
Not applicable
Malignant Metastatic,
such as:
• Lymphoma 130
Benign:
Not applicable

Co Congenital
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Not applicable Not applicable Cervical stenosis 121
UNCOMMON
Marfan’s syndrome 114 Not applicable Not applicable
RARE
Not applicable Not applicable Subluxations/dislocations:
• Congenital 128
• Degenerative 128
• Traumatic 128

Ne Neurogenic/Psychogenic
LOCAL LOCAL POSTERIOR AND
REMOTE ANTERIOR POSTEROLATERAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Post-traumatic Not applicable
dysautonomic
cephalgia 127
RARE
Not applicable Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Neck Pain through the cervical region. In addition to the


immediate involvement of the underlying
Pain in the cervical spine may involve any of structures, many of the structures and organs
the seven vertebral bodies, eight spinal nerves, in other parts of the body can refer pain to the
23 muscles, or three major vessels passing cervical spine because the cervical spine is an
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Chapter 10 Liver Injury 113

important conduit for innervation and vascu- This condition is a medical emergency due to

NECK PAIN
larization to and from the rest of the body. the risk for significant myocardial damage
Some systemic diseases such as rheumatoid and death.1
arthritis, fibromyalgia, and lupus erythemato-
sus can have profound effects and present with ■ Dissecting Aneurysm
pain in the cervical spine. of the Thoracic Aorta
Chief Clinical Characteristics
Description of Conditions That This presentation may involve severe tearing or
May Lead to Neck Pain crushing pain along the chest, anterior base of
the neck, or interscapular region. Symptoms
Remote may include ischemic neuropathy.
■ Acute Coronary Insufficiency Background Information
Chief Clinical Characteristics Dissecting aneurysms of the ascending aorta
This presentation is mainly characterized by may manifest with symptoms of brain is-
chest pain or tightness, but can present as pain chemia, whereas dissecting aneurysms of the
radiating down one or both arms or as neck or descending aorta typically present with
jaw pain. This constellation of symptoms is symptoms of spinal ischemia. Systolic blood
similar to those experienced for myocardial pressure may drop below 100 mm Hg, and
infarction. heart rate may increase to greater than 100
beats per minute. Risk factors include male
Background Information sex, hypertension, lung disease, arteriosclero-
Temporary occlusion or spasm of the coronary sis, and connective tissue disease. Abdominal
artery causes the onset of symptoms related to computed tomography confirms the diagno-
regional hypoxia of the myocardium. Symp- sis.2 This condition is a medical emergency.
toms may occur at rest or during activity. In
contrast to acute myocardial infarction, pain is INTERNAL ORGAN INJURIES
relieved by administration of nitroglycerin. ■ Diaphragm Injury
This health condition merits urgent consul-
Chief Clinical Characteristics
tation with a physician, particularly during
The presentation of diaphragm injuries typ-
the first onset of symptoms or changes in
ically includes shoulder or lateral neck pain
symptom pattern, due to the risk for hypoxic
with chest pain.
myocardial damage.
Background Information
■ Acute Myocardial Infarction
Diaphragm rupture can occur with blunt or
Chief Clinical Characteristics penetrating trauma to the chest or abdomen.
This presentation often includes chest pain or Deep breathing and changes in position also
tightness, but can present as pain radiating may aggravate the symptoms. The mecha-
down one or both arms or as neck or jaw pain. nism that causes shoulder pain may involve
This condition also may be associated with the phrenic nerve.3 Chest radiographs con-
dyspnea, diaphoresis, pallor, weakness, and firm the diagnosis, with characteristic find-
nausea. ings of irregular diaphragmatic contour or
Background Information elevation, contralateral mediastinal shift,
This health condition occurs when there is and gas collection in the hemithorax. This
an acute disruption of blood flow to the my- condition requires urgent referral for med-
ocardium. Women are more likely than men ical evaluation.
to report neck and jaw symptoms associated
with this condition. Symptoms may be ag- ■ Liver Injury
gravated by exertion but also may occur at Chief Clinical Characteristics
rest; symptoms are relieved neither by rest The presentation of liver injuries can include
nor with administration of nitroglycerin. pain in the midepigastric region or right
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114 Chapter 10 Lung Injury

upper quadrant, with referred pain to the ■ Marfan’s Syndrome


NECK PAIN

superior shoulder, interscapular, and upper Chief Clinical Characteristics


trapezius regions. This presentation may include tall height,
Background Information arachnodactyly, pectus excavatum, hypermobil-
Additional symptoms are right upper quad- ity, and skin stretch marks. It is potentially
rant tenderness and guarding, and signs of associated with anterior neck pain by way of
blood loss such as shock and hypotension. characteristic cardiac pathology.
Liver trauma accounts for 15% to 20% of Background Information
blunt abdominal injuries. Common mecha- Individuals with this rare congenital condition
nisms of injury include motor vehicle have a larger and more fragile aorta; aortic
accidents and receiving an abdominal aneurysm, aortic dissection, aortic regurgita-
blow while fighting. Abdominal computed tion, and mitral regurgitation are common.
tomography confirms the diagnosis.4 This Defects and abnormalities of the heart valves
condition requires urgent referral for med- can cause referred pain to the anterior aspect
ical evaluation. of the neck. This condition involves fibrillin
■ Lung Injury protein deficiency that affects the mesodermal
and ectodermal tissues, resulting in compro-
Chief Clinical Characteristics mised form and function of the heart
The presentation of lung injuries may involve valves, blood vessels, lungs, kidneys, eyes, and
sharp, pleuritic pain in the axilla, shoulder, skeleton.7 Individuals with this condition re-
or subscapular regions with possible referral quire interdisciplinary management due to its
pain to the neck.5 Clinical findings may also cardiovascular, optic, and musculoskeletal
include shoulder-arm pain, Horner’s syn- manifestations.
drome, and neurological deficits affecting the
C8 and T1 nerve roots in the case of apical lung ■ Medication Overuse Headache
involvement. Chief Clinical Characteristics
Background Information This presentation typically involves progres-
Chest radiographs or computed tomogra- sively worsening daily headaches and neck pain
phy confirm the diagnosis. This condition that present on more than 15 days per month
requires urgent referral for medical in the presence of regular overuse of a medica-
evaluation. tion for greater than 3 months.8

■ Spleen Injury Background Information


Headaches due to medication overuse resolve
Chief Clinical Characteristics or revert to their previous pattern within
The presentation of spleen injuries may be 2 months of ceasing the overused medication.
characterized by left shoulder pain possibly Inappropriate use of headache medications—
associated with neck pain. Symptoms are such as ergotamine, triptans, opioids, and
worse with coughing, deep breathing, and simple or combination analgesics—may con-
changes in position. tribute to the development of chronic daily
Background Information headaches.9 Medication overuse headache is a
Blunt trauma injury to the abdomen is the retrospective diagnosis made when the of-
most common cause of this health condi- fending agent is withdrawn and the headache
tion, although delayed splenic rupture after pattern ceases to be daily. This treatment
colonoscopy is becoming a better recognized should be undertaken with the advice of a
presentation. Abdominal distention and physician.
tenderness and hemodynamic instability
may be associated signs. Abdominal com- ■ Pericarditis
puted tomography confirms the diagnosis.6 Chief Clinical Characteristics
This condition requires urgent referral for This presentation typically involves sharp, stab-
medical evaluation. bing anterior chest pain that may radiate to the
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Chapter 10 Spontaneous Pneumomediastinum 115

neck, back, left shoulder, or left supraclavicular corticosteroids, tamoxifen, and levothyroxine.

NECK PAIN
region. Pain is worse with deep inspiration, Surgical resection of the affected thyroid may
coughing, trunk rotation or side-bending, and confirm the diagnosis and assist in the man-
lying supine. It is alleviated by sitting up or agement of this health condition.
leaning forward. This condition may be
associated with a history of recent respiratory ■ Shoulder Pathology
illness, fever, chills, neoplasm, or heart disease/ Chief Clinical Characteristics
myocardial infarction. This presentation typically involves shoulder
pain, instability, and/or crepitus in combination
Background Information
with paracentral neck pain.
Pericardial friction rub may be auscultated.
This health condition commonly occurs in re- Background Information
sponse to viral infection of the pericardium. A variety of conditions in the shoulder can
Chest computed tomography and magnetic res- lead to neck pain, usually through compensa-
onance imaging confirm the diagnosis.10 This tory actions of the pectorals and upper trapez-
condition is a medical emergency due to the ius. These actions also may cause cervico-
risk for cardiac tamponade. brachial neural tissue provocation, resulting in
pseudoradicular symptoms of the upper ex-
■ Pleurisy tremity. These conditions include upper
Chief Clinical Characteristics trapezius strain, levator scapula strain, gleno-
This presentation is mainly characterized by humeral or sternoclavicular osteoarthrosis/
pain in the chest over the affected site, short osteoarthritis, rotator cuff tear, and subacro-
rapid breathing, coughing, dyspnea, and fever, mial impingement. Usual treatment for neck
with pain referred to the shoulder and neck re- pain related to this condition involves address-
gions. Aggravating factors include coughing, ing the underlying shoulder pathology through
deep inspiration, and laughing. use of appropriate surgical or nonsurgical
interventions.
Background Information
This condition involves inflammation of the
■ Spontaneous
pleura. Common causes of pleurisy include in-
Pneumomediastinum
fection, trauma, rheumatoid arthritis, systemic
lupus erythematosus, and tumor. Chest radi- Chief Clinical Characteristics
ographs confirm the diagnosis.11 Treatment This presentation may involve moderate to
commonly involves addressing the underlying severe pain in the anterior neck and chest,
infective agent with antibiotic medication. accompanied by shortness of breath, throat
soreness, or dysphagia. Symptoms are aggravated
■ Riedel’s Struma by deep breathing, coughing, or lying supine, and
Chief Clinical Characteristics they are alleviated by sitting up or leaning
This presentation includes anterior neck pain forward.
in association with a firm mass, hoarseness, or
Background Information
signs of tracheal compression. Women in
This condition is most common in young
the sixth decade of life are most commonly
men. It is caused by the presence of free air
affected.
within the mediastinum due to alveolar rup-
Background Information ture. Violent coughing, acute asthma, or in-
This rare condition occurs due to an idio- halation of illicit drugs may contribute to the
pathic autoimmune process that results in etiology of this condition.13 Treatment in-
fibrosclerosis of the thyroid, impinging the re- cludes analgesics and bed rest. Spontaneous
current pharyngeal nerve, trachea, and other resolution typically occurs within 3 to 5 days,
adjacent structures. Individuals with this condi- but patients suspected of having this health
tion should be monitored for secondary condition should be referred for additional
hypothyroidism.12 Common treatment for medical evaluation emergently to monitor for
this health condition includes management with signs of serious complications.
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116 Chapter 10 Stylohyoid Syndrome (Eagle’s Syndrome)

■ Stylohyoid Syndrome (Eagle’s thoracic functional spinal units was observed


NECK PAIN

Syndrome) to produce beneficial effects in individuals ex-


Chief Clinical Characteristics periencing upper extremity pain.16 This idio-
This presentation can include pain in the pathic health condition is considered to be
upper region of the neck near the angle of the rare. The mechanism of relief is still not clear,
jaw. Symptoms may also occur in the ipsilateral but it is believed that modulation of the auto-
side of the face, ears, throat, temple, and ster- nomic nervous system may be involved.
nocleidomastoid. Pain may be aggravated by ■ Thoracic Outlet Syndrome
swallowing, talking, and turning the head
toward the painful side. Chief Clinical Characteristics
This presentation is characterized by lateral
Background Information neck pain that radiates into the arm and hand
Stylohyoid syndrome can be caused by the accompanied by paresthesias and numbness in
styloid impinging on the carotid vessels. Scar tis- the medial forearm and fourth and fifth
sue as a result of surgery also may be responsi- fingers with possible weakness of the intrinsic
ble. Lidocaine injection to the tip of the greater hand muscles.
horn of the hyoid bone may be necessary. Surgi-
cal shortening or removal of the styloid may be Background Information
necessary to alleviate the symptoms.14 This condition can result from a congenital
cervical rib or other anatomical structure
■ Submandibular Space Infection compressing the neurovascular structures be-
(Ludwig’s Angina) tween the cervical spine and axilla. It also can
Chief Clinical Characteristics be the result of trauma. Thoracic outlet syn-
This presentation is mainly characterized drome is broken down into three subcate-
by pain and edema of the mouth, sub- gories: arterial, venous, and neurogenic. Vas-
mandibular, and anterior neck regions in as- cular forms of this condition are rare and
sociation with sublingual firmness, excessive account for fewer than 5% of all cases. The di-
drooling, fever, malaise, and tachycardia. The agnosis is made clinically and usually requires
significant swelling associated with this con- a combination of tests for both the neurologi-
dition may impair breathing and swallowing cal and vascular components.17 Although a va-
rapidly. riety of surgical and nonsurgical procedures
are available to treat this health condition, the
Background Information first intervention of choice is usually nonsurgi-
This condition is caused by dental disease or cal in patients without significant neurological
infection that progresses to the deep tissues of and vascular compromise.
the anterior neck. Risk factors also include re-
cent dental work, oral piercing, and mandibu- TUMORS
lar fracture.15 The diagnosis is made based on
clinical examination. Treatments include an- ■ Breast Tumor
tibiotic medication, surgical drainage, and Chief Clinical Characteristics
airway access as needed to ensure adequate This presentation involves a nonpainful, pal-
respiration. pable, firm, irregular mass in the breast, and
deep shoulder and neck pain, jaundice, or
■ T4 Syndrome weight loss in individuals with advanced
Chief Clinical Characteristics disease.
This presentation involves pain in the cervical Background Information
spine and upper extremities, associated with The onset of aching pain in the shoulder
paresthesias and unremarkable reflex, sensation, with difficulty sleeping may be an indication
and myotomal strength tests. of metastases to the lymph or osseous struc-
Background Information tures of the shoulder. Risk factors include
T4 syndrome was first described by Maitland, personal and family history of cancer, late-
when mobilization/manipulation of upper onset pregnancy, and early menopause.
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Chapter 10 Bacterial Meningitis 117

Screening breast radiographs may detect early ■ Ankylosing Spondylitis

NECK PAIN
disease. Blood tests for carcinoembryonic Chief Clinical Characteristics
antigen, ferritin, and human chorionic go- This presentation includes an insidious onset of
nadotropin confirm the diagnosis in individ- spinal and symmetric posterior hip pain asso-
uals with metastasis. Treatment may include ciated with a slowly progressive and significant
resection of the tumor and involved axillary loss of general spinal mobility. Symptoms may
lymph nodes, mastectomy, chemotherapy, or be worse in the morning and improve with light
radiation. exercise.
■ Pancoast Tumor Background Information
Chief Clinical Characteristics While lumbar spine involvement is classic, the
This presentation includes sharp, pleuritic cervical spine also may be involved. This con-
pain in the axilla, shoulder, or subscapular dition is more common in males, as well as
regions with referral pain to the neck. Clin- people of American indigenous descent, those
ical findings also include shoulder-arm less than 40 years of age, or those who carry
pain, Horner’s syndrome, and neurological the human leukocyte antigen B27. It also may
deficits affecting the C8 and T1 nerve be associated with fever, malaise, and inflam-
roots.18 matory bowel disease. The diagnosis is con-
firmed with plain radiographs of the sacroiliac
Background Information joints and lumbar spine, which reveal charac-
True Pancoast tumors originate in the ex- teristic findings of sacroiliitis and “bamboo
trathoracic space. However, primary lung tu- spine.” Blood panels including erythrocyte
mors and abscesses in the superior thoracic sedimentation rate are useful to track disease
sulcus also may mimic this condition by in- activity. Individuals with this condition are en-
vading or impinging the subclavian space. couraged to exercise to improve posture and
Risk factors include smoking, exposure to maintain joint mobility in combination with a
secondary smoke, prolonged asbestos expo- regimen of nonsteroidal, steroidal, or biologi-
sure, and exposure to industrial elements. cal anti-inflammatory medications.
Cervical spine radiographs confirm the diag-
nosis.19 Treatment may include a course of
■ Arteritis
radiation therapy prior to surgical resection,
along with medical management of pain and Chief Clinical Characteristics
paraneoplastic syndromes. This presentation typically involves pain and
swelling of the carotid sheath and carotid artery,
Local and is characterized by inflammation of the
arterial wall that may result in occlusion or
■ Abscess or Cyst Infection aneurysm formation. Symptoms may include
Chief Clinical Characteristics general malaise and fever.
This presentation is mainly characterized by
Background Information
pain, tenderness, and swelling anteromedially
Essential hypertension and atherosclerosis are
to the sternocleidomastoid. Skin erythema is
risk factors. Carotid sheath infection may also
also common.
present with Horner’s syndrome and dysfunc-
Background Information tion of cranial nerves IX through XII. Rupture
CT scans may reveal an abscess or cyst in the of an aneurysm may result in bleeding
deep soft tissues of the neck. Suppurative from the nose and mouth.21,22 Individuals sus-
adenitis is the most common etiology. Other pected of this condition require urgent med-
causes include puncture or perforation ical evaluation.
from a foreign body, thrombophlebitis, or
osteomyelitis of the spine. This health condi- ■ Bacterial Meningitis
tion is typically treated with antibiotics, Chief Clinical Characteristics
and the abscess or cyst can be drained or This presentation may include neck pain
excised.20 and stiffness that is aggravated by flexion and
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118 Chapter 10 Blunt Trauma to Larynx or Trachea

rotation movements, associated with headache, of brachial plexus dysfunction may present as
NECK PAIN

fever, nausea, vomiting, photophobia, and cen- a constellation of sensory deficits, motor weak-
tral nervous system dysfunction including altered ness, reflex changes, and the pattern may vary
mental status, seizures, gait disturbances, and with the level of involvement and may present
paresis. Symptoms may progress rapidly or over either unilaterally or bilaterally.
a period of several days.
Background Information
Background Information Pain typically precedes motor and sensory
Bacteria can enter the host via the upper air- signs in brachial plexus injuries. Up to 50% of
way and then invade the subarachnoid space. patients with this condition will eventually
Lumbar puncture and obtaining cultures are present with central cord signs and symptoms.
important for correct diagnosis, as well as to Horner’s syndrome may be present if the
guide medical management.23 Usual treatment stellate ganglion is affected. Computed tomog-
for meningitis includes antibiotics for causes raphy, electromyography, and cervical myelog-
other than viral etiology, antipyretic and anti- raphy confirm the diagnosis.26 Treatment is
seizure agents, and supportive interventions to palliative. Recovery is sometimes incomplete
maintain oxygen and fluids at homeostatic and may take months.
levels.
■ Cervical Degenerative Disk
■ Blunt Trauma to Larynx Disease
or Trachea Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can include pain in the
This presentation is mainly characterized by posterior or posterolateral region of the neck
pain in the anterior neck/throat. Other symp- with or without radiculopathy.
toms can include hemoptysis, hoarseness of the
Background Information
voice when speaking, dysphagia, odynophagia,
This condition is associated with the degener-
and obstruction of the airway.
ative changes of the cervical spine, and most
Background Information commonly affects the C5–C6 and C6–C7
Hyperextension injuries or direct trauma to levels (Fig. 10-1). Patients may develop osteo-
the larynx occur most commonly with motor phytes, degenerative annular tears, loss of disk
vehicle accidents. Penetrating trauma injuries height, narrowing of the spinal canal, or
associated with violent crime are increasing in compression of the spinal cord or nerve roots,
frequency. Other common mechanisms of in- resulting in radiculopathy. The diagnosis is
jury are direct blows from a fist or other object confirmed with plain radiographs and mag-
and attempted strangulation. Physical exami- netic resonance imaging. Treatment is vari-
nation may reveal laryngeal swelling with able; operative intervention may be required
tenderness to palpation, tracheal shift or devi- for individuals with extensive or rapidly pro-
ation, or subcutaneous emphysema. Depend- gressing neurological symptoms, but nonsur-
ing on the mechanism of injury, radiographic gical intervention including rehabilitation is
findings may include pneumomediastinum or typically considered a primary treatment for
pneumothorax. Computed tomography, bron- most individuals with this health condition.
choscopy, and indirect and flexible laryn-
■ Cervical Degenerative Joint
goscopy can be used to assess the injury.5,24,25
Disease
Surgical repair with or without placement of a
stent may be required. Chief Clinical Characteristics
This presentation is characterized by pain in the
■ Brachial Plexus/Nerve Root posterior or posterolateral aspect of the neck. This
Lesion condition occurs in the older population and
usually without radicular symptoms.
Chief Clinical Characteristics
This presentation may involve a traumatic on- Background Information
set of variable symptoms in the cervical spine Osteoarthritis of the zygapophyseal joints,
and the upper extremities. Signs and symptoms hypertrophy of the ligamentum flavum, and
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NECK PAIN
C2-3

C3-4

C4-5

C5-6

C6-7

C7-T1

FIGURE 10-1 Reported referral patterns of the cervical intervertebral disks during discography.
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120 Chapter 10 Cervical Disk Herniation

osteophyte formation around the interverte- rapidly progressing neurological symptoms,


NECK PAIN

bral foramen can all contribute to symptoms but nonsurgical intervention including reha-
(Fig. 10-2).27 Plain radiographs and magnetic bilitation is typically considered a primary
resonance imaging confirm the diagnosis, with treatment for most individuals with this
joint space narrowing in the cervical facet or health condition.
intervertebral joints, subchondral sclerosis of
affected joints, and presence of periarticular ■ Cervical Dystonia
osteophytes and articular hypertrophy. Treat- Chief Clinical Characteristics
ment is variable; operative intervention may This presentation is mainly characterized by
be required for individuals with extensive or anterior or lateral neck pain and rotational
rapidly progressing neurological symptoms, position of the head and neck.
but nonsurgical intervention including reha-
Background Information
bilitation is typically considered a primary
The sternocleidomastoid muscle is contracted
treatment for most individuals with this health
or spastic, causing cervical spine ipsilateral
condition.
side-bending and contralateral rotation to the
■ Cervical Disk Herniation side of involvement. This idiopathic condition
also is known as torticollis. Congenital torti-
Chief Clinical Characteristics
collis occurs in infants, whereas acquired
This presentation involves pain in the poste-
(spastic) torticollis presents itself in adoles-
rior or posterolateral aspect of the neck with or
cents and adults. Certain illicit drugs may
without radicular symptoms down the arm or
cause temporary dystonia. Radiographic stud-
through the midthoracic region.
ies are important to rule out congenital anom-
Background Information alies and to detect unilateral atlantoaxial ro-
A cervical disk herniation can result from tary subluxation. Medications, botulinum
trauma, occur spontaneously in midlife (be- toxin, exercise, and cervical repositioning or-
tween 30 and 40 years of age), or result from thoses may be considered as treatment options
degenerative changes in the geriatric popula- for this health condition.
tion (spondylosis). C5–C6 and C6–C7 are
the most common levels of disk herniation in ■ Cervical Facet Dysfunction
the cervical spine.28 Magnetic resonance Chief Clinical Characteristics
imaging best confirms the diagnosis. Treat- This presentation involves sharp pain in
ment is variable; operative intervention may the posterior or posterolateral aspect of the
be required for individuals with extensive or cervical spine accompanied by ipsilateral range-
of-motion loss in the directions of side-bending
and rotation.
Background Information
This condition is more common in younger
C0-4 and active individuals. Symptoms of this con-
C2-4 C0-3
C3-5 C4-6
dition also may be referred to the shoulder and
C5-7
proximal brachium (see Fig. 10-2). Facet dys-
function implies injury that interrupts normal
joint mobility, possibly associated with motor
vehicle accidents, aggravating sleep positions,
degenerative joint disease, and infection. The
diagnosis is confirmed clinically with in-
creased symptoms during movements that
cause downgliding of the affected facets or
FIGURE 10-2 Referral patterns of the cervical facet with selective joint injections. Treatment for
joints. Cervical facet joints may refer pain to the jaw facet dysfunction includes mobilization or
region, and these symptoms may be associated with manipulation of the affected joints, exercise,
suboccipital headaches. and therapeutic modalities.
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Chapter 10 Cervicogenic Headache 121

■ Cervical Lymphadenitis diagnosis is confirmed with destruction of

NECK PAIN
Chief Clinical Characteristics vertebral bodies and disks on plain radiographs,
This presentation typically includes pain along the nuclear medicine scan, or magnetic resonance
sternocleidomastoid to the jaw with swelling and imaging. Typical treatment involves intra-
tenderness of the lymph nodes. Fever, sore throat, venous antibiotic therapy and may require
and pain with swallowing may also occur. surgical decompression with potential bone
grafting or fusion to stabilize the spine.
Background Information
Common causes are dental disease or infec- ■ Cervical Stenosis
tion, an oral ulceration, and an infection of the Chief Clinical Characteristics
skin of the face or scalp. Most diagnoses can be This presentation is characterized by pain in the
made with a careful history and physical ex- posterior or posterolateral region of the neck
amination that reveals tenderness of involved with or without radiculopathy.
lymph nodes (Fig. 10-3); however, ultrasonog-
raphy or fine-needle biopsy may be neces- Background Information
sary.29,30 Treatment is directed at resolving the Classically, symptoms increase with extension
underlying infection, including antimicrobial and decrease with flexion of the cervical
and antipyretic agents where indicated. spine. Symptoms can be bilateral as in central
stenosis or unilateral in peripheral stenosis.
■ Cervical Osteomyelitis This condition usually occurs in older indi-
Chief Clinical Characteristics viduals, but it may occur much earlier in
This presentation includes posterior, midline individuals with congenital decrease in the
neck pain and progresses to include radiculopathy/ central canal volume. This condition usually
paresthesias and weakness into one or more occurs in more than one spinal level. The
extremities. Loss of bowel and bladder control multilevel involvement in a patient with
follows. Symptoms may be aggravated by move- stenosis results in a variable clinical presenta-
ment and accompanied by low-grade fever. tion of neurological deficits.27 The diagnosis
is confirmed with magnetic resonance imaging.
Background Information Nonsurgical treatment usually is successful to
Risk factors include diabetes mellitus, trauma, address this condition, with surgical inter-
and infection outside the cervical spine. The vention potentially indicated for patients
with extensive or rapidly progressing neuro-
logical deficits.

■ Cervicogenic Headache
Chief Clinical Characteristics
This presentation typically includes unilateral
head pain associated with neck movement; sus-
tained or awkward cervical posture, restricted
cervical range of motion; and ipsilateral neck,
shoulder, or arm pain.31 Cervicogenic headaches
are characterized by moderate to severe episodic
pain that originates in the neck or suboccipital
region and spreads to the head. Pain attacks
last from 3 weeks to 3 months, and may vary in
frequency from occurring every 2 days to
2 months. People with cervicogenic headache
describe symptoms that do not change
sides during an attack,32 and may also present
with nausea, dizziness, and phonophobia or
photophobia that is unresolved with migraine
FIGURE 10-3 Cervical lymph nodes. medications.
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122 Chapter 10 Chronic Fatigue Syndrome

Background Information and may be addressed with a combination of


NECK PAIN

Initial management includes amelioration of avoidance or modification of aggravating ac-


cervical spine musculoskeletal impairments. tivities in combination with nonsteroidal anti-
Nerve blockade may be effective in recalcitrant inflammatory or beta-blocker medications.
cases. A favorable response to this intervention
also is pathognomic.31 ■ Complex Regional Pain Syndrome
Chief Clinical Characteristics
■ Chronic Fatigue Syndrome This presentation can involve a traumatic
Chief Clinical Characteristics onset of severe chronic neck and shoulder pain
This presentation is defined as a new onset accompanied by allodynia, hyperalgesia, and
of unexplained or persistent or recurrent trophic, vasomotor, and sudomotor changes in
physical or mental fatigue that substantially later stages.
reduces activity level and is characterized
Background Information
by postexertional malaise, which requires
This idiopathic health condition is character-
an extended recovery period. Exclusion of
ized by disproportionate responses to painful
other potentially explanatory medical or
stimuli. It is a regional neuropathic pain dis-
psychiatric conditions is required along with
order that presents either without direct nerve
at least one symptom from two of the follow-
trauma (Type I) or with direct nerve trauma
ing categories: autonomic manifestations,
(Type II) in any region of the body.34 Re-
neuroendocrine manifestations, and immune
searchers hypothesize that the sympathetic
manifestations.33
nervous system and immune system may
Background Information support the progressive increase in pain, sensi-
Other possible clinical features include joint tivity, and clinical signs that characterize this
and muscle pain (which may serve as the health condition. This health condition may
chief symptom that directs patients toward precipitate due to an event distant to the
physical therapists for management), diffi- affected area. Thermography may confirm
culty concentrating, tender lymph nodes, associated sympathetic dysfunction. Treat-
headaches, and sleep dysfunction. This ment options are variable for this population,
health condition is diagnosed on the basis and include topical and oral analgesics, antide-
of clinical examination. There is significant pressants, sympathetic nerve block, sympa-
diagnostic overlap with major depression, thectomy, and spinal cord stimulator.
fibromyalgia, and systemic lupus erythe-
matosus. Optimal treatment includes activity ■ Crowned Dens Syndrome
modification and stress management, anaer- Chief Clinical Characteristics
obic reconditioning, and medication for This presentation typically includes moderate
relief of associated symptoms. to severe neck pain and stiffness.

■ Coital Neck Pain Background Information


This condition occurs when hydroxyapatite or
Chief Clinical Characteristics calcium pyrophosphate dihydrate crystals form
This presentation includes a sudden onset a halo or crown around the posterior and lat-
of severe neck and head pain on one or both eral regions of the dens. Computed tomogra-
sides during sexual intercourse. Pain may phy confirms the diagnosis. Neck pain usually
be accompanied by nausea, vomiting, and resolves following treatment with nonsteroidal
anxiety. anti-inflammatory medications.
Background Information
This condition often mimics the symptoms ■ Digastric and Stylohyoid Muscle
of a subarachnoid hemorrhage without neck Strain
stiffness or photophobia, so this health condi- Chief Clinical Characteristics
tion must be excluded as an important differen- This presentation is mainly characterized by
tial diagnosis. This syndrome usually is benign, anterior and/or posterior neck and jaw pain.
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Chapter 10 Fibromyalgia 123

Background Information recalcitrant cases, sectioning of the inferior

NECK PAIN
This condition is commonly observed in oblique muscle may be required.
patients with temporomandibular joint dys-
function, and associated with bruxism, teeth ■ Spinal Accessory Nerve
clenching, and grinding. Management consists Chief Clinical Characteristics
of nonsteroidal anti-inflammatory medica- This presentation typically involves unilat-
tions, physical therapy, night splints, and relax- eral neck and shoulder pain, associated with
ation therapy. altered scapulohumeral mechanics during
elevation and abduction.
■ Dissection of the Internal Carotid
Artery Background Information
Injury to the nerve may occur during surgi-
Chief Clinical Characteristics
cal procedures such as cervical lymph node
This presentation involves a sudden onset of
biopsy or result from trauma such as gun-
knife-like, tearing, unilateral neck pain with
shot or stab wounds. Entrapment can occur
headache and face pain. Horner’s syndrome,
as the nerve travels through the posterior
cranial nerve palsy, hemicrania, and focal neu-
triangle. The diagnosis is confirmed with
rological abnormalities may follow the onset
electromyography, which may demonstrate
of pain. The blood pressure may be lower in
signs of chronic denervation.38 Surgical
one arm, and lying supine usually aggravates
exploration, neurolysis, grafting, or repair
symptoms.
may be required for open injuries.38
Background Information
Dissection may be caused by trauma or may ■ Epidural Hematoma
occur idiopathically. The diagnosis is con- Chief Clinical Characteristics
firmed with Doppler ultrasound, computed This presentation typically includes sudden on-
tomographic angiography, or conventional set of severe headache and/or severe localized
angiography.35,36 Individuals suspected of this neck pain with stiffness. A pattern of loss of
condition should be referred for immediate consciousness, followed by lucidity/alertness,
medical evaluation. then another loss of consciousness is typical,
though not present in all cases. Other symp-
ENTRAPMENTS
toms include dizziness, nausea or vomiting,
■ Greater Occipital Nerve confusion, enlarged pupil, seizures, and focal neu-
(Occipital Neuralgia) rological deficits. Delayed onset of radiating
Chief Clinical Characteristics pain, weakness, and numbness into one or more
This presentation includes unilateral or bilat- extremities can occur and progress to bowel and
eral headache, pain, and paresthesia and bladder incontinence.
hypoesthesia in the distribution of the greater Background Information
or lesser suboccipital nerve with tenderness Patients with ankylosing spondylitis, hyper-
to palpation of the affected area. Nausea, tension, coronary and peripheral vascular dis-
dizziness, or visual disturbances also may be ease, and patients taking anticoagulant drugs
associated with this condition. are at higher risk for developing this condi-
Background Information tion. Individuals suspected of this condition
Myofascial trigger points in the posterior require urgent referral for medical evaluation.
neck and trapezius muscles are common
clinical findings. Symptoms may occur fol- ■ Fibromyalgia
lowing whiplash injury, head trauma, or Chief Clinical Characteristics
with degenerative arthritis of the atlantoax- This presentation is mainly characterized by
ial joint.37 The diagnosis is confirmed with chronic widespread joint and muscle pain de-
selective injections to the suboccipital trian- fined as bilateral upper body, lower body, and
gle. Administration of local anesthetic via spine pain, associated with tenderness to pal-
injection resolves symptoms readily. In pation of 11 of 18 specific muscle-tendon sites.
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124 Chapter 10 Fibromyositis

Background Information during various recreational activities.43 The


NECK PAIN

Individuals with this condition demonstrate diagnosis is confirmed with plain radiographs
lowered mechanical and thermal pain thresh- of the cervical spine.
olds, high pain ratings for noxious stimuli,
Background Information
and altered temporal summation of pain
This condition is considered a medical emer-
stimuli.39 The etiology of this condition is
gency, and patients suspected of this health
unclear; multiple body systems appear to be
condition should be referred for immediate
involved. Indistinct clinical boundaries be-
medical evaluation. All fractures in the
tween this condition and similar conditions
lower cervical spine should be treated as
(eg, chronic fatigue syndrome, irritable bowel
unstable fractures until formally evaluated
syndrome, and chronic muscular headaches)
radiographically.
pose a diagnostic challenge.39 This condition
is diagnosed by exclusion on the basis of clin- ■ Upper Cervical Spine (C0–C2)
ical examination. Common pharmacologic
Chief Clinical Characteristics
interventions include antidepressants, opi-
This presentation includes suboccipital midline
oids, nonsteroidal anti-inflammatory drugs,
tenderness, muscle spasm, and neck pain that
sedatives, muscle relaxants, and antiepilep-
may or may not include neurological signs.
tics. Nonpharmacologic treatments can be
helpful, including exercise, physical therapy, Background Information
massage, acupuncture, and cognitive-behavioral Stable fractures may not present with neuro-
therapy.40 logical compromise, whereas fractures with
concomitant ligamentous rupture may result
■ Fibromyositis in central or peripheral neural compression.
Chief Clinical Characteristics Active range of motion is restricted and
This presentation is characterized by pain in all painful. Common mechanisms of injury in-
areas of the cervical spine, associated with gen- clude compression with flexion, distraction
eralized body pain. with flexion, and compression with exten-
sion. Compression fractures of the upper
Background Information
cervical spine include Jefferson fractures
The term fibromyositis is a combination of
(burst fractures of the ring of C1), which are
fibrositis—inflammation of the fibrous tissue—
clinically significant because they are associ-
and myositis—inflammation in the muscular
ated with neurological deficits. Distraction
tissue.41 Once the myofascial structure is in-
with hyperextension injuries of the upper
flamed, trigger points may develop. Trigger
cervical spine include hangman’s fractures,
points can be quantified with thermography or
which may be associated with cranial nerve,
measurement with pressure algometry or tissue
vertebral artery, and craniofacial injuries.
compliance meters.42 A combination of thermal
Fractures of the dens are generally divided
therapeutic modalities, postural exercises, and
into three categories depending on the
manual therapy procedures is commonly used
anatomical location of the break. Types I
to address this health condition.
and II are stable and do not require surgical
fusion. Type III fractures are unstable, and
FRACTURES rupture of the transverse ligament may cause
■ Lower Cervical Spine (C3–C7) spinal cord compression. This condition is a
Chief Clinical Characteristics medical emergency, and patients suspected
This presentation typically includes midline of this health condition should be referred
tenderness to palpation, neck pain with mus- for immediate medical evaluation.43
cle spasm, crepitus, step-off deformity, and
restricted and painful active range of motion. ■ Hemorrhage
Common mechanisms of injury include mo- Chief Clinical Characteristics
tor vehicle accidents, falls, diving accidents, This presentation involves a rapid onset of
blunt trauma, and accidents that occur unilateral neck pain and swelling.
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Chapter 10 Muscle Strain 125

Background Information treatment for Lyme disease is essential. Treat-

NECK PAIN
This health condition occurs when an artery ment usually includes a course of oral or intra-
ruptures, such as the sequel to an aneurysm. venous antibiotics. Inadequate treatment may
Arterial ruptures causing hemorrhage of the lead to chronic oligoarthritis and chronic dis-
cervical spine may include the internal and turbances.26 Patients who are diagnosed later
external carotid, vertebral, and thyroid arteries. in the course of this health condition carry an
An elevated risk of hemorrhage is associated elevated risk for neurological effects.
with hypertension, trauma, or thrombolytic
therapy. Ultrasonography can be useful in de- ■ Meningismus (Aseptic
termining the source of hemorrhage. This Meningitis)
condition is a medical emergency, and pa- Chief Clinical Characteristics
tients suspected of this health condition This presentation is characterized by neck pain
should be referred for immediate medical and stiffness, sore throat, headache, fever,
evaluation.23 nausea, vomiting, drowsiness, confusion, and
myalgia.
■ Internal Carotid Arteritis
Background Information
Chief Clinical Characteristics
Viral vectors may infect the meninges, causing
This presentation includes pain and swelling
aseptic meningitis syndrome. Other causes in-
of the carotid sheath and carotid artery.
clude fungi and certain types of medication.
Other symptoms include general malaise and
Cerebrospinal fluid analysis and gadolinium-
fever.
enhanced magnetic resonance imaging can
Background Information confirm the diagnosis.45,46 This condition is
Carotid sheath infection may also present with usually self-limiting, and supportive interven-
Horner’s syndrome and dysfunction of cranial tion is typically provided.
nerves IX through XII. Advanced disease may
result in bleeding from the nose and mouth or ■ Muscle Strain
stroke.21,23 Treatment typically involves ad- Chief Clinical Characteristics
ministration of steroidal medications. Individ- This presentation typically includes local pain
uals who are suspected of this health condition that may be present in various regions of the
should be referred for medical evaluation ur- cervical spine dependent on the location of the
gently to begin treatment and prevent the risks injury.
of advanced disease.
Background Information
■ Lyme Disease The mechanism of injury can range from sleep
and postural dysfunction to sports activities
Chief Clinical Characteristics
and motor vehicle accidents. Causes of neck
This presentation can involve fatigue, headache,
muscle strain include orthopedic shoulder
fever, neck stiffness, joint and muscle pain,
pathologies (eg, rotator cuff injuries, impinge-
anorexia, sore throat, and nausea.
ment syndrome, bursitis, and tendinopathy)
Background Information and temporomandibular joint dysfunction.
This condition is caused by a spirochete car- These health conditions can affect the cervical
ried by ticks during the summer months in the spine through faulty posture, inadequate
Western Hemisphere. Neck pain occurs due to shoulder- scapular kinematics, dyskinesia of
a local immune reaction in the cervical spine the shoulder and scapular musculatures, and
that produces both central and peripheral abnormal tension on the cervical spinal
nervous system symptoms. One or more cervi- nerves.47 The diagnosis is confirmed clinically
cal, thoracic or lumbar nerve roots may also be on palpation and resisted testing of the af-
involved. The diagnosis is confirmed by sero- fected muscle. This health condition is ad-
logic testing for Borrelia burgdorferi, preferably dressed nonsurgically with a combination of
by enzyme-linked immunosorbent assay exercise and anti-inflammatory or analgesic
followed by Western blot analysis.44 Early medications.
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126 Chapter 10 Osteomalacia

■ Osteomalacia ■ Pachymeningitis Cervicalis


NECK PAIN

Chief Clinical Characteristics Hypertrophica


This presentation often involves diffuse bone Chief Clinical Characteristics
pain and tenderness of affected bones with pro- This presentation includes posterior neck and
gressive muscle weakness and fracture with arm pain with numbness and weakness in the
minimal trauma. affected extremity.Paresthesias usually involve
three or more cervical nerve roots on the
Background Information
affected side and progress to bilateral upper
This condition is caused by a loss of
extremity involvement.
calcium from the bones, and is common
during pregnancy especially in regions Background Information
where nutrition is suboptimal as well as in Severe cases can progress to spinal cord com-
the urban population of elderly confined.48 pression with paresthesias and weakness in
Deformity may follow mineral loss; bone de- the lower extremities as well. Symptoms oc-
formation and fractures are the source of cur secondary to the lack of filling of the
pain in this health condition. Pseudofrac- dural sleeves surrounding the affected nerve
tures on plain radiographs are pathogno- roots, resulting in thickening of the dura sur-
monic.26 Treatment depends on the underly- rounding the spinal cord and nerve root
ing cause of the disorder and may include sheaths. Causes of this disorder include in-
vitamin D, calcium, and phosphorous fection, trauma, injection of intrathecal
supplementation.49 steroids, and rheumatoid arthritis.52 Surgical
interventions including durotomy may be
■ Osteoporosis considered for individuals with this health
Chief Clinical Characteristics condition.
This presentation typically includes pain
following a fracture of the cervical spine ■ Paget’s Disease
associated with limited bone mineral density in Chief Clinical Characteristics
the affected region. Fractures due to this condi- This presentation can include bone pain,
tion most frequently occur in the hip, spine, bone deformity, fracture, and deafness. 53
and wrist.50 Symptoms are described as continuous and,
unlike osteoarthritis pain, pagetic bone pain
Background Information
usually increases with rest, on weight bear-
Women are four times more likely to be af-
ing, when the limbs are warmed, and at
fected than men. Risk factors for primary os-
night.54
teoporosis include female sex, Caucasian or
Asian descent, early menopause, family his- Background Information
tory, underweight, excessive drinking or Spinal pain is thought to be due to facet
smoking, and overuse of steroids. Fractures arthropathy, engorgement of the vertebral
of the vertebrae due to osteoporosis can body caused by vascular processes, and hyper-
cause severe neck pain, loss of height, and active remodeling.55 Complications of Paget’s
spinal deformity. The diagnosis is confirmed disease can include spinal stenosis or nerve
with plain radiographs and a bone density root compression syndromes and fractures that
test. Treatment includes calcium and vitamin can occur in 10% to 30% of individuals.55 The
D supplementation, bisphosphonates, calci- diagnosis of Paget’s disease is based on an ele-
tonin, estrogen, and parathyroid hormone. vated serum alkaline phosphatase in patients
Exercise programs focus on increasing bone with radiographic findings of osteosclerosis,
mineral density by loading the skeletal sys- osteolysis, and bone expansion. Intervention
tem using weight-bearing exercises and re- usually includes a high-protein diet with vita-
sistance training.51 Improved strength, coor- min C supplementation, bisphosphonates and
dination, and balance may decrease the risk calcitonin, and surgical interventions to ad-
of other fractures by decreasing the likeli- dress the consequences of bone deformation
hood of falls. and fracture.
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Chapter 10 Rheumatoid Arthritis of the Cervical Spine 127

■ Pharyngoesophageal Background Information

NECK PAIN
Diverticulum The mechanism of injury is often postconcus-
Chief Clinical Characteristics sion and may be caused by injury to the sym-
This presentation typically includes a pathetic fibers in the neck. Trauma to the
sensation of a lump in the throat and carotid artery sheath in the anterior triangle of
may be accompanied by the presence of a the neck also may be contributory. Beta-
gurgling sound when the patient drinks blocker medications such as propranolol are
fluids. among primary treatments for this health
condition.57
Background Information
Pharyngoesophageal diverticulum occurs ■ Retropharyngeal Calcific
more frequently in women and the elderly. In Tendinitis
this health condition, the esophageal Chief Clinical Characteristics
mucosa herniates posteriorly, trapping This presentation typically includes neck
food. This can lead to an infection that causes pain and stiffness often associated with dys-
neck pain and odynophagia. Coughing may phagia and odynophagia. Symptom onset
lead to aspiration, resulting in bronchitis or may be spontaneous or associated with
pneumonia. Diagnosis is confirmed with bar- trauma such as whiplash. Aggravating fac-
ium swallow or endoscopic studies. Surgical tors include neck movements in any direction
removal of the infected diverticulum may be and swallowing.
necessary.23
Background Information
■ Polymyalgia Rheumatica Plain radiographs reveal calcification anterior
Chief Clinical Characteristics to the dens in the longus colli muscle, confirm-
This presentation is mainly characterized by ing the diagnosis.58,59 Treatment includes non-
widespread muscle aching and stiffness in the steroidal anti-inflammatory and analgesic
neck and shoulder regions and the low back, medication.
hips, and thighs, possibly associated with
weakness, fever, weight loss, stiffness, muscle ■ Rheumatoid Arthritis
tenderness, and anemia. of the Cervical Spine
Chief Clinical Characteristics
Background Information This presentation is mainly characterized by
The onset of pain can occur very suddenly morning stiffness and generalized pain through-
with symptoms appearing overnight. This out multiple joints in a symmetric distribution,
health condition typically affects people over with possible tenderness and swelling of
the age of 50, and women are more frequently affected joints.
affected than men. This condition is regarded
as a variant of giant cell arteritis, characterized Background Information
by a subacute granulomatous inflammation Women are twice as likely to be affected as
that affects the external carotid arterial sys- men. Symptoms associated with this pro-
tem.26 Corticosteroids are considered among gressive inflammatory joint disease are
primary treatments for this health condition. caused by synovial membrane thickening
This treatment course takes 1 to 2 years to re- and cytokine production in synovial
solve this pathology.56 fluid. Articular cartilage erosion, synovial
hypertrophy, and constant joint effusion 60
■ Post-Traumatic Dysautonomic eventually cause bony erosions and joint
Cephalgia deformities that have a significant impact
Chief Clinical Characteristics on daily function. Younger age of onset is
This presentation may involve headache and associated with a greater extent of disability
neck pain that resemble the classic migraine, but later. Treatment typically includes a combi-
include signs of pupil dilation, sweating, and nation of nonsteroidal, steroidal, or biolog-
transient Horner’s syndrome. ical anti-inflammatory medication and
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128 Chapter 10 Subarachnoid Hemorrhage

supportive rehabilitative intervention to though well-documented sequela of lumbar


NECK PAIN

maintain functional strength and joint punctures.66 Predisposing factors include ad-
motion. vanced age, alcoholism, and coagulation disor-
ders.64 Subdural hematomas form when
■ Subarachnoid Hemorrhage bridging veins rupture and blood accumulates
Chief Clinical Characteristics in the space between the arachnoid and the
This presentation includes severe headache dura. This health condition is a medical emer-
and/or neck pain with nuchal rigidity. Passive gency; individuals suspected of a subdural
and active flexion of the neck increase pain. hematoma should be referred for computed
Additional symptoms include nausea, vomiting, tomography without contrast.
photophobia, drowsiness, confusion, dizziness,
transient loss of consciousness, and enlarged SUBLUXATIONS/DISLOCATIONS
pupils. ■ Congenital
Background Information Chief Clinical Characteristics
Head trauma and intracranial aneurysms This presentation is mainly characterized by
are the most common causes, and the neck pain with or without neurological
incidence of nontraumatic hemorrhage in- deficits.
creases linearly from the age of 25 to 64. Background Information
Ruptured aneurysms and arteriovenous Congenital abnormalities such as Chiari
malformations can lead to this condition. malformations and osteogenesis imperfecta
Hypertension and diabetes mellitus are two can result in instability and subluxation. This
significant risk factors, 61 and polycystic condition is a medical emergency.43,67 Treat-
kidney disease, Ehlers-Danlos syndrome, ment usually involves surgical stabilization
systemic lupus erythematosus, and preg- of the affected region.
nancy are also known risk factors. 62
Polmear63 reported that between 50% and ■ Degenerative
60% of patients with this condition de- Chief Clinical Characteristics
scribed a previous history of an atypical This presentation may include neck pain with
headache days to weeks before the event. or without neurological deficits.
This warning sign is known as a sentinel
headache. Computed tomography and lum- Background Information
bar puncture confirm the diagnosis. This Common causes of degenerative subluxation
condition is a medical emergency.23 include transverse ligament rupture in
patients with rheumatoid arthritis and
■ Subdural Hematoma Down syndrome. This condition is a medical
Chief Clinical Characteristics emergency.43,67 Treatment usually involves
This presentation can involve neck pain surgical stabilization of the affected region.
and unilateral or occipital headache associated
with a decline in level of consciousness ■ Traumatic
and focal neurological deficits. Secondary Chief Clinical Characteristics
symptoms include autonomic signs, vomit- This presentation typically includes neck pain
ing, somnolence, or signs of personality with or without neurological deficits.
change.64
Background Information
Background Information Common causes of traumatic dislocation in-
The location of headache depends on the clude motor vehicle accidents and diving.
location of hematoma. Symptoms develop With traumatic injuries, concomitant disk
within hours to weeks after the precipitating herniation should be suspected and as-
event. This condition may be caused by sessed.43,67 This condition is a medical emer-
severe sneezing, coughing, strain from heavy gency. Treatment usually involves surgical
lifting, and whiplash injury.65 It is also a rare stabilization of the affected region.
1528_Ch10_108-132 11/05/12 3:54 PM Page 129

Chapter 10 Anaplastic Carcinoma 129

■ Systemic Lupus Erythematosus the thyroid. Subacutely, this presentation

NECK PAIN
Chief Clinical Characteristics typically includes dull, aching paratracheal neck
This presentation typically includes neck pain pain that radiates to one or both ears.
associated with fatigue and joint pain/ Other symptoms include sore throat, thyroid
swelling affecting the hands, feet, knees, and enlargement and tenderness, and pain with
shoulders. swallowing.

Background Information Background Information


This condition affects mostly women of child- It is most commonly a result of infection in a
bearing age, but men also may be affected. This cervical thymic cyst and is frequently accom-
condition is a chronic autoimmune disorder panied by fever and dysphagia. Pharyngogra-
that can affect virtually any organ system of the phy or ultrasonography may identify the pres-
body, including skin, joints, kidneys, brain, ence of thyroiditis.23,70 Initial management
heart, lungs, and blood. The diagnosis is con- involves antibiotic medications directed at the
firmed by the presence of skin lesions; heart, underlying infection.
lung, or kidney involvement; and laboratory ab- ■ Transverse Myelitis
normalities including low red or white cell
counts, low platelet counts, or positive antinu- Chief Clinical Characteristics
clear antibody and anti-DNA antibody tests.68 This presentation can include posterior neck
Blood test, urine test, and skin and kidney biop- pain with radiculopathy and paresthesias into
sies confirm the diagnosis. Treatment includes the upper extremities followed by lower
medication, physical therapy, and management extremity weakness and paresthesias.
of associated complications of the skin, lungs, Background Information
kidneys, joints, and nervous system. Flaccid paresis of the extremities develops into
spastic paresis. The pathogenesis of transverse
■ Temporomandibular Dysfunction myelitis is poorly understood; however, spon-
Chief Clinical Characteristics taneous recovery or improvement occurs in
This presentation may be characterized by one-half of all cases. No effective medical in-
headache radiating from the muscles of mas- tervention or therapy exists to act on the
tication, periauricular region, or the tem- pathophysiology of this health condition, al-
poromandibular joint associated with abnor- though corticosteroid medication and early
mal jaw function, asymmetric chewing, mobilization is thought to reduce inflammation
bruxism, neck pain, tinnitus, and vertigo. and enhance the likelihood of neurological
Background Information return.23
Headache pain related to this condition may
be caused by pathology of the temporo- TUMORS
mandibular joint or may be an associated ■ Anaplastic Carcinoma
symptom of another form of headache. This Chief Clinical Characteristics
condition is believed to be an aggravating This presentation may involve formation of
factor in headaches and only the cause if a mass or nodule that is painful and tender
clearly related to the clinical signs and symp- to palpation along the lateral borders of the
toms involving the masticatory system.69 The trachea. Airway compromise may result in
typical management involves a combination stridor.
of rehabilitative and pharmacologic inter-
ventions for headaches related to temporo- Background Information
mandibular dysfunction. This tumor is considered the most locally ag-
gressive and fatal of thyroid tumors. Previous
■ Thyroiditis thyroid pathology and low iodine intake are
Chief Clinical Characteristics among risk factors for development of this
This presentation involves anterior neck pain, health condition. Positron emission tomog-
swelling, and tenderness to palpation of raphy, computed tomography, and magnetic
1528_Ch10_108-132 11/05/12 3:54 PM Page 130

130 Chapter 10 Carcinoma of the Esophagus

resonance imaging can be useful in detecting Background Information


NECK PAIN

the presence of metastases.23,41 Lateral medullary syndrome is the most com-


mon neurological presentation, which in-
■ Carcinoma of the Esophagus cludes ataxia, diplopia, dysphagia, Horner’s
Chief Clinical Characteristics syndrome, and nystagmus. Ultrasound, com-
This presentation typically includes sharp, puted tomographic angiography, and magnetic
burning, or stabbing substernal, anterior neck, resonance angiography can confirm the diag-
and throat pain that can include radiating pain nosis.71 Individuals suspected of this condition
around the thorax to the middle of the back. require urgent referral for medical evaluation
Patients may report melena, odynophagia, to prevent the extension of neurological
and dysphagia. damage.
Background Information
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CHAPTER11
Shoulder Pain
■ Chris A. Sebelski, PT, DPT, OCS, CSCS

Description of the Symptom ■ Abnormal sensorimotor status of the


affected upper extremity
This chapter describes pathology that may lead ■ Warmth and erythema of the shoulder
to shoulder pain. Local causes of shoulder region
pain include pathology that occurs within the ■ Constitutional symptoms associated with
humerus, scapula, and clavicle; acromioclavic- onset of shoulder pain
ular joint, glenohumeral joint, and sternoclav- ■ History of trauma or recent seizure
icular joint; and associated soft tissue struc- ■ Unremitting pain not associated with direct
tures. Remote causes are defined as occurring movement of the shoulder
outside this region. ■ Severe sharp, stabbing pain of several min-
utes in duration
Special Concerns ■ Trauma associated with high irritability
■ Asymmetry in shoulder contour between and inability to move the shoulder
sides, or change in contour of the affected
shoulder, with loss of rotation range of
motion

133
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134 Chapter 11 Shoulder Pain

CHAPTER PREVIEW: Conditions That May Lead to Shoulder Pain


SHOULDER PAIN

T Trauma
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Cervical disk herniation 145 Dislocations: Biceps long tendon tear/
Status post–laparoscopic • Acromioclavicular dislocation 157 rupture 156
procedure 152 • Glenohumeral dislocation 157 Fractures:
Thoracic outlet • Sternoclavicular dislocation 157 • Bankart lesion 158
syndrome 153 Fractures: • Bennett lesion 158
• Bankart lesion 158 • Clavicle 159
• Bennett lesion 158 • Hills-Sachs lesion 159
• Clavicle 159 • Proximal humerus 159
• Hills-Sachs lesion 159 Glenoid labrum tears 159
• Proximal humerus 159 Impingement syndromes:
• Scapula 159 • Subacromial impingement 161
Glenoid labrum tears 159 Joint injuries:
Impingement syndromes: • Acromioclavicular sprain 161
• Subacromial impingement 161 • Glenohumeral
Joint injuries: sprain/subluxation 161
• Glenohumeral sprain/ Muscle strains 162
subluxation 161
Muscle strains 162
UNCOMMON
Internal organ injuries: Joint injuries: Joint injuries:
• Diaphragm injury 149 • Sternoclavicular sprain 162 • Sternoclavicular sprain 162
• Liver injury 149
• Lung injury 149
• Spleen injury 150
RARE
Not applicable Dislocations: Not applicable
• Sternoclavicular
dislocation 157

I Inflammation
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Aseptic Aseptic Aseptic
Rheumatoid arthritis of Adhesive capsulitis (frozen Adhesive capsulitis (frozen
the cervical spine 151 shoulder) 155 shoulder) 155
Bursitis (subdeltoid/ Bursitis (subdeltoid/
Septic subacromial) 156 subacromial) 156
Hepatitis 149 Chronic fatigue syndrome 156 Complex regional pain syndrome
Pleurisy 150 Complex regional pain syndrome (CRPS; also reflex sympathetic
Pneumonia 151 (CRPS; also reflex sympathetic dystrophy, algodystrophy,
Ulcers: dystrophy, algodystrophy, Sudeck’s atrophy) 157
• Duodenal ulcer 154 Sudeck’s atrophy)157 Myofascial pain syndrome 162
• Gastric ulcer 154 Fibromyalgia 158
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Chapter 11 Shoulder Pain 135

SHOULDER PAIN
LOCAL LOCAL LOCAL
LATERAL POSTERIOR SUPERIOR

Dislocations: Fractures: Dislocations:


• Acromioclavicular • Scapula 159 • Acromioclavicular
dislocation 157 Glenoid labrum tears 159 dislocation 157
• Glenohumeral dislocation 157 Impingement syndromes: • Glenohumeral
Fractures: • Internal impingement (posterior dislocation 157
• Proximal humerus 159 glenoid impingement) 160 Glenoid labrum tears 159
Glenoid labrum tears 159 Joint injuries: Joint injuries:
Impingement syndromes: • Glenohumeral • Acromioclavicular
• Subacromial impingement 161 sprain/subluxation 161 sprain 161
Joint injuries: Muscle strains 162 • Glenohumeral
• Acromioclavicular sprain 161 sprain/subluxation 161
• Glenohumeral Muscle strains 162
sprain/subluxation 161
Rotator cuff tear 167

Nerve injuries: Nerve injuries: Nerve injuries:


• Axillary nerve injury 162 • Suprascapular nerve injury 163 • Suprascapular nerve
injury 163

Not applicable Nerve injuries: Not applicable


• Spinal accessory nerve
trauma 163

LOCAL LOCAL LOCAL


LATERAL POSTERIOR SUPERIOR

Aseptic Aseptic Aseptic


Adhesive capsulitis (frozen Adhesive capsulitis (frozen Bursitis (subdeltoid/
shoulder) 155 shoulder) 155 subacromial) 156
Bursitis (subdeltoid/ Bursitis (subdeltoid/ Complex regional pain
subacromial) 156 subacromial) 156 syndrome (CRPS; also
Complex regional pain syndrome Complex regional pain syndrome reflex sympathetic
(CRPS; also reflex sympathetic (CRPS; also reflex sympathetic dystrophy, algodystrophy,
dystrophy, algodystrophy, dystrophy, algodystrophy, Sudeck’s atrophy) 157
Sudeck’s atrophy) 157 Sudeck’s atrophy) 157 Myofascial pain syndrome
Myofascial pain syndrome 162 Myofascial pain syndrome 162 162
Rheumatoid arthritis 165
Tendinopathies:
• Biceps long tendinitis 167
(continued)
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136 Chapter 11 Shoulder Pain

Inflammation (continued)
SHOULDER PAIN

LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Myofascial pain syndrome 162 Tendinopathies:
Rheumatoid arthritis 165 • Biceps long tendinitis 167
• Calcific tendinopathy 168
Septic
Osteomyelitis 164 Septic
Septic arthritis 167 Not applicable

UNCOMMON
Aseptic Aseptic Not applicable
Acute cholecystitis 143 Ankylosing spondylitis 155
Costochondritis 148 Chronic fatigue syndrome 156
Gaseous distention of the Neuralgic amyotrophy
stomach 149 (Parsonage-Turner
Rheumatoid arthritis–like syndrome) 163
diseases of the cervical Polymyalgia rheumatica 164
spine: Rheumatoid–like health
• Crohn’s disease conditions:
(regional enteritis) 151 • Dermatomyositis 166
• Psoriatic arthritis 152 • Polymyositis 166
• Scleroderma 152 • Psoriatic arthritis 166
• Systemic lupus • Scleroderma 166
erythematosus 152 • Systemic lupus
• Ulcerative colitis 152 erythematosus 167
Tietze’s syndrome 153
Septic
Septic Not applicable
Acute viral/idiopathic
pericarditis 144
RARE
Aseptic Aseptic Not applicable
Not applicable Reiter’s syndrome 165
Septic Septic
Cat-scratch disease 144 Ankylosing spondylitis 155
Cervical epidural Skeletal tuberculosis (Pott’s
abscess 145 disease) 167
Cervical lymphadenitis 145
Perihepatitis (Fitz-Hugh–
Curtis syndrome) 150
Subphrenic abscess 153

M Metabolic
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Not applicable Not applicable Not applicable
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Chapter 11 Shoulder Pain 137

SHOULDER PAIN
LOCAL LOCAL LOCAL
LATERAL POSTERIOR SUPERIOR

Tendinopathies: Tendinopathies: • Calcific tendinopathy 168


• Calcific tendinopathy 168 • Calcific tendinopathy 168 • Rotator cuff tendinitis 168
• Rotator cuff tendinitis 168 • Rotator cuff tendinitis 168
Septic
Septic Septic Osteomyelitis 164
Osteomyelitis 164 Osteomyelitis 164
Septic arthritis 167

Aseptic Aseptic Aseptic


Neuralgic amyotrophy Neuralgic amyotrophy (Parsonage- Neuralgic amyotrophy
(Parsonage-Turner Turner syndrome) 163 (Parsonage-Turner
syndrome) 163 syndrome) 163
Polymyalgia rheumatica 164 Septic Polymyalgia rheumatica
Rheumatoid arthritis 165 Not applicable 164
Rheumatoid–like health
conditions: Septic
• Dermatomyositis 166 Not applicable
• Polymyositis 166
• Psoriatic arthritis 166
• Scleroderma 166
• Systemic lupus
erythematosus 167
Septic
Not applicable

Aseptic Aseptic Aseptic


Not applicable Not applicable Not applicable
Septic Septic Septic
Skeletal tuberculosis (Pott’s Skeletal tuberculosis (Pott’s Skeletal tuberculosis (Pott’s
disease) 167 disease) 167 disease) 167

LOCAL LOCAL LOCAL


LATERAL POSTERIOR SUPERIOR

Not applicable Not applicable Not applicable


(continued)
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138 Chapter 11 Shoulder Pain

Metabolic (continued)
SHOULDER PAIN

LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
UNCOMMON
Osteomalacia 150 Hereditary neuralgic Hereditary neuralgic
amyotrophy 160 amyotrophy 160
Heterotopic ossification/
myositis ossificans 160
RARE
Ectopic pregnancy 148 Amyloid arthropathy 155 Not applicable
Gout 160
Pseudogout (calcium
pyrophosphate dihydrate
deposition disease) 165

Va Vascular
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Acute myocardial Not applicable Not applicable
infarction 144
Coronary artery
insufficiency 148
UNCOMMON
Not applicable Avascular necrosis of the Not applicable
humeral head 156
Quadrilateral space
syndrome 165
RARE
Aneurysm 144 Aneurysm 155 Aneurysm 155
Pulmonary embolus 151 Upper extremity deep venous Upper extremity deep venous
Upper extremity deep thrombosis (UEDVT; thrombosis (UEDVT; includes
venous thrombosis includes Paget-Schroetter Paget-Schroetter
(UEDVT; includes Paget- syndrome) 154 syndrome) 154
Schroetter syndrome) 154

De Degenerative
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Cervical osteoarthrosis/ Osteoarthrosis/osteoarthritis: Osteoarthrosis/osteoarthritis:
osteoarthritis 146 • Acromioclavicular joint 164 • Acromioclavicular joint 164
• Glenohumeral joint 164 • Glenohumeral joint 164
Rotator cuff tear 167 Tendinopathies:
• Biceps long tendinosis 168
• Calcific tendinopathy 168
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Chapter 11 Shoulder Pain 139

SHOULDER PAIN
LOCAL LOCAL LOCAL
LATERAL POSTERIOR SUPERIOR

Not applicable Heterotopic ossification/ Hereditary neuralgic


myositis ossificans 160 amyotrophy 160
Heterotopic ossification/
myositis ossificans 160

Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


LATERAL POSTERIOR SUPERIOR

Not applicable Not applicable Not applicable

Avascular necrosis of the Avascular necrosis of the humeral Not applicable


humeral head 156 head 156
Quadrilateral space syndrome 165

Aneurysm 155 Aneurysm 155 Aneurysm 155


Upper extremity deep venous Upper extremity deep venous Upper extremity deep
thrombosis (UEDVT; includes thrombosis (UEDVT; includes venous thrombosis
Paget-Schroetter Paget-Schroetter syndrome) 154 (UEDVT; includes Paget-
syndrome) 154 Schroetter syndrome) 154

LOCAL LOCAL LOCAL


LATERAL POSTERIOR SUPERIOR

Osteoarthrosis/osteoarthritis: Osteoarthrosis/osteoarthritis: Rotator cuff tear 167


• Acromioclavicular joint 164 • Glenohumeral joint 164 Tendinopathies:
• Glenohumeral joint 164 Rotator cuff tear 167 • Biceps long
Rotator cuff tear 167 Tendinopathies: tendinosis 168
Tendinopathies: • Rotator cuff tendinosis 168 • Calcific tendinopathy
• Rotator cuff tendinosis 168 168
• Rotator cuff
tendinosis 168
(continued)
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140 Chapter 11 Shoulder Pain

Degenerative (continued)
SHOULDER PAIN

LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
UNCOMMON
Not applicable Osteoarthrosis/osteoarthritis: Osteoarthrosis/osteoarthritis:
• Sternoclavicular 164 • Sternoclavicular 164
RARE
Not applicable Not applicable Not applicable

Tu Tumor
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Malignant Primary, such as: Malignant Primary, such as: Malignant Primary, such as:
• Breast tumor 153 • Chondrosarcoma 169 • Chondrosarcoma 169
• Pancoast tumor 154 • Lung tumor 169 • Lung tumor 169
Malignant Metastatic: • Osteosarcoma 170 • Osteosarcoma 170
Not applicable Malignant Metastatic, such as: Malignant Metastatic, such as:
Benign: • Metastases, including from • Metastases, including from
Not applicable primary breast, kidney, primary breast, kidney,
lung, prostate, and thyroid lung, prostate, and thyroid
disease 169 disease 169
Benign, such as: Benign, such as:
• Enchondroma 169 • Enchondroma 169
• Lipoma 169 • Lipoma 169
• Osteoblastoma 169 • Osteoblastoma 169
• Osteochondroma 170 • Osteochondroma 170
• Osteoid osteoma 170 • Osteoid osteoma 170
• Unicameral bone cyst 170 • Unicameral bone cyst 170

RARE
Not applicable Not applicable Not applicable

Co Congenital
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable
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Chapter 11 Shoulder Pain 141

SHOULDER PAIN
LOCAL LOCAL LOCAL
LATERAL POSTERIOR SUPERIOR

Osteoarthrosis/osteoarthritis: Not applicable Not applicable


• Sternoclavicular 164

Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


LATERAL POSTERIOR SUPERIOR

Not applicable Not applicable Not applicable

Malignant Primary, such as: Malignant Primary, such as: Malignant Primary, such as:
• Chondrosarcoma 169 • Chondrosarcoma 169 • Chondrosarcoma 169
• Lung tumor 169 • Lung tumor 169 • Lung tumor 169
• Osteosarcoma 170 • Osteosarcoma 170 • Osteosarcoma 170
Malignant Metastatic, such as: Malignant Metastatic, such as: Malignant Metastatic,
• Metastases, including from • Metastases, including from such as:
primary breast, kidney, primary breast, kidney, • Metastases, including
lung, prostate, and thyroid lung, prostate, and thyroid from primary breast,
disease 169 disease 169 kidney, lung, prostate,
Benign, such as: Benign, such as: and thyroid disease 169
• Enchondroma 169 • Enchondroma 169 Benign, such as:
• Lipoma 169 • Lipoma 169 • Enchondroma 169
• Osteoblastoma 169 • Osteoblastoma 169 • Lipoma 169
• Osteochondroma 170 • Osteochondroma 170 • Osteoblastoma 169
• Osteoid osteoma 170 • Osteoid osteoma 170 • Osteochondroma 170
• Unicameral bone cyst 170 • Unicameral bone cyst 170 • Osteoid osteoma 170
• Unicameral bone cyst 170

Not applicable Not applicable Not applicable

Not applicable Not applicable

LOCAL LOCAL LOCAL


LATERAL POSTERIOR SUPERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable


(continued)
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142 Chapter 11 Shoulder Pain


SHOULDER PAIN

Ne Neurogenic/Psychogenic
LOCAL LOCAL
REMOTE GENERALIZED ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Erb’s palsy 158 Erb’s palsy 158
Neuropathic arthropathy
(Charcot-Marie-Tooth
disease) 163
RARE
Not applicable Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Shoulder Pain tumors and infection. Certain anatomical


characteristics of the shoulder girdle place a
The shoulder girdle is composed of the clavi- special premium on muscular activity and
cle, acromion process of the scapula, and the passive restraint provided by ligaments to
proximal humerus. These bones form the the overall stability and optimal function of
acromioclavicular, sternoclavicular, and gleno- this region:
humeral joints. A physiological articulation 1. The only articular connection between the
between the scapula and thorax also exists. shoulder girdle and axial skeleton is the
The position of the shoulder girdle as the first sternoclavicular joint.
major set of articulations of the upper extrem- 2. The head of the humerus also is dispropor-
ity distal to the cervical spine makes cervical tionably large compared to the glenoid fossa.
spine pathology an important remote source
of symptoms that should be considered in These same anatomical characteristics
each patient presenting with shoulder pain. Its also make the shoulder girdle uniquely sus-
relatively proximal position within the body, ceptible to pathology secondary to micro-
proximity to lung and breast tissue, and rich trauma, macrotrauma, and degeneration that
blood supply place it at particularly elevated should be considered if health conditions in
risk for health conditions that are character- other pathological categories are ruled less
ized by hematogenous spread, such as metastatic likely.

Superior Superior
shoulder pain shoulder pain

Lateral
Lateral
shoulder
shoulder
pain
pain
Posterior
Anterior
shoulder
shoulder
pain
pain

A Anterior B Posterior
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Chapter 11 Acute Cholecystitis 143

SHOULDER PAIN
LOCAL LOCAL LOCAL
LATERAL POSTERIOR SUPERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Description of Conditions That health condition without compromise of


May Lead to Shoulder Pain shoulder motion (Fig. 11-1). Fever is typically
present, as well as vomiting, nausea, or visible
Remote jaundice in some cases.
■ Acute Cholecystitis Background Information
Chief Clinical Characteristics Radiation of pain to the area of the right
This presentation involves a gradual onset of pain acromioclavicular joint or tip of the shoulder
that progresses to a very severe level, which is is not as common. On abdominal examina-
mainly located in the right upper quadrant of tion, subcostal tenderness or guarding in the
the abdomen. Pain in the interscapular or right upper quadrant that increases with respi-
scapular region may be associated with this ration or thoracic movement (Murphy’s sign)

Liver Lungs and Lungs and Liver


diaphragm diaphragm
Gallbladder Heart Gallbladder
Heart

A Anterior B Posterior
FIGURE 11-1 Referral patterns of the viscera to the shoulder girdle region.
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144 Chapter 11 Acute Myocardial Infarction

may be present. Surgical removal of the gall- after presumed viral illness. Alleviating positions
SHOULDER PAIN

bladder may be indicated. Although biliary include kneeling with hands on floor, leaning
calculi are considered common, they become forward, or sitting upright. Pericardial friction
symptomatic in 1% to 4% of patients annually. rub may be auscultated. Episodes are longer in
The prevalence of gallstones is higher in duration than that of an acute myocardial in-
women; however, attacks tend to be more farction or anginal pain. Pericarditis may be due
prevalent and severe in men. Individuals with to an acute viral infection or it may be idiopathic
diabetes mellitus appear at elevated risk for in nature. Chest computed tomography and
this health condition.1 The main symptom is magnetic resonance imaging confirm the diag-
caused by obstruction of the gallbladder neck nosis.4 This condition is a medical emergency
by a stone. Abdominal sonography or hepato- due to the risk for cardiac tamponade.
biliary scintigraphy confirms the diagnosis.
■ Aneurysm
Shoulder pain is a common sequela to this
surgical procedure that may be addressed Chief Clinical Characteristics
effectively with injection of a short-term anes- This presentation can include localized
thetic agent into the gallbladder.2 shoulder pain and neuropathic or myopathic
symptoms either with or without functional
■ Acute Myocardial Infarction limitations; the distribution of symptoms de-
Chief Clinical Characteristics pends on the affected artery.5,6 Aortic and carotid
This presentation includes crushing, strangling, artery aneurysms have been documented to give
or stabbing pressure across the chest, which also rise to shoulder pain. Secondary signs may in-
may be present in the anterior or lateral left clude warmth, redness, and swelling.
shoulder that radiates to one or both arms in the
Background Information
T1 dermatome, neck, or jaw (see Fig. 11-1). As-
The potential compression of the brachial
sociated symptoms include diaphoresis, dysp-
plexus or cervical nerve roots or vascular com-
nea, weakness, palpitations, and dizziness.
promise may elicit these associated signs and
Background Information symptoms. Aneurysms or pseudoaneurysms
This severe paroxysmal pain may or may not may be present due to recent surgery, trauma,
be associated with a recent increase of activity. or iatrogenic complications.7 The diagnosis is
It may not be changed by rest, body position, confirmed with arteriography or Doppler ul-
breathing pattern, or with the administration trasound. This condition typically is managed
of nitroglycerin. This condition is a medical with surgical procedures such as stent place-
emergency due to the risk for significant ment, the goal of which is to reinforce the
myocardial damage.3 structural integrity of the arterial wall.
■ Acute Viral/Idiopathic ■ Cat-Scratch Disease
Pericarditis Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically involves a unilateral,
This presentation is mainly characterized by painless, subacute granulomatous lymphadenopa-
sharp and stabbing anterior chest pain that thy that is most common in the axillary lymph
may radiate to the neck, back, left shoulder, or nodes but may present in the cervical or in-
left supraclavicular region with worsening pain guinal lymph nodes. Constitutional symptoms
with deep inspiration, coughing, trunk rota- such as prolonged fever, headache, and malaise
tion or side-bending, and lying supine. The may be present. A slow to heal scratch may also
presentation closely mimics that of an acute be present with a history of recent cat scratch
myocardial infarction with a similar referral within the past several months.8
pattern to the left shoulder and in the T1 der-
Background Information
matome (see Fig. 11-1). This condition is more
Though less frequent in adults, this health
common in young males.
condition often may be initially misdiagnosed
Background Information as breast cancer.9 The diagnosis is confirmed
This condition is typically associated with with lymph node biopsy, blood culture, or
cough, fever, and pain usually 10 to 12 days imaging of the local soft tissues. Treatment
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Chapter 11 Cervical Lymphadenitis 145

may include antibiotic agents with surgical ex- spreading of an infectious organism, such as

SHOULDER PAIN
cision reserved for the most severe cases. Staphylococcus aureus, due to an untreated in-
fection of a skin lesion or intravenous drug
■ Cervical Disk Herniation abuse. This diagnosis is confirmed with mag-
Chief Clinical Characteristics netic resonance imaging, which reveals an
This presentation involves pain in the posterior abnormal region of high signal on T2-weighted
or posterolateral aspect of the neck with or images in the epidural space within the affected
without radicular symptoms down the arm or region of the cervical spine. Treatment typically
through the midthoracic region. Aching pain, includes some combination of intravenous an-
burning, numbness, or tingling radicular pain tibiotic medication and surgical decompres-
at the supraclavicular area (C4; Fig. 11-2A), the sion of the abscess.
anterior shoulder (C5, Fig. 11-2B), superior-
posterior shoulder into the lateral brachium ■ Cervical Lymphadenitis
(C6, Fig. 11-2C), posterior shoulder (C7-C8; Chief Clinical Characteristics
Fig. 11-2D and E) or the axilla (T1) also may This presentation may involve a supraclavicular
be present. Location of the signs and symptoms mass with neck pain radiating to the shoulder.
may be seen to correlate to the dermatome, The presence of a draining fistula is possible.
myotome, or sclerotome of the nerve root or Masses also may appear at other locations in-
adjacent root. Other signs may include muscle cluding the posterior neck and the submandibu-
weakness, muscle wasting, or diminution or lar area.12
loss of reflex response. Aggravating factors could
include neck rotation or compression. Background Information
Cervical lymphadenitis is an infection of
Background Information
the cervical lymph nodes that results in forma-
Cervical disk herniation can result from
tion of a mass. In adults, the mass may be
trauma, occur spontaneously in midlife (be-
secondary to tuberculosis or other form of
tween 30 and 40 years of age), or result from
bacterial infection. The bacterial infection may
degenerative changes in the geriatric popula-
not present with the usual constitutional
tion (spondylosis). C5–C6 and C6–C7 are the
most common levels of disk herniation in the
cervical spine.10 Magnetic resonance imaging
best confirms the diagnosis. Treatment is vari-
able; surgical intervention may be required for
individuals with extensive or rapidly progress-
ing neurological symptoms, but nonsurgical
intervention including rehabilitation is con-
sidered a primary treatment for most individ- Characteristic
C3
uals with this health condition. distribution
C4 of symptoms
■ Cervical Epidural Abscess
Chief Clinical Characteristics C5
Anterior Posterior
This presentation often may include pain in view
C6 view
the axial or appendicular region correspon-
ding to the segmental level of involvement in
the cervical spine and an elevated white cell C7
count. A fever may be present in up to 50% of
T1
cases.11 Sensorimotor signs including poten-
tial bowel and bladder disturbances may be
present.
Background Information
The abscess is usually an infection that gener- Somatosensory
A
ates a pus-filled sac in the epidural space. The findings
most common risk factor is hematogenous FIGURE 11-2 Patterns of cervical radiculopathy.
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146 Chapter 11 Cervical Osteoarthrosis/Osteoarthritis


SHOULDER PAIN

Characteristic
C3 distribution
of symptoms
Characteristic C4
C3 distribution Biceps C5, C6
C5
C4 of symptoms
C6
C5 Deltoid

C6 C7 Wrist extensors C6

T1
C7

T1 Motor findings Motor findings

Biceps tendon Brachio


Radialis C6

Reflex findings
Reflex findings
Anterior Posterior
Anterior Posterior
view view
view view

Somatosensory
findings Somatosensory
B C findings
FIGURE 11-2 cont’d

symptoms, but fever, weight loss, and fatigue pharmacologic management provides optimal
are possible if the condition results from outcomes.13
tuberculosis. This suggests the importance
for the clinician to clarify whether the patient ■ Cervical Osteoarthrosis/
was previously exposed to tuberculosis to en- Osteoarthritis
sure appropriate reporting and containment Chief Clinical Characteristics
in the presence of possible tuberculosis. The This presentation is characterized by pain in the
diagnosis is confirmed with imaging of chest posterior or posterolateral aspect of the neck,
and neck, blood cultures, and biopsy of the which may be related with aching pain, burn-
mass itself. A combination of surgical and ing, numbness, or tingling radicular pain at the
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Chapter 11 Cervical Osteoarthrosis/Osteoarthritis 147

SHOULDER PAIN
Characteristic Characteristic
C3 C3
distribution distribution
of symptoms C4 of symptoms
C4
C5 Finger Thumb
C5 Wrist flexors C8 extension
flexors C7
C6 C6

C7 C7

T1 Finger T1 Motor findings


extensors
C7
Finger flexors C8
Motor findings

Triceps C7

Reflex findings

Anterior Posterior
Reflex findings view view

Anterior Posterior
view view

Somatosensory
E findings

Somatosensory
D findings
FIGURE 11-2 cont’d

supraclavicular area (C4), the anterior shoul- Background Information


der (C5), superior-posterior shoulder into the Osteoarthrosis/osteoarthritis of the zy-
lateral brachium (C6), posterior shoulder (C7), gapophyseal joints, hypertrophy of the liga-
or the axilla (see Fig. 11-2A to E). Location of mentum flavum, and osteophyte formation
the signs and symptoms may be seen to corre- around the intervertebral foramen can all con-
late to the dermatome, myotome, or sclerotome tribute to symptoms (Fig. 11-3).14 Plain radi-
of the nerve root or adjacent root. Other signs ographs and magnetic resonance imaging
may include muscle weakness, muscle wasting, confirm the diagnosis, with joint space nar-
or diminution or loss of reflex response. This rowing in the cervical facet or intervertebral
condition occurs in the older population and joints, subchondral sclerosis of affected joints,
usually without radicular symptoms. and presence of periarticular osteophytes and
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148 Chapter 11 Coronary Artery Insufficiency

■ Costochondritis
SHOULDER PAIN

C0-4
Chief Clinical Characteristics
C2-4 C0-3 This presentation commonly includes dull
C3-5 C4-6 pain with anterior chest wall tenderness that
C5-7 is frequently perceived at the anterior shoul-
der. Tenderness without swelling typically is
focused along the third, fourth, or fifth costo-
chondral joints with possible corresponding
deeper pain at the posterior midscapular
area.16 Shoulder pain may be bilateral but
is less specific. Provocation of anterior chest
FIGURE 11-3 Referral patterns of the cervical pain occurs with shoulder mobility versus
facet joints. Cervical facet joints may refer pain to the shoulder pain limiting mobility. Reproduc-
shoulder region. tion of pain is typically with shoulder abduc-
tion, horizontal adduction, or movements
articular hypertrophy. Treatment is variable; against resistance.
surgical intervention may be required for Background Information
individuals with extensive or rapidly progress- Cervical spine extension and ipsilateral rota-
ing neurological symptoms; otherwise, non- tion may also reproduce the pain. Costochon-
surgical intervention including rehabilitation dritis may be idiopathic or trauma induced,
is considered a primary treatment for most such as with coughing or other overexertion of
individuals with this health condition. the chest wall. Costochondritis is often consid-
ered synonymous with Tietze’s syndrome, al-
■ Coronary Artery Insufficiency though Tietze’s syndrome presents with a
Chief Clinical Characteristics probable aseptic inflammatory etiology with a
This presentation typically involves chest pain hallmark of swelling in the area.16 Each diag-
or tightness, but may also present as radiating nosis is confirmed with clinical examination.
pain into the shoulder or shoulder girdle. Most cases are self-limiting with resolution
Episodes may be accompanied by anginal type within 1 year of onset or cortisone injection
symptoms similar to myocardial infarction, into effected costochondral joint.
such as diaphoresis, dyspnea, palpitations, and
presyncope. Women are more greatly affected ■ Ectopic Pregnancy
than men, especially when older than 50 years, Chief Clinical Characteristics
postmenopausal on hormone replacement This presentation may involve pain that
therapy, and with diabetes mellitus.15 extends from the superior shoulder to the
proximal brachium or axilla, accompanied by
Background Information
hypotensive symptoms including nausea, dizzi-
Shoulder movement will not be specifically re-
ness, fainting, pallor, weak pulse, or other signs
stricted. This health condition occurs due to
of shock or hemorrhage.17
occlusion or spasm of the coronary artery.3 In
contrast to acute myocardial infarction, pain Background Information
from coronary artery insufficiency is relieved This health condition occurs when an ectopic
by administration of nitroglycerin. This health pregnancy ruptures or becomes large enough
condition is a progressive disorder with more that it disrupts surrounding tissues. Some cases
severe cardiac disease to be expected without of ectopic pregnancy present with unilateral or
treatment. The diagnosis is confirmed with re- bilateral shoulder pain due to irritation of the
sponse to stress testing and cardiac imaging. diaphragm from bleeding. A patient with an as-
Treatment may include prevention for those yet unruptured ectopic pregnancy may report
with the greatest risk factors, in combination having vague abdominal pains for several
with pharmacologic and surgical management weeks prior to the rupture. Other possible symp-
to reduce symptoms and ensure adequate toms include missed menstruation and vaginal
cardiac perfusion. spotting. Treatment of this health condition
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Chapter 11 Lung Injury 149

typically includes surgical excision; a ruptured abuse, and recent blood transfusions may assist

SHOULDER PAIN
ectopic pregnancy is a medical emergency. typing of hepatitis. In turn, typing of hepatitis
guides clinical management.
■ Gaseous Distention
of the Stomach INTERNAL ORGAN INJURIES
Chief Clinical Characteristics See Figure 11-1.
This presentation can be characterized by mide-
pigastric abdominal pain, associated with uni- ■ Diaphragm Injury
lateral axilla or anterolateral chest wall pain that Chief Clinical Characteristics
is unrelated to activity or mobility. Alleviating This presentation typically includes lateral
factors may involve belching and antacids. neck or shoulder pain with chest pain.
Background Information Background Information
This health condition occurs when excessive Diaphragm rupture can occur with blunt or
gastric gases cause distention of the stomach to penetrating trauma to the chest or abdomen.
the extent that it irritates the inferior portion of Deep breathing and changes in position also
the diaphragm. In turn, diaphragm irritation may aggravate the symptoms. The mecha-
may result in referred pain to the shoulder. nism that causes shoulder pain may involve
Common risk factors for shoulder pain related the phrenic nerve.18 Chest radiographs con-
to excessive stomach gas include constipation, firm the diagnosis, with characteristic find-
lactose intolerance, and status post–laparoscopy ings of irregular diaphragmatic contour or
procedure. Association of pain with meal habits elevation, contralateral mediastinal shift,
and diet are important clinical considerations. and gas collection in the hemithorax. This
Typical treatment is directed at the underlying condition requires urgent referral for med-
cause of excessive gastric gases, including ical evaluation.
antacid and antibiotic agents.
■ Hepatitis ■ Liver Injury
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation commonly involves tender- This presentation may be characterized
ness and pain in the right upper abdominal by pain in the midepigastric region or right
quadrant, scapular region, and right shoulder, upper quadrant, with referred pain to the
possibly associated with headaches, myalgias, superior shoulder, interscapular, and upper
arthralgias, fatigue, anorexia, nausea, jaundice, trapezius regions. Additional symptoms are
and fever. right upper quadrant tenderness and guard-
ing, and signs of blood loss such as shock and
Background Information hypotension.
Hepatitis refers to a family of pathological con-
ditions that is related to inflammation of the Background Information
liver, consisting of five main types. Patients pre- Liver trauma accounts for 15% to 20% of blunt
senting with hepatitis-related shoulder or mid- abdominal injuries. Common mechanisms of
back pain may be clinically undiagnosed and injury include motor vehicle accidents and
appear healthy (see Fig. 11-1). There is also an fighting. Abdominal computed tomography
acutely progressive type of hepatitis in which confirms the diagnosis.19 This condition re-
the patient may be extremely ill within 2 to 8 quires urgent referral for medical evaluation.
weeks of exposure. Each type of hepatitis,
named for the infectious agent, has specific epi- ■ Lung Injury
demiological characteristics. Treatment varies Chief Clinical Characteristics
by type and involves pharmacologic therapy. This presentation can involve sharp, pleuritic
Depending on the type, hepatitis may be con- pain in the axilla, shoulder, or subscapular
tracted via personal or sexual contact, food, as regions with possible referral pain to the
well as airborne or fecal–oral transmission. As- neck.20 Clinical findings may also include
sessing the patient history regarding the extent shoulder-arm pain, Horner’s syndrome,
of alcohol use/abuse, intravenous drug use/ and neurological deficits affecting the C8
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150 Chapter 11 Spleen Injury

and T1 nerve roots in the case of apical lung bowing of the lower quadrant, and a heart-
SHOULDER PAIN

involvement. shaped pelvis. Presentation of shoulder pain in


isolation of any of the other factors is rare.
Background Information Treatment is directed at the underlying etiol-
Chest radiographs or computed tomography ogy and therefore a consultation with other
confirm the diagnosis. This condition requires health care professionals is necessary for ap-
urgent referral for medical evaluation. propriate management of a patient with this
■ Spleen Injury health condition.
Chief Clinical Characteristics ■ Perihepatitis (Fitz-Hugh–Curtis
This presentation may include left shoulder Syndrome)
pain associated with at least one of the follow- Chief Clinical Characteristics
ing: unilateral neck pain, upper left abdomen This presentation may involve an insidious on-
pain and tenderness, nausea, vomiting, set of general shoulder pain that may extend into
dizziness, and syncope. Symptoms are worse the area of the clavicle. The pain may progress
with coughing, deep breathing, and changes to “sharp” in nature over the course of a few
in position and may be provoked via lying in days versus months. This condition presents
the Trendelenburg position. almost exclusively in females, with concurrent
Background Information symptoms that may include right upper quad-
Symptoms may be delayed. Splenic rupture rant pain, reports of vague abdominal symptoms,
after colonoscopy is becoming more recog- and reproduction of right shoulder pain with
nized as a potential consequence. Abdominal palpation of the right upper quadrant of the
computed tomography confirms the diagno- abdomen. Aggravating movements include
sis.21 This condition requires urgent referral right side-lying, deep breathing, or reports of
for medical evaluation. shortness of breath.

■ Osteomalacia Background Information


Limitations of shoulder range of motion or
Chief Clinical Characteristics tenderness to palpation at the shoulder will not
This presentation can involve an initial clini- be present. In case studies, procedures involv-
cal picture of diffuse, general aching and global ing the liver have produced right shoulder pain
fatigue. Typically affecting the axial skeleton that may be of viscerosomatic referral.23 This
and lower extremities, the patient rarely may condition is an infectious type of perihepatitis
report generalized shoulder region discomfort. and has been noted to be associated with pelvic
Symptoms should be present with the following inflammatory disorders,24 gonococcus, and
risk factors: advanced age, cold geographic area, Chlamydia infections. Treatment typically in-
vitamin D deficiency, gastrectomy, and intestinal cludes pharmacologic management.
malabsorption pathology
Background Information ■ Pleurisy
This diagnosis is rarely made quickly due to Chief Clinical Characteristics
the vague initial presentation. A thorough his- This presentation is mainly characterized by a
tory should query other risk factors including moderate aching pain focused in the chest over
use of medications such as anticonvulsants, the affected site. Associated symptoms include
tranquilizers, sedatives, muscle relaxants, di- short rapid breathing, coughing, dyspnea, and
uretics, and antacids and a medical history of fever with pain referred to the shoulder and
hyperparathyroidism, chronic renal failure, neck regions. Aggravating factors include
and renal tubular defects. Bone and articular coughing, deep inspiration, and laughing.
tenderness may occur in the spine, ribs, pelvis, Alleviating factors include side-lying on the
and proximal extremities including the affected side.
humerus.22 As the disease progresses one
might notice postural deformities, which result Background Information
from the muscular weakness and softening of After several hours, this condition may pro-
the bone, such as increased thoracic kyphosis, gress to a constant pain with any movement
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Chapter 11 Crohn’s Disease (Regional Enteritis) 151

of shoulder/neck. This condition involves in- Long-duration stiffness is especially prevalent

SHOULDER PAIN
flammation of the pleura. Common causes of in the morning.
pleurisy include infection, trauma, rheuma-
toid arthritis, systemic lupus erythematosus, Background Information
and tumor. Chest radiographs confirm the Involvement of the cervical spine as the first
diagnosis.25 Pleurisy is treated by the under- site of pathology is rare. Protective posturing
lying cause; a bacterial infection is treated of the upper extremity in internal rotation
via antibiotics and a viral-based infection is held against the stomach is common. Females
typically monitored without pharmacologic are more affected than males with a peak age
interference. of 35 to 45 years old. Criteria for diagnosis of
this condition includes prolonged morning
■ Pneumonia stiffness (greater than 60 to 90 minutes), soft
Chief Clinical Characteristics tissue swelling, symmetric arthritis, subcuta-
This presentation may involve severe shoulder neous nodules, positive rheumatoid factor, or
pain at the tip of the acromioclavicular joint that radiographic evidence. Treatment should be
is associated with chest pain, cough, sputum tailored to the patient and his or her specific
production, or breathlessness. symptoms, but typically involves steroidal,
nonsteroidal, and biological anti-inflammatory
Background Information medications.
The referred pain to the shoulder is theorized
to be due to irritation of the diaphragm and INFLAMMATORY DISEASES
the C4 sensory axons of the phrenic nerve. ■ Crohn’s Disease (Regional
Diagnosis is via clinical presentation, radiog- Enteritis)
raphy, and blood cultures with the causative Chief Clinical Characteristics
agent typically being bacterial. Treatment is via This presentation may be characterized by
antibiotics and as the pneumonia resolves, generalized aching pain in the shoulder. This
shoulder function should return. symptom is infrequently the initial present-
■ Pulmonary Embolus ing symptom but may be an associated
arthralgia during an acute episode of Crohn’s
Chief Clinical Characteristics disease. Other associated issues may include
This presentation may involve crushing pain erythema nodosum, fever, weight loss,
that may mimic myocardial infarction or arthritis, and complications from long-term
angina-type pain. Typically, the symptoms are corticosteroid use.27
substernal but may present anywhere in the
trunk including the shoulder. It is associated Background Information
with dyspnea, wheezing, and a marked drop in Intestinal symptoms may vary according to
blood pressure.26 the involved area of the gastrointestinal
tract but may include nausea, cramping,
Background Information anorexia, and diarrhea without blood in the
This condition occurs when an embolus of stool. This condition is a subcategory of
clotted blood or less commonly cholesterol inflammatory bowel disease where the in-
plaque becomes lodged in the pulmonary flammation and ulceration occur primarily
vascular tree, resulting in reduced oxygen ex- in the terminal ileum and colon, although
change. Risk factors include immobilization or any portion of the intestinal tract can be
recent surgery. Treatment commonly involves affected.26 The etiology has not been identi-
anticoagulant medications, and this condition fied though it is suspected to include ge-
is considered a medical emergency. netic, microbial, inflammatory, immune,
and permeability abnormalities.26 Treat-
■ Rheumatoid Arthritis ment of the disease process focuses on
of the Cervical Spine pharmacologic, homeopathic, or surgical
Chief Clinical Characteristics management. Management of the patho-
This presentation involves an onset of tran- physiology of this condition decreases the
sitory pain and stiffness in the shoulder. extraintestinal presentations. Therefore, the
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152 Chapter 11 Ulcerative Colitis

therapist should advocate a multidiscipli- associated with fatigue and joint pain/swelling
SHOULDER PAIN

nary approach. affecting the hands, feet, knees, and shoulders.

■ Psoriatic Arthritis Background Information


This condition affects mostly women of child-
Chief Clinical Characteristics bearing age. It is a chronic autoimmune disor-
This presentation may involve an insidious on- der that can affect any organ system, including
set of lumbopelvic and hip pain associated skin, joints, kidneys, brain, heart, lungs, and
with psoriasis. The severity of arthritis is blood. The diagnosis is confirmed by the pres-
uncorrelated with the extent of skin involve- ence of skin lesions; heart, lung, or kidney in-
ment.28 Pitting nail lesions occur in 80% of volvement; and laboratory abnormalities in-
individuals with this condition. Dactylitis, cluding low red or white cell counts, low
tenosynovitis, and peripheral arthritis also platelet counts, or positive antinuclear anti-
are common. body and anti-DNA antibody tests.29
Background Information
Radiographs of the distal phalanges may re- ■ Ulcerative Colitis
veal a characteristic “pencil in cup” deformity. Chief Clinical Characteristics
Blood panels including erythrocyte sedimen- This presentation may include generalized
tation rate are useful to track disease activity. aching pain. This condition is infrequently the
initial presenting symptom but may be an as-
■ Scleroderma sociated arthralgia during an acute episode
Chief Clinical Characteristics or progression of ulcerative colitis. Other as-
This presentation typically includes myalgia, sociated symptoms may include fever, weight
arthralgia, fatigue, weight loss, limited loss, arthritis, hepatobiliary disease, and com-
mobility, and hardened skin about the hands, plications from long-term corticosteroid use.27
knees, or elbows. This condition occurs in Background Information
individuals between 25 and 55 years of age, Intestinal symptoms include blood in the stool,
and is four to five times more likely in women abdominal cramping especially during bowel
than men. Additional symptoms may movements, and episodic diarrhea.27 Ulcera-
include dry mouth and eyes, as well as tive colitis is a subcategory of inflammatory
Raynaud’s phenomenon. bowel disease in which the inflammation and
Background Information ulceration that occur are confined to the colon.
This rare and progressive autoimmune disor- The etiology is unknown but is suspected to
der affects blood vessels and many internal include genetic, microbial, inflammatory, im-
organs, including the lungs and the gastroin- mune, and permeability abnormalities.26
testinal system. Overproduction of collagen in Treatment of the disease process focuses on
this condition eventually leads to poor blood pharmacologic, homeopathic, or surgical
flow in the extremities, which can cause ulcers management. Management of the pathophysi-
in the fingers, changes in skin color, and a dis- ology of this condition decreases the extrain-
appearance of creases in the skin. Continued testinal presentations. Therefore, the therapist
damage to small-diameter vasculature leads to should advocate a multidisciplinary approach.
scar tissue production that impairs joint
range of motion. Blood tests confirm the di- ■ Status Post–Laparoscopic
agnosis. Treatment includes both steroidal Procedure
and nonsteroidal anti-inflammatory medica- Chief Clinical Characteristics
tions and gentle exercise. This presentation can include moderate to
severe shoulder pain that escalates 1 to 3 days
■ Systemic Lupus Erythematosus following a surgical procedure.27
Chief Clinical Characteristics Background Information
This presentation can include lower back pain Theoretically, the pain is generated from
radiating to the hip and groin pain. It is phrenic nerve irritation. Prevention and
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Chapter 11 Breast Tumor 153

resolution of the pain into the shoulder is ■ Tietze’s Syndrome

SHOULDER PAIN
dependent on the surgical procedure and Chief Clinical Characteristics
management postsurgically. This presentation may be associated with an in-
■ Subphrenic Abscess sidious and rapid onset of dull pain with ante-
rior chest wall tenderness that may be perceived
Chief Clinical Characteristics at the anterior shoulder.33 An inflammatory
This presentation involves aching and reaction with noted swelling is present at the
persistent pain at the “tip” of the shoulder second and third costochondral joints with con-
(see Fig. 11-1). Further specific location may comitant irritation at the chondrosternal, ster-
depend on the area inferior to the diaphragm noclavicular, or manubriosternal areas. Tietze’s
that is affected. Abdominal guarding or is aggravated by a history of prolonged cough-
pain will be present.30 Patient may or may ing or overexertion. It is bilateral in 30% of
not have a fever, recent trauma, or surgical cases. Provocation of anterior chest pain occurs
intervention. with shoulder mobility versus shoulder pain
Background Information that limits mobility. Reproduction of pain is
This condition is an area of localized pus infe- typically with shoulder abduction, horizontal
rior to the diaphragm. It may be diagnosed via adduction, or movements against resistance.
imaging. Early intervention by way of surgical Background Information
or nonsurgical management gives the best This syndrome is similar to costochondritis
outcomes. and the two are often considered to be synony-
mous conditions. However, Tietze’s may in-
■ Thoracic Outlet Syndrome volve an aseptic inflammatory etiology. This
Chief Clinical Characteristics condition is equally common in both sexes,
This presentation may be characterized by and commonly presents within the second to
diffuse pain in the shoulder or axilla, neck, arm, fourth decades of life. The diagnosis is con-
or chest. Symptoms may also include paresthe- firmed with clinical examination. The cases
sias, discoloration, numbness, weakness, non- are self-limiting with a relapsing, remitting
pitting edema, and/or fatigue.31 presentation.16 Case studies34,35 now support
the view that malignancy should be considered
Background Information when Tietze’s is considered in the differential
Postural abnormalities, macrotrauma, or diagnosis.
microtrauma from repetitive overhead activ-
ities are possible contributors. These may TUMORS
result in a variety of syndromes in which the ■ Breast Tumor
name of the syndrome is derived from the
hypothetical source of neurovascular entrap- Chief Clinical Characteristics
ment, such as cervical rib syndrome, scalenus This presentation is usually characterized by
anticus syndrome, pectoralis minor syn- initial report of a nonpainful palpable
drome, or first thoracic rib syndrome. This is firm, irregular mass in the breast, which is
a clinical diagnosis with neurological, vascu- the most common presentation of breast
lar, or nonspecific symptoms that may be cancer. Reports of shoulder pain, deep bone
secondary to either upper or lower trunk pain, and jaundice or weight loss are less com-
compression of the brachial plexus and vas- monly seen as the initial presentation.
culature. Multiple clinical exam measures Background Information
have been developed though few are sensitive The onset of aching pain in the shoulder
or specific to diagnose this condition. The with difficulty sleeping may be an indication
90-degree abduction test with external of metastases to the lymph or osseous struc-
rotation appears to have the best predictive tures of the shoulder. Upper extremity
value.32 Surgical intervention is indicated for edema is rarely seen as an initial sign. Blood
those with symptoms that do not respond to tests for the markers (carcinoembryonic
nonoperative treatments. antigen, ferritin, and human chorionic
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154 Chapter 11 Pancoast Tumor

gonadotropin) have been noted as also be present in the midscapular region or


SHOULDER PAIN

elevated in as many as 70% of patients with in the epigastric region.41


metastasis. Risk factors include female with
age greater than 40, personal and family his- Background Information
tory of cancer, late-onset pregnancy, and An ulcer may become more symptomatic,
early menopause. Breast cancer treatments that is, with greater incidence of referred
include needle biopsy, lumpectomy, mastec- pain to the shoulder, following perforation
tomy, and lymph node removal. Radiation into the subdiaphragmatic region. Ulcers de-
and chemotherapy with continued and velop when the lining of the digestive tract is
monitored oral medications follow surgical exposed to acids. Specifically, duodenal ul-
intervention. Early range of motion is rec- cers are caused by Helicobacter pylori bacte-
ommended to prevent limitations on the ria in the stomach. The onset of symptoms
involved side during the postsurgical phase. will coincide when the stomach is empty and
will temporarily resolve with eating or taking
■ Pancoast Tumor of antacids. The reproduction of symptoms
Chief Clinical Characteristics will not be demonstrated through range of
This presentation may involve diffuse, sharp motion in the shoulder or scapula. Treat-
pleuretic pain reported in the ipsilateral ment is typically pharmacologic.
axillary, shoulder, or subscapular region with ■ Gastric Ulcer
possible referred pain to the neck. Each of Chief Clinical Characteristics
these symptoms may be the initial presenting This presentation may most commonly in-
symptom. Clinical findings also include volve aching pain at the “tip” of the on the left
shoulder-arm pain, Horner’s syndrome, and shoulder,42 although this symptom is rare as
neurological deficits affecting the C8 and an initial presentation (see Fig. 11-1). Pain also
T1 nerve roots.36 Risk factors include smok- may be present in the interscapular region, an-
ing, exposure to secondary smoke, prolonged terior chest, or into the facial region.41 The on-
asbestos exposure, and exposure to industrial set of symptoms differs from duodenal ulcers
elements.37 Absence of breath sounds over the in that with a gastric ulcer symptoms may
affected lung and absence of pulmonary air coincide with when the stomach is full rather
in the lobe of the affected lung via radiograph than empty. The reproduction of symptoms will
are primary methods of diagnosis. not be demonstrated through range of motion
Background Information in the shoulder or scapula.
True Pancoast tumors originate in the ex-
trathoracic space with lysis at the ribs evident Background Information
via anteroposterior cervical radiographs.38 An ulcer may become more symptomatic,
The tumor typically also invades the apex of that is, with greater incidence of referred
the affected lung, lower brachial plexus, first pain to the shoulder, following perforation
ribs, vertebrae, and vessels.39 However, pri- into the subdiaphragmatic region. Ulcers de-
mary lung tumors and abscesses in the velop when the lining of the digestive tract is
superior thoracic sulcus also may mimic this exposed to acids. This condition may be sec-
condition by invading or impinging the sub- ondary to bacteria or due to malignancy.
clavian space.38 Palliative radiotherapy Treatment is typically pharmacologic after
may produce pain relief at the shoulder.40 exploring the underlying etiology.
Preoperative radiation, chemotherapy with ■ Upper Extremity Deep Venous
excision, and follow-up treatment are typical. Thrombosis (UEDVT; Includes
Paget-Schroetter Syndrome)
ULCERS
Chief Clinical Characteristics
■ Duodenal Ulcer This presentation may include pain in the
Chief Clinical Characteristics shoulder, neck, or upper extremity. Swelling,
This presentation can involve gnawing, dull, discoloration, tenderness, and venous distention
or aching pain in the supraclavicular area on may be present. It may mimic a muscular strain
the right (see Fig. 11-1). Similar pain may or it may be completely asymptomatic.43
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Chapter 11 Ankylosing Spondylitis 155

Background Information ■ Amyloid Arthropathy

SHOULDER PAIN
Subclavian vessels are the most commonly Chief Clinical Characteristics
affected with axillary and brachial vessels next This presentation involves the presence of the
in the order of frequency.43 Primary forms of “shoulder pad” sign, which is localized anterior
this condition include idiopathic and Paget- swelling at the shoulder. This sign is pathognomic
Schroetter syndrome or “effort” thrombosis. for the rare disorder of amyloidosis. There may
“Effort” thrombosis is typically seen in healthy be associated shoulder pain and functional
young men with overdeveloped scalene muscu- limitation with systemic signs and symptoms of
lature,43 which compresses the subclavian vein fatigue, edema, and weight loss.
during overexertion, leading to thrombosis de-
velopment. Secondary forms of this condition Background Information
account for the majority of episodes. Age Amyloidosis is a deposit of insoluble fragments
greater than 50, diseased state, and a slightly of a protein in tissues and may affect nerves,
elevated risk for females may be included in the muscles, and ligaments. Underlying disease
epidemiology.43 Risk factors include cancer, processes may include renal insufficiency, car-
central venous catheters, anatomical abnormal- diomyopathy, hepatomegaly, peripheral neu-
ities, recent trauma, acquired hypercoagulable ropathy, and autonomic failure.46 Biochemical
states, and spontaneous events. Diagnosis is via analysis of blood is required for diagnosis.
noninvasive techniques. One serious complica-
tion is pulmonary embolus. Suspicion of ■ Aneurysm
thrombosis should be considered an emergent Chief Clinical Characteristics
referral. This presentation can include localized shoulder
pain and neuropathic or myopathic symptoms
Local either with or without functional limitations;
the distribution of symptoms depends on the
■ Adhesive Capsulitis (Frozen affected artery.5,6 Axillary and subclavian
Shoulder) artery aneurysms have been documented to
Chief Clinical Characteristics give rise to shoulder pain. Secondary signs may
This condition presents as insidious and painful include warmth, redness, and swelling.
loss of both active and passive range of motion Background Information
in a capsular pattern. There are two types: if not The potential compression of the brachial
associated with a traumatic event, then it is plexus or cervical nerve roots or vascular com-
considered primary adhesive capsulitis; if asso- promise may elicit these associated signs and
ciated with a traumatic or surgical event, then symptoms. Aneurysms or pseudoaneurysms
it is considered secondary adhesive capsulitis. may be present due to recent surgery, trauma,
Initial presentation mimics subacromial im- or iatrogenic complications.7 The diagnosis
pingement syndrome. is confirmed with arteriography or Doppler
Background Information ultrasound. This condition typically is man-
Females are more affected than males with aged with surgical procedures such as stenting,
the dominant arm most involved. Other asso- the goal of which is to reinforce the structural
ciated factors include age greater than 40, integrity of the arterial wall.
trauma, prolonged immobilization, diabetes, ■ Ankylosing Spondylitis
thyroid disorders, autoimmune disorders, and
stroke/myocardial infarction.44 Diagnosis may Chief Clinical Characteristics
include imaging, exploratory arthroscopic This presentation typically includes an insidious
procedure, and blood panel. Pathology of the onset of shoulder pain associated with a slowly
midshaft of the humerus has been known to progressive and significant loss of general spinal
be associated and must be excluded.45 Etiology mobility. Low back symptoms may be worse in
remains unknown. Staging of the condition is the morning and improve with light exercise.
important for determination of treatment Background Information
though both operative and nonoperative treat- This condition is more common in males,
ment remain controversial. as well as people of American indigenous
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156 Chapter 11 Avascular Necrosis of the Humeral Head

descent, those less than 40 years of age, or Background Information


SHOULDER PAIN

those who carry the human leukocyte antigen Risk factors include recurrent biceps tendini-
B27. It also may be associated with fever, tis, anabolic steroid use, age, and heavy
malaise, and inflammatory bowel disease. lifting.51 Partial ruptures are rare. Gleno-
Shoulder joint arthritis may develop during humeral instability and anterior shoulder
the later stages of the disease in up to 60% of swelling resulting from complete rupture mak-
patients.47 The diagnosis is confirmed with ing the diagnosis more difficult. Ultrasound
plain radiographs of the sacroiliac joints and and magnetic resonance imaging may be uti-
lumbar spine, which reveal characteristic find- lized to assist with diagnosis. Objective exam
ings of sacroiliitis and “bamboo spine.” Blood techniques such as Speed’s test are utilized but
panels including erythrocyte sedimentation are nonspecific.52 Questions regarding activity
rate are useful to track disease activity. level and sport participation may be helpful
in determining management. Nonsurgical
■ Avascular Necrosis management is successful for optimizing the
of the Humeral Head surrounding structures for compensation.
Chief Clinical Characteristics Surgical management is repair of the tendon.
This presentation may include limited motion
at the glenohumeral joint, deep joint pain, and ■ Bursitis (Subdeltoid/
increasing pain with motion and time of weight Subacromial)
bearing. Such symptoms should lead to suspi- Chief Clinical Characteristics
cion of osteonecrosis. Reports of difficulty This presentation typically includes an
sleeping and pain at rest are common.48 insidious onset of pain at the anterior or lateral
Patients are typically asymptomatic at the shoulder. Passive movements are painful espe-
shoulder until the later stages of the disease.49 cially at the end range of motion into abduction,
internal rotation, and horizontal adduction.
Background Information
Active movements typically present with pain
Associated nontraumatic and traumatic
during flexion and abduction.
pathologies include proximal humerus trauma,
sickle cell disease, history of radiation, Gaucher’s Background Information
disease, dysbaric disorders, alcohol intake, corti- Secondary bursitis as a sign of underlying
costeroid use, or a history of systemic lupus ery- shoulder pathologies is more common than
thematosus. The presence of avascular necrosis primary. For primary bursitis, typical treat-
in other joints, especially of the hip, are com- ment is a course of local anesthetic injections.
mon.49 Complete blood screen and plain For secondary bursitis, treatment consists of
radiographs assist with diagnosis. Nonsurgical treatment of the underlying contributing
treatment is often difficult due to the delayed shoulder pathology.
presentation of the patient for treatment.
However, with early diagnosis, rest, immobiliza- ■ Chronic Fatigue Syndrome
tion, nonsteroidal anti-inflammatory medica- Chief Clinical Characteristics
tion, and therapy are recommended. Surgical This presentation is defined as a new onset of
management depends on the severity of the unexplained, persistent, or recurrent physical or
destruction.48,49 mental fatigue that substantially reduces ac-
tivity level, postexertional malaise, and exclu-
■ Biceps Long Tendon Tear/Rupture sion of other potentially explanatory medical or
Chief Clinical Characteristics psychiatric conditions; also requires at least one
This presentation may be characterized by pain symptom from two of the following categories:
in the anterior shoulder in middle-aged to older autonomic manifestations, neuroendocrine
males following heavy lifting or quick onset of manifestations, and immune manifestations.53
an eccentric load. A “pop” may be heard with
weakness of supination and elbow flexion noted. Background Information
Observable distal muscle convexity may be Other possible clinical features include pain,
seen due to retraction of the muscle toward the which may serve as the chief symptom that
remaining attachment.50 directs patients toward physical therapists
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Chapter 11 Sternoclavicular Dislocation 157

for management, and sleep dysfunction. Radiographs are required to confirm the

SHOULDER PAIN
This health condition is diagnosed on the presence of fracture and grade of injury.
basis of clinical examination. There is signifi- Grades I and II require immobilization and
cant diagnostic overlap with major depression, physical therapy. Grade III may require oper-
fibromyalgia, and systemic lupus erythemato- ative or nonoperative treatment with immo-
sus. Optimal treatment includes activity bilization and therapy. Grades IV through
modification and stress management, anaerobic VI require operative management due to
reconditioning, and medication for relief of disruption of muscular structures and sever-
associated symptomatology. ity of displacement of the clavicle.
■ Complex Regional Pain Syndrome ■ Glenohumeral Dislocation
(CRPS; Also Reflex Sympathetic Chief Clinical Characteristics
Dystrophy, Algodystrophy, This presentation may include patients,
Sudeck’s Atrophy) typically male and in their 20s, who may ex-
Chief Clinical Characteristics perience mechanical symptoms along with
This presentation may include a “burning or pain from soft tissue disruption in overhead
throbbing pain.” Symptoms typically manifest activities.
in the distal extremity including escalated
Background Information
pain pattern, swelling, autonomic vasomotor
The injury can occur across the life span,
dysfunction, and impaired upper extremity
though the soft tissues injured differ. In the
function.54
adolescent age group, injuries to the gleno-
Background Information humeral ligament and capsular avulsions are
There are two types of CRPS. Type I is precipi- more common. In the middle-aged group,
tated by a noxious event and Type II occurs rotator cuff pathology is more common.56
specifically from a peripheral nerve injury. The Up to 96% of all dislocations are due to
noxious event or nerve injury may occur in the acute trauma to the shoulder.57 Typically the
shoulder.55 Staging of the disorder is according mechanism is in the anterior direction
to skin changes, pain response, and edema. The (98%) secondary to the mechanism of force-
diagnosis is one of exclusion; therefore, compet- ful abduction and external rotation.58 Other
ing differential diagnoses must be ruled out. mechanisms include a fall on an out-
Treatment is nonsurgical for type I; for type II, stretched arm, traction, or some form of
identification of the underlying peripheral nerve wrenching. Diagnosis is dependent on his-
pathology may indicate surgical intervention.55 tory exam due to intolerance by the patient
for many of the clinical exam procedures
DISLOCATIONS following an acute trauma.59 Imaging may
■ Acromioclavicular Dislocation be necessary to detect associated lesions,
Chief Clinical Characteristics such as impaction fractures of the humeral
This presentation can involve acute onset of head or inferior glenoid surface. Complica-
superior lateral shoulder pain with sharp re- tions include axillary nerve injury or
production during attempts of movement. supraspinatus injury. Outcomes of nonsur-
Observable deformity at the acromioclavic- gical management of unilateral-direction
ular joint and/or clavicle will assist with grad- traumatic dislocation in adolescents are typ-
ing the severity of injury. Mechanisms may ically poor, so surgical intervention is often
include traumatic high-velocity injury in recommended. Multidirectional instability
younger patients or older patients with low to may be managed nonsurgically initially.57
moderate velocity injury, such as a fall on an ■ Sternoclavicular Dislocation
outstretched hand.
Chief Clinical Characteristics
Background Information This presentation typically involves anterior
Grades of injury are from I to VI with pro- shoulder pain coupled with the patient’s pref-
gressive involvement of ligamentous, capsu- erence toward placing his or her affective up-
lar, muscular, bony, and neural structures. per extremity in a protective posture to avoid
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158 Chapter 11 Erb’s Palsy

medial clavicle pain. The neck may be postured unclear; multiple body systems appear to be
SHOULDER PAIN

toward the affected side. Swelling and/or a involved. Indistinct clinical boundaries be-
lump over the joint region is palpated.60 The tween this condition and similar conditions
patient may have difficulty with lying supine (eg, chronic fatigue syndrome, irritable bowel
and on the affected shoulder. Atraumatic dis- syndrome, and chronic muscular headaches)
locations typically occur in young females pose a diagnostic challenge.63 This condition is
with generalized joint laxity.61 diagnosed by exclusion. Treatment will often
include polypharmacy and elements to im-
Background Information
prove self-efficacy, physical training, and
The mechanism is typically from an acute
cognitive-behavioral techniques.64
injury such as a sports injury or motor ve-
hicle accident. Though both are rarely seen, FRACTURES
anterior dislocation is of greater prevalence
■ Bankart Lesion
than posterior. Posterior dislocation must
be treated as an emergent situation due to Chief Clinical Characteristics
the proximity to neurovasculature struc- This presentation may be asymptomatic or
tures. Once effectively diagnosed via imag- minimally symptomatic with overhead mo-
ing, intervention for a mild to moderate tions. The patient more typically presents with
displacement is typically a sling and protec- symptoms of glenohumeral instability second-
tive rest. For dislocations, the decision of ary to repetitive microtrauma or dislocation.
closed reduction versus surgical interven- Background Information
tion may be dependent on the direction of Bankart lesion is a capsular avulsion of the
the dislocation. anterior and inferior portions of the labrum.
■ Erb’s Palsy It is strongly associated with traumatic dislo-
cations. A “bony” Bankart lesion is a disrup-
Chief Clinical Characteristics tion of the bone of the inferior glenoid rim
This presentation may involve shoulder pain following a dislocation.56 Imaging is neces-
secondary to a known neurological injury, sary to diagnose this condition. Repair is
possibly occurring at birth, resulting in signif- considered if surgical intervention is neces-
icant weakness of the shoulder musculature. sary for the treatment of the shoulder dislo-
Adaptive posturing will be present typically of cation or instability.
30 degrees of abduction, flexion, 60 degrees of
humeral internal rotation, and potential elbow ■ Bennett Lesion
flexion contracture.62 Pain and limited function Chief Clinical Characteristics
will be present due to secondary osteoarthritis. This presentation more commonly presents in
Background Information patients who are male, long-term throwers
Resolution of deteriorating function via shoul- (ie, baseball pitchers) with posterior shoulder
der arthrodesis is common. pain during forced extension or with full
external rotation and abduction. Pain is
■ Fibromyalgia absent in daily activities.
Chief Clinical Characteristics
Background Information
This presentation involves chronic widespread
Failure of nonsurgical management is typi-
joint and muscle pain defined as bilateral up-
cal. Diagnosis is difficult because this lesion
per body, lower body, and spine pain, associated
may be asymptomatic. Etiology is unknown
with tenderness to palpation of 11 of 18 specific
but the biomechanics and mechanical
muscle-tendon sites.
stresses of the throwing arm are suspected. A
Background Information local anesthetic test with lidocaine injection
Individuals with this condition will demon- into the lesion via fluoroscope is assistive in
strate lowered mechanical and thermal pain determining symptomatic contribution of
thresholds, high pain ratings for noxious the lesion to the shoulder pain. Surgical
stimuli, and altered temporal summation of management with full return to sports
pain stimuli.63 The etiology of this condition is should be expected.65
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Chapter 11 Glenoid Labrum Tears 159

■ Clavicle that of a fall.67 Incidence increases with age,

SHOULDER PAIN
Chief Clinical Characteristics and women are more affected than men.68
This presentation includes immediate pain, Background Information
inability to move shoulder and possibly the Dependent on the severity of the fracture,
neck with swelling, and observable deformity. there may not be observable deformity, ne-
Tenting of the skin is commonly seen.66 cessitating plain radiographs for diagnosis.
Background Information Neer’s classification system assists with deter-
In adults, clavicle fractures account for mining severity of injury and therefore the
~10% of all trauma. Grading of the severity appropriate nonsurgical or surgical manage-
of a clavicle fractures is dependent on the de- ment.69 Complications include neurovascular-
gree of displacement, number and severity of associated injuries.
the involved ligaments, and the presence of ■ Scapula
acromioclavicular joint involvement. Open
Chief Clinical Characteristics
clavicle fractures are uncommon with the
This condition commonly presents as poste-
risk of pneumothorax at ~3% with high-
rior shoulder pain in patients who hold their
energy mechanisms of injury. Radiographs
involved upper extremity in adduction with
are necessary for appropriate diagnosis.
severely restricted motion. Weak rotator cuff
Treatment is dependent on the grade of the
function may be present, yet tenderness is
fracture with complications including de-
localized to the scapula. Edema and ecchymo-
layed healing and nonunion. The majority of
sis may be absent. Pain may be present with
clavicle fractures are nondisplaced and man-
inspiration due to respiratory muscular attach-
aged nonsurgically via a sling or figure-eight
ments. A flattened shoulder is typical of a
harness with therapy to follow the period of
displaced glenoid neck or acromial fracture.70
immobilization.
Background Information
■ Hills-Sachs Lesion These uncommon injuries are typically seen
Chief Clinical Characteristics in males from 20 to 40 years of age due to
This presentation involves shoulder instabil- direct trauma with considerable energy. This
ity; the lesion itself is typically asymptomatic. condition is often overlooked on initial eval-
Poorly localized shoulder pain, either anterior uation, but clinicians are advised that the
or posterior, is present, especially with late- presence of a scapular fracture should raise
phase cocking or the early acceleration phases the suspicion of associated injuries if not
of throwing or overhead activities. already evident.70 Complications include
pneumothorax, which may be delayed up to
Background Information
3 days,65 and associated fractures including
Occurring in approximately 80% of trau-
of the skull and brachial plexus. Nonsurgical
matic dislocations, this condition is a
treatment is still preferred despite the ad-
compression fracture of the posterolateral
vances with open reduction internal fixation
corner of the humeral head created when
procedures.
the humeral head passes over the lip of the
glenoid.56 The lesion contributes to insta- ■ Glenoid Labrum Tears
bility if greater than 30% of the articular
Chief Clinical Characteristics
surface is involved.57 If nonsurgical man-
This presentation may include poorly localized
agement fails, then several surgical options
anterior or posterior pain in the shoulder that
are available.
may range from constant to episodic and
■ Proximal Humerus sharp.51 It is typically activity-related shoulder
pain that increases with overhead motion.
Chief Clinical Characteristics
This presentation may be characterized by Background Information
pain and bony tenderness in the axilla, crepi- The most common is a superior labrum an-
tus, and significant limitation of range of teroposterior tear of the labrum. History from
motion. The mechanism of injury is typically the patient should include a description of
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160 Chapter 11 Gout

compressive or traction force on the shoulder. Background Information


SHOULDER PAIN

A history of trauma or repetitive overhead This condition is a rare autosomal dominant


motion also may be indicated as the mecha- disorder that may affect entire families.72 Full
nism of injury. The labrum demonstrates in- recovery is expected between episodes; how-
creasing degenerative changes with advancing ever, it is not uncommon to see residual weak-
age.51 It is speculated that there is a strong re- ness and atrophy. Two types of this condition
lationship between underlying glenohumeral have been reported: “classic” relapsing-remit-
instability and labral tears. A variety of clinical ting or chronic undulating with exacerba-
examination procedures have been reported tions. Symptoms may be bilateral or unilateral
but an ideal test remains elusive. Imaging is lasting up to 6 months. Treatment involves
utilized to assist with diagnosing labral tears. self-monitoring.
Nonsurgical treatment is successful with the
identification of the underlying associated ■ Heterotopic Ossification/Myositis
pathologies. Surgical intervention is common Ossificans
if nonsurgical management is unsuccessful. Chief Clinical Characteristics
This presentation may commonly include
■ Gout pain with limited passive and active range
Chief Clinical Characteristics of motion with a history of trauma and/or
This presentation may include mechanical im- immobilization.
pingement signs, such as sharp pain at the Background Information
anterior shoulder with movement from 90 to Development at the proximal humerus is not
120 degrees humeral abduction, limited overhead atypical following shoulder arthroplasty,
movement, and possible associated rotator cuff but there is significantly less risk than that
tendinitis.66 Typical patient presentation: male, which is found after lower extremity total joint
40 to 60 years old, obese, probable excessive surgery.73 Risk factors for development follow-
alcohol intake, with history of gout attacks ing arthroplasty may include rotator cuff
in other joints. Fever is infrequently present. tears.73 The development of clavicle hetero-
Local warmth and edema may be present. topic ossification after acromioplasty or resec-
Background Information tion is associated with patients with a history
Gout is the presence of monosodium crystals of chronic pulmonary disease.74 Surgical man-
within the joint or soft tissue. Synovial fluid agement is dependent on level of functional
analysis is critical for an intra-articular differ- limitation.
ential diagnosis. Diagnosis of a soft tissue
IMPINGEMENT SYNDROMES
lesion is more challenging and may require
imaging. This is an unusual presentation of ■ Internal Impingement
shoulder pain because tophaceous gout is (Posterior Glenoid
more common in the finger, carpal tunnel, and Impingement)
spine. Management is through urate lowering Chief Clinical Characteristics
therapy which includes pharmacology, diet This presentation includes posterior shoul-
modifications, and treatment of associated im- der pain and less commonly joint line pain
pairments due to pain and secondary shoulder associated with repetitive positioning of
impingement.71 the shoulder into an abducted and externally
rotated position. Patients are typically athletes
■ Hereditary Neuralgic Amyotrophy under the age of 40; though there are case re-
Chief Clinical Characteristics ports of internal impingement in the non-
This presentation may involve recurrent sudden athletic population.75 Progression of symptoms
episodes of pain and weakness in the shoulder may be acute onset (more common with non-
girdle. Patients with this condition are typi- throwers) or insidious (more common with
cally ages 20 to 40 years. Episodes may be throwers). Jobe76 classified three stages for
brought on by physical exertion, viral illness, or presentation: Stage I, reports of stiffness with
extreme stress. pain occurring specifically during the late
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Chapter 11 Glenohumeral Sprain/Subluxation 161

cocking and early acceleration phases; JOINT INJURIES

SHOULDER PAIN
stage II, progression to significant posterior ■ Acromioclavicular Sprain
joint-line pain with activity; and stage III,
failure to improve with conservative means. Chief Clinical Characteristics
This presentation involves an acute onset
Background Information of superior lateral shoulder pain with sharp
The etiology of pain is theorized as being reproduction during attempts at movement.
multifactorial and complex with impinge- Observable deformity at the acromioclavic-
ment of the undersurface (deep layer) of the ular joint and/or clavicle will assist with
posterior supraspinatus tendon and/or grading the severity of injury. Mechanisms
the anterior infraspinatus tendon by the may include traumatic high-velocity injury in
posterosuperior glenoid margin. Differential younger patients or older patients with low-
diagnosis should include cervical radiculopa- to moderate-velocity injury, such as a fall on
thy, neurological conditions, tendinopathy an outstretched hand.
of rotator cuff musculature, and shoulder Background Information
instability.77 Grades of injury are from I to VI with pro-
gressive involvement of ligamentous, capsu-
■ Subacromial Impingement
lar, muscular, bony, and neural structures.
Chief Clinical Characteristics Radiographs are required to confirm the
This presentation may be characterized in presence of fracture and grade of injury.
the earliest stages as sharp episodic pain at the Grades I and II require immobilization and
anterolateral acromion with radiating pain physical therapy. Grade III may be operative
to the midlateral humerus. In the later stages, or nonoperative treatment with immobiliza-
the pain is “toothache like” and the limitations tion and therapy. Grades IV through VI
in physical activity are more apparent.77 require operative management due to dis-
Affected demographics are variable for ruption of muscular structures and severity
age, severity of functional limitations, and of displacement of the clavicle.
pain reports.
■ Glenohumeral Sprain/
Background Information Subluxation
With any stage, mechanism is typically of an
Chief Clinical Characteristics
insidious onset though traumatic onset is
This presentation may involve vague shoul-
possible. Resting pain is not common. Night
der pain, especially with overhead activities,
pain is exacerbated by lying on the shoulder
due to the disruption of the soft tissue struc-
or sleeping with arm overhead and is allevi-
tures. If the mechanism is secondary to
ated by position changes. Overhead activities
neurological injury, then the presentation
especially with movements into the frontal
may be of generalized shoulder pain, even
plane are commonly limited with an im-
at rest.
pingement arc present: pain is experienced
during 70 to 120 degrees of humeral motion. Background Information
Pain is generated when the structures be- Subluxation is described as minor disrup-
neath the subacromial arch become com- tion of the joint where the articular surface
pressed within the subacromial space. remains intact and soft tissues are dis-
Causative factors may include rotator cuff rupted.56 Mechanisms may include trauma
pathology, degenerative changes of the to the joint in the younger athletic popula-
acromioclavicular joint, or improper tempo- tion or from sustained hemiplegia or spastic-
ral sequencing of the scapulothoracic muscu- ity after a stroke. Differential diagnosis
lature. Differential diagnosis should include should include dislocation, labral pathology,
cervical radiculopathy, neurological condi- axillary nerve injury, and rotator cuff pathol-
tions, tendinopathy of rotator cuff muscula- ogy. Imaging is utilized to exclude associated
ture, and shoulder instability.77 Nonsurgical pathologies including Bennett, Bankart,
treatment is commonly successful. or superior labrum anterior to posterior
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162 Chapter 11 Sternoclavicular Sprain

lesions. Treatment is determined by the ■ Myofascial Pain Syndrome


SHOULDER PAIN

underlying mechanism of the subluxation Chief Clinical Characteristics


and may vary significantly from neuromus- This presentation may include diffuse, deep
cular stimulation to the neurologically aching or soreness in the axioscapular
impaired shoulder to strengthening activities muscles. Also included in the presentation are
overhead for the athletic shoulder. palpable tender taut bands in the muscles
■ Sternoclavicular Sprain and trigger points, paresthesias in the area of
trigger points, and weakness of the affected
Chief Clinical Characteristics axioscapular muscle.
This presentation may include acute onset
of medial clavicular pain with possible Background Information
production of sharp pain with attempts Affected muscles may be sensitive to prolonged
at ipsilateral shoulder girdle motion, side- activation or passive stretching, creating adap-
lying on the affected side, and lying supine. tive shortening over the course of the pathol-
The neck may be postured toward the affected ogy.78 Differential diagnosis should include
side. Swelling and/or a lump over the joint fibromyalgia. Treatment is injection of saline,
region may be palpated.60 anesthetic, or Botox into the offending trigger
Background Information point.79
The mechanism is typically a sports injury.
NERVE INJURIES
Once effectively diagnosed via imaging,
intervention for a mild to moderate dis- ■ Axillary Nerve Injury
placement is typically a sling and protec- Chief Clinical Characteristics
tive rest. This presentation can involve shoulder pain
with abduction and external rotation or low
■ Muscle Strains endurance with overhead activities.80 Dener-
Chief Clinical Characteristics vation to the teres minor and the deltoid and
This presentation involves localized pain sensation to the lateral shoulder may or may
that is dependent on the location of the muscle not be spared.
affected. Limited active range of motion
will be present and may be correlated with the Background Information
action of the injured muscle. Tenderness with The axillary nerve is most commonly in-
palpation and muscular inhibition upon isomet- jured during an anterior dislocation of the
ric contraction may be present. glenohumeral joint, via fracture of the
proximal humerus, due to blunt trauma to
Background Information the anterolateral deltoid or as a complica-
The mechanisms of injury include postural tion of shoulder surgery.81 Neurological
dysfunction, repetitive motion, sports activi- injury is more common in patients greater
ties, or motor vehicle accidents. Examination than 50 years of age or if the shoulder
of proximal structures is necessary to ensure a remains dislocated for greater than
thorough comprehension of the etiology due 12 hours.82 The axillary nerve is one of the
to the association between the axial and the structures implicated in quadrilateral space
scapular musculature. Occurrence in any age syndrome secondary to proposed fibrous
group is possible in the event of a recent, finite bands constricting the axillary nerve or less
mechanism of injury. Common sites of muscle commonly from a space-occupying le-
strain include the levator scapula, pectoralis sion.80 Differential diagnosis should in-
major and minor, rotator cuff, and upper clude neuralgic amyotrophy, quadrilateral
trapezius. The diagnosis is confirmed clinically space syndrome, and cervical radiculopa-
on palpation and resistance testing of the thy. For atraumatic injury of the axillary
affected muscle. This health condition is nerve, nonsurgical management should be
addressed nonsurgically with a combination of attempted for 3 months while awaiting
exercise and anti-inflammatory or analgesic the return of muscle function.80 If there is
medications. no clinical or electromyographic evidence
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Chapter 11 Neuropathic Arthropathy (Charcot-Marie-Tooth Disease) 163

of axillary nerve recovery by 3 to 6 months, ■ Neuralgic Amyotrophy

SHOULDER PAIN
then surgical intervention is recommended.82 (Parsonage-Turner Syndrome)
■ Spinal Accessory Nerve Trauma Chief Clinical Characteristics
This presentation can involve sudden acute on-
Chief Clinical Characteristics set of burning and severe pain at the shoulder
This presentation may commonly involve and into the upper arm with unknown etiology.
shoulder pain, scapular winging, depressed This pain resolves and precedes the weakness in
shoulder girdle, and limitations in shoulder the shoulder and shoulder girdle due to dener-
range of motion. These characteristics are vation of the muscles.85
typical following traumatic disruption of the
spinal accessory nerve affecting the function Background Information
of the trapezius.83 The deltoid, infraspinatus, and supraspinatus
are typically affected with serratus anterior in-
Background Information volvement as noted by scapular winging. Pro-
Mechanisms of injury include direct trauma gression of symptoms may be within days or
to the nerve, shoulder, or neck or neurapraxia weeks. Relatively uncommon, this health condi-
from surgical intervention. Manual muscle tion affects males more often than females and
testing and electromyographic testing of the occurs primarily between 20 and 60 years of age.
trapezius muscle will reveal weakness. Asso- Acute brachial neuritis is of unknown etiology,
ciated symptoms may include subacromial although hereditary, viral, infectious, and im-
impingement, neck pain, and tendinopathy mune causes are being investigated.85 It is often
due to the inadequate scapulothoracic misdiagnosed and cervical spine radiculopathy
muscular sequencing of the shoulder. Identi- must be considered as a differential diagnosis.
fication of muscular weakness and determi- Electrodiagnostic testing will be assistive in
nation of its cause guide treatment and localizing to the brachial plexus, and magnetic
prognosis. resonance imaging may reveal edema within the
involved musculature.86 Gradual improvement
■ Suprascapular Nerve Injury
occurs over 3 to 4 months with pharmacologic
Chief Clinical Characteristics management and physical therapy.
This presentation may include deep, poorly
localized shoulder pain with muscular wast- ■ Neuropathic Arthropathy
ing of the infraspinatus or supraspinatus. (Charcot-Marie-Tooth Disease)
Background Information Chief Clinical Characteristics
Patient will demonstrate inadequate strength This presentation may be characterized by gen-
into abduction and external rotation of the eralized pain, burning, paresthesia, swelling,
shoulder. Irritation or compression of the and functional limitation of the involved joint.
nerve most frequently occurs at the supras- The symptoms may progress over several
capular foramen, affecting the supraspinatus months or years.
and the infraspinatus. However, isolated Background Information
muscle involvement may occur via impinge- Typical onset of symptoms is a benign or unre-
ment at the spinoglenoid notch or various lated mechanism of injury. Neuropathic
presentations from microtrauma, repetitive arthropathy is a destructive joint disease with
trauma, or distal trauma to the upper ex- decreased sensory innervation and propriocep-
tremity. The injury may be due to a com- tion to the involved joint. Pathogenesis remains
pression lesion, trauma, the presence of a controversial, involving potential neurovascu-
lipoma, or neuritis. Electromyographic stud- lar, neurotrophic, or central nervous system
ies may be used to determine the extent of pathology. Syringomyelia with or without
the injury and the potential for recovery.84 complication of Arnold-Chiari malformation
Anterior lesions most commonly affect is a likely underlying contribution to neuro-
the supraspinatus, and the posterior lesions pathic arthropathy at the shoulder and elbow.87
affect the infraspinatus. The etiology must Diagnosis requires imaging and follow-up for
be determined for the best outcomes. the destruction of the joint. Treatment is based
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164 Chapter 11 Acromioclavicular Joint

on the discovery of the underlying causative Background Information


SHOULDER PAIN

factor of the destruction of the joint. Nonsurgical and surgical treatments exist for
this health condition.
OSTEOARTHROSIS/
OSTEOARTHRITIS ■ Sternoclavicular
■ Acromioclavicular Joint Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may commonly range from
This presentation involves localized tenderness, absence of pain to possible localized tender-
pain, or aching in the lateral deltoid region with ness to mild aching pain typically of insidious
overhead activities and/or shoulder adduction. onset with female patients age 40 or older.
There may be reports of difficulty lying in an Swelling may be present over the joint and may
ipsilateral side-lying position. A history of go unnoticed by the patient. Aggravating fac-
heavy lifting or repetitive overhead activity tors may be ipsilateral shoulder motion. Other
may be present. joints will not be affected and radiographic ev-
idence may not match the clinical picture.33
Background Information
Radiographic evidence may not match the Background Information
clinical picture.88 Treatment focuses on Treatment focuses on resolution of impair-
resolution of impairments such as posture, ments such as posture, including shoulder
including shoulder girdle position and girdle position.
axioscapular strength. ■ Osteomyelitis
■ Glenohumeral Joint Chief Clinical Characteristics
This presentation involves pain, as well as lim-
Chief Clinical Characteristics ited motion of the shoulder with or without
This presentation may include insidious on- warmth or swelling. Up to 50% of patients
set of shoulder pain and progressive loss of with this health condition may present with
range of motion, most commonly in an older nausea, anorexia, and night sweats.90
individual. Marked loss of external rotation
typically is present. Disuse atrophy of the Background Information
rotator cuff may also be present. Though primarily a condition in childhood, it
may affect drug abusers or adults with sickle
Background Information cell disease or may follow intensive surgery.
Radiographs provide evidence of the de- Diagnosis may be delayed for several months.90
structive pattern of posterior glenoid erosion Blood tests, aspiration biopsies, and imaging
and central baldness of the humeral carti- assist with diagnosis. Treatment is dependent
lage.89 Nonsurgical and surgical treatments on the offending organism and may include
exist for this health condition. pharmacologic or operative intervention.

■ Secondary Osteoarthritis/ ■ Polymyalgia Rheumatica


Osteoarthrosis of the Chief Clinical Characteristics
Glenohumeral Joint This presentation typically includes aching and
Chief Clinical Characteristics stiffness at the bilateral shoulder joints with
This presentation commonly involves shoul- radiating pain distally into the elbows. Patients
der pain ranging from mild to severe in the with this condition are typically over the age of
presence of a contributory event, such as a 50 years with a peak incidence at age 70 to
history of significant trauma and/or surgi- 80 years. Involvement of the pelvis and hips is
cal intervention. This health condition is typical with similar symptoms of aching/
typically associated with a younger patient stiffness with radiation of pain into the knees.
population than primary osteoarthritis/ Distal pitting edema and asymmetric periph-
osteoarthrosis. As is true in primary eral arthritis are less typical.
osteoarthritis/osteoarthrosis, significant Background Information
stiffness and the loss of active/passive range The stiffness will require greater than 1 hour
of motion is seen on physical exam.89 to resolve and is typical in the morning. Both
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Chapter 11 Rheumatoid Arthritis 165

passive and active range of motion will be re- results in atrophy and denervation of the teres

SHOULDER PAIN
stricted at the involved shoulder(s) due to pain. minor with or without deltoid involvement.81
Imaging studies reveal a greater appearance of This uncommon injury is difficult to diagnose
subacromial or subdeltoid bursitis rather than clinically and is frequently misdiagnosed as
synovitis of the glenohumeral joint. This is an rotator cuff pathology or impingement
inflammatory condition of unknown etiology syndrome. Electromyography and magnetic
with a suspicion of environmental and genetic resonance imaging are the recommended
causes. An erythrocyte sedimentation rate diagnostic tests. Nonsurgical treatment is the
greater than 40 mm/hr, imaging studies to initial plan of care with surgical intervention
detect the synovitis, and a rapid response to recommended only for those with long-term
corticosteroids combined with the clinical pathology.81
presentation above assist with the differential
diagnosis.91 ■ Reiter’s Syndrome
Chief Clinical Characteristics
■ Pseudogout (Calcium This presentation may involve acute onset with
Pyrophosphate Dihydrate pain and swelling in an asymmetric multiple-
Deposition Disease) joint pattern. Prolonged stiffness following
Chief Clinical Characteristics inactivity is a common complaint for any of
This presentation may involve episodic aching the involved joints.
and limited mobility of the shoulder without
Background Information
a specific mechanism of injury. This condition
This condition is rarely present in the shoul-
is most common in women and with advanc-
der, except possibly in the advanced stages of
ing age.
the disease. This syndrome is a combination of
Background Information four syndromes: peripheral arthritis syn-
The shoulder may be the first affected joint. drome, painful bone syndrome, back and
Causes for the contributory calcium py- pelvis pain, and intestinal and/or genitouri-
rophosphate crystal deposition in synovial nary symptoms. Asymmetrical arthritis of
joints are unknown. Diagnosis is based on the the lower extremity with urethritis and
clinical presentation, the pattern of joints conjunctivitis often occurs early in the
involved, radiographic intra-articular and disease. Typically males are affected more
periarticular involvement, and aspiration of commonly than females. A history of infection
calcium pyrophosphate crystals in the joint (venereal or dysenteric) is associated with in-
fluid.92 Anti-inflammatory medication com- creased risk. Range-of-motion exercises and
prises the usual treatment for this condition. stretching are emphasized along with nons-
Unlike gout, uric acid–lowering medications teroidal anti-inflammatory medications. Rest/
are not used. immobilization is discouraged.93
■ Quadrilateral Space Syndrome ■ Rheumatoid Arthritis
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation will include poorly localized This presentation is characterized by an insid-
shoulder pain with paresthesias in the involved ious onset of transitory pain and stiffness in
extremity in a nondermatomal pattern. Weak- the shoulder. Long-duration stiffness is espe-
ness may be of sudden onset with episodic his- cially prevalent in the morning. Shoulder pain
tory of shoulder pain. Commonly, abduction is rarely the first report of pain in individuals
and external rotation of the shoulder are the presenting with this condition.
actions affected.
Background Information
Background Information Protective posturing of the upper extremity in
The proposed etiology of quadrilateral space internal rotation held against the stomach is
syndrome is vascular occlusion or compres- common. Females are more affected than
sion of the posterior circumflex humeral males with a peak age of 35 to 45 years. Criteria
artery and the axillary nerve possibly due to for diagnosis of this condition include pro-
fibrous bands, scarring, or adhesions. This longed morning stiffness, soft tissue swelling,
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166 Chapter 11 Dermatomyositis

symmetric arthritis, subcutaneous nodules, Background Information


SHOULDER PAIN

positive rheumatoid factor, and/or radi- Although proximal extremity weakness is


ographic evidence. The four types of shoul- a classic finding, up to 50% also demon-
der involvement in rheumatoid arthritis are strate distal weakness that may be equally
dry, wet, resorptive, and bursal. Treatment as severe.96 Blood panels help confirm the
should be tailored to the patient and their diagnosis and track disease activity, reveal-
symptoms, but usually includes steroidal, ing elevated serum levels of creatine phos-
nonsteroidal, or biological anti-inflammatory phokinase. Treatment typically involves
medications. steroidal, nonsteroidal, and biological anti-
inflammatory medications.
RHEUMATOID–LIKE HEALTH
CONDITIONS ■ Psoriatic Arthritis
■ Dermatomyositis Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may involve an insidious on-
This presentation involves myalgia coupled set of lumbopelvic and hip pain associated
with a significant decline in function such with psoriasis. The severity of arthritis is
as difficulty standing from a chair, stepping uncorrelated with the extent of skin involve-
off curbs, and performing overhead activi- ment.28 Pitting nail lesions occur in 80% of
ties of daily living. A skin rash most com- individuals with this condition. Dactylitis,
monly precedes the muscular manifestations tenosynovitis, and peripheral arthritis also
and may be present on the eyelids, in a V sign are common.
or a shawl sign.94 The heliotrope rash is pres-
ent in 25% of cases, and in 30% of cases, Background Information
lesions appear on the metacarpophalangeal, Radiographs of the distal phalanges may re-
proximal, and distal interphalangeal veal a characteristic “pencil in cup” deformity.
joints.95 Sensation remains normal and deep Blood panels including erythrocyte sedimen-
tendon reflexes are rarely affected early in tation rate are useful to track disease activity.
the disease.
■ Scleroderma
Background Information Chief Clinical Characteristics
This condition affects women more fre- This presentation typically includes myalgia,
quently than men. Though considered arthralgia, fatigue, weight loss, limited mobil-
rare, dermatomyositis is the most common ity, and hardened skin about the hands, knees,
of the idiopathic inflammatory diseases. or elbows. This condition occurs in individu-
It is a multisystem autoimmune disease als between 25 and 55 years of age, and is four
characterized by inflammation of the mus- to five times more likely in women than men.
cles and the skin and is similar in presen- Additional symptoms may include dry mouth
tation to polymyositis. Diagnosis may be and eyes, as well as Raynaud’s phenomenon.
via several different tests such as elec-
tromyographic studies, laboratory tests, Background Information
and muscle biopsy.95 Treatment is pharma- This rare and progressive autoimmune disor-
cologic. der affects blood vessels and many internal
organs, including the lungs and the gastroin-
■ Polymyositis testinal system. Overproduction of collagen in
Chief Clinical Characteristics this condition eventually leads to poor blood
This presentation includes a gradual onset of flow in the extremities, which can cause ulcers
mild muscle pain in the shoulder girdle and in the fingers, changes in skin color, and a dis-
other proximal muscles, associated with prox- appearance of creases in the skin. Continued
imal muscle weakness that causes difficulty damage to small-diameter vasculature leads
with daily activities such as walking, ascend- to scar tissue production that impairs joint
ing and descending stairs, and rising from range of motion. Blood tests confirm the di-
chairs. agnosis. Treatment includes both steroidal
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Chapter 11 Biceps Long Tendinitis 167

and nonsteroidal anti-inflammatory medica- 90% of the cases. During the acute phase, the

SHOULDER PAIN
tions and gentle exercise. patient might note incapacitating shoulder pain
with fever and chills.
■ Systemic Lupus Erythematosus
Chief Clinical Characteristics Background Information
This presentation can include lower back This is the most common of the septic inflam-
pain radiating to the hip and groin and is matory causes of shoulder pain. A blood cul-
associated with fatigue and joint pain/ ture will be positive in 50% of the cases. This
swelling affecting the hands, feet, knees, and infection can be caused by dissemination from
shoulders. a different organ system with such examples
including skin breakdown, urinary tract infec-
Background Information tion, or pneumonia. Comorbidities that may
This condition affects mostly women of be associated with spontaneous septic arthritis
childbearing age. It is a chronic autoimmune include a prosthetic joint, cancer, cirrhosis,
disorder that can affect any organ system, in- rheumatoid arthritis, impaired immune sys-
cluding skin, joints, kidneys, brain, heart, tem, diabetes, or the presence of indwelling in-
lungs, and blood. The diagnosis is confirmed travenous or catheter lines. This is typically a
by the presence of skin lesions; heart, lung, disease of the older patient with primary
or kidney involvement; and laboratory ab- shoulder sepsis occurring in between 10% and
normalities including low red or white cell 15% of all joint infections. Clinical presenta-
counts, low platelet counts, or positive anti- tion and subjective questioning must assist
nuclear antibody and anti-DNA antibody with differentiating a soft tissue lesion from
tests.29 infection.
■ Rotator Cuff Tear ■ Skeletal Tuberculosis (Pott’s
Chief Clinical Characteristics Disease)
This presentation may involve weakness, pos- Chief Clinical Characteristics
terior atrophy over the involved muscle, and This presentation is characterized by limited
pain at the lateral brachium with attempts at range of motion, pain, and the presence of a
movement. Night pain, mechanical impingement soft tissue abscess. Typically there is an absence
signs, and crepitus are common. of constitutional symptoms.97
Background Information Background Information
Reaching overhead in the frontal plane and Erythrocyte sedimentation levels may be ele-
with a long lever arm may be most signifi- vated, although the tuberculosis skin test may
cantly affected. Age-related changes in the tis- be negative. A history of trauma may be pres-
sue predispose the rotator cuff to tears on the ent. Osteomyelitis and osteoarticular forms of
articular side. Severity of involvement may be this condition are seldom separated in the lit-
noted via radiographs or computed tomogra- erature. Peripheral referral is uncommon
phy. Nonsurgical management may return full though if affected the shoulder is more com-
patient function. Surgical management and mon than other joints of the upper extremity.
preferred technique are dependent on surgeon Diagnosis is accomplished via blood value
skill and number of tendons involved. tests, chest x-ray, and local imaging. Surgical
management includes debridement.97
■ Septic Arthritis
TENDINOPATHIES
Chief Clinical Characteristics
This presentation may involve a primary re- ■ Biceps Long Tendinitis
port of limited range of motion at the shoulder Chief Clinical Characteristics
because an absence of pain as the primary This presentation can involve aggravating ac-
complaint is not uncommon. Local signs of tivities of lifting, pulling, and reaching with pain
infection will be present. A low-grade or tran- and tenderness to palpation at the anterior
sient fever may occur in anywhere from 40% to shoulder at the level of the bicipital groove.
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168 Chapter 11 Biceps Long Tendinosis

Background Information ■ Rotator Cuff Tendinitis


SHOULDER PAIN

This inflammatory condition typically re- Chief Clinical Characteristics


sults from repetitive activities and may be as- This presentation may involve weakness, at-
sociated with poor mechanics. This condi- rophy, palpatory tenderness over the involved
tion is often diagnosed as subacromial tendon, and pain at the lateral brachium,
impingement syndrome. Diagnosis is typi- night pain, painful arc of movement between
cally based on the clinical exam including 80 and 120 degrees of upper extremity eleva-
special orthopedic tests that have a low tion, and crepitus.99
specificity. The nonoperative management
of this condition focuses on optimizing Background Information
shoulder function and decreasing the local Functional overhead activities are often dif-
inflammatory response. ficult and lead to increased pain. Tender-
ness to palpation is most frequently over
■ Biceps Long Tendinosis the supraspinatus musculotendinous junc-
Chief Clinical Characteristics tion. Athletes or manual laborers in the
This presentation may include aggravating ac- fourth to seventh decade of life are most
tivities of lifting, pulling, and reaching commonly affected. Imaging studies are
with pain and tenderness to palpation at the helpful to determine if a tear is present.
anterior shoulder at the level of the bicipital Limitation in motion may be present,
groove. Patient may report a history of episodic although strength may be preserved or
discomfort with waxing/waning periods inhibited by pain. Underlying laxity should
increasing in frequency. Physical exam be ruled out in the younger population.
will not reveal local warmth or swelling over Initially, nonsurgical management is at-
the tendon. tempted. Failure following 6 to 9 months of
treatment may suggest the need to evaluate
Background Information the patient for surgical management.100
This condition is often diagnosed as sub-
acromial impingement syndrome. Diagnosis ■ Rotator Cuff Tendinosis
is based on the clinical exam including Chief Clinical Characteristics
special orthopedic tests that have a low This presentation can include weakness,
specificity, especially in determination of atrophy, pain at the lateral brachium,
tendinosis versus tendinitis. The goal of night pain, painful arc of movement
nonsurgical management is optimization between 80 and 120 degrees of upper
of shoulder function and a decrease in the extremity elevation, crepitus, and less fre-
local inflammatory response. quently tenderness to palpation of the
involved tendon.99
■ Calcific Tendinopathy
Background Information
Chief Clinical Characteristics
Functional overhead activities are often
This presentation may commonly include
limited with reports of pain or weakness.
pain and limited range of motion. These char-
Athletes or manual laborers in the fourth to
acteristics are present in approximately 50%
seventh decade of life are most commonly
of the patients affected by calcific tendinitis
affected. Imaging studies are helpful to de-
in the shoulder. This condition typically
termine if a tear is present. Limitation in
occurs in the fourth decade.
motion may be present, although strength
Background Information may be preserved or inhibited by pain. Un-
The supraspinatus tendon is theorized to be derlying laxity should be ruled out in the
primarily affected by hypoperfusion of the younger population. Initially, nonsurgical
musculotendinous junction. Differential di- management is attempted. Failure following
agnosis should include rotator cuff pathology. 6 to 9 months of treatment may suggest the
Evidence does support the use of ultrasound need to evaluate the patient for surgical
as a successful therapeutic treatment.98 management.100
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Chapter 11 Osteoblastoma 169

TUMORS ■ Lung Tumor

SHOULDER PAIN
■ Chondrosarcoma Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may be characterized by
This presentation may involve shoulder pain shoulder pain associated with changes in func-
and restricted motion located in various tion, shortness of breath, and probable Horner’s
areas of the proximal humerus in a middle- sign.
aged patient. A nonpainful mass may be the Background Information
initial presenting sign. Symptoms may be associated with direct ex-
Background Information tension of the tumor into the ribs and soft
Tapping along the bone appears to be sensi- tissues or bones of the shoulder girdle; how-
tive to prediction of neoplasm along with a ever, there are some reports of shoulder pain
more significant score for fatigue and low without evidence of direct extension of the
energy.101 This condition is caused by a tumor into the shoulder region.40 Typical
slow growing and destructive primary bone treatment includes a combination of radia-
tumor. Abnormal findings in radiographic tion, chemotherapy, and surgical excision of
images should increase suspicion. Intra- the tumor.
articular invasion should be suspected until ■ Metastases, Including From
proven otherwise. Preoperative chemother- Primary Breast, Kidney, Lung,
apy and radiation are used to reduce tumor Prostate, and Thyroid Disease
size with resection afterward.
Chief Clinical Characteristics
■ Enchondroma This presentation typically includes unremit-
Chief Clinical Characteristics ting pain in individuals with the risk factors
This presentation may involve pain that is of previous history of cancer, age 50 years or
unassociated with the lesion itself, since the older, failure to improve with conservative
lesion is typically asymptomatic. Yet associ- therapy, and unexplained weight change of
ation of pain with this type of lesion needs to more than 10 pounds in 6 months.103
be explored for potential malignancy. Loss of Background Information
range of motion and night pain are not The skeletal system is the third most com-
consistently present. mon site of metastatic disease.104 Symptoms
Background Information also may be related to pathological fracture
This condition is most common during in affected sites. Common primary sites
the third decade of life. Treatment involves causing metastases to bone include breast,
surgical excision after detection. prostate, lung, and kidney. Bone scan con-
firms the diagnosis.
■ Lipoma
Chief Clinical Characteristics ■ Osteoblastoma
This presentation can include a small, mobile, Chief Clinical Characteristics
and palpable mass if superficial; however, This presentation may include pain and
these are typically intramuscular, smaller than limited shoulder range of motion. Night pain
5 cm, and asymptomatic. is typically present.
Background Information Background Information
Lipomas are commonly present in the trunk, Often termed a giant osteoid osteoma, this is
shoulder, and upper arm.102 Any lack of a rare primary bone tumor that may reoccur
range of motion or production of pain may after treatment. Differential diagnosis is
be due to invasion of the joint space or extra- difficult to distinguish from a low-grade
articular block of motion. Surgical removal osteosarcoma until surgical excision and
is successful as a treatment; however, these biopsy confirm the diagnosis.105 Surgical
lesions may recur. management is typical.
1528_Ch11_133-173 11/05/12 3:55 PM Page 170

170 Chapter 11 Osteochondroma

■ Osteochondroma suspicion. Intra-articular invasion should be


SHOULDER PAIN

Chief Clinical Characteristics suspected until proven otherwise. Preopera-


This presentation may involve shoulder pain, tive chemotherapy and radiation are used to
limited range of motion, and crepitus in reduce tumor size with resection afterward.
skeletally immature and young adults with ■ Unicameral Bone Cyst
presentation of an enlarging mass. Pain may
be present with palpation. Chief Clinical Characteristics
This presentation may include an acute
Background Information onset of arm pain with inadequate range of
Onset of neuropathic symptoms preceded by motion secondary to the pain. A mass or
upper extremity exercise have been reported tenderness to palpation may not be present.110
in case studies as the precipitating event of
discovery of the osteochondroma.106 Though Background Information
most typical in the proximal humerus, it is This condition is defined as a fluid-filled
the most common tumor of the scapula.107 lesion in the proximal humerus that is gener-
Surgical interventions have been successful in ally considered benign despite typical reoc-
resolution of patient symptoms. currence following surgical removal. Radi-
ographs and biopsy of tissue are necessary
■ Osteoid Osteoma to confirm diagnosis and rule out more
Chief Clinical Characteristics aggressive forms of cancer. Surgical removal is
This presentation involves shoulder pain typically recommended.
and limited motion. Night pain is typically
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CHAPTER12
Elbow Pain
■ Julia L. Burlette, PT, DPT, OCS ■ John M. Itamura, MD

Description of the Symptom Special Concerns


■ Decreased pulses
This chapter describes pathology that may lead ■ Marked swelling and paresthesias
to elbow pain. Local causes of elbow pain in- ■ Recent change of status from previous status
clude the distal one-third of the humerus, ■ Skin breakdown or wound problems
proximal one-third of the radium and ulna, ■ Warmth and swelling associated with a
and the corresponding articular and periartic- fever
ular structures. Remote causes are defined as
occurring outside these regions.

CHAPTER PREVIEW: Conditions That May Lead to Elbow Pain

T Trauma
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Cervical radiculopathies: Elbow dislocation 184 Muscle strains:
• C6 radiculopathy 182 • Flexor/pronator muscle
strain 190

UNCOMMON
Cervical radiculopathies: Fractures: Biceps tendon distal rupture
• C8 radiculopathy 182 • Intercondylar fracture of the 182
humerus 185 Nerve entrapments:
• Monteggia fracture- • Median nerve compression in
dislocation 186 the proximal forearm 191
• Supracondylar fracture of the
humerus 188
• Transolecranon fracture-
dislocation 188
Post-traumatic
osteoarthrosis/osteoarthritis
193
Terrible triad 196

174
1528_Ch12_174-200 11/05/12 3:55 PM Page 175

ELBOW PAIN
Anterior
Anterior Anterior
Lateral Posterior
Medial

Medial

Lateral Posterior
Anteromedial view Lateral view Posteromedial view

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Fractures: Bursitis: Ligament injuries:


• Radial head fracture 187 • Traumatic aseptic olecranon • Medial collateral ligament
Muscle strains: bursitis 183 insufficiency 189
• Extensor muscle strain 190 Fractures: Muscle strains:
• Olecranon fracture 186 • Flexor/pronator muscle strain 190
Nerve entrapments: Nerve entrapments:
• Ulnar neuropathy/neuritis 192 • Ulnar neuropathy/neuritis 192

Ligament injuries: Valgus extension overload Ligament injuries:


• Lateral ulnar collateral ligament syndrome 198 • Medial collateral ligament
insufficiency (posterolateral insufficiency 189
rotatory instability) 189
Nerve entrapments:
• Deep radial nerve entrapment
(posterior interosseous nerve
paralysis, supinator syndrome,
radial tunnel syndrome) 190
Posterior interosseous nerve
injury postsurgical 192

(continued)

175
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176 Chapter 12 Elbow Pain

Trauma (continued)
ELBOW PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


RARE
Not applicable Snapping triceps syndrome 194 Biceps muscle tear 182
Fractures:
• Coronoid fracture 185

I Inflammation
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Aseptic Not applicable
Rheumatoid arthritis 193
Septic
Not applicable

UNCOMMON
Not applicable Aseptic Not applicable
Not applicable
Septic
Osteomyelitis of the distal
humerus, proximal radius,
and ulna 192
Septic arthritis 193
RARE
Not applicable Not applicable Aseptic
Tendinopathies:
• Distal biceps tendinitis 194
Septic
Not applicable

M Metabolic
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Gout 189 Not applicable
Heterotopic ossification 189
RARE
Not applicable Pseudogout 193 Not applicable
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Chapter 12 Elbow Pain 177

ELBOW PAIN
LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Fractures: Triceps rupture 196 Nerve entrapments:


• Capitellar fracture 184 • Median nerve compression in
Nerve entrapments: the supracondylar tunnel 191
• Musculocutaneous or lateral Snapping triceps syndrome 194
antebrachial cutaneous
nerve entrapment 191

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Aseptic Aseptic Aseptic


Tendinopathies: Tendinopathies: Tendinopathies:
• Lateral epicondylitis (tennis • Triceps tendinitis 195 • Medial epicondylitis (golfer’s
elbow) 194 elbow) 195
Septic
Septic Not applicable Septic
Not applicable Not applicable

Not applicable Aseptic Not applicable


Bursitis:
• Chronic aseptic olecranon
bursitis 183
Septic
Bursitis:
• Septic olecranon bursitis 183

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable


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178 Chapter 12 Elbow Pain


ELBOW PAIN

Va Vascular
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Arterial injury 182 Superficial thrombophlebitis
Compartment syndrome 183 of the cephalic or basilic
veins 194
RARE
Not applicable Not applicable Not applicable

De Degenerative
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable

UNCOMMON
Not applicable Primary osteoarthrosis/ Not applicable
osteoarthritis 193
RARE
Not applicable Not applicable Tendinopathies:
• Distal biceps tendinosis 194

Tu Tumor
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Malignant Primary, such as: Not applicable
• Malignant lymphoma 197
• Osteosarcoma 198
• Synovial sarcoma 198
Malignant Metastatic:
Not applicable
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Chapter 12 Elbow Pain 179

ELBOW PAIN
LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Tendinopathies: Tendinopathies: Tendinopathies:


• Lateral epicondylosis 195 • Triceps tendinosis 196 • Medial epicondylosis 195

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

(continued)
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180 Chapter 12 Elbow Pain

Tumor (continued)
ELBOW PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


RARE
Benign, such as:
• Ganglion cyst 196
• Giant cell tumor 197
• Lipoma 197
• Osteoblastoma 197
• Osteochondroma 197
• Osteoid osteoma 197
• Synovial chondromatosis 198

Co Congenital
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Elbow Pain incision is posterior and then skin flaps are
made to approach posterior, medial, or lateral
Successful management of elbow injuries regions. The bone and tissue quality will also
requires a thorough screening and detailed determine the appropriate interventions and
history. Fractures can be misdiagnosed or the progression.
extent of the injury and associated injuries Early range of motion following injury and
may not be recognized. Obtaining the opera- surgery is indicated to prevent loss of motion;
tive notes following surgery and communica- however, aggressive mobilization may cause
tion with the physician are paramount. It increased inflammation and contribute to the
may be useful to review the radiology reports. development of heterotopic ossification. Static
Certain fracture patterns are more difficult to versus dynamic splints are more beneficial and
stabilize and the surgical incision may not nec- should be used to assist in achieving elbow
essarily indicate the areas of fixation. Often the range of motion. While functional elbow
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Chapter 12 Elbow Pain 181

ELBOW PAIN
LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

range is considered to be 30 degrees of exten- lack of inflammatory cells. Because of com-


sion to 130 degrees of flexion, the patients’ mon usage of tendinitis to describe any tendon
individual goals and range needed to return condition the literature is difficult to interpret.
to their prior level of function should be con- The term tendinopathy has been suggested to
sidered. Certain occupations and extracurric- encompass both the inflammatory and degen-
ular activities require greater ranges of elbow erative conditions. Treatment of overuse in-
motion. juries at the elbow necessitates addressing
Overuse injuries at the elbow are common. proximal areas of weakness and decreased
Tendinitis is an acute inflammatory process flexibility to restore alignment and improve
characterized by the presence of inflammatory mechanics at the elbow, thereby decreasing
cells. Tendinosis is a degenerative process result- stress at the elbow. Focusing treatment inter-
ing in incomplete/failed tendon healing charac- ventions solely on the elbow will lead to less
terized by disorganized collagen fibers and the than optimal outcomes and will not assist in
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182 Chapter 12 C6 Radiculopathy

preventing recurrence of the injury and Background Information


ELBOW PAIN

chronic tendon symptoms. The presentation may also include motor loss
in the muscles innervated by the C8 nerve.
Description of Conditions That Cervical disk herniation, infection, hemor-
May Lead to Elbow Pain rhage, or other space-occupying lesions may
cause cervical radiculopathy. C8 radiculopathy
Remote is uncommon.2 The diagnosis is confirmed
with magnetic resonance imaging. Treatment
CERVICAL RADICULOPATHIES
includes inflammation control, restoration of
■ C6 Radiculopathy mobility, and strengthening. Surgical inter-
Chief Clinical Characteristics vention may be necessary in cases with severe
This presentation, while variable, typically or progressive neurological deficits.
includes pain in the neck, shoulder, medial bor-
der of the scapula, radial side of the upper Local
arm and forearm, thumb, and index finger. ■ Arterial Injury
Pain is increased with cervical movement and
use of the extremity. Coughing or sneezing Chief Clinical Characteristics
may also increase symptoms. Sensory changes This presentation includes pain out of propor-
may be present in the thumb and index tion to the injury, decreased or absent pulses,
finger and the radial aspect of the hand and decreased skin temperature, and pallor. Pain
forearm. increases with passive stretching of the involved
muscles.
Background Information
In severe cases, the brachioradialis or biceps Background Information
reflexes may be absent and motor loss evi- The presentation may also include weakness
dent in the muscles innervated by the C6 and hypoesthesia. The mechanism of injury is
nerve. Cervical radiculopathy may occur usually traumatic. Arteriography confirms the
from cervical disk herniation or other diagnosis. Operative repair may be required.
lesions (space-occupying, infection, hemor- ■ Biceps Muscle Tear
rhage). A test item cluster consisting of an
upper limb tension test, Spurling test, Chief Clinical Characteristics
distraction test, and cervical rotation ipsilat- This presentation includes distal upper arm or
eral to the side of involvement less than anterior elbow pain, tenderness, swelling, and
60 degrees is useful to identify this condi- ecchymosis. The muscle defect in the distal
tion.1 The diagnosis is confirmed with mag- biceps may be palpable. Elbow flexion and fore-
netic resonance imaging. Treatment includes arm supination strength are impaired. The
inflammation control, restoration of mobil- patient may report a tearing or popping sensa-
ity, and strengthening. Severe or progressive tion above the anterior elbow.3
neurological deficits may require surgical Background Information
intervention. This injury is rare and is reported most fre-
quently by military parachutists.3 It may also
■ C8 Radiculopathy be associated with anabolic steroid use. Clinical
Chief Clinical Characteristics examination and magnetic resonance imaging
This presentation may be characterized by or ultrasonography confirm the diagnosis.
pain in the neck, scapula, ulnar aspect of the
upper arm and forearm, and fourth and fifth ■ Biceps Tendon Distal Rupture
fingers. Pain is increased with cervical move- Chief Clinical Characteristics
ment and use of the extremity. Coughing or This presentation typically includes local tender-
sneezing may also increase symptoms. Sensory ness in the antecubital fossa and ecchymosis in
disturbances may be reported in the fourth and the antecubital fossa and medial elbow. The de-
fifth fingers as well as the medial aspect of fect may be palpated and visible during active
the forearm. elbow flexion with the biceps muscle retracted
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Chapter 12 Compartment Syndrome 183

up in the proximal upper arm. The retracted ■ Septic Olecranon Bursitis

ELBOW PAIN
tendon may be a palpable mass. Decreased flex- Chief Clinical Characteristics
ion, supination, and grip strength occur in vary- This presentation is characterized by pain
ing degrees with supination most impacted. Pa- and swelling over the tip of the olecranon
tients typically report a sharp or tearing pain in and a palpable bursa. The area is often
the antecubital fossa. Patients may report an warm and erythematous. The patient may
inability to perform activities of daily living. have a fever and axillary or epitrochlear
Background Information adenopathy. Cellulitis overlying the bursa
The mechanism of injury is typically flexion of may be present.7 End-range elbow flexion
the elbow against resistance with the elbow in may be limited.
flexion but may also occur with repetitive
heavy use of the elbow.4 Abuse of anabolic Background Information
steroids and preexisting degenerative changes Onset can occur from a laceration or injec-
in the tendon predispose it to rupture. Distal tion or from hematogenous seeding. Trau-
biceps tendon rupture is uncommon.5 Tendon matic aseptic olecranon bursitis can develop
avulsion from the radial tuberosity is the most into septic olecranon bursitis and occurs
common distal biceps tendon injury. A com- more frequently in immunocompromised
plete avulsion is more common than a partial patients. Clinical examination and culture of
tear. The clinical presentation of a partial dis- bursal fluid aspirate confirm the diagnosis.
tal biceps tendon tear is more subtle than a Treatment includes bursectomy and antibi-
complete rupture and the injury may be mis- otic therapy.
diagnosed. Magnetic resonance imaging has
■ Traumatic Aseptic Olecranon
been found to be useful in accurately diagnos-
Bursitis
ing this injury.4 Complete ruptures are man-
aged surgically for the most optimal outcomes. Chief Clinical Characteristics
Partial tears can be managed nonsurgically but This presentation includes pain and swelling
if conservative therapy is ineffective surgical over the tip of the olecranon and a large
intervention can provide good results. palpable bursa. Warmth and redness may
also be present in the same region.
BURSITIS Background Information
■ Chronic Aseptic Olecranon The mechanism of injury is either a direct
Bursitis blow or acute overexertion. It can also
Chief Clinical Characteristics develop following surgical procedures utiliz-
This presentation includes a distended olecra- ing a posterior approach. The bursa may re-
non bursa and may include tenderness. The quire aspiration if elbow motion is limited or
patient will report a gradual onset of symp- in recalcitrant cases. Clinical examination and
toms and may report pain with elbow move- resolution of symptoms with conservative
ment. Fever and axillary adenopathy are not management confirms the diagnosis. Blood
present, differentiating it from septic olecra- tests and cultures of aspirated fluid may be
non bursitis. indicated. In the presence of diabetes or renal
failure, aseptic olecranon bursitis may de-
Background Information velop into septic olecranon bursitis. Immuno-
The mechanism of injury is repeated suppressant and anti-inflammatory medica-
trauma. Chronic bursitis typically coexists tions may mask signs and symptoms, making
with chronic inflammation of adjacent tis- assessment difficult. Treatment includes icing,
sues.6 Treatment includes compression compression dressing, and an elbow pad.
dressing, elbow pad, and icing. Eliminating
the repetitive trauma to the region is neces- ■ Compartment Syndrome
sary to prevent continued inflammation. Chief Clinical Characteristics
Surgical excision may be needed in cases that This presentation includes pain, feelings of in-
do not respond to nonoperative treatment. creased pressure, tense compartment, weakness,
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184 Chapter 12 Elbow Dislocation

numbness, and swelling. Pain is out of propor- has studies documenting reduction follow-up
ELBOW PAIN

tion to that expected for injury and increases with radiographs are necessary to identify any
passive stretching of the muscles. residual instability. Treatment options range
from surgical to nonsurgical forms of manage-
Background Information
ment, depending on the associated injuries
Immobilization does not relieve the pain.
and the patient’s lifestyle.
Peripheral pulses are usually intact except if
there has been arterial injury. The mechanisms FRACTURES
of injury include fracture, crush injury, burns,
■ Capitellar Fracture
and vascular injury. Prolonged external pres-
sure from a compressive cast or bandage may Chief Clinical Characteristics
also cause compartment syndrome. Clinical This presentation includes pain and swelling
examination and compartmental pressure at the lateral elbow and antecubital fossa,
measurement confirm the diagnosis. Immedi- and crepitus during motion, especially flex-
ate identification of compartment syndrome is ion and extension. Pain is increased during
critical to prevent muscle ischemia. If com- forearm rotation.8
partment syndrome is suspected, an immedi- Background Information
ate referral to an emergency department
should be made. A decompressive fasciotomy
is required to alleviate the pressure. The major
complication of compartment syndrome is a
Volkmann contracture.
■ Elbow Dislocation
Chief Clinical Characteristics
This presentation includes pain, swelling, ecchy-
mosis, and deformity.
Background Information
The mechanism of injury is typically a fall
onto the outstretched hand. Motor vehicle
accidents and direct trauma can also cause
elbow dislocation. In adults, the elbow joint is Capitellar fracture
the second most commonly dislocated joint.
Simple elbow dislocation can result in injury The mechanism of injury is typically a fall
to the medial and lateral collateral ligaments, onto an outstretched hand, with the elbow
and anterior and posterior capsule. Other partially flexed and the forearm pronated.
associated injuries are common including Capitellar fractures account for less than 1%
radial head and neck fractures, coronoid frac- of all elbow fractures and are more common
tures, osteochondral fractures, avulsion frag- in females.9,10 The fracture can involve the
ments from the medial and lateral epicondyles, osseous and cartilaginous portions of the
and wrist and shoulder injuries. Triceps capitellum or less frequently present as a
avulsion can also occur but is rare. Dislocation shearing off of the articular cartilage and a
with associated fractures can lead to instability thin layer of subchondral bone. This condi-
and poor outcomes. Complications include tion may occur in isolation but may also be
contracture, instability, heterotopic ossifica- associated with other injuries. Medial joint
tion, neurovascular injury, and compartment line tenderness would suggest a medial col-
syndrome. Clinical examination, history, and lateral ligament injury. Identification of a
plain radiographs confirm the diagnosis. If the ligament injury is important to prevent
elbow has relocated, plain radiographs may future instability. Plain radiographs confirm
not be helpful because there may only be liga- the diagnosis. Capitellar fractures typically
mentous injury. However, even if the patient require surgical intervention.
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Chapter 12 Intercondylar Fracture of the Humerus 185

■ Coronoid Fracture Background Information

ELBOW PAIN
Chief Clinical Characteristics
This presentation may include pain and
swelling at the antecubital fossa and instabil-
ity. Crepitus may occur with end-range
elbow extension.
Background Information

This injury rarely occurs in isolation and is


associated with other injuries including
elbow dislocation or olecranon fractures.11
Coronoid fractures are commonly unrecog-
nized and may be the cause of continued in-
stability following elbow trauma.12 The size
and location of the coronoid fracture will im-
plicate what other structures may be involved
(capsule versus ulnar collateral ligament) and
the severity of the injury.13 Plain radiographs
confirm the diagnosis; however, computer-
ized tomography and/or magnetic resonance
imaging may be indicated to identify all in- The fracture usually occurs from forces
jured structures. Nondisplaced fractures that against the posterior aspect of the flexed
do not have instability may be managed non- elbow or from a fall on the outstretched
surgically, while comminuted fractures or hand. The diagnosis is confirmed with trac-
those associated with dislocation are typically tion films and possibly a computerized
managed with surgical stabilization. tomography scan. This fracture is managed
surgically. Certain fracture patterns are easily
■ Intercondylar Fracture of the addressed but others, especially the low-H
Humerus pattern, are difficult to stabilize, necessitat-
Chief Clinical Characteristics ing extreme caution during postsurgical
This presentation includes pain, swelling, rehabilitation, particularly in patients with
paresthesias, and tenderness. osteoporotic bone.
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186 Chapter 12 Monteggia Fracture-Dislocation

■ Monteggia Fracture-Dislocation diagnosis. Stable, anatomical fixation of the


ELBOW PAIN

Chief Clinical Characteristics ulnar fracture including any associated coro-


This presentation includes pain, swelling, ten- noid fracture results in satisfactory out-
derness, angulation of the ulna, and impaired comes in most adults.15 Impaired rotation
supination and pronation. postsurgically may indicate instability of the
proximal radioulnar joint or radiocapitellar
Background Information joint secondary to malreduction of the ulna.
■ Olecranon Fracture
Chief Clinical Characteristics
This presentation includes posterior elbow
pain, swelling, tenderness, and ecchymosis.
The bone may have a palpable defect. An in-
ability to actively extend the elbow occurs
with a displaced fracture.
Background Information

The dislocated radial head can be palpated.


Radial nerve injury may also occur, resulting
primarily in weakness. A Monteggia lesion
involves a fracture of the proximal third of
the ulna and dislocation of the radial head. It
is classified according to the direction of the
radial head dislocation and the trauma to the
ulna. The mechanism of injury is typically a
fall onto the outstretched hand with the
forearm pronated or direct trauma to the
ulna. The most commonly reported associ-
ated nerve injury is dysfunction of the
posterior interosseous nerve (Fig. 12-1).14
Monteggia fracture-dislocation is managed
surgically. Plain radiographs confirm the
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Chapter 12 Radial Head Fracture 187

ELBOW PAIN
Arcade of
Frohse

Supinator

Posterior
interosseous
nerve

Pronated Supinated
FIGURE 12-1 Anatomical course of the posterior
interosseous nerve.

The mechanism of injury is typically either a


direct impact with the tip of the elbow or a
strong contraction of the triceps at the time
of a fall on the extended arm. Plain radi-
ographs confirm the diagnosis. Nondis-
placed fractures are usually managed non-
surgically and displaced fractures are
managed surgically. Complications include
failure of fixation, loss of motion, hetero-
topic ossification, and ulnar neuropathy.16
■ Radial Head Fracture
Chief Clinical Characteristics
This presentation includes pain, swelling, and
tenderness on the lateral side of the elbow
over the radial head. Supination and prona-
tion may be limited secondary to pain or a
mechanical block.
Background Information
Ecchymosis and tenderness on the medial
side of the elbow are suggestive of medial
collateral ligament involvement. Radial head
fracture usually results from a fall onto the
outstretched hand with the elbow partially
flexed and pronated or directly onto the
lateral elbow. Plain radiographs confirm the
diagnosis. Associated injuries are common
and include dislocation, capitellum fracture,
Essex-Lopresti injury, and medial and/
or lateral collateral ligament disruption.
The incidence of associated concomitant
osseous, osteochondral, and ligamentous
injuries has been demonstrated to be high.17
Nondisplaced fractures may be managed
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188 Chapter 12 Supracondylar Fracture of the Humerus

nonsurgically, but must be closely moni- This fracture occurs with a fall on the out-
ELBOW PAIN

tored for delayed fracture displacement. stretched hand with the elbow extended or
Early range of motion for patients with with a force against the posterior aspect of
nondisplaced fractures is necessary to pre- the flexed elbow.18 Supracondylar fracture
vent contracture but can increase the risk of of the humerus is uncommon in adults,
displacement. A loss of motion, an increase although it can occur during manipulation
in pain, or the occurrence of crepitus would of a stiff osteoporotic joint. The neurovascu-
indicate a need for imaging. The physician lar status must be evaluated thoroughly be-
may routinely order radiographs to moni- cause the median, ulnar, and radial nerves
tor the fracture during the healing stage. and the brachial artery may be injured with
Displaced fractures are usually managed this fracture. Plain radiographs confirm the
surgically. diagnosis. Displaced supracondylar fractures
■ Supracondylar Fracture are managed surgically.
of the Humerus ■ Transolecranon
Chief Clinical Characteristics Fracture-Dislocation
This presentation is mainly characterized by Chief Clinical Characteristics
pain, swelling, visible deformity, ecchymosis, This presentation includes pain, swelling, and
and tenderness of the distal humerus. tenderness at the elbow joint.
Background Information Background Information
This injury typically results from a high-
energy trauma.19 A transolecranon fracture-
dislocation is an anterior fracture-dislocation
of the olecranon and is associated with frac-
ture of the coronoid process. The olecranon
fracture is often comminuted.16 The injury
may be misdiagnosed as a Monteggia frac-
ture but usually has more chondral injury
and a poorer prognosis. Plain radiographs
confirm the diagnosis. Transolecranon
fracture-dislocations require surgical inter-
vention. Despite the complexity of the in-
Supracondylar fracture of the humerus jury, good results are obtained in most cases

Transolecranon fracture-dislocation
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Chapter 12 Medial Collateral Ligament Insufficiency 189

if stable, anatomical reconstruction is Heterotopic bone development around the

ELBOW PAIN
achieved.19 Active and active-assisted range- elbow has been associated with severe trauma
of-motion exercises should be initiated im- with extensive soft tissue injury, infected
mediately postsurgically if stable fixation is fracture, closed head or spinal cord injury,
achieved. burn injury, and genetic conditions such as
fibrodysplasia ossificans progressiva and his-
■ Gout tory of ectopic bone formation.20,21 It mani-
Chief Clinical Characteristics fests clinically 3 to 4 weeks following injury
This presentation involves a sudden onset of or surgery but may occur earlier. Plain radi-
pain, swelling, redness of the superficial tissues, ographs and sometimes computerized to-
and tenderness of the joint or bursal cavity. mography scan confirm the diagnosis. Resec-
Advanced cases may have palpable firm tophi. tion of the heterotopic bone may be necessary
to restore functional motion, but frequently
Background Information
recurs.
Gout commonly manifests as acute olecranon
bursitis. The olecranon bursa is a common LIGAMENT INJURIES
location for tophi. The toes should also be as-
■ Lateral Ulnar Collateral
sessed for metatarsal joint involvement. Gout
Ligament Insufficiency
is usually associated with elevated uric acid
(Posterolateral Rotatory
levels. Increased prevalence is associated with
Instability)
advanced age, male gender, and regular alcohol
consumption. Polarized microscopic examina- Chief Clinical Characteristics
tion of aspirated synovial fluid confirms the This presentation mainly involves pain and
diagnosis. Treatment includes inflammation reports of catching, clicking, and locking. The
control, medication, and diet modification. patient may also report slipping of the elbow
joint. Apprehension with the elbow supinated
■ Heterotopic Ossification and fully extended may occur.
Chief Clinical Characteristics Background Information
This presentation typically includes loss of range Lateral ulnar collateral ligament injury can
of motion, swelling, and local warmth. The result from dislocation, fracture, or surgery
patient may report pain. to the lateral aspect of the elbow including
Background Information lateral epicondyle revision. Injections for
lateral epicondylitis may also weaken the
ligament. The most sensitive clinical test is
the overhead lateral pivot-shift test per-
formed with the patient supine.22 Magnetic
resonance imaging or fluoroscopic exami-
nation confirms the diagnosis. Active
patients will require surgery to restore
the stability. Sedentary patients may be able
to modify their activities and be managed
nonsurgically.
■ Medial Collateral Ligament
Insufficiency
Chief Clinical Characteristics
This presentation can include medial elbow
pain, tenderness, instability to valgus stress,
and possible ecchymosis (Fig. 12-2). The
patient commonly reports symptoms with
reaching out and back during activities of
daily living. Chronic cases may present with
ulnar nerve involvement.
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190 Chapter 12 Extensor Muscle Strain

specifically the wrist extensors, 1 to 2 days


ELBOW PAIN

prior to the onset of symptoms. Clinical


examination confirms the diagnosis. Symp-
toms typically resolve within 1 to 3 days with
avoidance of aggravating activities.
■ Flexor/Pronator Muscle Strain
Chief Clinical Characteristics
This presentation involves muscle soreness
and tenderness at the wrist flexor/pronator
muscle group. Symptoms are reproduced
with active wrist flexion and forearm prona-
FIGURE 12-2 Elbow ecchymosis in a patient with tion, with symptoms greater when resist-
medial collateral ligament insufficiency. ance is provided. Passive wrist extension
and forearm supination may reproduce
Background Information symptoms.
This condition usually occurs from a valgus Background Information
force on the extended arm, which may occur This condition is commonly associated with
from a fall on the outstretched hand with a increased forearm use 1 to 2 days prior to the
valgus force at the elbow or with throwing onset of symptoms. Clinical examination
sports. It may be associated with a flexor/ confirms the diagnosis. Symptoms will
pronator avulsion. Commonly a history of resolve within 1 to 3 days with avoidance of
repetitive overhead throwing activities will aggravating activities.
be reported. The moving valgus stress test is
highly sensitive for medial elbow pain aris- NERVE ENTRAPMENTS
ing from the medial collateral ligament.23 ■ Deep Radial Nerve Entrapment
Stress radiographs and often magnetic reso- (Posterior Interosseous Nerve
nance imaging assist in confirming the Paralysis, Supinator Syndrome,
diagnosis. A throwing athlete returning to Radial Tunnel Syndrome)
competition will usually require surgery, Chief Clinical Characteristics
however, nonoperative treatment has been This presentation may involve minimal to
successful in returning athletes to throwing severe aching pain in the lateral forearm and
competitively.24 It is important to obtain the tenderness distal to the lateral epicondyle and
operative notes to determine if the flexor/ in the supinator region.25 Symptoms may be
pronator mass was taken down or split dur- reproduced with resisted supination of the
ing repair of the ligament. forearm or resisted wrist extension with the
MUSCLE STRAINS elbow extended. Passive wrist flexion with
ulnar deviation and the elbow extended may
■ Extensor Muscle Strain reproduce symptoms that are increased when
Chief Clinical Characteristics the shoulder is depressed and abducted.
This presentation can include muscle sore- Partial or complete paralysis of the muscles
ness and tenderness at the wrist extensor innervated by the deep radial nerve may be
muscle group. Reproduction of symptoms present. Sensation is not impaired.
occurs with active wrist extension, with in-
Background Information
creased symptoms when resistance is applied.
Patients may report participating in repeti-
Symptoms may be produced with passive wrist
tive forearm pronation and supination
flexion.
prior to onset of symptoms. Deep radial
Background Information nerve compression has multiple causes
Extensor muscle strain is commonly associ- including trauma, space-occupying lesions,
ated with increased use of the forearm, inflammation, and excessive muscular
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Chapter 12 Musculocutaneous or Lateral Antebrachial Cutaneous Nerve Entrapment 191

activity. Compression at the arcade of respond to the above treatment or who

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Frohse (the proximal edge of the supinator) demonstrate progressive weakness.
occurs most frequently (see Fig. 12-1).26
Isolated entrapment is rare. This condition ■ Median Nerve Compression in
is often difficult to differentiate from lateral the Supracondylar Tunnel
epicondylitis; they may occur independ- Chief Clinical Characteristics
ently or simultaneously. Tennis elbow This presentation includes deep pain in the
straps may cause increased symptoms in a region of the supracondylar tunnel (comprised
patient with deep radial nerve entrapment. of the supracondylar process, the medial epi-
It is essential to rule out cervical radicu- condyle, and the ligament of Struthers) and
lopathy and other potential sites of com- pain and paresthesias in the median nerve
pression. Clinical examination and selective dermatome. Pain is typically worse at night.
injections confirm the diagnosis. Treatment Weakness of the muscles innervated by the
should initially focus on rest and inflamma- median nerve may be present.
tion control. Surgical interventions are
Background Information
rarely indicated.
The supracondylar process is rare, occurring
in 0.3% to 2.7% of individuals.25 The mech-
■ Median Nerve Compression
anism of injury is typically a fracture of the
in the Proximal Forearm
supracondylar process but may occur from
Chief Clinical Characteristics surgical treatment of an intra-articular distal
This presentation typically includes nonlo- humerus fracture. Clinical examination and
calized aching pain in the anterior forearm and plain radiographs to assess for a supracondy-
pain with palpation to the forearm along the lar process confirm the diagnosis. Treatment
course of the nerve. Symptoms may be asso- initially consists of inflammation control
ciated with weakness in the muscles innervated and avoidance of aggravating positions.
by the median nerve and numbness in the Surgical decompression to remove the
hand, primarily the first and second digits. supracondylar process may be indicated if
Reproduction of symptoms may vary depend- the patient is not responsive to conservative
ing on the site of compression. treatment.
Background Information ■ Musculocutaneous or Lateral
The mechanism of injury may be acute Antebrachial Cutaneous Nerve
trauma or chronic, repetitive microtrauma. Entrapment
Common sites of entrapment include the
Chief Clinical Characteristics
pronator teres, flexor superficialis arch, and
This presentation is mainly characterized by
the bicipital aponeurosis.25 Symptoms with
pain and tenderness in the anterolateral
resisted forearm, elbow, or finger motions
elbow and forearm. Symptoms may be
may implicate the structures involved in the
aggravated with full elbow extension and
entrapment.27 Activities that require repeti-
forearm pronation. Acute compression may
tive, resisted pronation and supination of the
cause burning pain. Chronic irritation of the
forearm may play a role in this entrapment.
nerve results in hypoesthesia in the wrist and
It is important to rule out cervical radicu-
forearm and pain.25
lopathy and other potential sites of compres-
sion. Clinical examination and selective in- Background Information
jections confirm the diagnosis. Median nerve The mechanism of injury may be acute
entrapment should initially be managed or chronic trauma to the elbow involving
with inflammation control and activity forced elbow extension and pronation.
modification. Stretching and strengthening Chronic trauma may occur from repetitive
should be initiated once the initial symp- pronation and supination of the forearm.
toms have decreased. Surgical intervention Elbow flexor hypertrophy may also be a con-
may be necessary for patients who do not tributing factor in compression of the nerve.
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192 Chapter 12 Ulnar Neuropathy/Neuritis

Clinical examination and electrodiagnostic positions. Strengthening and stretching to


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evaluation confirm the diagnosis.28 Selective improve trunk and upper extremity align-
injections may also be helpful to confirm the ment should be included to prevent recur-
diagnosis. Treatment includes avoidance of rence. Surgical intervention, although con-
aggravating activities and inflammation troversial, may be indicated if conservative
control. Stretching and strengthening should treatment does not provide relief or the
be initiated when the inflammatory stage weakness is progressing.
resolves. Surgical decompression may be
indicated if symptoms do not respond to ■ Osteomyelitis of the Distal
nonoperative measures. Humerus, Proximal Radius,
and Ulna
■ Ulnar Neuropathy/Neuritis
Chief Clinical Characteristics
Chief Clinical Characteristics This presentation involves local pain, tenderness,
This presentation initially includes paresthe- swelling, warmth, and axillary and/or
sias in the sensory distribution of the ulnar epitrochlear adenopathy. The elbow may be
nerve. Aching pain may occur in the medial held in flexion.
elbow and forearm. Less commonly, pain may
occur in the posterior elbow. Tinel’s sign over Background Information
the ulnar sulcus or distally may reproduce Gentle passive range of motion is tolerated.
symptoms. Weakness of the muscles inner- Bone infection occurs hematogenously, after
vated by the ulnar nerve and atrophy may be surgery or open fracture, or by spread from a
found if the nerve compression is severe or local process.31 It should be suspected in
continues for an extended period. any postoperative case directly involving
osseous structures. An increased prevalence is
Background Information observed in individuals with a history of im-
The mechanism of injury may be acute munocompromise and rheumatoid arthritis.
trauma (elbow dislocation, fracture, direct The diagnosis is confirmed with blood work.
blow, direct pressure for extended period), An indium-labeled white blood cell scan
chronic nerve microtrauma, or ulnar nerve and magnetic resonance imaging may also
entrapment. Activities that involve repeti- be indicated. Early diagnosis and surgical
tive elbow flexion and extension, leaning on treatment with adjuvant antibiotic therapy
the elbow, and ulnar nerve subluxation improve outcomes.
may contribute to microtrauma of the
nerve. Ulnar nerve entrapment at the elbow ■ Posterior Interosseous Nerve
can occur at the medial intermuscular sep- Injury Postsurgical
tum, arcade of Struthers, the cubital tunnel,
and under the transverse fascia of the flexor Chief Clinical Characteristics
carpi ulnaris.27 The cubital tunnel is the This presentation includes partial or complete
paralysis of the muscles innervated by the pos-
most common compression site. Decreased
terior interosseous nerve. This is differentiated
volume of the cubital tunnel and compres-
from radial nerve palsy by the ability to extend
sion of the ulnar nerve have been found to
the wrist (see Fig. 12-1).
occur with increasing elbow flexion.29 The
intraneural pressure of the ulnar nerve Background Information
within the cubital tunnel and proximal to Iatrogenic radial nerve injury may result from
it also increases as the elbow is flexed arthroscopic surgery or surgery utilizing a
120 degrees or more.29 Entrapment of the dorsal or volar approach. Electromyographic
ulnar nerve at the cubital tunnel has also and nerve conduction studies are indicated if
been associated with medial elbow ganglia return of function has not occurred 4 to
and osteoarthritis.30 Clinical examination 6 weeks following injury. Initially the extrem-
and electrodiagnostic tests confirm the ity should be protected and monitored for
diagnosis. Treatment should initially in- recovery. Neurapraxia may take several
clude soft bracing, night splinting, and months to resolve. Surgical intervention may
avoidance of aggravating activities and be indicated for restoring function.
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Chapter 12 Septic Arthritis 193

■ Post-Traumatic Osteoarthrosis/ synovitis is caused by deposition of calcium

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Osteoarthritis pyrophosphate dihydrate crystals. The onset
Chief Clinical Characteristics typically occurs during the sixth and seventh
This presentation typically includes pain and loss decade. Pseudogout occurs less commonly in
of elbow motion, most commonly extension. the elbow compared to the knees and wrists.
Polarized microscopic examination of aspirated
Background Information synovial fluid confirms the diagnosis. Treatment
Post-traumatic osteoarthrosis of the elbow can includes inflammation control and medication
occur following dislocation or intra-articular directed at the underlying pathophysiological
fracture. Inadequate stabilization or restora- process responsible for crystal deposition.
tion of the joint following injury contributes
to increased arthrosis.32 Plain radiographs ■ Rheumatoid Arthritis
typically show osteophytes and joint space Chief Clinical Characteristics
narrowing with this condition. Nonoperative This presentation, while variable, can involve
treatment should include activity modifica- joint pain, swelling, and tenderness. Inflam-
tion, stretching, and strengthening. Significant matory symptoms are episodic. Morning stiff-
post-traumatic arthrosis may require surgical ness, decreased stiffness with use of the involved
interventions, such as debridement and inter- joint, and symmetrical joint involvement are
positional arthroplasty. common findings. The earliest finding with
elbow involvement is loss of elbow extension.
■ Primary Osteoarthrosis/ With progression of the disease process, joint
Osteoarthritis deformity and loss of function occur.
Chief Clinical Characteristics
Background Information
This presentation may include pain at termi-
Loss of rotation may be observed secondary
nal elbow flexion and/or extension but may
to radial head subluxation and involvement
also occur throughout the range or at rest. A loss
of the distal radioulnar joint. The onset may
of elbow extension and reports of locking are
be acute or insidious. The elbow is frequently
common findings.
involved in patients with rheumatoid arthri-
Background Information tis.34 Plain radiographs indicating the lack of
Ulnar nerve irritation may be present. Pri- osteophytes and blood tests confirm the
mary degenerative arthrosis of the elbow is an diagnosis. Treatment includes medication,
uncommon disorder reported primarily in activity modification, and general condition-
middle-aged men.33 The mechanism of injury ing. Psychosocial support groups are benefi-
is repetitive use of the upper extremity. Plain cial. Surgical intervention may be necessary
radiographs that show olecranon and coro- to improve joint function. Advancements
noid osteophytes and intra-articular loose bod- in total elbow arthroplasty have resulted in
ies confirm the diagnosis.33 Nonoperative improved outcomes.35,36
treatment should include activity modifica-
tion, stretching, and strengthening. Surgical ■ Septic Arthritis
intervention may be necessary to restore func- Chief Clinical Characteristics
tional motion and eliminate locking. This presentation includes severe aching pain,
swelling, warmth, axillary adenopathy, and
■ Pseudogout fever. The fever may be low grade. The elbow is
Chief Clinical Characteristics typically held in about 80 degrees of flexion and
This presentation includes a sudden onset of passive motion is painful.37
pain, swelling, tenderness, and redness of the Background Information
superficial tissues. The elbow commonly lacks Common risk factors include rheumatoid
full extension. arthritis, immunodeficiency, intravenous drug
Background Information use, and joint replacement. Clinical examina-
This condition’s presentation is often indistin- tion, blood tests, fluid cell count, polarized
guishable from gout; however, the acute microscopic analysis, and culture of the
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194 Chapter 12 Snapping Triceps Syndrome

aspirated synovial fluid confirm the diagnosis. workers who carry heavy loads with flexed
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The treatment includes antibiotic therapy and elbows.6 Chronic cases or a history of inter-
aspiration. Surgical intervention may be neces- mittent symptoms should be classified as
sary in cases that do not respond to aspiration. distal biceps tendinosis. Treatment for distal
biceps tendinitis includes activity modifica-
■ Snapping Triceps Syndrome tion and inflammation control. Any strength
Chief Clinical Characteristics deficits should be addressed when the acute
This presentation includes a snapping sensation inflammatory stage has resolved.
over the medial epicondyle, and may occur with
concomitant chronic ulnar neuritis. The snap- ■ Distal Biceps Tendinosis
ping may be painless. Chief Clinical Characteristics
This presentation may include pain and ten-
Background Information derness deep in the anterior elbow. Repro-
The most common etiology is subluxation of duction of symptoms may occur with resisted
the medial head of the triceps over the medial elbow flexion and forearm supination.
epicondyle.38 The diagnosis is confirmed by
magnetic resonance imaging or computed Background Information
tomography.39 Selective injections may be Symptoms result from forceful and repetitive
helpful in diagnosis when the snapping is elbow flexion movements. It is a rare injury
painful. If conservative measures fail, treat- and occurs most often in power athletes and
ment involves rerouting the medial head later- workers who carry heavy loads with flexed
ally and, if needed, ulnar nerve management. elbows.6 It may be a factor in spontaneous
distal biceps ruptures. Treatment is similar to
■ Superficial Thrombophlebitis that for distal biceps tendinitis but there may
of the Cephalic or Basilic Veins not be an acute inflammatory stage. Eccen-
Chief Clinical Characteristics tric strengthening may be beneficial for
This presentation may involve aching pain in distal biceps tendinosis.
the antecubital region and/or forearm. A raised,
■ Lateral Epicondylitis
warm, red, tender cord will be palpable along
(Tennis Elbow)
the course of the involved vein.40
Chief Clinical Characteristics
Background Information This presentation is characterized by pain
This condition is associated with chronic and point tenderness at the lateral epicondyle
intravenous treatment.41 Although rare, the and the involved tendon(s), most commonly
potential exists for pulmonary embolization the extensor carpi radialis brevis. Pain is worse
or extension to deep veins. Clinical examina- with use of the arm. Reproduction of symp-
tion and Doppler ultrasonography confirm toms will occur with resisted wrist extension
the diagnosis. Treatment includes control of and passive wrist flexion with increased symp-
local pain and inflammation. Anticoagulants toms when the elbow is extended.
may be utilized in some cases; rarely,
thrombectomy is indicated. Background Information
This condition is also called tennis elbow,
TENDINOPATHIES but symptoms can result from any excessive
■ Distal Biceps Tendinitis forearm use including gardening, gripping a
heavy briefcase, and using a screwdriver. In-
Chief Clinical Characteristics
frequently, acute onset may be associated
This presentation includes pain and tender-
with a direct blow to the lateral elbow. Pos-
ness deep in the anterior elbow. Symptoms
terolateral rotatory instability may mimic or
may occur with resisted elbow flexion and
be associated with lateral epicondylitis.42,43
forearm supination.
Lateral epicondylitis occurs most commonly
Background Information between the ages of 30 and 55 years.44 If
Distal biceps tendinitis is a rare injury and symptoms are long-standing or the patient
occurs most often in power athletes and has a history of excessive forearm use, the
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Chapter 12 Triceps Tendinitis 195

classification should be lateral epicondylosis. only 15% to 20% as common as lateral

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Treatment includes inflammation control, epicondylitis.45 It occurs most commonly
stretching, postural training, bracing, and between the ages of 30 and 59 years and
strengthening when the acute inflammatory twice as often in males. Ulnar nerve symp-
phase has resolved. Avoidance of painful toms have been associated in up to 50% of
activities, modification of provoking activities cases.45 It is important to differentiate from
that cannot be avoided, and environmental medial collateral ligament rupture and insta-
adaptations (at work and leisure) are an bility. Treatment includes activity modifica-
integral part of successfully treating lateral tion, inflammation control, postural train-
epicondylitis. Infrequently, surgical treat- ing, stretching, and strengthening when the
ment is utilized to treat patients who have acute inflammatory phase has resolved.
not responded to nonoperative intervention. Chronic symptoms or a history of intermit-
tent symptoms would indicate medial epi-
■ Lateral Epicondylosis condylosis. Surgical intervention is used in
Chief Clinical Characteristics cases where symptoms have lasted for longer
This presentation may include pain and point than 1 year and have not responded to the
tenderness at the lateral epicondyle. Symptoms above interventions.
will be reproduced with resisted wrist exten-
sion and passive wrist flexion with increased ■ Medial Epicondylosis
symptoms when the elbow is extended. Chief Clinical Characteristics
This presentation may include pain and point
Background Information tenderness at the medial epicondyle. Symptoms
This condition occurs in response to repeti- will be reproduced with resisted wrist flex-
tive microtrauma from excessive use of the ion, resisted forearm pronation, and passive
wrist extensors and forearm supinators. It wrist extension with the elbow extended.
can be work induced or sports induced. Symptoms may also occur when making a
Degeneration of tendons is very common tight fist. Mild extension loss at the elbow
and may be initially asymptomatic. Lateral may occur with chronic cases.
epicondylosis is associated with aging but is
seen most commonly between the ages of Background Information
30 and 55 years. Treatment is similar to Microtrauma results from repetitive valgus
that for lateral epicondylitis but may not stress on the medial elbow and wrist flexors
have an acute inflammatory phase. Eccentric and pronators. It can be work induced or
strengthening may be beneficial in the treat- sports induced. Degeneration of tendons
ment of lateral epicondylosis. may be initially asymptomatic. Medial epi-
condylosis is seen most commonly between
■ Medial Epicondylitis the ages of 30 and 59 years. Treatment is sim-
(Golfer’s Elbow) ilar to that for medial epicondylitis. Eccen-
Chief Clinical Characteristics tric strengthening may be beneficial in the
This presentation may include pain, point treatment of medial epicondylosis.
tenderness at the common flexor origin, and
inflammation. Tenderness may also occur dis- ■ Triceps Tendinitis
tal to the medial epicondyle in the proximal Chief Clinical Characteristics
flexor and pronator mass. Reproduction of This presentation includes pain and tender-
symptoms will occur with resisted wrist flex- ness at the insertion of the triceps. Symptoms
ion, resisted forearm pronation, and passive will occur with resisted elbow extension. Symp-
wrist extension with the elbow extended. toms may be produced with full passive elbow
Making a tight fist increases pain. flexion. Elbow motion is typically not affected.
Background Information Background Information
Also called golfer’s elbow, pain may result Also called posterior tennis elbow, triceps
from throwing, tennis, golf, or any other tendinitis is uncommon as an isolated event
excessive forearm use. Medial epicondylitis is and is usually associated with other posterior
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196 Chapter 12 Triceps Tendinosis

elbow disorders (loose bodies and synovitis).44 account of the injury and plain radiographs
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It is seen more commonly in throwers but confirm the diagnosis.


can occur with any repetitive elbow exten-
sion movements. Chronic cases or a history ■ Triceps Rupture
of intermittent symptoms should be classi- Chief Clinical Characteristics
fied as triceps tendinosis. Treatment for tri- This presentation includes pain and tenderness
ceps tendinitis includes activity modification at the site of the osseous tendon insertion.
and inflammation control. When the acute Swelling and ecchymosis will be observed, as
inflammatory stage has resolved, treatment well as an inability to extend the elbow against
should also include strengthening. gravity.
■ Triceps Tendinosis Background Information
A palpable defect may be present above the
Chief Clinical Characteristics
elbow. The mechanism of injury is typically a
This presentation may be characterized by
fall on the outstretched hand, but may also
pain and tenderness at the tip at the triceps
result from direct trauma or elbow dislocation
insertion. Reproduction of symptoms will
or it may occur spontaneously or following
occur with resisted elbow extension.
surgical release and reattachment. The rupture
Background Information is not always obvious when the injury is acute
Symptoms result from forceful and repetitive and may be misdiagnosed.47 Spontaneous
elbow extension movements. It is seen more ruptures may be associated with chronic dis-
commonly in throwers and is associated with eases such as renal osteodystrophy and sec-
loose bodies and synovitis of the elbow.44 ondary hyperthyroidism.48 Clinical examina-
Radiographs may demonstrate insertional tion and magnetic resonance imaging or
calcifications. Treatment includes activity ultrasonography confirm the diagnosis.
modification, stretching, and strengthening. Complete triceps rupture is managed surgi-
Inflammation control may also be necessary. cally (Fig. 12-3); partial tendon rupture can be
Rarely, chronic cases may require surgical treated nonoperatively.47
excision of the degenerated tissue.
TUMORS
■ Terrible Triad ■ Ganglion Cyst
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation can be characterized by pain, This presentation includes a small, tender
swelling, ecchymosis, tenderness, and deformity. mass in the elbow region.
Background Information
The terrible triad is a combination of injuries
including posterior dislocation of the ulno-
humeral joint, fracture of the radial head, and
fracture of the coronoid. A comminuted radial
head fracture should be assumed a triad injury
until proven otherwise. The mechanism of
injury is typically a fall onto the outstretched
hand or direct trauma. This injury pattern is
uncommon and difficult to manage. Inade-
quate fixation results in redislocation or
chronic subluxation, and ulnohumeral arthro-
sis develops quickly in most patients.46 Loss of
motion is common with this condition. Exter-
nal fixation following surgical management
of the radial head and coronoid fractures and
lateral collateral ligament allows early motion FIGURE 12-3 Surgical approach for surgical repair
without compromising stability. A historical of a triceps tendon rupture.
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Chapter 12 Osteoid Osteoma 197

Background Information tissues. It occurs more frequently in middle-

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Ganglion cysts are single or multilobulated aged or elderly adults. Diagnosis is con-
structures that are idiopathic mucin-filled firmed with biopsy. Treatment is dependent
outgrowths of tendon sheaths. Usually they on the stage of the disease process but
are asymptomatic, but they may become generally includes radiation therapy.
painful and limit joint range of motion in
some individuals. Elbow ganglions are more ■ Osteoblastoma
common postsurgically, especially following Chief Clinical Characteristics
arthroscopic procedures. The diagnosis is This presentation is characterized by pain
confirmed with clinical examination and as- and loss of elbow motion. The pain is less
piration. Surgical excision may be indicated severe than with an osteoid osteoma.
for symptom relief.
Background Information
■ Giant Cell Tumor Osteoblastoma is an uncommon, benign
tumor. It is histologically similar to an
Chief Clinical Characteristics
osteoid osteoma but typically greater than
This presentation may include pain and tender-
2 cm in diameter. There is equal incidence
ness but the tumor is typically asymptomatic.
of this condition between sexes, usually
Background Information between the ages of 10 to 35 years.49 Plain ra-
Pain may occur from the associated inflam- diographs and computerized tomography
matory response. Giant cell tumor is rare in scan confirm the diagnosis. The tumor
the upper extremity. It affects females more requires surgical intervention.
commonly than males and occurs most
often in persons over 20 years of age.49 The ■ Osteochondroma
tumor typically occurs in the epiphyseal re- Chief Clinical Characteristics
gion and may extend to the articular surface This presentation includes pain and may im-
of the bone. Biopsy confirms the diagnosis. pair elbow motion. Symptoms are caused by
Surgical excision is required. pressure on neighboring structures.
■ Lipoma Background Information
Osteochondroma is the most common
Chief Clinical Characteristics benign bone tumor but is rare in the elbow.
This presentation typically involves a small, The tumor can occur at any age but growth
asymptomatic soft tissue mass. Occasionally the usually ceases when skeletal maturity is
lipoma may grow and become symptomatic. achieved. The tumor develops from the sur-
Background Information face of the bone and tends to extend away
Lipoma is a benign soft tissue neoplasm from the joint. Plain radiographs confirm the
comprised of a localized collection of adi- diagnosis. Surgical excision may be necessary
pose tissue. It is the most common tumor in to relieve symptoms and/or restore motion.
the elbow region.50 Biopsy or magnetic reso-
nance imaging confirms the diagnosis. Sur- ■ Osteoid Osteoma
gical excision may be indicated if the lipoma Chief Clinical Characteristics
increases in size, becomes symptomatic, or This presentation may include sharp,
interferes with function. unremitting pain that is worse at night and
relieved significantly with aspirin. The pain
■ Malignant Lymphoma often increases with alcohol intake. The
Chief Clinical Characteristics patient may have a progressive loss of elbow
This presentation typically includes pain and flexion or extension motion.
sometimes swelling in the region of the lesion.
Background Information
Background Information Osteoid osteoma is a benign tumor, usually
Malignant lymphoma is a malignant bone no greater than 1 cm in diameter. It typically
tumor. The tumor may extend into the soft affects persons 5 to 25 years of age, with a
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198 Chapter 12 Osteosarcoma

greater occurrence in males. Plain radi- extremity, but it does occur in the elbow
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ographs and computerized tomography scan region. The tumor is more common in
confirm the diagnosis. The tumor typically young or middle-aged adults. Biopsy con-
requires surgical excision. firms the diagnosis. The most optimal prog-
nosis is achieved with wide excision or
■ Osteosarcoma radical surgery. Radiation therapy may be
Chief Clinical Characteristics combined with the surgery.
This presentation can involve pain with pro-
gressing severity and eventual swelling. The ■ Valgus Extension Overload
overlying skin may be warm. Loss of elbow mo- Syndrome
tion may occur secondary to swelling. The Chief Clinical Characteristics
patient may report weight loss. This presentation includes posterior elbow pain,
Background Information tenderness of the olecranon, flexion contrac-
Osteosarcoma is one of the most common ture, and pain with passive elbow extension.
bone malignancies but is uncommon in the Increased valgus laxity and painful locking may
elbow. The tumor frequently metastasizes. also occur. Reproduction of symptoms will occur
Osteosarcoma is more common in males with the valgus extension snap maneuver in
and usually occurs between the ages of 10 which a firm valgus stress is placed on the elbow
and 20 years of age. Diagnosis is confirmed and the elbow is then snapped into extension.52
with biopsy. Treatment usually consists of Background Information
surgical ablation and chemotherapy. This syndrome occurs most commonly in ath-
■ Synovial Chondromatosis letes who do a lot of overhead throwing. The
repetitive hyperextension stress combined
Chief Clinical Characteristics with medial elbow laxity results in impinge-
This presentation includes pain, swelling, and ment of the olecranon in the olecranon fossa.
limited elbow motion. Patients may report The impingement results in osteophytes and
locking or catching.51 The patient may report loose bodies. Valgus extension overload syn-
symptoms lasting several years. drome is almost always progressive. Clinical
Background Information examination and radiographs confirm the
Synovial chondromatosis is benign and in- diagnosis. Treatment includes inflammation
volves the subsynovial connective tissue of control and activity modification. Once the
joints, tendon sheaths, or bursa. It can occur inflammatory stage has resolved, stretching,
at any joint. The tumor typically occurs in strengthening, and a gradual return to throw-
adults in their 20s to 40s, with males affected ing are indicated. Surgical intervention may be
more commonly than females.49 Clinical ex- necessary for patients who do not respond to
amination and plain radiographs confirm nonoperative treatment.
the diagnosis. The treatment includes surgi-
cal removal of the involved synovium and References
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loose osteochondromatous bodies. Long- Allison S. Reliability and diagnostic accuracy of the clini-
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the ulna. J Bone Joint Surg Am. Oct 1989;71(9):1348–1354. 31. Perry CR. Bone and Joint Infections. St. Louis, MO:
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oblique compression fracture of the coronoid process of 32. Doornberg J, Ring D, Jupiter JB. Effective treatment of
the ulna. J Shoulder Elbow Surg. Jan–Feb 2005;14(1): fracture-dislocations of the olecranon requires a stable
60–64. trochlear notch. Clin Orthop Relat Res. Dec 2004(429):
13. O’Driscoll SW, Jupiter JB, Cohen MS, Ring D, McKee 292–300.
MD. Difficult elbow fractures: pearls and pitfalls. Instr 33. Antuna SA, Morrey BF, Adams RA, O’Driscoll SW.
Course Lect. 2003;52:113–134. Ulnohumeral arthroplasty for primary degenerative
14. Ristic S, Strauch RJ, Rosenwasser MP. The assessment arthritis of the elbow: long-term outcome and complica-
and treatment of nerve dysfunction after trauma tions. J Bone Joint Surg Am. Dec 2002;84-A(12):2168–2173.
around the elbow. Clin Orthop Relat Res. Jan 2000(370): 34. Lems WF, Dijkmans BAC. Rheumatoid arthritis: clinical
138–153. picture and its variants. In: Firestein GS, Panayi GS,
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adults. J Bone Joint Surg Am. Dec 1998;80(12):1733–1744. in Pathogenesis and Treatment. Oxford, UK: Oxford
16. Harness N, Ring D, Jupiter JB. Olecranon fractures. In: University Press; 2000.
Mirzayan R, Itamura J, eds. Shoulder and Elbow Trauma. 35. Hargreaves D, Emery R. Total elbow replacement in the
New York, NY: Thieme Medical Publishers; 2004:53–66. treatment of rheumatoid disease. Clin Orthop Relat Res.
17. Itamura J, Roidis N, Mirzayan R, Vaishnav S, Learch T, Sep 1999(366):61–71.
Shean C. Radial head fractures: MRI evaluation of asso- 36. Gill DR, Morrey BF. The Coonrad-Morrey total elbow
ciated injuries. J Shoulder Elbow Surg. Jul–Aug arthroplasty in patients who have rheumatoid arthritis.
2005;14(4):421–424. A ten to fifteen-year follow-up study. J Bone Joint Surg
18. Early SD, Tolo VT. Pediatric elbow fractures. In: Mirza- Am. Sep 1998;80(9):1327–1335.
yan R, Itamura J, eds. Shoulder and Elbow Trauma. New 37. Butters KP, Morrey BF. Septic arthritis. In: Morrey BF,
York, NY: Thieme Medical Publishers; 2004:115–131. ed. The Elbow and Its Disorders. 3rd ed. Philadelphia, PA:
19. Ring D, Jupiter JB, Sanders RW, Mast J, Simpson NS. Saunders; 2000:809–817.
Transolecranon fracture-dislocation of the elbow. J 38. Dreyfuss U, Kessler I. Snapping elbow due to dislocation
Orthop Trauma. Nov 1997;11(8):545–550. of the medial head of the triceps. A report of two cases.
20. Morrey BF. Ectopic ossification about the elbow. In: J Bone Joint Surg Br. Feb 1978;60(1):56–57.
Morrey BF, ed. The Elbow and Its Disorders. 3rd ed. 39. Spinner RJ, Goldner RD. Snapping of the medial head of
Philadelphia, PA: Saunders; 2000:437–446. the triceps and recurrent dislocation of the ulnar nerve.
21. Chen FS, Mirzayan R, Itamura JM. The post-traumatic Anatomical and dynamic factors. J Bone Joint Surg Am.
stiff elbow: overview and management. In: Mirzayan R, Feb 1998;80(2):239–247.
Itamura JM, eds. Shoulder and Elbow Trauma. New York: 40. Goodman CC, Snyder TEK. Differential diagnosis in phys-
Thieme Medical Publishers; 2004:99–114. ical therapy. 2nd ed. Philadelphia, PA: Saunders; 1995.
22. O’Driscoll SW. Classification and evaluation of recur- 41. De Sanctis MT, Cesarone MR, Incandela L, Belcaro G,
rent instability of the elbow. Clin Orthop Relat Res. Griffin M. Treatment of superficial vein throm-
Jan 2000(370):34–43. bophlebitis of the arm with Essaven gel—a placebo-
23. O’Driscoll SW, Lawton RL, Smith AM. The “moving controlled, randomized study. Angiology. Dec 2001;52
valgus stress test” for medial collateral ligament tears of (suppl 3):S63–67.
the elbow. Am J Sports Med. Feb 2005;33(2):231–239. 42. Baker CLJ, Nirschl RP. Lateral tendon injury: open and
24. Rettig AC, Sherrill C, Snead DS, Mendler JC, Mieling P. arthroscopic treatment. In: Altchek DW, Andrews JR,
Nonoperative treatment of ulnar collateral ligament in- eds. The Athlete’s Elbow. Philadelphia, PA: Lippincott
juries in throwing athletes. Am J Sports Med. Jan–Feb Williams & Wilkins; 2001:91–103.
2001;29(1):15–17. 43. Kalainov DM, Cohen MS. Posterolateral rotatory insta-
25. Pecina MM, Krmpotic-Nemanic J, Markiewitz AD. Tun- bility of the elbow in association with lateral epicondyli-
nel Syndromes Peripheral Nerve Compression Syndromes. tis. A report of three cases. J Bone Joint Surg Am. May
2nd ed. Boca Raton, FL: CRC Press; 1997. 2005;87(5):1120–1125.
26. Ritts GD, Wood MB, Linscheid RL. Radial tunnel syn- 44. Nirschl RP. Muscle and tendon trauma: tennis elbow
drome. A ten-year surgical experience. Clin Orthop Re- tendinosis. In: Morrey BF, ed. The Elbow and Its Disor-
lat Res. Jun 1987(219):201–205. ders. 3rd ed. Philadelphia, PA: Saunders; 2000:523–535.
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45. Gabel GT, Morrey BF. Medial Epicondylitis. In: Morrey 49. Levesque J, Marx R, Bell RS, Wunder JS, Kandel R,
ELBOW PAIN

BF, ed. The Elbow and Its Disorders. 3rd ed. Philadelphia, White LM. A Clinical Guide to Primary Bone Tumors.
PA: Saunders; 2000:537–542. Baltimore, MD: Williams & Wilkins; 1998.
46. Ring D, Jupiter JB, Zilberfarb J. Posterior dislocation 50. Pritchard DJ, Unni KK. Neoplasms of the elbow. In:
of the elbow with fractures of the radial head and Morrey BF, ed. The Elbow and Its Disorders. 3rd ed.
coronoid. J Bone Joint Surg Am. Apr 2002;84-A(4): Philadelphia, PA: Saunders; 2000:873–889.
547–551. 51. Kamineni S, O’Driscoll SW, Morrey BF. Synovial osteo-
47. van Riet RP, Morrey BF, Ho E, O’Driscoll SW. Surgical chondromatosis of the elbow. J Bone Joint Surg Br.
treatment of distal triceps ruptures. J Bone Joint Surg Sep 2002;84(7):961–966.
Am. Oct 2003;85-A(10):1961–1967. 52. Wilk K, Andrews JA. Elbow injuries. In: Brotzman SB, Wilk
48. Pina A, Garcia I, Sabater M. Traumatic avulsion of the tri- K, eds. Clinical Orthopedic Rehabilitation. Philadelphia, PA:
ceps brachii. J Orthop Trauma. Apr 2002;16(4):273–276. Mosby; 2003:85–123.
1528_Ch13_201-239 11/05/12 3:57 PM Page 201

CHAPTER 13
Wrist and Hand Pain
■ Robin I. Burks, PT, DPT, CHT ■ Stephen Schnall, MD

Description of the Symptom wrist and hand pain are defined as pathology
that occurs within the distal one-quarter of the
Wrist and hand pain, as discussed in this radius and ulna, carpals, metacarpals, and
chapter, includes diffuse/general wrist and phalanges; component articulations of the
hand pain, central wrist pain, radial wrist pain, wrist and hand; and associated soft tissue
ulnar wrist pain, dorsal hand pain, palmar structures. Remote causes are defined as those
hand pain, and digit pain. Local causes of occurring outside this region.

Digital Palmar Radial Dorsal Digital


pain hand pain wrist pain hand pain pain

Ulnar Central
wrist pain wrist pain
Palmar ulnar view Dorsal ulnar view

Special Concerns ■ Skin break and fever


■ Decreased pulses ■ Sudden or progressive paresthesia or loss of
■ Fracture with suspected skin break sensation associated with trauma
■ Pain in bilateral hands provoked with ■ Warmth and swelling associated with a
cervical movements fever or purulent drainage
■ Pain out of proportion to the injury
■ Severe edema following trauma in areas
with fascial muscle compartments

201
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202 Chapter 13 Wrist and Hand Pain

CHAPTER PREVIEW: Conditions That May Lead to Wrist and Hand Pain
WRIST AND HAND PAIN

T Trauma
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Radiculopathies: Not applicable Fractures: Fractures:
• C6 radiculopathy 214 • Capitate and lunate • Distal radius
Ulnar nerve fractures 221 fracture 222
compression at the • Distal radioulnar joint • Scaphoid
elbow (cubital fracture/ fracture 223
tunnel syndrome) 218 dislocation 221 • Thumb metacarpal
Lunate dislocation 225 base fracture
Nerve injuries: (Bennett’s or
• Median nerve Rolando’s
compression fracture) 224
at the wrist Nerve injuries:
(carpal tunnel • Radial sensory
syndrome) 226 nerve injury
Scapholunate (without
ligament sprain 231 entrapment) 227

UNCOMMON
Pronator syndrome 214 Not applicable Not applicable Nerve injuries:
Radiculopathies: • Radial sensory
• C7 radiculopathy 214 nerve entrapment
• C8 radiculopathy 215 (Wartenberg’s
Thoracic outlet syndrome) 226
syndrome 216

RARE
Not applicable Not applicable Not applicable Not applicable
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Chapter 13 Wrist and Hand Pain 203

WRIST AND HAND PAIN


LOCAL LOCAL LOCAL
ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Fractures: Fractures: Fractures: Acute central slip


• Distal radioulnar • Metacarpal fractures • Hamate fracture rupture (Boutonnière
joint fracture/ (including Boxer’s (hook) 222 deformity) 218
dislocation 221 fracture) 223 Nerve injuries: Flexor digitorum
• Distal radius Nerve injuries: • Median nerve profundus
fracture 222 • Radial sensory nerve compression at the avulsion/rupture
• Distal ulna entrapment wrist (carpal tunnel (jersey finger) 221
fracture 222 (Wartenberg’s syndrome) 226 Fractures:
• Hamate fracture syndrome) 226 • Phalangeal
(non-hook) 222 • Radial sensory nerve fracture 223
• Pisiform injury (without • Thumb metacarpal
fracture 223 entrapment) 227 base fracture
• Triquetrum (Bennett’s or
fracture 224 Rolando’s
Lunotriquetral fracture) 224
ligament tear (and Interphalangeal
lunotriquetral joint dislocation 225
dissociation) 226 Nerve injuries:
• Median nerve
compression at the
wrist (carpal tunnel
syndrome) 226
• Radial sensory nerve
injury (without
entrapment) 227
Terminal extensor
tendon rupture
(Mallet finger) 234
Ulnar collateral
ligament tear
(Gamekeeper’s or
Skier’s thumb) 237

Nerve injuries: Not applicable Not applicable Nerve injuries:


• Ulnar nerve • Ulnar digital nerve
compression at compression (Bowler’s
the wrist (Guyon’s thumb) 227
canal) 227 Subungual
Triangular hematoma 232
fibrocartilage
complex tear 234

Not applicable Not applicable Not applicable Not applicable


(continued)
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204 Chapter 13 Wrist and Hand Pain


WRIST AND HAND PAIN

I Inflammation
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Aseptic Not applicable Aseptic Aseptic
Trigger points 216 Tendinopathies: Tendinopathies:
• Extensor digitorum • Extensor pollicis
Septic communis longus
Not applicable tendinitis 233 tendinitis 233
• First dorsal
Septic compartment
Not applicable (De Quervain’s)
tenosynovitis 234
Septic
Not applicable
UNCOMMON
Aseptic Aseptic Aseptic Aseptic
Not applicable Complex regional pain Not applicable Flexor carpi
syndrome 220 radialis tunnel
Septic Inflammatory arthritis Septic syndrome 220
Herpes zoster/ (including Osteomyelitis:
post-herpetic rheumatoid, • Wrist and hand 229 Septic
neuralgia 213 psoriatic, Septic arthritis 231 Osteomyelitis:
scleroderma, and • Wrist and hand 229
systemic lupus Septic arthritis 231
erythematosus) 225
Septic
Septic arthritis 231

RARE
Aseptic Aseptic Aseptic Aseptic
Not applicable Arthritis associated Arthritis associated Arthritis associated
with inflammatory with inflammatory with inflammatory
Septic bowel disease 218 bowel disease 218 bowel disease 218
Cervical epidural Reiter’s syndrome 231 Foreign body Foreign body
abscess 213 reaction 221 reaction 221
Cervical osteomyelitis 213 Septic Intersection
Cellulitis 219 Septic syndrome 225
Lyme disease 226 Not applicable
Septic
Not applicable
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Chapter 13 Wrist and Hand Pain 205

WRIST AND HAND PAIN


LOCAL LOCAL LOCAL
ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Aseptic Aseptic Aseptic Aseptic


Tendinopathies: Tendinopathies: Stenosing Stenosing
• Extensor carpi • Extensor digitorum tenosynovitis tenosynovitis
ulnaris communis (trigger (trigger
tendinitis 232 tendinitis 233 finger/thumb) 231 finger/thumb) 231
• Flexor carpi ulnaris
tendinitis 234 Septic Septic Septic
Not applicable Not applicable Paronychia 230
Septic
Not applicable

Aseptic Aseptic Aseptic Aseptic


Ulnocarpal Not applicable Not applicable Inflammatory arthritis
impaction/abutment (including
syndrome 237 Septic Septic rheumatoid,
Osteomyelitis: Osteomyelitis: psoriatic,
Septic • Wrist and hand • Wrist and hand scleroderma, and
Osteomyelitis: 229 229 systemic lupus
• Wrist and hand Septic arthritis 231 Septic arthritis 231 erythematosus) 225
229
Septic arthritis 231 Septic
Animal bite
infections 218
Felon 220
Human bite
infections 224
Osteomyelitis:
• Wrist and hand 229
Septic arthritis 231
Suppurative
digital flexor
tenosynovitis 232

Aseptic Aseptic Aseptic Aseptic


Arthritis associated Arthritis associated Arthritis associated Arthritis associated
with inflammatory with inflammatory with inflammatory with inflammatory
bowel disease 218 bowel disease 218 bowel disease 218 bowel disease 218
Foreign body Foreign body Foreign body Foreign body reaction
reaction 221 reaction 221 reaction 221 221
Septic Septic Septic Septic
Not applicable Not applicable Not applicable Not applicable

(continued)
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206 Chapter 13 Wrist and Hand Pain


WRIST AND HAND PAIN

M Metabolic
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Not applicable Gout 224 Gout 224 Gout 224
Pseudogout 230 Pseudogout 230 Pseudogout 230
UNCOMMON
Not applicable Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable Not applicable

Va Vascular
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Not applicable Not applicable Not applicable Not applicable
UNCOMMON
Myocardial infarction 214 Vascular Avascular necrosis: Vascular
malformation 238 • Avascular necrosis malformation 238
of the lunate
(Kienböck’s
disease) 219
Vascular
malformation 238

RARE
Not applicable Compartment Not applicable Avascular necrosis:
syndrome 219 • Avascular necrosis
Emboli 220 of the scaphoid
(Preiser’s
disease) 219

De Degenerative
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Not applicable Not applicable Osteoarthrosis/ Osteoarthrosis/
osteoarthritis: osteoarthritis:
• Intercarpal • Intercarpal
osteoarthrosis/ osteoarthrosis/
osteoarthritis 228 osteoarthritis 228
Tendinopathies: • Radioscaphoid
• Extensor digitorum osteoarthrosis/
communis osteoarthritis 228
tendinosis 233
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Chapter 13 Wrist and Hand Pain 207

WRIST AND HAND PAIN


LOCAL LOCAL LOCAL
ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Gout 224 Gout 224 Gout 224 Gout 224


Pseudogout 230 Pseudogout 230 Pseudogout 230 Pseudogout 230

Not applicable Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Not applicable Not applicable Not applicable Not applicable

Vascular Vascular Vascular Hypothenar hammer


malformation 238 malformation 238 malformation 238 syndrome 224
Peripheral vascular
disease 230
Raynaud’s phenomenon/
disease 230
Vascular
malformation 238

Not applicable Not applicable Not applicable Emboli 220

LOCAL LOCAL LOCAL


ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Osteoarthrosis/ Tendinopathies: Not applicable Osteoarthrosis/


osteoarthritis: • Extensor digitorum osteoarthritis:
• Distal radioulnar communis • Distal interphalangeal
joint osteoarthrosis/ tendinosis 233 joint osteoarthrosis/
osteoarthritis 227 osteoarthritis 227
• Intercarpal • Metacarpophalangeal
osteoarthrosis/ joint osteoarthrosis/
osteoarthritis 228 osteoarthritis 228

(continued)
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208 Chapter 13 Wrist and Hand Pain

Degenerative (continued)
WRIST AND HAND PAIN

LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
• Thumb
carpometacarpal
joint osteoarthrosis/
osteoarthritis
(basilar joint or
CMC arthrosis) 229

UNCOMMON
Cervical degenerative Osteoarthrosis/ Osteoarthrosis/ Not applicable
disk disease 212 osteoarthritis: osteoarthritis:
Cervical degenerative • Scapholunate • Distal radioulnar
joint disease 213 advanced collapse joint osteoarthrosis/
(SLAC) wrist 229 osteoarthritis 227
RARE
Not applicable Not applicable Not applicable Not applicable

Tu Tumor
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Not applicable Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Malignant Primary: Malignant Primary:
Not applicable Not applicable
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign, such as: Benign, such as:
• Ganglion cyst 235 • Ganglion cyst 235
RARE
Malignant Primary, Malignant Primary: Malignant Primary: Malignant Primary:
such as: Not applicable Not applicable Not applicable
• Pancoast tumor 217 Malignant Metastatic, Malignant Malignant
Malignant Metastatic, such as: Metastatic, such as: Metastatic, such as:
such as: • Metastases to the • Metastases to the • Metastases to the
• Metastases to the wrist and hand, wrist and hand, wrist and hand,
cervical spine or other including from primary including from primary including from
tissue adjacent to breast, kidney, lung, breast, kidney, lung, primary breast, kidney,
nerves to the upper prostate, and thyroid prostate, and thyroid lung, prostate, and
extremity 217 disease 236 disease 236 thyroid disease 236
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Chapter 13 Wrist and Hand Pain 209

WRIST AND HAND PAIN


LOCAL LOCAL LOCAL
ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

• Pisotriquetral • Proximal
osteoarthrosis/ interphalangeal joint
osteoarthritis 228 osteoarthrosis/
Tendinopathies: osteoarthritis 228
• Extensor • Sesamoid
carpi ulnaris osteoarthrosis/
tendinosis 232 osteoarthritis
(thumb) 229
• Thumb carpometacarpal
joint osteoarthrosis/
osteoarthritis (basilar
joint or CMC
arthrosis) 229

Tendinopathies: Not applicable Not applicable Not applicable


• Flexor carpi ulnaris
tendinosis 234

Not applicable Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Not applicable Not applicable Not applicable Not applicable

Malignant Primary: Malignant Primary: Malignant Primary: Malignant Primary:


Not applicable Not applicable Not applicable Not applicable
Malignant Metastatic: Malignant Metastatic: Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable Not applicable Not applicable
Benign, such as: Benign, such as: Benign, such as: Benign, such as:
• Ganglion cyst 235 • Ganglion cyst 235 • Ganglion cyst 235 • Ganglion cyst 235

Malignant Primary: Malignant Primary, Malignant Primary, Malignant Primary,


Not applicable such as: such as: such as:
Malignant • Chondrosarcoma 235 • Chondrosarcoma • Chondrosarcoma 235
Metastatic, such as: • Epithelioid 235 • Epithelioid sarcoma 235
• Metastases to the sarcoma 235 • Osteosarcoma 237 • Osteosarcoma 237
wrist and hand, • Osteosarcoma 237 • Squamous cell
including from primary • Sarcoidosis 231 carcinoma 237
breast, kidney, lung, • Squamous cell
prostate, and thyroid carcinoma 237
disease 236
(continued)
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210 Chapter 13 Wrist and Hand Pain

Tumor (continued)
WRIST AND HAND PAIN

LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
RARE
Benign, such as: Benign, such as: Benign, such as: Benign, such as:
• Meningioma 217 • Sarcoidosis 231 • Osteoblastoma 236 • Osteoblastoma 236
• Syringomyelia 217 • Osteoid • Osteoid
osteoma 237 osteoma 237
• Sarcoidosis 231 • Sarcoidosis 231

Co Congenital
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Not applicable Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
COMMON
Not applicable Not applicable Not applicable Not applicable
UNCOMMON
Somatoform disorders: Not applicable Not applicable Not applicable
• Hypochondriasis 215
• Malingering 215
• Pain disorder 216
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Chapter 13 Wrist and Hand Pain 211

WRIST AND HAND PAIN


LOCAL LOCAL LOCAL
ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Benign, such as: Malignant Metastatic, Malignant Metastatic, Malignant Metastatic,


• Osteoblastoma 236 such as: such as: such as:
• Sarcoidosis 231 • Metastases to wrist • Metastases to the • Metastases to the
and hand, including wrist and hand, wrist and hand,
from primary breast, including from including from primary
kidney, lung, primary breast, breast, kidney, lung,
prostate, and thyroid kidney, lung, prostate, and thyroid
disease 236 prostate, and thyroid disease 236
Benign, such as: disease 236 Benign, such as:
• Enchondroma 235 Benign, such as: • Enchondroma 235
• Epithelial inclusion • Enchondroma 235 • Epithelial inclusion
cyst 235 • Epithelial inclusion cyst 235
• Osteoblastoma 236 cyst 235 • Giant cell tumor of the
• Giant cell tumor tendon sheath 235
of the tendon • Glomus tumor 236
sheath 235 • Mucous cyst 236
• Osteoblastoma 236 • Osteochondroma 236
• Sarcoidosis 231 • Osteoid osteoma 237
• Sarcoidosis 231

LOCAL LOCAL LOCAL


ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Not applicable Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Not applicable Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable Not applicable

(continued)
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212 Chapter 13 Cervical Degenerative Disk Disease

Neurogenic/Psychogenic (continued)
WRIST AND HAND PAIN

LOCAL DIFFUSE/
GENERAL WRIST LOCAL LOCAL
REMOTE AND HAND CENTRAL WRIST RADIAL WRIST
RARE
Somatoform disorders: Not applicable Not applicable Not applicable
• Factitious disorder 215
• Somatization
disorder 216

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Wrist and Hand Pain degenerative annular tears, loss of disk height,
narrowing of the spinal canal, or compression of
Due to the delicacy of structures in the hand, the spinal cord or nerve roots, resulting in
both examination and treatment of the wrist radiculopathy. Upper extremity symptoms
and hand should be performed with great care. occur when pathology progresses to the point of
A partial ligament or tendon rupture that could encroachment on the neural foramina. The
have been treated conservatively may become a diagnosis is confirmed with plain radiographs
complete injury requiring surgery following an that reveal decreased disk height and osteophyte
overly vigorous examination. Plain radiographs formation along the anterior and posterior mar-
should be taken after any injury to the hand to gins of the intervertebral joint.1 Surgery may be
rule out fractures, dislocations, foreign bodies, necessary in severe and resistant cases that do
or other pathology. Injuries with fractures or not respond to nonoperative treatment, includ-
suspected tendon or ligament injuries should ing severe or worsening neurological deficits.
be referred to a hand surgeon for further con-
sultation. Splinting is an essential part of treat-
ment for many hand problems (Box 13-1). BOX 13-1 Conditions of the Wrist and
Some can be treated with prefabricated splints, Hand That May Require
but many require splints to be custom-made by Splinting
the therapist. Therapists unfamiliar with splint- ● Acute central slip rupture (Boutonnière
ing should consider referring these cases to a deformity)
colleague, at least for the splint fabrication. ● Extensor carpi ulnaris tendinitis/tendinosis

● Extensor digitorum communis tendinitis/


Description of Conditions That tendinosis
May Lead to Wrist and Hand Pain ● Extensor pollicis longus tendinitis/tendinosis

Remote ● First dorsal compartment (De Quervain’s)


tenosynovitis
■ Cervical Degenerative Disk ● Flexor carpi radialis tunnel syndrome
Disease
● Flexor carpi ulnaris tendinitis/tendinosis
Chief Clinical Characteristics
● Interphalangeal joint dislocation
This presentation typically includes pain in the
● Median nerve compression at the wrist
posterior or posterolateral region of the neck
(carpal tunnel syndrome)
with or without radiculopathy.
● Ulnar collateral ligament tear (gamekeeper’s
Background Information or skier’s thumb)
This condition is associated with advanced age ● Ulnar nerve compression at the elbow (cubital
and most commonly affects the C5–C6 and tunnel syndrome)
C6–C7 levels. Patients may develop osteophytes,
1528_Ch13_201-239 11/05/12 3:57 PM Page 213

Chapter 13 Herpes Zoster/Post-Herpetic Neuralgia 213

WRIST AND HAND PAIN


LOCAL LOCAL LOCAL
ULNAR WRIST DORSAL HAND PALMAR HAND LOCAL DIGITS

Not applicable Not applicable Not applicable Not applicable

■ Cervical Degenerative Joint immunodeficiency syndrome or diabetes mel-


Disease litus. This condition is caused by an infection
Chief Clinical Characteristics that usually results from bloodborne spread of
This presentation can involve pain in the bacteria from an infection site elsewhere in the
posterior or posterolateral aspect of the neck body. It also may be associated with intra-
with possible radiation to the upper extremity, venous drug use. The diagnosis is confirmed
possibly associated with dermatomal sensory loss with magnetic resonance imaging.3 This con-
and myotomal weakness. dition is a medical emergency.

Background Information ■ Cervical Osteomyelitis


This condition becomes more common with Chief Clinical Characteristics
advancing age. Osteoarthrosis (osteoarthritis) This presentation typically includes upper extrem-
of the facet joints, hypertrophy of the ligamen- ity pain, sensory loss, and weakness possibly
tum flavum, and osteophyte formation around associated with fever and neck pain. Fever may
the intervertebral foramen can contribute to or may not be present. Extremity symptoms may
symptoms. It usually presents without radicu- occur in single or multiple dermatomal patterns.
lar symptoms; upper extremity symptoms
occur when pathology progresses to the point Background Information
of encroachment on the neural foramina. Cervical osteomyelitis is often associated with
Plain radiographs and magnetic resonance im- immune suppression (acquired immunodefi-
aging confirm the diagnosis.2 Surgery may be ciency syndrome, diabetes mellitus, liver fail-
necessary in severe and resistant cases that do ure), intravenous drug abuse, or infection in
not respond to nonoperative treatment, espe- other areas. Epidural abscess, discitis, segmental
cially those that involve severe or progressing deformity, or instability may develop as a result
neurological deficits. of this condition. The diagnosis is confirmed
with plain radiographs or magnetic resonance
■ Cervical Epidural Abscess imaging of the cervical spine.4,5 Individuals
suspected of having this condition must be re-
Chief Clinical Characteristics
ferred to a physician for medical management,
This presentation may include neck pain and
which includes antibiotics and surgery.
fever. Extremity pain develops a few days later
due to nerve root irritation or compression. ■ Herpes Zoster/Post-Herpetic
Symptoms may be unilateral or bilateral, involv- Neuralgia
ing single or multiple dermatomes depending
Chief Clinical Characteristics
on the specific location of the infection.
This presentation is characterized by hypersen-
Background Information sitivity and pain in a unilateral dermatomal
This condition is often associated with im- distribution, associated with a vivid red skin rash
mune suppressive conditions such as acquired that is distinguished by small vesicles filled with
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214 Chapter 13 Myocardial Infarction

clear fluid. Pain may persist for up to a year distinguish this condition from carpal tunnel
WRIST AND HAND PAIN

following resolution of the rash (post-herpetic syndrome.8 Diagnosis is usually made based
neuralgia), although the typical duration of on clinical findings; electromyogram/nerve
symptoms is a few months. conduction studies are used to confirm the
diagnosis. Surgical release at the site of com-
Background Information
pression is generally indicated if symptoms are
This condition occurs when varicella virus is
severe.
latently reactivated in a spinal ganglion. This
condition may present initially without the RADICULOPATHIES
rash, leading to an incorrect diagnosis of
■ C6 Radiculopathy
radiculopathy due to the distribution of symp-
toms. The presence of the rash, extreme pain, Chief Clinical Characteristics
unilateral involvement, general malaise, and This presentation typically includes pain,
unclear association with spinal movement aids paresthesia, and numbness of the neck, shoul-
in differential diagnosis.6 Treatment with an- der, upper arm, radial forearm and wrist,
tiviral and anti-inflammatory medications is entire surface of the thumb, and possibly in-
most effective if started as soon as possible. dex finger. Weakness of forearm pronation or
wrist extension, or changes in the biceps or
■ Myocardial Infarction brachioradialis reflex may be noted. Arm and
Chief Clinical Characteristics hand use may worsen symptoms.
This presentation typically involves pain and Background Information
pressure in the chest or between shoulder blades This condition may be caused by cervical disk
that may radiate to upper extremities, neck, protrusion, arthritic spurs on the cervical
torso, or jaw. Infrequently, symptoms may re- facet joints, and tumors or other space-
fer distally to the wrist and hand. Pain may be occupying lesions in the spine.9 A negative
worsened by physical exertion. upper limb tension test is useful in ruling out
Background Information the condition. The combination of positive
Other findings include diaphoresis, dyspnea, upper limb tension test, cervical rotation to-
dizziness, loss of consciousness, pallor, and ward the involved side less than 60 degrees,
tachycardia. Blood pressure may be normal, decreased symptoms with cervical distrac-
decreased, or elevated. Symptoms are respon- tion, and positive Spurling’s test increases the
sive to nitroglycerin.7 This health condition likelihood of cervical radiculopathy to 90%.10
occurs when occlusion of a coronary artery re- Cervical spine magnetic resonance imaging
sults in myocardial ischemia. Echocardiogra- confirms the diagnosis. Depending on the eti-
phy, serial blood tests, and angiogram confirm ology, cervical radiculopathy can often be
the diagnosis. This condition is a medical managed nonsurgically, but must be moni-
emergency. tored for worsening symptoms, especially
constant numbness and developing weakness.
■ Pronator Syndrome
Chief Clinical Characteristics ■ C7 Radiculopathy
This presentation typically includes pain, tin- Chief Clinical Characteristics
gling, and numbness in the volar aspect of the This presentation typically includes pain,
thumb, index, and middle and the radial half paresthesia, and numbness of the neck,
of the ring fingers, as well as pain in the ante- shoulder, upper arm, dorsal forearm and
rior surface of the distal arm and proximal fore- wrist, and the entire surface of the middle
arm and weakness of the flexor pollicis longus finger. Weakness with elbow extension, wrist
and the flexor digitorum profundus to the flexion, and finger extension or changes in
index and middle fingers. the triceps reflex may be noted. Arm and hand
use may worsen symptoms.
Background Information
This condition involves compression of the Background Information
median nerve in the antecubital area. Negative This condition may be caused by cervical disk
Phalen’s and Tinel’s tests at the wrist will help protrusion, arthritic spurs on the cervical
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Chapter 13 Malingering 215

facet joints, and tumors or other space- can affect the patient’s perception of physical

WRIST AND HAND PAIN


occupying lesions in the spine.9 A negative well-being and lead to exaggeration or mis-
upper limb tension test is useful in ruling out representation of symptoms. This can com-
the condition. The combination of positive plicate the diagnostic process. Clinical psy-
upper limb tension test, cervical rotation to- chiatric disorders may, in addition, be a
ward the involved side less than 60 degrees, primary cause of perceived wrist and hand
decreased symptoms with cervical distrac- pain. Somatoform disorders involve psychi-
tion, and positive Spurling’s test increases the atric problems that manifest as apparent
likelihood of cervical radiculopathy to 90%.10 physical symptoms.
Cervical spine magnetic resonance imaging
confirms the diagnosis. Depending on the eti- ■ Factitious Disorder
ology, cervical radiculopathy can often be Chief Clinical Characteristics
managed nonsurgically, but must be moni- This presentation is characterized by inten-
tored for worsening symptoms, especially tional production or feigning of physical
constant numbness and developing weakness. symptoms that could include wrist and hand
symptoms. These patients are motivated by a
■ C8 Radiculopathy desire to assume the sick role. When perceived
Chief Clinical Characteristics as being sick, these patients derive emotional
This presentation typically includes pain, rewards such as increased tolerance, attention, or
numbness, and tingling throughout the small compassion from family, friends, or coworkers.
finger, ulnar border of the hand, and medial
Background Information
aspect of the forearm. There may be weakness
External motivation (eg, obtaining narcotic
of extensor indicis, flexor pollicis longus, and
medication, financial gain through litigation or
hand intrinsics. Arm and hand use may
disability payments) to assume the sick role is
worsen symptoms.
absent in this condition. Evaluation by a clini-
Background Information cal psychologist or psychiatrist is necessary to
This condition may be caused by cervical disk confirm the diagnosis.11 This health condition
protrusion, arthritic spurs on the cervical facet may be amenable to a combination of pharma-
joints, and tumors or other space-occupying cologic and cognitive-behavioral management.
lesions in the spine.9 A negative upper limb
tension test is useful in ruling out the condi- ■ Hypochondriasis
tion. The combination of positive upper limb Chief Clinical Characteristics
tension test, cervical rotation toward the in- This presentation typically involves fear of hav-
volved side less than 60 degrees, decreased ing a serious medical condition despite its
symptoms with cervical distraction, and posi- absence and reassurance that one does not ex-
tive Spurling’s test increases the likelihood of ist; this may include wrist and hand disorders.
cervical radiculopathy to 90%.10 Cervical The fear associated with this condition causes
spine magnetic resonance imaging confirms clinically significant distress or impairment in
the diagnosis. Depending on the etiology, cer- social, occupational, or other important areas
vical radiculopathy can often be managed of functioning lasting greater than 6 months.
nonsurgically, but must be monitored for
worsening symptoms, especially constant Background Information
numbness and developing weakness. Individuals with this condition either may or
may not recognize that their behavior is ex-
SOMATOFORM DISORDERS cessive or dysfunctional. Evaluation by a
Psychological factors may contribute to a clinical psychologist or psychiatrist is neces-
patient’s perception or report of pain and sary to confirm the diagnosis.11
other symptoms in the hand and wrist. These
factors should be considered when a patient’s ■ Malingering
symptoms do not seem to conform to Chief Clinical Characteristics
recognized pathologies or when they fail This presentation typically involves inten-
to respond to treatment. Psychological issues tionally producing or feigning physical
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216 Chapter 13 Pain Disorder

symptoms. These complaints could include pharmacologic and cognitive-behavioral


WRIST AND HAND PAIN

wrist and hand symptoms. management.


Background Information ■ Thoracic Outlet Syndrome
Patients with this condition are motivated to
Chief Clinical Characteristics
assume the sick role to achieve external re-
This presentation typically includes aching,
wards such as financial gain, narcotic med-
throbbing pain and limited numbness in the
ication, or decreased demands to function in
hand, especially on the ulnar side. There may
the home or workplace. Evaluation by a clin-
be atrophy of the intrinsic muscles of the hand.
ical psychologist or psychiatrist is necessary
Fine-motor dexterity in the hand may be
to confirm the diagnosis.11
decreased.
■ Pain Disorder Background Information
Chief Clinical Characteristics Circulatory changes including edema, Raynaud’s
This presentation typically involves reports phenomenon, and pallor may be noted. This
of pain in one or more areas (which may condition can result from compression of the
occur in the wrist or hand) that is not faked brachial plexus at several points along its
or intentionally produced, but in which course, including between the anterior and
psychological factors are felt to play a middle scalene muscles and against the first
contributory role in the intensity, begin- rib. Contributing factors may include a cervi-
ning, worsening, or continuation of the pain. cal rib, clavicular fracture malunion or callus,
The pain must result in a significant loss of neck trauma, poor posture, or abnormal
function. breathing patterns that utilize muscles of deep
inspiration for normal breathing.8 The diag-
Background Information nosis is confirmed with the clinical examina-
Evaluation by a clinical psychologist or tion, although its diagnostic accuracy is con-
psychiatrist is necessary to confirm the diag- troversial. Imaging modalities also may
nosis.11 A combination of pharmacologic implicate involved structures. Surgery is indi-
and cognitive-behavioral management may cated if symptoms do not respond to nonsur-
be necessary. gical treatment.
■ Somatization Disorder ■ Trigger Points
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation may include reports of pain This presentation involves variable, poorly lo-
in one or more areas (which may occur in the calized, and mobile symptoms in the wrist and
wrist or hand), as well as a variety of other hand that may be associated with severe local
physical symptoms such as gastrointestinal tenderness of muscles distant to the site of wrist
dysfunction, balance disorders, and sexual and hand pain.
dysfunction in addition to pain. These
symptoms are not adequately explained by a Background Information
physical diagnosis. Tenderness at the trigger point will be intense,
but may not always reproduce the hand symp-
Background Information toms. Tenderness to palpation in the hand, if
There must be a history of multiple com- present, is generally less intense than would be
plaints beginning before the fourth decade anticipated based on patient reports. Trigger
of life. Complaints must involve several areas points in muscles throughout the upper quar-
or organ symptoms, and must have resulted ter may produce pain in the wrist and hand.12
in impairment of function or in the seeking This diagnosis should only be accepted after
of treatment. They must not be intentionally other more serious pathologies are rejected.
produced or faked. Evaluation by a clinical The diagnosis is confirmed through the clini-
psychologist or psychiatrist is necessary to cal examination, and management involves
confirm the diagnosis.11 This health condi- options ranging from rehabilitation interven-
tion may be amenable to a combination of tions to injections.
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Chapter 13 Syringomyelia 217

TUMORS ■ Pancoast Tumor

WRIST AND HAND PAIN


■ Meningioma Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically involves sharp,
This presentation typically includes variable pleuritic pain in the axilla, shoulder, or sub-
symptoms, including upper extremity radic- scapular regions with referral pain to the
ular symptoms. neck. Clinical findings also include shoulder-
arm pain, Horner’s syndrome, and neurolog-
Background Information ical deficits affecting the C8 and T1 nerve
This condition involves a benign tumor of roots.16 Neck movements, and cervical com-
the meninges, and symptoms depend on the pression or distraction may not change
specific nerve roots affected. Onset is usually symptoms. Symptoms may be similar to tho-
in the second to third decade of life.13 racic outlet syndrome. Pain is severe and
Despite the benign nature of the tumor, this constant; patients often support the involved
condition may involve significant morbidity. elbow with the opposite hand to relieve
The diagnosis is confirmed with magnetic pressure.
resonance imaging of the cervical spine.
Surgery, radiation, or chemotherapy may be Background Information
necessary to address this health condition. Because of their location at the extreme apex
of the lung, much of the mass of Pancoast
■ Metastases to the Cervical Spine tumors is located in the extrathoracic
or Other Tissue Adjacent to space.17 Risk factors for lung tumors include
Nerves to the Upper Extremity smoking, exposure to secondary smoke, pro-
Chief Clinical Characteristics longed asbestos exposure, and exposure to
This presentation can include unremitting industrial elements. Chest radiographs con-
pain in individuals with these risk factors: firm the diagnosis.17–19 Surgery, chemother-
previous history or cancer, age 50 years or apy, and radiation therapy are all possible
older, failure to improve with conservative options for treatment.
therapy, and unexplained weight change of
more than 10 pounds in 6 months.14 ■ Syringomyelia
Chief Clinical Characteristics
Background Information This presentation may involve bilateral but
The skeletal system is the third most com- asymmetrical loss of pain and temperature
mon site of metastatic disease.15 Symptoms sensation in the upper extremity, possibly
also may be related to pathological fracture in associated with pain. Weakness may also de-
affected sites. Metastatic tumors may develop velop, especially in the hand intrinsic muscles;
in the cervical spine or near the brachial this can produce a clawing deformity of the
plexus or radial, median, or ulnar nerves. fingers.
Common primary sites causing metastases to
bone include breast, prostate, lung, and kid- Background Information
ney. Nerve symptoms not explained by other Upper extremity deep tendon reflexes may
diagnoses, or symptoms accompanied by pal- be absent. Painless ulcers may occur on the
pable tumors along the path of the nerve, hands. Hyperhidrosis and edema may de-
should be referred to a physician for further velop. This condition usually is caused by
workup. Bone scan confirms the diagnosis in trauma to the cervical spine. Patients with
the event of bony metastases. Electromyo- bilateral upper extremity symptoms, espe-
gram/nerve conduction studies can be useful cially weakness, should be referred to a
in localizing nerve compression; plain radi- physician to rule out possible spinal cord
ography, computed tomography, and mag- pathology. This diagnosis is confirmed
netic resonance imaging are also useful for with magnetic resonance imaging of the
assessing etiology. Intervention may involve cervical spine.20 Surgical decompression of
options such as chemotherapy, radiation the characteristic lesion (syrinx) may be
therapy, and surgical resection. indicated.
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218 Chapter 13 Ulnar Nerve Compression at the Elbow (Cubital Tunnel Syndrome)

■ Ulnar Nerve Compression at the there may also be collateral ligament injury. If
WRIST AND HAND PAIN

Elbow (Cubital Tunnel Syndrome) the central slip tear is not properly treated
Chief Clinical Characteristics acutely, permanent deformity can develop as
This presentation can include pain, numbness, the central slip retracts proximally, the trian-
and tingling in the small and ulnar half of the gular ligament attenuates, and the lateral
ring fingers, and in the ulnar half of the dorsum bands slip volarly to become PIP flexors.21
of the hand (this is differentiated from ulnar Plain radiographs are useful to rule out frac-
nerve compression at the wrist, which does not tures that may be associated with this health
include symptoms on the dorsum of the hand). condition. This condition may be managed
Weakness may be present in the flexor digito- nonoperatively with splinting of the PIP in ex-
rum profundus to the ring and small fingers, the tension while allowing distal interphalangeal
thumb adductor, the interosseous muscles, joint flexion. Surgery is indicated if a large
the lumbricals to the ring and small fingers, bone fragment is avulsed.
and the hypothenar muscles. ■ Animal Bite Infections
Background Information Chief Clinical Characteristics
If there is severe compression, the ring and Animal bite infections involve a variable clin-
small fingers may be held in a “claw defor- ical presentation, depending on the depth of
mity,” with the metacarpophalangeal joints bite, organism introduced, and tissue affected.
hyperextended and the interphalangeal joints
flexed; the patient will be unable to simultane- Background Information
ously flex the metacarpophalangeal joints and Animal bites produce complex wounds. They
extend the interphalangeal joints. The condi- must be thoroughly assessed to determine the
tion may begin acutely following a blow to the involved structures, and tendon or nerve in-
posterior elbow, or develop chronically due to juries or fractures must be treated appropri-
prolonged positioning in elbow flexion or ately. These wounds are prone to infection; a
weight bearing on the elbow. Diagnosis is usu- physician must prescribe proper antibiotics.
ally made based on clinical findings; elec- Cat bites may be deep without much bleeding
tromyogram/nerve conduction studies are and introduce bacteria deep into the affected
used to confirm the diagnosis. Nonoperative tissue due to the slenderness of their teeth.
treatment consists of splinting and activity Therefore infection in the joint, flexor sheath,
modification to decrease pressure on the or other anatomical space can result from a
nerve. Surgery is indicated if numbness is con- seemingly insignificant wound. If not treated
stant or if significant weakness is noted. promptly, the developing infection can lead to
extensive tissue damage and may threaten the
Local viability of the finger or even the limb. All bite
wounds should be referred to a physician for
■ Acute Central Slip Rupture evaluation. Treatment involves systemic an-
(Boutonnière Deformity) tibiotics as soon as possible, even if no signs of
Chief Clinical Characteristics infection have developed.
This presentation typically includes pain,
edema, and bruising over the dorsum of the ■ Arthritis Associated with
proximal interphalangeal joint and inability Inflammatory Bowel Disease
to actively extend the proximal interphalangeal Chief Clinical Characteristics
joint. This presentation may include asymmetrical
migratory polyarthritis associated with a
Background Information
history of inflammatory bowel disease (IBD).
This injury usually results from a blow to the
dorsum of the middle phalanx that forces the Background Information
proximal interphalangeal (PIP) joint to flex Although lower extremity joints are affected
during attempted active extension, or from a more frequently, the upper extremity may be
volar dislocation of the PIP joint that may have involved as well. Flare-ups tend to last 2 to
spontaneously reduced. With dislocation, 3 months, although recurrence and chronic
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Chapter 13 Compartment Syndrome 219

arthritis are possible. About 20% of patients ■ Avascular Necrosis of the

WRIST AND HAND PAIN


with IBD develop arthritis.22 Patients with IBD Scaphoid (Preiser’s Disease)
and arthritic symptoms should be referred for Chief Clinical Characteristics
medical management. Medical management This presentation can involve pain in the ra-
may include palliative medication, as well as dial side of the wrist and tenderness to palpa-
corticosteroids or immune modifying agents, tion over the scaphoid, especially at the
in combination with dietary advice. anatomical snuffbox. Edema may be present.
AVASCULAR NECROSIS Pain may be worse with wrist extension and
radial deviation, or with end-range pronation
■ Avascular Necrosis of the Lunate and supination.
(Kienböck’s Disease)
Chief Clinical Characteristics Background Information
This presentation typically includes dorsal This condition usually results from a fall on
central wrist pain worsened with activity, the extended, radially deviated wrist, leading
tenderness to palpation over the lunate to a scaphoid fracture. It also may develop
(Fig. 13-1), decreased wrist range of motion idiopathically.23 Plain radiographs confirm
and grip strength, and dorsal wrist edema. the diagnosis. Surgery is indicated in all cases
to prevent degenerative changes in the wrist.
Background Information
Onset is usually in the second or third decade ■ Cellulitis
of life. The cause of this condition is unclear. It Chief Clinical Characteristics
is associated with negative ulnar variance This presentation typically involves swelling,
(ulna shorter than radius) and may accom- pain, and erythema. The edges are usually not
pany lunate fracture. Plain radiographs con- clearly demarcated. As the infection progresses,
firm this diagnosis. Kienböck’s may be initially blisters may develop in the central portion of the
managed nonoperatively with cast immobi- affected tissue. Fever and/or serous or purulent
lization, but must be closely monitored for drainage may be present.
improvement. Surgery is indicated if degener-
ation continues, or if lunate collapse has Background Information
occurred. Complications may include lunate This health condition results from an acute in-
collapse and wrist osteoarthrosis/osteoarthritis. fection of the skin and subdermal tissues. This
secondary infection may result from a nearby
contaminated wound, or through the blood-
Extensor Scapholunate Ulnar collateral stream from a remote site of infection. This
pollicis ligament ligament condition occurs more frequently among indi-
longus sprain/tear (thumb MP) tear viduals with immune compromise.24 Individ-
tendinitis/ (gamekeeper’s
uals suspected of cellulitis should be referred
osis or skier’s thumb)
immediately for medical treatment.

■ Compartment Syndrome
Chief Clinical Characteristics
This presentation typically includes swelling
(often producing tissue rigidity) and pain at
initial presentation, with increasing pain and
numbness as the condition progresses.
Avascular Extensor Extensor Background Information
necrosis carpi ulnaris digitorum Passive stretching may increase pain; active
of the lunate tendinitis/ communis contraction of involved muscles may be too
(Kienböck’s osis tendinitis/osis painful to perform. Palpable distal pulses do
disease)
not preclude a diagnosis of compartment syn-
FIGURE 13-1 Palpation guide: causes of dorsal hand drome since blood pressure in the large arteries
pain. may be higher than compartment pressure.
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220 Chapter 13 Complex Regional Pain Syndrome

This condition may follow a closed injury to system until they occlude a smaller vessel. This
WRIST AND HAND PAIN

the wrist and hand, especially a crush injury. It condition can occur anywhere in the hand, but
develops when pressure due to edema or bleed- is more likely to result from blockage of arter-
ing in the fascial compartments exceeds the ies supplying the fingers due to the redun-
perfusion pressure of muscle and nerve tissue dancy of more proximal circulation. Loss of
in the compartment; this produces ischemia. blood flow can eventually lead to cell death,
This condition is more likely in the presence of chronic wounds, and dry gangrene. The
multiple fractures. It also may result from diagnosis is confirmed by vascular studies and
external pressure such as an overly restrictive laser Doppler. This condition is a medical
cast or bandage, high-pressure injection in- emergency.
juries (such as with a paint or grease gun), ex-
travasated intravenous lines, and pressure due ■ Felon
to an unconscious or inebriated patient lying Chief Clinical Characteristics
on the affected arm for a prolonged period. An- This presentation typically includes deep aching
ticoagulant use increases risk. The diagnosis is or throbbing pain, accompanied by erythema
made based on clinical findings, confirmed by and tense swelling of the tip and extreme
tonometer measurement of compartment tenderness to touch. Individuals with this con-
pressures. This condition is a medical emer- dition will avoid using the involved digit.
gency, typically treated by fasciotomy. Compli-
Background Information
cations of delayed fasciotomy may include
This is an infection of the distal pulp space; it
Volkmann’s ischemic contracture (irreversible
results from introduction of commonly occur-
fibrotic changes and contracture of involved
ring skin bacteria into the deep compartments
muscles) and renal damage (resulting from the
of the finger tip via a puncture wound; bacte-
toxic by-products of muscle necrosis).25
ria can also enter this space from an untreated
■ Complex Regional Pain Syndrome paronychia. A felon differs from a paronychia
in that the erythema and swelling are through-
Chief Clinical Characteristics
out the finger tip, rather than just near the nail.
This presentation can involve a traumatic
This condition may progress to osteomyelitis
onset of severe chronic wrist and hand pain
of the distal phalanx or infection of the distal
accompanied by allodynia, hyperalgesia, as well
interphalangeal joint if left untreated.29 Diag-
as trophic, vasomotor, and sudomotor changes
nosis is based on clinical findings. Treatment
in later stages.
consists of irrigation, debridement, and
Background Information systemic antibiotics.
This condition is characterized by dispropor-
tionate responses to painful stimuli. It is a re- ■ Flexor Carpi Radialis Tunnel
gional neuropathic pain disorder that presents Syndrome
either without direct nerve trauma (type I) or Chief Clinical Characteristics
with direct nerve trauma (type II) in any This presentation may be characterized by pain
region of the body.26 This condition may pre- on the volar/radial aspect of the wrist, from the
cipitate as a result of an event distant to the proximal pole of the scaphoid up the forearm
affected area. Thermography may confirm as- 1 to 4 cm. The pain will worsen with resisted wrist
sociated sympathetic dysfunction. Plain radi- flexion and radial deviation.
ographs may reveal associated osteopenia.27,28
Background Information
■ Emboli This condition usually results from overuse
and awkward hand postures, especially with
Chief Clinical Characteristics
constant active wrist flexion. The diagnosis
This presentation may include pain, pallor, and
is based on clinical findings.30 This health
coldness in the involved tissues.
condition is usually managed nonoperatively
Background Information with anti-inflammatory medications and
This condition occurs when small blood clots splinting/casting. Surgery is indicated if con-
travel through the larger vessels of the arterial servative measures fail.
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Chapter 13 Distal Radioulnar Joint Fracture/Dislocation 221

■ Flexor Digitorum Profundus infection is suspected, referral to a physician is

WRIST AND HAND PAIN


Avulsion/Rupture (Jersey Finger) necessary. If not removed, the foreign body can
Chief Clinical Characteristics produce a granuloma that can compress sur-
This presentation involves edema and ecchymo- rounding structures and produce ongoing pain
sis over the volar aspect of the distal interpha- and stiffness.31
langeal (DIP) joint, associated with an inabil- FRACTURES
ity to actively flex the DIP.
■ Capitate and Lunate Fractures
Background Information Chief Clinical Characteristics
There may be a tender, palpable mass on the This presentation may involve pain, swe-
volar aspect of the DIP joint, the proximal lling, and bruising throughout the wrist,
interphalangeal joint, or in the distal palm. especially centered dorsally and volarly.
Onset is usually in the second to third decade There is tenderness to palpation over these
of life, but may occur at any age. This injury bones. Wrist movement is markedly limited
usually results from forcible extension of the due to pain.
DIP during active flexion, as when a football
player attempts to grab an opponent’s jersey Background Information
and slips off. When the tendon is avulsed, Bleeding into the carpal tunnel, or edema
plain radiographs may reveal a bone fragment resulting from the injury, may produce
drawn proximally as the tendon retracts. Oth- symptoms of median nerve compression.
erwise, diagnosis is based on clinical findings. These fractures are uncommon in isolation;
Complications may result from late or absent due to their relatively protected position in
repair, including more complicated surgery the hand, an injury with sufficient force to
with little likelihood of full recovery of range fracture these bones will also produce other
of motion. If the flexor digitorum profundus injuries such as distal radius fracture, liga-
is not repaired, a boutonnière deformity may ment tears, and carpal dislocations. The di-
develop.21 Surgical repair is always indicated, agnosis is confirmed by plain radiographs.32
and should be performed within 10 days of These injuries require cast immobilization
injury. or surgical repair.

■ Foreign Body Reaction ■ Distal Radioulnar Joint


Fracture/Dislocation
Chief Clinical Characteristics
This presentation includes localized pain. Signs Chief Clinical Characteristics
include local redness, swelling, and tenderness. This presentation is characterized by pain,
An entrance wound may be noted, but it al- swelling, and bruising along the central dor-
ready may have healed. sal aspect of the distal forearm. The area may
be tender to palpation. Deformity may be
Background Information present if the ulna is dislocated out of the sig-
Hands are especially susceptible to penetration moid notch of the radius. Pain is worse with
by foreign bodies due to their functional role in forearm movement, especially supination,
manipulating objects. Slivers of wood, metal, or and range of motion will be limited.
glass, cactus or other plant spines and thorns,
and fish spines are frequently involved. Individ- Background Information
uals with this condition do not always remem- Fracture may be due to a fall on an out-
ber the initial injury. Foreign body penetration stretched hand, especially with a rotation
may introduce a variety of organisms into the force. The fracture/dislocation may also be
wound, leading to infection; however, a foreign associated with tears of the triangular fibro-
body reaction is aseptic. If the foreign body can cartilage complex, dorsal radioulnar liga-
be visualized, it is within the scope of physical ments, or fracture of the ulnar styloid. The di-
therapy practice to remove it using clean tech- agnosis is confirmed by plain radiographs.32
nique and sterile instruments. However, if the Following fracture healing, therapy may be
object is not apparent from the surface, or if indicated to restore motion and function.
1528_Ch13_201-239 11/05/12 3:57 PM Page 222

222 Chapter 13 Distal Radius Fracture

■ Distal Radius Fracture proximal hypothenar area (Fig. 13-2). If


WRIST AND HAND PAIN

Chief Clinical Characteristics the ulnar nerve is compressed, there may


This presentation may include pain through- be numbness, tingling, or pain in the palmar
out the wrist, edema, tenderness to palpa- aspect of the small and ulnar aspect of the
tion, bruising, and deformity. Wrist move- ring fingers.
ments may be especially painful. Deformity Background Information
may be apparent if there is significant This condition usually results from impact
displacement of fracture fragments. with a held object (golf club, tennis racket, or
Background Information baseball bat) or fall on an outstretched hand.
This fracture usually results from a fall on an Complications may include rupture of the
outstretched hand. Fractures of the scaphoid flexor tendons to the ring and small fingers
and ulnar styloid and tears of wrist liga- due to abrasion on rough fracture edges.32
ments can occur concurrently. Complica- The fracture may be visible on plain radi-
tions include nonunion, malunion (eg, ra- ographs (carpal tunnel view), but CT scan is
dial shortening and altered radial palmar necessary to rule out fracture when plain ra-
tilt), and median nerve compression. A frac- diographs are negative. This condition may
ture involving Lister’s tubercle may lead to be managed nonoperatively with cast immo-
later rupture of the extensor pollicis longus bilization as long as ulnar nerve compression
tendon. The diagnosis is confirmed by plain is not present. Surgery is indicated if there is
radiographs.32 This condition may be man- displacement, if nonunion occurs, or if the
aged with closed reduction and casting but ulnar nerve is involved.
must be closely monitored for loss of ■ Hamate Fracture (Non-Hook)
anatomical reduction. Surgery is indicated if
stable reduction cannot be achieved. Chief Clinical Characteristics
This presentation typically includes pain,
■ Distal Ulna Fracture swelling, and bruising along the ulnar border
Chief Clinical Characteristics of the hand with localized tenderness to
This presentation can involve pain through- palpation over the hamate.
out the wrist, edema, tenderness to palpa- Background Information
tion, bruising, and deformity. Pronation and This condition is uncommon in isolation;
supination may be especially painful. a blow to the ulnar side of the hand may
Background Information
This fracture usually results from a fall on an Stenosing tenosynovitis
outstretched wrist. This condition may be (trigger finger/ thumb)
associated with distal radius fracture, tear of Flexor carpi radialis
the triangular fibrocartilage complex, or dis- tunnel syndrome
location of the distal radioulnar joint. Com-
plications include ulnar shortening (which
can lead to Kienböck’s disease).32 The diag-
nosis is confirmed by plain radiographs. This
condition may be managed with closed re-
duction and casting but must be closely
monitored for loss of anatomical reduction.
Surgery is indicated if stable reduction can-
not be achieved. Hamate Ulnocarpal Flexor
fracture impaction/ carpi ulnaris
■ Hamate Fracture (Hook) (hook) abutment tendinitis/osis
syndrome
Chief Clinical Characteristics
This presentation may include pain and ten- FIGURE 13-2 Palpation guide: causes of palmar
derness to localized deep palpation in the pain.
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Chapter 13 Scaphoid Fracture 223

fracture the hamate in addition to the bases amount and type of displacement, and

WRIST AND HAND PAIN


of the fourth and fifth metacarpals or the tri- whether the fracture is intra-articular.
quetrum.32 The diagnosis is confirmed by
plain radiographs or computed tomography. ■ Pisiform Fracture
These injuries require cast immobilization Chief Clinical Characteristics
or surgical repair. This presentation typically includes local
pain and tenderness to palpation over the
■ Metacarpal Fractures (Including pisiform, and edema in the proximal
Boxer’s Fracture) hypothenar area.
Chief Clinical Characteristics
Background Information
This presentation typically includes pain,
This uncommon fracture can result from a
tenderness, ecchymosis, and swelling over the
blow to the palm or a fall on an outstretched
fractured metacarpal. There may be visible or
hand.32 Plain radiographs or CT scan con-
palpable deformity if the fracture is suffi-
firm the diagnosis. Treatment involves
ciently angulated, usually on the dorsum of the
casting for 6 weeks.
hand.
Background Information ■ Scaphoid Fracture
These fractures usually result from impact to Chief Clinical Characteristics
the hand, frequently from punching a hard This presentation can include pain in the ra-
object such as a wall. Onset is usually in the dial side of the wrist and tenderness to palpa-
second or third decades of life.32 A boxer’s tion over the scaphoid, especially at the
fracture is specifically a fracture of the fifth anatomical snuffbox (Fig. 13-3). Edema may
metacarpal neck. Fractures sustained in a be present. Pain may be worse with wrist
fight may also be associated with tooth extension and radial deviation, or with end-
punctures of the metacarpophalangeal joint; range thumb movements, pronation, and
infection should be assumed in these cases. supination.
Complications may include rotational
Background Information
malalignment; the finger may appear normal
Onset is usually in the second or third
in extension, but cross over an adjacent fin-
decade of life, but can occur at any age. This
ger when the patient attempts to make a fist.
condition usually results from a fall on the
The diagnosis is confirmed by plain radi-
extended, radially deviated wrist. Nonunion
ographs. Surgical correction is required if
of the fracture can result in necrosis of one
rotational malalignment is functionally and
of the fragments; this leads to severe degen-
cosmetically unacceptable.
eration of the wrist joints.32 The diagnosis is
■ Phalangeal Fracture
Chief Clinical Characteristics Intersection Extensor pollicis longus
syndrome tendinitis/osis
This presentation may involve pain, tender-
ness, and swelling in the involved digit; ecchy-
mosis or bony deformity may be visible, but
significant redness or heat is unlikely.

Background Information
These injuries may result from falls, crush in-
Scaphoid Thumb carpo-
juries, or other impact to the digit. Fractures fracture metacarpal
near the joints may also be associated with First dorsal
osteoarthrosis
dislocations or tendon avulsions.32 The diag- compartment
(DeQuervain’s)
nosis is confirmed by plain radiographs.
tenosynovitis
Management of these fractures is determined
by characteristics of the individual fracture FIGURE 13-3 Palpation guide: causes of radial wrist
including stability, degree of comminution, and hand pain.
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224 Chapter 13 Thumb Metacarpal Base Fracture (Bennett’s or Rolando’s Fracture)

confirmed by plain radiographs; however, include deforming arthritic changes at multi-


WRIST AND HAND PAIN

fractures are often difficult to identify on ple joints.


plain films immediately after injury. If clini-
Background Information
cal signs of scaphoid fracture are present, a
Initial onset is typically in middle age or later,
cast should be applied to the affected hand
and 95% of cases occur in men. Initial presen-
and imaging repeated in 2 weeks.
tation in the hand is uncommon, so a history
■ Thumb Metacarpal Base of involvement in other joints, especially the
Fracture (Bennett’s or Rolando’s great toe metatarsophalangeal joint, may help
Fracture) guide the diagnosis. This condition results
from overproduction of uric acid, resulting in
Chief Clinical Characteristics
sodium urate crystal deposition in affected tis-
This presentation mainly includes pain, ten-
sues. This condition is commonly mistaken for
derness, and swelling at the base of the thumb;
an infection. Microscopic examination of aspi-
ecchymosis or bony deformity may be visible,
rated synovial joint fluid confirms the diagno-
but significant redness or heat is unlikely.
sis.31 Treatment involves medication to correct
Background Information the metabolic disorder. Surgery is indicated in
This fracture usually results from axial load- cases with advanced joint destruction.
ing of a partially flexed thumb. A Bennett’s
fracture is extra-articular, whereas a ■ Human Bite Infections
Rolando’s fracture is intra-articular.32 The Chief Clinical Characteristics
diagnosis is confirmed by plain radiographs. This presentation may involve minor pain as-
A variety of treatment options are available sociated with a small laceration over the
depending on the characteristics of the par- metacarpophalangeal joint of one of the fingers,
ticular fracture. Physical therapy may be and increasing erythema and swelling at the
beneficial in restoring range and function joint. Range of motion will become limited and
following fracture healing. painful.
■ Triquetrum Fracture Background Information
Chief Clinical Characteristics This injury results from impact of the knuckle
This presentation includes pain, swelling, and with a tooth; it is not uncommon for the tooth
tenderness to palpation on the ulnar border to penetrate the joint capsule, introducing
of the wrist. bacteria into the joint space. Onset is usually in
the second to third decade of life. Eventually,
Background Information septic arthritis and osteomyelitis may result if
Ecchymosis may be present with this con- left untreated. Clinical examination confirms
dition. This fracture is uncommon in isola- the diagnosis. Treatment includes irrigation,
tion. A blow to the ulnar side of the hand debridement, and systemic antibiotics.
may fracture the triquetrum in addition
to the bases of the fourth and fifth ■ Hypothenar Hammer Syndrome
metacarpals or the hamate.32 The diagnosis Chief Clinical Characteristics
is confirmed by plain radiographs. Isolated This presentation includes burning pain in
injuries require cast immobilization; in- the proximal hypothenar area, numbness in
juries that are more complex may need the palmar aspect of the small finger and ulnar-
surgical repair. palmar aspect of the ring finger, cold intolerance,
■ Gout and ischemia to the fingers.
Chief Clinical Characteristics Background Information
This presentation can involve a red, swollen, This condition occurs with trauma to the
acutely painful joint. As this condition pro- ulnar artery in the area of Guyon’s canal,
gresses, subcutaneous deposits of white pasty which may produce thrombosis, vasospasm, or
material (tophi) may develop and occasionally aneurysm. The ulnar nerve may be com-
erupt through the skin. Advanced cases may pressed by the enlarged vascular structures.
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Chapter 13 Lunate Dislocation 225

Clinically, there may be a positive finding of Background Information

WRIST AND HAND PAIN


occlusion of the ulnar artery on an Allen’s test. This injury can result from a variety of forces
An arteriogram confirms the diagnosis. This across the joint including impact, traction,
condition usually results from isolated or re- torsion, and linear forces pushing the joint
peated trauma to the hypothenar area where beyond physiological limits. Complications
the heel of the hand is used as a hammer, such include residual joint stiffness, especially
as in karate or in workplace activities.30 The proximal interphalangeal joint flexion contrac-
injury may be managed nonoperatively in tures.34 Fractures also may accompany a
mild cases, and the use of padded gear may al- dislocation, so plain radiographs are generally
low return to previous activity. Injury that is indicated. These injuries are usually managed
more serious requires surgery to repair or re- nonoperatively with a brief period of splinting
construct the vascular damage. followed by buddy taping and active range of
motion. Surgery is indicated if soft tissue or
■ Inflammatory Arthritis fracture injuries require repair.
(Including Rheumatoid, Psoriatic,
Scleroderma, and Systemic Lupus ■ Intersection Syndrome
Erythematosus) Chief Clinical Characteristics
Chief Clinical Characteristics This presentation mainly involves sharp pain in
Inflammatory arthritides typically present with the radial aspect of the distal forearm about
pain in multiple joints in the wrists and hands; 1 to 2 inches proximal to the radial styloid
symptoms are usually symmetrical in rheuma- (see Fig. 13-3). Pain may be reproduced with
toid arthritis, systemic lupus erythematosus resisted wrist extension. Edema may be present.
(SLE), and scleroderma, but may not be in other Background Information
diseases. In addition to pain, there may be There will be tenderness to palpation where
swelling, stiffness, and erythema. the tendons of the extensor carpi radialis
Background Information longus and brevis pass deep to the extensor
These diseases produce progressive deformity pollicis brevis and abductor pollicis longus.30
in the wrist and digital joints. These conditions Diagnosis is made based on clinical findings.
are often associated with Raynaud’s phenome- Intersection syndrome is usually managed
non. They may be managed nonoperatively nonoperatively with corticosteroid injections
with medication and splinting; paraffin baths or iontophoresis, oral anti-inflammatory med-
often provide temporary relief of pain. Surgery ication, splinting to limit wrist movement, and
is often valuable to correct deformity and re- thermal modalities.
pair damaged structures; joint replacements
■ Lunate Dislocation
are performed for metacarpophalangeal joints
and, less often, for the wrist.33 Therapy may in- Chief Clinical Characteristics
crease range and strength and help to reduce This presentation is characterized by pain and
pain. Complications differ for specific dis- swelling in the wrist and hard volar bulge of the
eases, but may include joint erosion, deformity lunate in the area of the carpal tunnel. There
(ulnar drift at the metacarpophalangeal joints, may be decreased finger flexion due to pressure
boutonnière and swan neck deformities), and on the flexor tendons, or pressure on the median
attritional tendon ruptures. nerve may produce pain, numbness, and weak-
ness in a median distribution.
■ Interphalangeal Joint Dislocation Background Information
Chief Clinical Characteristics This injury usually results from a fall on an
This presentation typically includes edema, outstretched hand. Complications may in-
tenderness, and possibly ecchymosis at the in- clude nerve injury and loss of range of motion
volved joint. The affected joint may be visibly in the wrist. The diagnosis is confirmed by
dislocated, but more often either spontaneous plain radiographs.34 A surgeon should manage
or deliberate reduction of the dislocation may this injury; if nerve compression is suspected,
be reported. the referral should be urgent.
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226 Chapter 13 Lunotriquetral Ligament Tear (and Lunotriquetral Dissociation)

■ Lunotriquetral Ligament Tear aspect of the ring finger. Pain may be present
WRIST AND HAND PAIN

(and Lunotriquetral Dissociation) in the wrist, and may radiate up the arm to
Chief Clinical Characteristics the shoulder. In advanced cases, atrophy of the
This presentation typically includes pain in the thenar muscles may be noted. Reproduction
ulnar wrist and tenderness over the lunotri- of symptoms when holding a full fist suggests
quetral joint. The ligaments supporting the that the lumbrical attachment on the flexor
joint may be merely sprained; however, a painful digitorum profundus (FDP) tendon may be too
click with ulnar and radial deviation may in- proximal, causing the muscle bellies to be
dicate complete rupture. drawn into the tunnel with full FDP contrac-
tion. Patients will often report difficulty
Background Information manipulating small objects such as buttons or
This injury usually results from a fall on an earrings. Symptoms are frequently worse
outstretched hand, or from forceful twisting of at night.
the wrist. The diagnosis is confirmed by plain
radiographs. Sprains may be managed nonop- Background Information
eratively. Surgery is indicated if instability is This condition may result from repetitive
present. Complications may develop if insta- activity or from frequent exposure to vibra-
bility is not addressed; abnormal lunate move- tion. It can also occur due to sustained
ment can eventually lead to degenerative wrist flexion in sleep, pregnancy, and
osteoarthrosis/osteoarthritis requiring salvage hypothyroidism. Diagnosis is through clini-
surgery.35 cal examination, and may be confirmed by
electromyogram/nerve conduction studies.
■ Lyme Disease Worsening symptoms or symptoms that do
not respond to nonsurgical treatment within
Chief Clinical Characteristics 6 weeks also indicate a need for surgical
This presentation involves myalgia and migra- referral.30,38
tory joint pain associated with a characteristic
“bull’s-eye” shaped rash (erythema migrans), ■ Radial Sensory Nerve
fever, chills, and lymphadenopathy. Entrapment (Wartenberg’s
Background Information Syndrome)
This condition results from infection with Chief Clinical Characteristics
Borrelia burgdorferi bacteria following a tick This presentation involves pain, tingling, and
bite. One to 4 months following inoculation, numbness over the dorsoradial aspect of the
neurological symptoms including radicu- wrist, index finger, and thumb.
loneuritis may develop, as well as conjunctivi- Background Information
tis, cardiac abnormalities, and myocarditis. With this condition a positive Tinel’s sign is
Long-standing disease (greater than 6 months) often seen in the distal third of the forearm.
may result in chronic synovitis, chronic arthri- This syndrome results from compression of
tis, chronic fatigue, and encephalopathy. This the radial sensory nerve as it passes between
diagnosis is confirmed with the clinical exami- the brachioradialis and extensor carpi radi-
nation in early stages, and serologic tests in later alis longus. Acute injury or chronic overuse
stages.36,37 Individuals with multijoint pain may contribute to the development of this
should always be referred for medical workup. condition.30 Diagnosis is made based on
NERVE INJURIES clinical findings and confirmed by elec-
tromyogram/nerve conduction studies. This
■ Median Nerve Compression at condition may be managed nonoperatively
the Wrist (Carpal Tunnel with corticosteroid injection or iontophore-
Syndrome) sis, splinting to hold the wrist in a neutral
Chief Clinical Characteristics position, and removal of sources of com-
This presentation mainly includes pain, pares- pression such as jewelry or tight clothing.
thesia, or numbness in the volar aspect of the Surgery is indicated if nonsurgical treatment
thumb, index and middle fingers, and radial is unsuccessful.
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Chapter 13 Distal Radioulnar Joint Osteoarthrosis/Osteoarthritis 227

■ Radial Sensory Nerve Injury interosseous muscles, the lumbricals to the

WRIST AND HAND PAIN


(Without Entrapment) ring and small fingers, and the hypothenar
Chief Clinical Characteristics muscles.
This presentation includes pain (frequently de- Background Information
scribed as burning), tingling, and numbness If there is severe compression, the ring and
in the radial dorsal aspect of the hand and the small fingers may be held in a “claw defor-
dorsum of the thumb and index finger. mity,” with the metacarpophalangeal joints
Background Information hyperextended and the IP joints flexed; the
The radial sensory nerve can be injured at patient will be unable to flex the MPs with
any point along its course, from just distal to the IPs extended. The condition may begin
the elbow to the dorsoradial aspect of the acutely following a blow to the wrist, or
hand. This nerve is easy to irritate and slow result from ulnar artery thrombosis or
to heal. This injury (contusion or stretch) aneurysm or a ganglion cyst in Guyon’s
can result from impact, prolonged pressure canal.35 Diagnosis is based on clinical find-
(including from an overly tight cast or ings and confirmed with electromyogram/
splint), surgery to the radial wrist, or place- nerve conduction studies. Nonsurgical treat-
ment of an external fixator.32 Diagnosis is ment following direct trauma consists of
made based on clinical findings, and may be splinting and activity modification to de-
confirmed by electromyogram/nerve con- crease pressure on the nerve. Surgery is indi-
duction studies or injection of local anes- cated if imaging reveals a mass in the tunnel,
thetic. This injury is generally managed if numbness is constant, or if significant
without intervention. However, recovery can weakness is noted.
be very slow. Iontophoresis with dexametha-
sone via a low-current (24-hour) delivery
OSTEOARTHROSIS/
system may help reduce recovery time.
OSTEOARTHRITIS
■ Distal Interphalangeal Joint
■ Ulnar Digital Nerve Osteoarthrosis/Osteoarthritis
Compression (Bowler’s Thumb) Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically includes pain,
This presentation can involve pain and numb- swelling, stiffness, and palpable crepitus with
ness in the ulnar side of the thumb from the movement at the distal interphalangeal joint.
metacarpophalangeal joint to the tip.
Background Information
Background Information The condition usually develops after the
This results from compression of the nerve, fourth decade of life. The underlying pathol-
frequently against the edge of the hole in the ogy in distal interphalangeal joint degenera-
bowling ball.39 Diagnosis is based on history tion is usually osteoarthrosis/osteoarthritis,
and clinical findings. Protective splinting of but similar changes also may occur with pso-
the thumb and redrilling of the bowling ball riatic arthritis.8 The diagnosis is confirmed
to relieve pressure and allow return to sport. with plain radiographs that reveal subchon-
dral sclerosis and osteophyte formation
■ Ulnar Nerve Compression at the about the affected joints. It may be managed
Wrist (Guyon’s Canal) nonsurgically, or the joint may be fused if
Chief Clinical Characteristics pain is debilitating.
This presentation mainly involves pain, numb-
ness, and tingling in the small and ulnar half ■ Distal Radioulnar Joint
of the ring fingers, but not in the ulnar half of Osteoarthrosis/Osteoarthritis
the dorsum of the hand. This is differentiated Chief Clinical Characteristics
from ulnar nerve compression at the elbow, This presentation can involve pain and
which includes dorsal symptoms. There may swelling along the central dorsal aspect of the
be weakness in the adductor pollicis, the distal forearm.
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228 Chapter 13 Intercarpal Osteoarthrosis/Osteoarthritis

Background Information nonoperatively as long as adequate pain con-


WRIST AND HAND PAIN

The area may be tender to palpation. Pain trol and functional ability are maintained.
is worse with forearm movement, especially Joint replacement is indicated if deformity,
at the end of range. This osteoarthrosis/ pain, and loss of function are unacceptable.
osteoarthritis can develop following trauma,
but may occur without prior injury.35 The di- ■ Pisotriquetral Osteoarthrosis/
agnosis is confirmed with plain radiographs Osteoarthritis
that reveal subchondral sclerosis and osteo- Chief Clinical Characteristics
phyte formation about the affected joints. This presentation is characterized by pain
Nonsurgical treatments include physical and tenderness in the area of the pisiform;
therapy and nonsteroidal anti-inflammatory crepitus may be present.
medication. Surgery may be indicated if
nonoperative measures are inadequate. Background Information
The flexor carpi ulnaris tendon will not be
■ Intercarpal Osteoarthrosis/ tender. Osteoarthrosis/osteoarthritis in this
Osteoarthritis joint usually develops insidiously as the re-
sult of chronic bearing of the weight of the
Chief Clinical Characteristics
hand through the joint (eg, using a computer
This presentation is mainly characterized by
mouse).35 Plain radiographs confirm the di-
aching pain that is worse in the morning and
agnosis. It may be managed nonsurgically
improved with heat, such as after bathing.
with anti-inflammatory medication, or the
Background Information pisiform may be excised.
This condition may occur in any joint of
the wrist and usually affects older individuals. ■ Proximal Interphalangeal Joint
It may be associated with recent or past injury Osteoarthrosis/Osteoarthritis
to the area, but symptoms can begin without Chief Clinical Characteristics
identifiable cause. A history of heavy physical This presentation typically includes pain,
labor or avid participation in vigorous sports swelling, stiffness, and palpable crepitus with
or hobbies may increase the likelihood of de- movement at the proximal interphalangeal
veloping osteoarthrosis/osteoarthritis.35 The joint.
diagnosis is confirmed with plain radiographs.
Intercarpal osteoarthrosis is usually managed Background Information
nonsurgically with anti-inflammatory med- Degeneration at this joint can result from ei-
ication, activity modification, and splinting. ther rheumatoid arthritis or osteoarthrosis/
osteoarthritis.8 The diagnosis is confirmed
■ Metacarpophalangeal Joint with plain radiographs, which reveal reduced
Osteoarthrosis/Osteoarthritis joint space, subchondral sclerosis, and osteo-
Chief Clinical Characteristics phyte formation about affected joints. It is
This presentation can involve pain, swelling, usually managed nonsurgically. Surgery is in-
stiffness, and palpable crepitus with move- dicated if symptoms are severe; joint fusion is
ment at the metatarsophalangeal joint. more common than arthroplasty.

Background Information ■ Radioscaphoid Osteoarthrosis/


A history of isolated or repetitive trauma is Osteoarthritis
frequently reported. Metacarpophalangeal
Chief Clinical Characteristics
joint osteoarthrosis/osteoarthritis may be
This presentation is characterized by aching
associated with hemochromatosis.8 This
to sharp pain in the radial side of the wrist with
condition is uncommon; rheumatoid arthri-
all movements.
tis should be considered as a contributing
pathology when arthritic changes in this Background Information
joint are noted, especially if multiple joints Ulnar deviation may be less painful. Pain may
are involved. The diagnosis is confirmed be worse early in the morning and with cold,
by plain radiographs. It may be managed wet weather. This condition may result from
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Chapter 13 Wrist and Hand 229

abnormal joint forces following a number of This condition may be managed nonsurgi-

WRIST AND HAND PAIN


traumatic injuries including distal radius frac- cally with steroid injection, protective
ture malunion, scaphoid fracture nonunion, splinting, and activity modification.
or scapholunate ligament rupture. It may
develop because of chronic overuse. Plain ■ Thumb Carpometacarpal Joint
radiographs, which are used to confirm the di- Osteoarthrosis/Osteoarthritis
agnosis, reveal reduced joint space, osteophyte (Basilar Joint or CMC Arthrosis)
formation, and subchondral sclerosis within Chief Clinical Characteristics
affected joints. This condition may be man- This presentation can involve pain in the base
aged nonsurgically with anti-inflammatory of the thumb, just distal to the scaphoid
medication, splints, heat modalities, and activ- (see Fig. 13-3). Symptoms may radiate distally
ity modification. Surgery generally involves into the thumb. Pain is typically worst with
salvage procedures such as proximal row pinching activities such as turning a key, and
carpectomy or scaphoid resection and four- with activities involving grip and turn such
corner fusion if nonoperative treatment fails. as opening jars and door knobs.

■ Scapholunate Advanced Background Information


Collapse (SLAC) Wrist This condition usually develops insidiously,
although it can follow specific joint trauma.
Chief Clinical Characteristics This condition is frequently bilateral and its in-
This presentation typically includes pain cidence is higher among women. Pantrapezial
throughout the wrist, which may be worse on osteoarthrosis/osteoarthritis—degenerative
the ulnar side. There will be a generalized changes to the articulations of the trapezium
decrease in wrist range of motion. There may with the trapezoid and scaphoid—shares a
be palpable crepitus in the radiocarpal and similar presentation.8 Plain radiographs con-
intercarpal joints with active movement or firm the diagnosis. Surgery is indicated if
compressive testing. nonoperative management does not provide
Background Information acceptable relief.
This condition is the result of inadequate
treatment for scapholunate dissociation. OSTEOMYELITIS
Rupture of the scapholunate ligament causes ■ Digit
instability and altered mechanics throughout Chief Clinical Characteristics
the wrist, leading to arthritic changes.40 Plain This presentation involves redness, swelling,
radiographs confirm the diagnosis with find- and pain over the involved bone, stiffness at
ings of osteoarthritis/osteoarthrosis. It may be adjacent joints, and signs of systemic illness
managed nonoperatively with anti-inflam- such as fever and malaise.
matory medication, and surgery is indicated
if nonoperative measures are inadequate. Background Information
This condition commonly results from a
■ Sesamoid Osteoarthrosis/ fracture with an open wound. However, con-
Osteoarthritis (Thumb) taminated small cutaneous wounds may
provide a sufficient portal of entry for bacte-
Chief Clinical Characteristics
ria, and distant infection can seed the site via
This presentation typically includes pain
the bloodstream. Individuals with compro-
over the volar surface of the thumb metacar-
mised immune responses are more suscepti-
pophalangeal joint worsened by compres-
ble to this condition. Plain radiographs con-
sion or resisted thumb flexion, and tender-
firm the diagnosis.31 This condition requires
ness to palpation.
urgent medical attention.
Background Information
This condition usually results from overuse. ■ Wrist and Hand
It may also be associated with a direct blow. Chief Clinical Characteristics
Clinical findings suggest the diagnosis, which This presentation typically includes redness,
can be confirmed with plain radiographs. swelling, and pain over the involved bone,
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230 Chapter 13 Paronychia

stiffness at adjacent joints, and signs of condition may be managed nonoperatively,


WRIST AND HAND PAIN

systemic illness such as fever and malaise. but must be closely monitored for worsening
symptoms and tissue necrosis. Tobacco use is a
Background Information
major contributing factor. Smoking cessation,
This condition commonly results from a
maintaining local warmth, and avoidance
fracture with an open wound. However, con-
of constrictive garments should be recom-
taminated small cutaneous wounds may
mended. Medication may enhance tissue
provide a sufficient portal of entry for bacte-
perfusion. Surgery may be useful in restoring
ria, and distant infection can seed the site via
circulation in cases of discrete blockage or to
the bloodstream. Individuals with compro-
amputate nonviable structures.
mised immune responses are more suscepti-
ble to this condition. Plain radiographs con- ■ Pseudogout
firm the diagnosis.31 This condition requires
Chief Clinical Characteristics
urgent medical evaluation and management.
This presentation may include uni- or multiar-
■ Paronychia ticular joint pain in the wrists, knees, and hips,
although any joint may be affected. Pain devel-
Chief Clinical Characteristics
ops over a period of days, and is accompanied
This presentation typically involves erythema
by redness, swelling, warmth, and tenderness to
around the nail, perhaps with a central
palpation. Fever may be present.
whitening of the skin, associated with exquisite
tenderness, disuse of the affected digit, and a Background Information
palpable core of pus that also may drain from The presentation is similar to gout or septic
the nail fold. arthritis. This condition is uncommon in
Background Information patients younger than 50 years of age, and be-
This infection results from the introduction of comes increasingly common with increasing
common skin bacteria beneath the nail fold by age. Pseudogout results from calcium py-
a minor trauma such as a torn hangnail, nail rophosphate crystal deposits that mediate its
biting, or a puncture wound. In acute infec- characteristic joint pain and articular cartilage
tions, the responsible organism is usually destruction. Microscopic examination of aspi-
Staphylococcus aureus; in chronic infections a rated synovial joint fluid confirms the diagno-
variety of bacteria and fungi may be involved. sis.41 Treatment involves medication to correct
This condition may develop into a felon or os- the underlying metabolic disorder.
teomyelitis if left untreated.29 Diagnosis is
■ Raynaud’s Phenomenon/Disease
based on clinical findings. Depending on how
far the infection has progressed, management Chief Clinical Characteristics
options for paronychia include warm soaks This presentation typically includes painful
and oral antibiotics. Infections that do not blanching of the fingers, toes, nose, and ears in
respond to these measures may require lancing response to cold, vibration, or stress.
to drain the pus.
Background Information
■ Peripheral Vascular Disease This condition results from vasospasm in the
digital arteries. It usually occurs as a complica-
Chief Clinical Characteristics
tion of collagen vascular diseases or thoracic
This presentation can include cold intolerance,
outlet syndrome (Raynaud’s phenomenon),
pain, and impaired wound healing, associated
but can occur in isolation (Raynaud’s disease).
with pale and cold digits, Raynaud’s phenom-
Diagnosis is based on history and clinical find-
enon, and chronic ulceration; dry gangrene can
ings. Treatment generally involves smoking
develop in severe cases.
cessation if applicable, and avoidance of trig-
Background Information gering stimuli. Patients may find wearing
This condition results from arteriosclerosis warm gloves particularly helpful. Digital sym-
in the arteries supplying the hand. Doppler pathectomy may be indicated if pain is un-
ultrasonography confirms the diagnosis. This remitting or if tissue necrosis is developing.
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Chapter 13 Stenosing Tenosynovitis (Trigger Finger/Thumb) 231

■ Reiter’s Syndrome Background Information

WRIST AND HAND PAIN


Chief Clinical Characteristics The degree of ligament injury varies, from
This presentation involves asymmetric joint partial tear of a single ligament to complete
pain and enthesitis at multiple sites in the spine rupture of all three ligaments (dorsal scaphol-
and extremities. A triad of symptoms charac- unate, volar radioscapholunate, and in-
terizes Reiter’s syndrome: joint pain, urethritis, terosseous). This injury usually results from a
and conjunctivitis. fall on an outstretched hand; however, the
trauma may be remote, having been initially
Background Information dismissed as a trivial sprain. Left untreated,
Musculoskeletal symptoms usually begin 1 to complete tears produce instability in the car-
4 weeks following a gastrointestinal or geni- pus. Over time, this leads to osteoarthrosis/
tourinary tract infection. Diagnosis is con- osteoarthritis in the radiocarpal and inter-
firmed by hematologic studies including carpal joints, and eventually to erosion of the
erythrocyte sedimentation rate, C-reactive carpals and a scapholunate advanced collapse
protein, rheumatoid factor, antibody to nu- (SLAC) wrist. Plain radiographs may help to
clear antigens, and human leucocyte antigen distinguish this injury from scaphoid frac-
B27.33 Individuals with this triad of symptoms ture, but should be repeated 2 weeks follow-
should be referred to a physician for medical ing injury if initially negative.34 Partial tears
management. Nonsteroidal anti-inflammatory can be managed with casting; complete tears
agents are commonly used to address joint require surgery.
symptoms.
■ Septic Arthritis
■ Sarcoidosis Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can involve joint redness,
This presentation mainly includes symmetric, pain, swelling, and stiffness. Fever may be
migratory, and intermittent joint and periar- present, and a tract to the surface may develop,
ticular pain and swelling. allowing purulent or serous fluid to drain.
Background Information Background Information
There may also be associated flexor and exten- This condition usually results from a penetrat-
sor tendinitis, which can produce median ing wound by a contaminated object near the
nerve compression if located in the carpal tun- joint, such as from a nail, fish hook, thorn, or
nel. Although onset can occur at any age, it is tooth. The underlying infection may originate
most common in the second through fourth in or spread to other tissues producing os-
decades of life.42–44 This condition can pro- teomyelitis or cellulitis. Complications may
duce granulomatous lesions in any tissue, al- include degenerative changes in the joint and
though the primary involvement is in the significant residual pain stiffness and loss of
lungs. Bone involvement occurs in 5% and function.45 This diagnosis is confirmed with a
joint involvement occurs in 25% to 50% of blood test to detect the responsible infective
patients, most often in the hands and feet. agent. Antibiotic therapy should be initiated
Individuals suspected of this diagnosis should immediately, and the joint will often require
be referred to a hand surgeon. Hand manifes- surgical exploration.
tations of this condition are generally man-
aged symptomatically with nonsteroidal anti- ■ Stenosing Tenosynovitis (Trigger
inflammatory medications. Finger/Thumb)
Chief Clinical Characteristics
■ Scapholunate Ligament Sprain This presentation may involve pain over the
Chief Clinical Characteristics volar aspect of the metacarpophalangeal joints
This presentation is characterized by pain and of the involved digit(s) (see Fig. 13-2) that is
edema in the area of the anatomical snuffbox worse with active flexion and frequently worse
(see Fig. 13-1). There may be limited wrist range on waking. Pain may refer distally into the digit.
of motion. There may be reports of the finger “getting
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232 Chapter 13 Subungual Hematoma

stuck” in flexion, requiring passive extension with Background Information


WRIST AND HAND PAIN

the other hand. The infection responsible for this condition


can result from a bite wound or other punc-
Background Information
ture that introduces bacteria into the flexor
The volar aspect of the MP will be tender to
tendon sheath. Diagnosis is based on clinical
palpation, and a mobile nodule may be felt to
findings. Scarring in the tendon sheath follow-
move proximally with contraction of the flexor
ing these infections may permanently limit
muscles. This condition results when a thick-
range of motion. Patients suspected of this
ening of the tendon sheath moves through the
condition should be referred to a hand sur-
lumen of the A1 pulley in the palm. It may de-
geon on an emergent basis. Delayed treatment
velop because of isolated trauma to the area or
can result in loss of the digit or occasionally
from chronic overuse.30 Diagnosis is based on
the hand.29
history and clinical findings. This condition
may be managed nonoperatively with anti- TENDINOPATHIES
inflammatory medication and splinting. Sur-
■ Extensor Carpi Ulnaris
gery to release the A1 pulley is indicated if
Tendinitis
these measures fail or if the condition recurs.
Chief Clinical Characteristics
■ Subungual Hematoma This presentation can include pain localized
Chief Clinical Characteristics to the ulnar/dorsal aspect of the wrist, fre-
This presentation involves purple or black col- quently near the ulnar styloid. Pain may be
oration deep to the finger or thumbnail. There worse with resisted wrist extension or ulnar
may be excruciating, often throbbing, pain. deviation. A “popping” may be reported and
palpated during resisted pronation and
Background Information supination if the tendon is subluxing out of
This injury usually results from a crushing its groove on the distal ulna; these movements
blow to the tip of the finger. If the discol- may also be painful. This condition frequently
oration involves half or more of the nailbed, a results from overuse. It also may be associated
nailbed tear or distal phalanx fracture should with ulnar styloid fractures and rheumatoid
be suspected. A melanoma under the nail can arthritis.35
be similar in appearance to a subungual
hematoma, although there will not be a history Background Information
of trauma. To differentiate between the two, Diagnosis is based on clinical findings. It
a line may be scratched in the nail with a nail may be managed nonoperatively with
file at the proximal margin of the darkened splinting/casting and corticosteroid injec-
area. A hematoma will move distally as the nail tion. Surgery is indicated if these measures
grows out, so the scratch will not cross it. A fail, but may increase the tendency for the
melanoma will be stationary under the nail, tendon to sublux. Over time, this condition
and the scratch will move across the darkened can develop into a tendinosis.
area as the nail grows. Plain radiographs are
indicated if fracture is suspected. Subungual ■ Extensor Carpi Ulnaris
hematomas may be managed nonoperatively Tendinosis
by draining the accumulated blood to relieve Chief Clinical Characteristics
pressure. This presentation typically includes pain lo-
calized to the ulnar/dorsal aspect of the wrist
■ Suppurative Digital Flexor (see Figures 13-1 and 13-4), frequently near
Tenosynovitis the ulnar styloid. Pain may be worse with re-
Chief Clinical Characteristics sisted wrist extension or ulnar deviation. A
This presentation typically includes tenderness “popping” may be reported and palpated dur-
over the volar aspect of the finger, flexed posture ing resisted pronation and supination if the
of the finger, severe edema, and pain with pas- tendon is subluxing out of its groove on the
sive extension of the finger (the classic “four distal ulna; these movements may also be
signs of Kanavel”).46 painful. This condition frequently results from
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Chapter 13 Extensor Pollicis Longus Tendinitis 233

Triangular Extensor carpi ulnaris respond to conservative treatment. Over time,

WRIST AND HAND PAIN


fibrocartilage tendinitis/osis this condition can develop into a tendinosis.
complex tear
■ Extensor Digitorum Communis
Tendinosis
Chief Clinical Characteristics
This presentation can include pain over the
dorsum of the wrist and hand (see Fig. 13-1).
Flexor carpi ulnaris
tendinitis/osis Background Information
Metacarpal fracture This condition usually results from overuse
(Boxer’s fracture) involving prolonged finger extension such as
FIGURE 13-4 Palpation guide: causes of ulnar wrist keyboarding with the keyboard positioned
and hand pain. too low. It may also occur following trauma
to the dorsum of the hand. Individuals with
overuse. It also may be associated with ulnar rheumatoid arthritis may develop extensor
styloid fractures and rheumatoid arthritis.35 digitorum communis tendinosis as a result
of abrasion over roughened areas on the car-
Background Information pus.30 The diagnosis is generally made based
Diagnosis is based on clinical findings. It on clinical findings. Pain will be worse with
may be managed nonoperatively with splint- resisted MP extension. Edema and tender-
ing/casting and corticosteroid injection. ness to palpation may be present over the
Surgery is indicated if these measures fail, fourth dorsal compartment. This condition
but may increase the tendency for the ten- may be managed nonoperatively with physi-
don to sublux. cal therapy, nonsteroidal anti-inflammatory
medication, and corticosteroid injection, but
■ Extensor Digitorum Communis must be closely monitored for worsening
Tendinitis symptoms in rheumatoid patients, in whom
Chief Clinical Characteristics it may indicate impending tendon rupture.
This presentation mainly involves pain over the Surgery is indicated if symptoms do not
dorsum of the wrist and hand. Pain will be respond to conservative treatment.
worse with resisted MP extension. There may
be edema and tenderness to palpation over ■ Extensor Pollicis Longus
the fourth dorsal compartment. This condition Tendinitis
usually results from overuse involving pro- Chief Clinical Characteristics
longed finger extension such as keyboarding This presentation involves pain on the dorsum
with the keyboard positioned too low. It may of the wrist at and just ulnar to Lister’s tuber-
also occur following trauma to the dorsum cle (see Fig. 13-3).
of the hand. Individuals with rheumatoid
arthritis may develop extensor digitorum com- Background Information
munis tendinitis as a result of abrasion over Diagnosis is based on clinical findings. Pain
roughened areas on the carpus.30 will be worse with resisted thumb extension,
especially interphalangeal joint extension, or
Background Information with active and passive thumb flexion. This
The diagnosis is generally made based on condition usually results from chronic over-
clinical findings. This condition may be use in situations requiring thumb extension;
managed nonoperatively with physical ther- it may also be associated with a distal radius
apy, nonsteroidal anti-inflammatory med- fracture when malunion creates a roughened
ication, and corticosteroid injection, but area in the tendon’s path. Surgery is manda-
must be closely monitored for worsening tory due to a high risk of tendon rupture, es-
symptoms in rheumatoid patients, in whom pecially following a distal radius fracture.
it may indicate impending tendon rupture. Rupture of the tendon produces a condition
Surgery is indicated if symptoms do not known as drummer’s palsy, characterized by
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234 Chapter 13 First Dorsal Compartment (De Quervain’s) Tenosynovitis

inability to actively extend the thumb inter- ■ Flexor Carpi Ulnaris Tendinosis
WRIST AND HAND PAIN

phalangeal joint. Chief Clinical Characteristics


■ First Dorsal Compartment (De This presentation typically includes pain on
Quervain’s) Tenosynovitis the volar/ulnar aspect of the wrist, proximal
to the pisiform (see Fig. 13-2).
Chief Clinical Characteristics
This presentation can involve sharp pain in Background Information
the radial aspect of the wrist (see Fig. 13-3) Diagnosis is based on clinical findings.
with thumb flexion, wrist ulnar deviation, Provocative actions are resisted wrist flexion
and (less often) thumb radial abduction. The and ulnar deviation, and passive wrist exten-
pain will be worse with resistance to thumb sion. This condition usually results from
metacarpophalangeal joint extension, but lit- overuse, especially with awkward hand
tle or no pain is evoked with resisted isolated postures.35 This condition is usually man-
interphalangeal joint extension. aged nonoperatively with anti-inflammatory
medications and splinting/casting. Surgery is
Background Information indicated if nonoperative measures fail.
There will be tenderness to palpation in this
area; there may be swelling or crepitus. The ■ Terminal Extensor Tendon
patient may report difficulty with grasping Rupture (Mallet Finger)
activities. This diagnosis is based on clinical Chief Clinical Characteristics
findings, and most notably a positive Finkel- This presentation includes pain, edema, and
stein’s test.30 This condition usually results bruising over the dorsum of the distal inter-
from overuse of the abductor pollicis longus phalangeal (DIP) joint and inability to actively
and extensor pollicis brevis. Treatment may extend the DIP.
include steroid injection or iontophoresis,
oral anti-inflammatory medications, and Background Information
splinting with a thumb spica splint to pre- This usually results from a direct blow to the
vent ulnar deviation and thumb use. Activity tip of the finger, forcing the dip into flexion
modification is essential. Surgery to release during active extension. Onset is usually in the
the first dorsal compartment is indicated if second to third decade of life. Diagnosis is
the condition does not respond to conserva- based on clinical findings. It may be managed
tive treatment. nonsurgically by splinting the DIP in constant
full extension for at least 6 weeks.21
■ Flexor Carpi Ulnaris Tendinitis
Chief Clinical Characteristics ■ Triangular Fibrocartilage
This presentation typically includes pain Complex Tear
on the volar/ulnar aspect of the wrist, Chief Clinical Characteristics
proximal to the pisiform (see Figures 13-2 This presentation typically involves ulnar-sided
and 13-4). wrist pain (see Fig. 13-4) that is worse with
pronation, supination, and possibly wrist
Background Information
flexion.
Diagnosis is based on clinical findings.
Provocative actions are resisted wrist flexion Background Information
and ulnar deviation, and passive wrist ex- Through careful palpation of the extensor
tension. This condition usually results from carpi ulnaris tendon, a painful click or snap
overuse, especially with awkward hand pos- may be noted with pronation or supination.
tures.35 This condition is usually managed This injury results from a fall on an out-
nonoperatively with anti-inflammatory stretched hand, especially with a rotation force
medications and splinting/casting. Surgery component. Chronic tears may be associated
is indicated if nonoperative measures fail. with ulnocarpal abutment.35 The diagnosis is
Over time, this condition can develop into a confirmed with magnetic resonance imaging,
tendinosis. arthrography, or diagnostic arthroscopy.
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Chapter 13 Giant Cell Tumor of the Tendon Sheath 235

Initially, these injuries may be managed with referred to a hand surgeon for potential exci-

WRIST AND HAND PAIN


casting or splinting. Arthroscopic surgery is sion and drainage.
indicated if symptoms persist.
■ Epithelioid Sarcoma
TUMORS Chief Clinical Characteristics
■ Chondrosarcoma This presentation can involve a slow-growing,
Chief Clinical Characteristics firm, pain-free subcutaneous lump on the
This presentation may include a gradually palm or palmar surface of the digits. Pain
enlarging mass on the metacarpals or proxi- may result from impingement of the tumor on
mal phalanx that is usually painless. surrounding structures. These tumors even-
tually may develop draining ulcers.
Background Information
Pain may result from impingement of sur- Background Information
rounding structures or pathological fracture. Onset is usually in the second to fourth
Onset is usually in the fourth to fifth decades decades of life. The rate of metastasis with
of life. Diagnosis is confirmed by plain radi- epithelioid sarcoma is high, in part because
ographs and biopsy.47 Individuals suspected of the tumor is often overlooked or misdiag-
this condition should be referred to a hand nosed as a benign lesion. Following metasta-
surgeon for additional evaluation and man- sis, there is a high mortality rate. Diagnosis is
agement, which may include surgical excision. confirmed by biopsy.48,49 Any lump that
presents the possibility of epithelioid sar-
■ Enchondroma coma should be referred to a hand surgeon.
Chief Clinical Characteristics ■ Ganglion Cyst
This presentation typically includes wrist and
hand pain following pathological fracture. Chief Clinical Characteristics
Onset is most often in the second to third This presentation involves a lump on the wrist
decades of life. and hand, most commonly on the dorsum of
the wrist although it can also occur on the
Background Information volar wrist or at any other joint in the hand.
Although usually benign, this condition can
rarely undergo malignant transformation. The Background Information
diagnosis is confirmed by plain radiographs.47 On palpation, the lump will be firm but not
Individuals suspected of this diagnosis should rigid. This cyst may increase and decrease in
be referred to a hand surgeon. Treatment size over time. Pain may be present or ab-
of pathological fracture associated with this sent; if present, it usually results from irrita-
condition consists of immobilization to allow tion to adjacent structures. The etiology of
fracture healing, followed by surgery to ad- this condition is unclear, although trauma or
dress the tumor. irritation may be involved. Onset is usually
in the second to third decades of life, but the
■ Epithelial Inclusion Cyst patient may not seek treatment at initial
onset if pain is not present. Magnetic reso-
Chief Clinical Characteristics
nance imaging confirms the diagnosis.31 In-
This presentation is mainly characterized by
dividuals suspected of this diagnosis should
a firm, mobile, pain-free mass on the wrist and
be referred to a hand surgeon. Surgery is
hand.
indicated if pain, functional interference, or
Background Information cosmesis is unacceptable for the patient.
Trauma or medical procedures may trans-
port epithelial cells into deeper tissue, typi- ■ Giant Cell Tumor of the Tendon
cally in the palm, that eventually produce this Sheath
tumor. Diagnosis is based on clinical findings Chief Clinical Characteristics
and history; biopsy is definitive.31 Individuals This presentation typically includes a mobile
suspected of this health condition should be benign tumor found on the volar aspect of
1528_Ch13_201-239 11/05/12 3:57 PM Page 236

236 Chapter 13 Glomus Tumor

the fingers, associated with tenderness to ■ Mucous Cyst


WRIST AND HAND PAIN

impact and pressure. Chief Clinical Characteristics


Background Information This presentation may involve localized digit
Onset is usually in the fourth to fifth decades pain and limited joint range of motion, asso-
of life. Diagnosis is made based on clinical ciated with possible nail growth distortion.
findings, confirmed by biopsy.50 Individuals Background Information
suspected of this diagnosis should be re- This tumor can appear translucent when
ferred to a hand surgeon. The tumor is transilluminated with a penlight. The cyst
generally managed with excisional biopsy. may deform the fingernail through pressure
on or deformation of the germinal matrix.8
■ Glomus Tumor Onset is usually after the fifth decade of life.
Chief Clinical Characteristics It is a ganglion cyst arising from the distal in-
This presentation mainly involves a painful, terphalangeal (DIP) joint, generally located
tender soft tissue mass that is sensitive to cold. on the dorsum of the finger. The tumor is
It may occur throughout the hand, but is most usually associated with an osteophyte on the
common in the fingertip and nailbed. DIP joint. Plain radiographs may confirm
Background Information the osteophyte. Complications may include
It can develop at any age, but is more likely rupture and infection. Individuals suspected
in adults.51 Diagnosis is based on clinical of this diagnosis should be referred to a hand
findings and history, especially a report of surgeon. Surgical excision (including the un-
exquisite pain with cold exposure. Individu- derlying osteophyte) is indicated.
als suspected of this diagnosis should be ■ Osteoblastoma
referred to a hand surgeon. Surgical excision
is indicated. Chief Clinical Characteristics
This presentation is characterized by a painful
■ Metastases to the Wrist and swollen area over a carpal, primarily the
Hand, Including from Primary scaphoid.
Breast, Kidney, Lung, Prostate, Background Information
and Thyroid Disease Onset is usually in the second to third
Chief Clinical Characteristics decades of life. These tumors are rare in the
This presentation typically includes un- hand. The diagnosis is confirmed by plain
remitting pain in individuals with these risk radiographs. Diagnosis is made following
factors: previous history or cancer, age plain radiograph, computed tomography, or
50 years or older, failure to improve with magnetic resonance imaging.52 Individuals
conservative therapy, and unexplained suspected of this diagnosis should be re-
weight change of more than 10 pounds in ferred to a hand surgeon. Surgical options
6 months.14 include curettage with or without bone graft,
or wider excision. If excessive bone destruc-
Background Information
tion has occurred, surgical reconstruction of
The skeletal system is the third most com-
the bone may be necessary.
mon site of metastatic disease,15 although
metastasis to the wrist and hand is rare. ■ Osteochondroma
Symptoms also may be related to patho-
Chief Clinical Characteristics
logical fracture in affected sites. Common
This presentation typically includes a hard
primary sites causing metastases to bone
prominence on bone that may produce pain
include breast, prostate, lung, and kidney.
in the surrounding soft tissues.
Bone scan, plain radiographs, or magnetic
resonance imaging confirms the diagnosis. Background Information
Individuals suspected of this diagnosis Onset is most common in the second decade of
should be referred to a hand surgeon. life.47 Solitary lesions are rare, and assessment
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Chapter 13 Ulnocarpal Impaction/Abutment Syndrome 237

for multiple lesions is warranted. Pathological Background Information

WRIST AND HAND PAIN


fractures may occur, but malignant transfor- This condition also may appear as horn-like
mation is rare. The diagnosis is confirmed by protrusions from the skin or disruptions of
plain radiographs. Individuals suspected of the nailbed. Individuals with lighter skin pig-
this diagnosis should be referred to a hand sur- mentation are at greater risk, and incidence in-
geon. Surgical excision is indicated for pain or creases with amount of lifetime sun exposure
pathological fracture. of the skin. Onset is progressively more likely
with age, and is most common in the seventh
■ Osteoid Osteoma decade of life or later.54 Tentative diagnosis is
Chief Clinical Characteristics made based on appearance and confirmed by
This presentation may include a focal area of biopsy. Individuals suspected of this diagnosis
painful bone with possible swelling that is often should be referred to a hand surgeon.
worse at night and substantially relieved by as-
pirin or other nonsteroidal anti-inflammatories. ■ Ulnar Collateral Ligament Tear
(Gamekeeper’s or Skier’s Thumb)
Background Information
There frequently is tenderness to palpation. Chief Clinical Characteristics
Joint effusion and loss of range of motion This presentation mainly includes pain,
may be present if the lesion is within the swelling, tenderness to palpation, and possibly
joint capsule. Any bone in the hand or wrist ecchymosis on the ulnar aspect of the thumb
may be involved. Onset is usually in the sec- metacarpophalangeal joint (see Fig. 13-1). With
ond or third decade of life.52,53 The diagno- a partial tear, radially directed force distal to the
sis is confirmed by plain radiographs. Indi- metacarpophalangeal joint will increase pain
viduals suspected of this diagnosis should be but not demonstrate laxity as compared to the
referred to a hand surgeon. Although they uninjured side. If the tear is complete, radially
may be self-limiting, osteoid osteomas are directed force may not increase pain, but will pro-
usually surgically excised. duce radial deviation at the metacarpopha-
langeal joint that is at least 30 degrees greater
■ Osteosarcoma than that on the uninjured side.
Chief Clinical Characteristics Background Information
This presentation involves pain and swelling Stress testing should be done very cautiously to
over the metacarpals and digits. A palpable avoid worsening a partial or nondisplaced com-
tender mass is often present. Symptoms may plete tear. This injury results from forceful
have been present for several months, and radial deviation at the metacarpophalangeal
may be associated with a pathological fracture. joint, such as when a skier falls on a ski pole
Background Information held in the first web space. It also can develop
This tumor is rare in the hand. Onset of os- following repeated radial stress. This condition
teosarcoma in the hand is in the fifth to sev- may be associated with volar plate or radial col-
enth decades of life. Plain radiographs and lateral ligament injuries; a fragment of the artic-
magnetic resonance images may be helpful, ular surface of the proximal phalanx may be
but definitive diagnosis is made following avulsed. The diagnosis is confirmed with clini-
biopsy.52 Individuals suspected of this diag- cal examination. A partial or nondisplaced
nosis should be referred to a hand surgeon complete tear can be managed nonoperatively
for potential surgical excision. with splinting or casting. Displaced complete
lesions will not heal, and require surgical repair.
■ Squamous Cell Carcinoma
Chief Clinical Characteristics ■ Ulnocarpal Impaction/Abutment
This presentation mainly involves patches of Syndrome
pink, red, gray, tan, or brown scaly skin on ar- Chief Clinical Characteristics
eas of the skin frequently exposed to the sun, This presentation typically includes pain
such as the dorsum of the hand. in the central to ulnar aspects of the wrist
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238 Chapter 13 Vascular Malformation

(see Fig. 13-2) that is worse with active or pas- 8. Jebson PJL, Kasdan ML. Hand Secrets. 2nd ed.
WRIST AND HAND PAIN

sive ulnar deviation, possibly associated with a Philadelphia, PA: Hanley & Belfus; 2002.
9. Malanga GA. Cervical radiculopathy. http://www.emed-
mechanical limitation to ulnar deviation. icine.com/sports/topic21.htm. Accessed March 4, 2006.
10. Wainner RS, Fritz JM, Irrgang JJ, Boninger ML, Delitto
Background Information A, Allison S. Reliability and diagnostic accuracy of the
This condition results when an excessively long clinical examination and patient self-report measures
ulna (ulnar positive variance) contacts the for cervical radiculopathy. Spine. Jan 1, 2003;28(1):
lunate or triquetrum during movement. Ulno- 52–62.
11. American Psychiatric Association, American Psychiatric
carpal abutment may occur following malunion Association, Task Force on DSM-IV. Diagnostic and Sta-
of a distal radius fracture with shortening, or tistical Manual of Mental Disorders: DSM-IV-TR. 4th ed.
when developmental anomalies lead to excessive Washington, DC: American Psychiatric Association;
ulnar length.35 The diagnosis is confirmed with 2000.
12. Simons DG, Travell JG, Simons LS, Travell JG. Travell &
plain radiographs and magnetic resonance im- Simons’ Myofascial Pain and Dysfunction: The Trigger
aging. Surgery is indicated to correct the length Point Manual. 2nd ed. Baltimore, MD: Williams &
discrepancy between the radius and ulna. Wilkins; 1999.
13. Zee C-S, Xu M. Meningiomas, spine. http://www.
emedicine.com/radio/topic440.htm. Accessed February
■ Vascular Malformation 4, 2006.
Chief Clinical Characteristics 14. Joines JD, McNutt RA, Carey TS, Deyo RA, Rouhani R.
This presentation may involve one or more soft, Finding cancer in primary care outpatients with low
back pain: a comparison of diagnostic strategies. J Gen
compressible masses in the wrist and hand that Intern Med. 2001;16(1):14–23.
may be tender or produce pain. 15. Holman PJ, Suki D, McCutcheon I, Wolinsky JP, Rhines
LD, Gokaslan ZL. Surgical management of metastatic
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cervical radiculopathy. Arch Phys Med Rehabil. Jul
condition is usually present at birth, but is 1990;71(8):606–609.
usually diagnosed in the first decade of life.55 17. Bhimji S. Pancoast tumor. http://www.emedicine.com/
The diagnosis is usually made through clinical med/topic3576.htm. Accessed March 4, 2006.
exam, in particular, the finding of palpable 18. Villas C, Collia A, Aquerreta JD, et al. Cervicobrachial-
gia and Pancoast tumor: value of standard anteroposte-
thrills and bruits. This condition may be man- rior cervical radiographs in early diagnosis. Orthopedics.
aged nonoperatively with splinting and pres- Oct 2004;27(10):1092–1095.
sure garments. Surgery is indicated if pain is 19. Guerrero M, Williams SC. Pancoast tumor. http://www.
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20. Galhom AA, Wagner FC. Syringomyelia. http://www.
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2447–2448. Descriptions of Chronic Pain Syndromes and Definitions
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emedicine.com/med/topic1567.htm. Accessed March 8, 27. Kasdan ML, Johnson AL. Reflex sympathetic dystrophy.
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1528_Ch14_240-260 07/05/12 1:46 PM Page 240

CHAPTER 14
Anterior Thorax Pain
■ Yogi Matharu, PT, DPT, OCS

Description of the Symptom Special Concerns


■ Acute chest pain with nausea, pallor, anxi-
This chapter describes pathology that may lead ety, vomiting, or diaphoresis
to anterior thorax pain. Local causes are de- ■ Severe fatigue or shortness of breath
fined as pathology occurring within the chest ■ Pain unresolved by rest or change in
wall structures. Remote causes are defined as position, or worse at night
pathology occurring outside this region, most ■ Pain that worsens following meals or while
notably deep to the parietal pleura. inhaling
■ Fainting, dizziness, or vertigo
■ Persistent cough that is dry in the presence
of limited medical follow-up, productive, or
includes blood

Anterior thorax

CHAPTER PREVIEW: Conditions That May Lead to Anterior Thorax Pain

T Trauma
REMOTE LOCAL
COMMON
Not applicable Delayed-onset muscle soreness 254
Dorsal nerve root irritation 254
Fracture of the rib or sternum 255
UNCOMMON
Not applicable Not applicable
RARE
Pneumothorax 250 Not applicable

240
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Chapter 14 Anterior Thorax Pain 241

ANTERIOR THORAX PAIN


I Inflammation
REMOTE LOCAL
COMMON
Aseptic Aseptic
Gastroesophageal reflux disease 246 Costochondritis 253
Septic Septic
Bronchitis 245 Not applicable
UNCOMMON
Aseptic Aseptic
Pleuritis secondary to rheumatic disease 250 Fibromyalgia 255
Galactocele 255
Septic Rheumatoid arthritis–like diseases:
Pericarditis 249 • Dermatomyositis 257
Pleurisy/pleurodynia 250
Pneumonia 250 Septic
Tracheobronchitis 251 Candida breast infection 253
Tuberculosis 252 Herpes zoster 256
Mastitis 256
Vertebral osteomyelitis 258
RARE
Aseptic Aseptic
Esophageal rupture 246 Precordial catch syndrome 256
Rheumatoid arthritis–like diseases:
Septic • Inclusion body myositis 257
Infectious esophagitis 247 • Polymyositis 257
Infectious esophagitis (secondary):
• Candida species infection 247 Septic
• Cytomegalovirus 247 Breast abscess 253
• Herpes simplex virus 247 Septic mediastinitis 258
Subdiaphragmatic abscess 251

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Cyclic breast pain 254
Early pregnancy 254
UNCOMMON
Medication or stimulant use/abuse: Iatrogenic muscle pain 256
• Illicit substances:
• Amphetamine, cocaine, or “crack” use/abuse 248
• Ecstasy/3,4-methylenedioxymethamphetamine
(MDMA) use/abuse 248
• Over-the-counter substances:
• Caffeine use/abuse 248
• Monosodium glutamate intake 248
• Pseudoephedrine (allergy/cold medicine)
use/abuse 249
(continued)
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242 Chapter 14 Anterior Thorax Pain

Metabolic (continued)
ANTERIOR THORAX PAIN

REMOTE LOCAL
UNCOMMON
• Prescription substances:
• Beta-agonist use 249
• Bronchodilators/nonspecific beta-agonist use 249
• Withdrawal from beta blockers 249
RARE
Not applicable Not applicable

Va Vascular
REMOTE LOCAL
COMMON
Angina: Not applicable
• Stable angina 244
• Unstable angina/acute coronary insufficiency 244
• Variant angina 244
Myocardial infarction 249
Pulmonary embolism/infarction 251
UNCOMMON
Aortic dissection (ascending aorta or thoracic Not applicable
descending aorta) 245
RARE
Sickle cell pain crisis 251 Mondor’s disease 256

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Thoracic disk herniation 258
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Malignant Primary, such as:
• Breast adenocarcinoma (women) 253
Malignant Metastatic:
Not applicable
Benign, such as:
• Fibrocystic breast disease 254
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Chapter 14 Anterior Thorax Pain 243

Tumor (continued)

ANTERIOR THORAX PAIN


REMOTE LOCAL
RARE
Malignant Primary, such as: Malignant Primary, such as:
• Esophageal tumor 252 • Breast adenocarcinoma (men) 253
• Lung tumor 252 Malignant Metastatic:
• Mesothelioma 252 Not applicable
Malignant Metastatic: Benign
Not applicable Not applicable
Benign:
Not applicable

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Hyperventilation 246 Not applicable
UNCOMMON
Chest pain with panic attack 245 Not applicable
Chest pain without panic attack 245
RARE
Hypochondriasis 246 Not applicable
Malingering 247

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Anterior Thorax Pain physical therapists have historically been well
trained to identify acute and dangerous condi-
Physical therapists often come into contact tions. However, making a sound judgment in
with patients reporting anterior thorax pain. less dangerous, although still urgent, condi-
In some cases, this may be their chief concern. tions can be difficult. As the diagnosis list
More likely, it will be a concern that may be demonstrates, many conditions are amenable
reported during treatment of another diagnosis. to physical therapy intervention if correctly
Recent studies reveal that myocardial infarc- identified and may not require physician inter-
tions are unrecognized in 20% to 60% of vention. Others may benefit from physical
the population.1 Because the consequence of therapy after a physician has begun treatment.
untreated cardiopulmonary disease is severe, If there is any doubt in the clinician’s mind
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244 Chapter 14 Stable Angina

about the diagnosis, signs, or symptoms, request that the individual stop activity, sit
ANTERIOR THORAX PAIN

urgent referral to a physician is indicated, par- down, and use his or her prescribed nitro-
ticularly in the presence of risk factors for glycerin spray or pills. If symptoms fail to
cardiovascular disease, such as cigarette smok- resolve or this is the first presentation of
ing, hypertension, hypercholesterolemia, dia- symptoms, the physical therapist should
betes, family history, obesity, and prolonged ex- activate the emergency medical service.
posure to stress. Finally, when the patient is in
the office, a physical therapist must decide if the ■ Unstable Angina/Acute
emergency medical system must be activated Coronary Insufficiency
(Box 14-1), if the physician should be contacted Chief Clinical Characteristics
immediately, or if the patient can wait until his This presentation is usually characterized by
or her next scheduled physician visit. pain and pressure in the chest and intrascapu-
lar region with possible radiation to the arms,
Description of Conditions That neck, torso, or jaw with symptoms lasting 20
May Lead to Anterior Thorax Pain to 30 minutes or occurring at rest.
Background Information
Remote
Individuals suspected of this condition may be
ANGINA unable to relieve their symptoms with nitro-
■ Stable Angina glycerin. Often the individual has a clot or
spasm superimposed on a region of existing
Chief Clinical Characteristics coronary artery plaque.2,5 The diagnosis is
This presentation typically includes pain and confirmed with echocardiography,1 serial
pressure in the chest or between shoulder blood tests (cardiac troponin T or I or
blades that may or may not radiate to arms, CK-MB),2–4 and angiogram.2 Infarction is not
neck, torso, or jaw with symptoms lasting 2 to present. This condition is defined as new on-
10 minutes. Symptoms are aggravated by set, angina at rest, recent increase in frequency,
activity, emotional distress, or large meals. duration, or intensity of angina.2,3,5 Initial
Background Information management by physical therapists involves
The diagnosis is made with echocardiogra- activation of the emergency medical service.
phy, serial blood tests (cardiac troponin T or
■ Variant Angina
I, or CK-MB),2–4 angiogram,2 symptom
provocation during a cardiac stress test, and Chief Clinical Characteristics
symptom relief following nitroglycerin ad- This presentation involves pain and pressure
ministration. Symptoms are consistently in the chest or between shoulder blades that
present at a certain rate–pressure product. may or may not radiate to arms, neck, torso,
If an individual with this condition develops or jaw that occurs spontaneously, often in the
symptoms, a physical therapist should morning hours, causing the patient to be
awakened with pain.2,5 Symptoms are vari-
able and do not always occur at the same
BOX 14-1 Conditions Requiring activity level.
Activation of Emergency Background Information
Medical Services
Symptoms may not be resolved with nitro-
● Angina glycerin. Diagnosis is made by echocardiog-
● Aortic dissection raphy, echocardiographic changes or symp-
● Esophageal rupture
tom provocation during a cardiac stress test,
angiogram,2,4,5 serial blood tests (cardiac
● Medication or stimulant use/abuse
troponin T or I or CK-MB),2–4 or symptom
● Myocardial infarction provocation with use of medication that
● Pneumothorax produces artery spasm. Initial management
● Pulmonary embolism/infarction by physical therapists involves activating the
emergency medical service.
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Chapter 14 Chest Pain Without Panic Attack 245

■ Aortic Dissection (Ascending condition may also have exertional dyspnea and

ANTERIOR THORAX PAIN


Aorta or Thoracic Descending difficulty sleeping.
Aorta) Background Information
Chief Clinical Characteristics Acute cases may be treated with antibiotics7;
This presentation typically includes sudden “tear- therefore, physical therapists should refer acute
ing” or “ripping” pain in the anterior chest with cases to a physician for initial management.
radiation to the intrascapular region, accompa- Treatment includes use of medications such as
nied by diaphoresis, syncope, or weakness. Pain bronchodilators, glucocorticoids, supplemental
may migrate into abdomen and lower back as dis- oxygen, transplantation, and surgery to decrease
section progresses. Symptoms peak immediately lung volume. Chronic cases of bronchitis, as well
and are not affected by change in position. as associated issues such as decreased bone mass
Background Information secondary to prolonged glucocorticoid use or
Other findings include hypertension or hy- airway clearance, can be addressed through
potension, loss of pulses, and pulmonary physical therapy intervention after the disease
edema. Individuals most at risk for this diag- process has been evaluated by a physician.
nosis are those with Marfan’s syndrome, ■ Chest Pain With Panic Attack
Ehlers-Danlos syndrome, systemic hyperten- Chief Clinical Characteristics
sion, congenital aortic anomalies, and women This presentation may include pain in the left an-
in their third trimester of pregnancy. This terior chest of a sharp, stabbing nature. In addi-
condition most commonly occurs in people tion, patient may have palpitations, sweating,
between 60 and 70 years of age. Men are twice light-headedness, gastrointestinal distress, nau-
more likely to be affected than women. Com- sea, chills or hot flushes, vomiting, shortness of
pression of surrounding structures and/or breath, and restlessness or a feeling of nervousness.
ischemia may cause neurological findings
(compression of nerves or cord ischemia) or Background Information
other complications. Chest plain radiographs Tachycardia, elevated blood pressure, and
are usually the first examination performed, moist palms may be present. Diagnosis is con-
followed by computed tomography or mag- firmed by negative tests for cardiac disease
netic resonance imaging to clarify location and such as echocardiography and blood tests, al-
extent of damage. Abdominal examination though some patients may present with
is usually ineffective to determine if an aortic echocardiographic changes. Individuals with
aneurysm is present.6 The “Ritter Rules,” recurrent attacks are advised to seek counsel-
named for the actor John Ritter, were created ing, avoid stimulants, and may be prescribed
for patients to recognize, treat, and prevent this medication. Thyroid disease and hypo-
condition. Emergent surgical correction is the glycemia must also be ruled out as causes.
typical treatment for dissections. Physical ther- Physical therapists can assist as part of a team-
apists should activate the emergency medical based approach by prescribing an exercise
service so that individuals suspected of this program to address anxiety and mood disor-
condition can be taken immediately to an der. Physical therapists may be treating an
emergency room for evaluation and treatment. individual for a different condition when
the chest pain presents. Individuals who experi-
■ Bronchitis enced recurrent medical workup may be reluc-
Chief Clinical Characteristics tant to seek additional medical assessment
This presentation typically involves chest tight- when symptoms recur; individuals suspected of
ness and burning anterior chest pain that is this condition should be encouraged to follow
worsened by a dry cough that later becomes pro- up with a physician when symptoms are noted.
ductive. Patient may also develop a fever or
bronchospasm as the disease progresses. These ■ Chest Pain Without Panic Attack
symptoms may be associated with muscular and Chief Clinical Characteristics
joint dysfunctions related to increased coughing This presentation can involve pain in the left an-
and pressure on the chest. Individuals with this terior chest of a sharp, stabbing nature. Pain may
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246 Chapter 14 Esophageal Rupture

be sporadic, lasting only for a few seconds, but dyspnea, halitosis, difficulty swallowing, and
ANTERIOR THORAX PAIN

recurring several times per minute for extended (rarely) hematemesis.9,10


periods of time. Often pain is a constant ache
Background Information
lasting for hours or days. Another presentation
Symptoms are caused by acid reflux into the
is a subjective description of pressure in throat
esophagus. This may be related to acid hyper-
and chest. Pain and paresthesias may radiate
secretion, hiatal hernia, obesity, decreased
to arm.
lower esophageal tone, and/or presence of He-
Background Information licobacter pylori bacteria in the stomach. Intra-
Diagnosis is confirmed by negative tests for gastric and intraesophageal pH monitoring
cardiac disease such as echocardiography and (>4), endoscopy (only effective if erosive
blood tests, although some patients may pres- changes are present), or trial treatment with
ent with echocardiographic changes. Patients proton pump inhibitors confirms the diagno-
with chest pain should be referred to a physi- sis.9–14 Patients should avoid large meals, caf-
cian for a complete cardiac examination. feine, alcohol, chocolate, fatty foods, citrus
foods, and excessive use of nonsteroidal anti-
■ Esophageal Rupture inflammatory medications. Physical therapists
Chief Clinical Characteristics should consider whether development of this
This presentation includes anterior chest pain disorder may be related to nonsteroidal anti-
in the retrosternal region. Depending on the inflammatory medication use prescribed for
location of rupture, the pain may be severe or other inflammation or pain disorders and
mild. Often pain will worsen with swallowing recommend (to patient and physician) alterna-
or breathing. tive means for treating pain and inflammation.
Background Information ■ Hyperventilation
Patients with this condition may present with
Chief Clinical Characteristics
dyspnea and cyanosis. Air entering the medi-
This presentation can include chest pain,
astinum may cause crackling sounds on aus-
abdominal pain, rapid or deep breathing, light-
cultation and pneumothorax. A mediastinal
headedness, and arm/face tingling.
shift may also occur. The rupture may be
caused by an instrument during endoscopy, Background Information
external trauma, increased pressure during Echocardiography may be altered, resulting in
forceful vomiting or weight lifting, or by dis- ST-T wave abnormalities. Clinical tests include
eases of the esophagus (ulcer, esophagitis, and deep breathing (as deep and fast as possible)
neoplasm). The diagnosis is confirmed with for 2 minutes. If symptoms are reproduced,
plain radiographs of the chest or barium swal- patient may be having symptoms secondary to
lowing study. Depending on the severity of hyperventilation. Diagnosis is confirmed with
the injury, surgical intervention may be re- negative cardiac tests. Treatment includes hav-
quired.8 Physical therapists should facilitate ing the patient breathe into a paper bag that
immediate evaluation by a physician including covers the mouth and nose. This will increase
activation of the emergency medical service the partial pressure of carbon dioxide in the
depending on the severity of the presenting inspired air. Recurrent symptoms are treated
symptoms. with psychotherapy. Physical therapists should
encourage individuals suspected of this condi-
■ Gastroesophageal Reflux Disease tion to have a physician evaluate all new
Chief Clinical Characteristics episodes of symptoms.
This presentation may be characterized by a
dull anterior chest pain usually in the region of ■ Hypochondriasis
the lower sternum. The pain may radiate and Chief Clinical Characteristics
is often associated with food intake, especially This presentation may include a variety of chest
large meals. Symptoms may be worsened by pain complaints and the belief that these symp-
bending forward or lying down. Some patients toms are related to a very severe medical illness
may report flatulence, hoarseness, sleep apnea, despite medical evaluation and reassurance.
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Chapter 14 Malingering 247

Background Information of this condition will have some degree of

ANTERIOR THORAX PAIN


The patient reports somatic symptoms that can- immunodeficiency.15–18 Treatment includes
not be explained by a known medical condition. the appropriate antifungal agents.
Often, individuals with this condition have a
history of inappropriate and inadequate medical ■ Cytomegalovirus
treatment. Physical therapy intervention should Chief Clinical Characteristics
be coordinated within a team approach to care This presentation may be characterized
in order to ensure that the individual is not by anterior chest pain, painful swallowing, he-
given conflicting information, because conflict- matemesis, nausea, and vomiting.
ing data may fuel a perception that inappropri-
Background Information
ate care is being provided. Individuals suspected
Most individuals suspected of this condition
of this condition should be referred to a mental
will have some degree of immunodeficiency,
health professional to confirm the diagnosis.
such as with human immunodeficiency virus
■ Infectious Esophagitis or patients who received transplants.15,17
Transmission occurs by way of multiple
Chief Clinical Characteristics routes, such as droplets, sexual contact, and
This presentation may involve anterior chest blood transfusions. Sites of infection may in-
pain with possible dysphasia, painful swallow- clude the eye, brain, and gastrointestinal
ing, and weight loss. tract. Several antiviral agents have shown
Background Information efficacy against this health condition.
Individuals with carcinoma related to
acquired immunodeficiency syndrome, dia- ■ Herpes Simplex Virus
betes mellitus, acid suppression, gastric sur- Chief Clinical Characteristics
gery, or steroid use (oral or inhaled) are at This presentation may include anterior chest
increased risk. The diagnosis is confirmed pain, painful swallowing, dysphasia, vomit-
with a barium swallowing examination, en- ing, fever, and chills.15,19
doscopy with brush specimens of tissue, or
biopsy. Infectious esophagitis has several eti- Background Information
ologies, and treatment is directed at the iden- Outbreaks may be caused by herpes sim-
tified organism. Patients suspected of having plex virus 1, which is classically associated
infectious esophagitis or anyone with swal- with mouth sores, or herpes simplex virus
lowing difficulty or pain should be referred to 2, which is classically associated with geni-
a physician. If very difficult or unable to swal- tal herpes. Blisters usually heal within 6 to
low, contact physician immediately. 10 days. Over time, outbreaks are thought
to become less severe and may change
INFECTIOUS ESOPHAGITIS from sores to a scar-like presentation as
(SECONDARY) immunity against the virus improves.
This condition is self-limiting and often
■ Candida Species Infection untreated. Initial occurrence or new symp-
Chief Clinical Characteristics toms should be evaluated by a physician.
This presentation can include anterior chest
pain and may include dysphagia, painful ■ Malingering
swallowing, oral thrush, or bleeding. Chief Clinical Characteristics
This presentation may involve consciously and
Background Information
intentionally produced physical symptoms in
This condition is the most common cause of
order to obtain some external reward.
esophagitis; therefore, a trial treatment is
often given rather than subjecting the Background Information
patient to endoscopy. Further examination is The reward is often secondary gain such as dis-
reserved for individuals with persistent dis- ability payments, free medical treatment at an
comfort. Endoscopy reveals whitish plaques emergency department, narcotic medication,
and exudates. Most individuals suspected or compensation via a lawsuit. Symptoms are
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248 Chapter 14 Amphetamine, Cocaine, or “Crack” Use/Abuse

not always “faked”; an individual suspected of dysfunction.23,24 This condition is thought to


ANTERIOR THORAX PAIN

this condition may deliberately exacerbate an be related to coronary artery vasospasm sec-
actual physical condition. Because chest pain is ondary to noradrenaline release associated
alarming to medical personnel and is sufficient with the drugs’ main effect. Because most
to secure immediate access to an emergency cases of chest pain induced by illicit stimu-
department, this may be a presenting symp- lants do not progress into myocardial infarc-
tom. Individuals suspected of this condition tion, individuals suspected of having this con-
should be referred to a mental health profes- dition are usually observed for 9 to 12 hours
sional to confirm the diagnosis. and then released.22,25
MEDICATION OR STIMULANT ■ Ecstasy/3,4-Methylene-
USE/ABUSE dioxymethamphetamine
Chief Clinical Characteristics (MDMA) Use/Abuse
This presentation may include nonspecific Chief Clinical Characteristics
chest pain after prescriptive or recreational use This presentation may be characterized by
of stimulant medication. The patient may be chest pain in adolescents and young adults in
of any age group, ethnicity, or sex. a broad range of ethnic groups.26
Background Information Background Information
Many illicit, over-the-counter, and prescrip- Ecstasy/MDMA may cause chest pain, palpita-
tion drugs can cause chest pain, tachycardia, tions, hypertension, faintness, panic attacks,
pulmonary changes, and cardiac palpitations loss of consciousness, seizures, and heart failure.
of sufficient severity that a patient may seek
medical care.20 Individuals suspected of this Over-the-Counter Substances
condition may come to the clinic because of ■ Caffeine Use/Abuse
these symptoms or they may develop during Chief Clinical Characteristics
treatment for another condition. It is difficult This presentation has been inconsistently cor-
to differentiate drugs based on clinical signs related to an increase in angina pectoris and
and symptoms because intoxication often re- acute coronary syndromes.27
sults in tachycardia, dilated pupils, marked
confusion, bizarre and sometimes violent be- Background Information
havior, psychosis, and hallucinations. Emer- The stimulant effects of caffeine may cause
gency medical service should be activated. palpitations that could alarm an individual
enough that he or she would seek medical
Illicit Substances treatment. It would be difficult for a physical
■ Amphetamine, Cocaine, or therapist to make this determination in their
“Crack” Use/Abuse office so it should be treated as if it were true
angina.
Chief Clinical Characteristics
This presentation can involve chest pain, pal- ■ Monosodium Glutamate Intake
pitations, hypertension, faintness, panic at- Chief Clinical Characteristics
tacks, loss of consciousness, seizures, and heart This presentation may be characterized by
failure.21 acute chest pain, burning, pressure, and short-
Background Information ness of breath.
Up to 25% of acute myocardial infarction Background Information
cases in 18- to 45-year-old patients are related Symptoms mimic an acute myocardial in-
to cocaine use. Many cases of cocaine-related farction. Laboratory tests, echocardiography,
chest pain are from cardiac causes such as and imaging are negative. Individuals with
heart failure, angina, arrhythmias, and palpi- this condition report a recent history of eat-
tations.22 However, there is evidence that the ing a meal high in monosodium glutamate,
inhalation of amphetamines and crack co- which is a common ingredient in Chinese
caine can also directly cause cardiopulmonary food and a variety of other ethnic and
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Chapter 14 Pericarditis 249

processed foods. This is a benign condition individual who recently ceased taking beta-

ANTERIOR THORAX PAIN


that resolves spontaneously.28 blocker medication.
■ Pseudoephedrine (Allergy/Cold Background Information
Medicine) Use/Abuse Angina may be associated with ischemic activ-
ity, which can result in serious cardiovascular
Chief Clinical Characteristics
injury.32 Physical therapists should screen in-
This presentation may involve sufficient chest
dividuals suspected of this condition to ensure
pain in adults and adolescents to cause the
they are taking their medication as prescribed.
patient to seek emergency care.29
Background Information ■ Myocardial Infarction
The Centers for Disease Control and Preven- Chief Clinical Characteristics
tion have received reports of adverse cardiac This presentation typically includes pain and
effects, including death, secondary to pseu- pressure in the chest or between shoulder blades
doephedrine use. Pseudoephedrine is contained that may or may not radiate to arms, neck,
in dietary supplements and allergy/ cold med- torso, or jaw. Pain may be slightly eased by flex-
icines. Many individuals will take these drugs ing the shoulders and worsened by deep breaths
without awareness of the side effects. These and/or activity.
conditions should be treated as angina.
Background Information
Prescription Substances Other findings include diaphoresis, dyspnea,
■ Beta-Agonist Use dizziness, loss of consciousness, pallor, and
tachycardia. Blood pressure may be normal,
Chief Clinical Characteristics
decreased, or elevated.2,5 Symptoms are un-
This presentation may include chest pain in
remitting to nitroglycerin. Echocardiography,1
pregnant women.
serial blood tests (cardiac troponin T or I, or
Background Information CK-MB),2–4 and angiogram2 confirm the diag-
This substance is sometimes used during nosis. Physical therapists should activate the
pregnancy to prevent preterm labor. It is esti- emergency medical services for patients with
mated that 5% of the total obstetrics popula- symptoms of acute chest pain as described
tion will take a beta agonist during their above lasting for more than 2 minutes. Recog-
pregnancy. Although serious adverse reactions nition of this disease is imperative, because
are rare, pregnant patients presenting with immediate medical intervention improves
chest pain should immediately contact their prognosis.33,34
physician or present to an emergency room.
■ Pericarditis
■ Bronchodilators/Nonspecific Chief Clinical Characteristics
Beta-Agonist Use This presentation can be characterized by an-
Chief Clinical Characteristics terior chest pain, which may radiate up toward
This presentation typically involves an in- the neck, that is relieved with sitting up, stand-
crease in heart rate and palpitations that may ing, and leaning forward; the pain is worsened
be associated with chest pain in an individ- with lying supine. Pain also may be worsened
ual with asthma who recently used an inhaler with deep breathing or coughing.
or handheld nebulizer.30,31
Background Information
Background Information A friction rub is audible during cardiac auscul-
One substance, Proventil (albuterol), also tation. In 9 out of 10 cases, the cause is unde-
may cause paradoxical bronchospasm. termined but may include viral, bacterial,
trauma, and post-transmural myocardial in-
■ Withdrawal From Beta Blockers farction.35 Viral and idiopathic causes may be
Chief Clinical Characteristics accompanied by a fever starting at approxi-
The presentation of this condition typically mately the same time as the pain. Bacterial
involves a rebound increase in angina in an causes may involve a fever that precedes the
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250 Chapter 14 Pleurisy/Pleurodynia

pain by several days. Tuberculosis-related peri- ■ Pneumonia


ANTERIOR THORAX PAIN

carditis may be accompanied by chest pain, pro- Chief Clinical Characteristics


ductive or nonproductive cough, malaise, and This presentation typically involves “stabbing”
dyspnea. Fluid in the lungs may be noted on the or “sharp” unilateral or bilateral anterior chest
radiographs and a tuberculin skin test is posi- pain that may radiate to the shoulders; it is
tive. Physical therapists should refer patient to a worsened with arm and neck movements or
physician and facilitate immediate evaluation. deep inspiration. Onset may be gradual or sud-
■ Pleurisy/Pleurodynia den, beginning with a dry cough and often other
symptoms such as myalgias, headache, or gas-
Chief Clinical Characteristics trointestinal distress.
This presentation typically includes sharp and
stabbing pain in the anterior or lateral chest (uni- Background Information
lateral or bilateral) that is made worse by deep Local tenderness may be present at the shoul-
inspiration, by movements, and in some cases der, neck, or chest wall. Fever, chills, and vom-
by lying down. Pain may be constant or inter- iting may or may not be present. Disease is
mittent, lasting 2 to 10 hours at a time. Fever caused by bacterial or viral infections that are
may or may not be present. believed to be aspirated from oropharynx.
Consequently, people with impaired con-
Background Information sciousness, such as individuals who abuse
Individuals with this condition may be sensi- alcohol and drugs, individuals fed with naso-
tive to palpation of the chest wall in the area of gastric or endotracheal tubes, and individuals
pain and may present with myalgias, dyspnea, with neurological impairments are at an
and fatigue. Pleuritic rub or crackles may be elevated risk for developing this condition. As-
heard on auscultation. Negative lung perfu- piration pneumonia may follow an episode of
sion scan and plain radiographs showing small vomiting or occur because of poor swallowing
effusion confirm the diagnosis.36 Physical function. Chest plain radiographs, sputum ex-
therapists should refer individuals suspected amination, and blood cultures confirm the di-
of this condition to a physician for evaluation. agnosis. In cases with radiographic changes
that are unresolved with treatment after 4 to
■ Pleuritis Secondary to Rheumatic 8 weeks, bronchoscopic evaluation is recom-
Disease mended to exclude the presence of unusual in-
Chief Clinical Characteristics fections or a noninfectious process.37 Physical
This presentation may involve pain in the an- therapists should refer individuals suspected
terior or lateral chest (unilateral or bilateral), of having this condition to a physician for
typically in an individual with a history of evaluation, but can often assist with managing
rheumatoid arthritis or systemic lupus erythe- the symptoms and disease process.
matosus. Pain is sharp and stabbing in nature.
It is made worse by deep inspiration, by move- ■ Pneumothorax
ments, and in some cases by lying down. Pain Chief Clinical Characteristics
may be constant or intermittent, lasting 2 to This presentation typically includes sudden,
10 hours at a time. sharp unilateral chest pain in the anterior or
lateral chest, worsened with deep inspiration and
Background Information
potentially accompanied by shortness of breath
Fever may or may not be present. The patient
or rapid breathing.
may demonstrate tenderness with palpation of
the chest wall in area of pain and may present Background Information
with myalgias, dyspnea, and fatigue. Pleuritic Tracheal deviation contralateral to the side of
rub or crackles may be heard on auscultation. the pneumothorax and jugular venous disten-
The diagnosis is confirmed with negative lung sion may be observed (Fig. 14-1). Tachycardia,
perfusion scan, and plain radiographs showing hypotension, and tachypnea may be present,
small effusion.36 Physical therapists should refer and breath sounds may be absent. Sponta-
individuals suspected of this condition for eval- neous pneumothorax occurs most commonly
uation by a physician, usually a rheumatologist. in people 20 to 40 years of age often preceded
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Chapter 14 Tracheobronchitis 251

Tracheal ■ Sickle Cell Pain Crisis

ANTERIOR THORAX PAIN


division
Chief Clinical Characteristics
Midline Suprasternal This presentation may involve diffuse pain that
notch progresses to pain in limbs and in the chest and
sometimes back. The pain is aggravated by
movement and deep breathing.
Background Information
The chest wall may be tender to palpation
especially along the ribs and intercostal region.
Pain is often caused by rib infarctions.39 Dysp-
nea and fever also may be present. Auscultation
of lungs sounds may reveal atelectasis. The
diagnosis is confirmed with history of sickle
cell disease and absence of other causes of
Collapsed pain. Treatment includes analgesics, intra-
lung venous fluids, and incentive spirometry to
FIGURE 14-1 Characteristic midline shift of medi- prevent pneumonia and mitigate atelectasis.
astinum with pneumothorax. Episodes resolve spontaneously in approxi-
mately 1 week, but may recur. Individuals
suspected of this condition should be referred
by strenuous activity, coughing, or prolonged to a physician for evaluation and management.
Valsalva maneuver (as is common during co-
caine use). Tension pneumothorax may also be ■ Subdiaphragmatic Abscess
a result of blunt trauma, penetrating trauma Chief Clinical Characteristics
(knife, bullet, or iatrogenic puncture during This presentation includes chest and shoulder
placement of central lines), use of mechanical pain that is made worse by movement, breath-
ventilators, or CPR. Plain radiographs confirm ing, coughing, and sneezing. Upper abdomen
diagnosis when they reveal the characteristic may be tender and patient may have a fever.
finding of a visceral pleural line with absence
of distal lung markings.38 Physical therapists Background Information
should activate emergency medical service for This condition may occur secondary to ab-
immediate management of the condition. dominal surgery or perforation of the bowel or
gallbladder. Computed tomography confirms
■ Pulmonary Embolism/Infarction the diagnosis. Physical therapists should refer
Chief Clinical Characteristics individuals suspected of having this condition
This presentation may be characterized by chest to an emergency medical service for evaluation.
and throat pain often accompanied by dyspnea
and worsened by thoracic spine movements and ■ Tracheobronchitis
deep breathing. Chief Clinical Characteristics
This presentation involves anterior chest
Background Information pain that may be increased by cough or breath-
Evaluation may reveal tachycardia, cough, ing, productive cough, fever, and sometimes
fever, diaphoresis, cyanosis, and clubbing of bronchospasm.
fingernails. There may be local tenderness on
the rib cage. Pulmonary embolus is a compli- Background Information
cation of calf deep venous thrombosis, so This condition is often caused by viral or
physical therapists should screen all at-risk pa- bacterial infection. Crackles, rhonchi, and
tients for this pathology. The diagnosis is con- wheezes may be heard to auscultation in
firmed with plain radiographs, negative midline position. Physical therapists should
echocardiography, and lung perfusion scan. refer individuals suspected of having this con-
Immediate physical therapy intervention is dition to a physician for evaluation and initial
activation of the emergency medical service. management.
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252 Chapter 14 Tuberculosis

■ Tuberculosis individuals suspected of having this condi-


ANTERIOR THORAX PAIN

Chief Clinical Characteristics tion to a physician for evaluation.


This presentation typically is characterized by ■ Lung Tumor
chest pain, productive or nonproductive cough,
malaise, and dyspnea. Chest pain is caused by Chief Clinical Characteristics
irritation of the pleura. This presentation includes chest pain, arm
pain, dyspnea, hoarseness, and cough, some-
Background Information times with blood-tinged sputum.
A friction rub can often be heard during
auscultation. This condition is most common Background Information
in patients under 30 years of age. Chest plain Pain may be caused by pleural irritation or
radiographs confirm the diagnosis. Fluid in may be caused my pressure of the tumor on
the lungs may be noted on radiographs and a local structures such as a nerve root. Pleu-
tuberculin skin test is positive. Individuals ritic pain includes sharp and stabbing pain
with this condition may have active pul- in the anterior or lateral chest (unilateral or
monary lesions and present with pericarditis bilateral) that is made worse by deep inspira-
as part of this disease process. Physical thera- tion, by movements, and in some cases by
pists should refer any individuals with chronic lying down. Pain may be constant or inter-
cough for physician evaluation, especially if mittent, lasting 2 to 10 hours at a time. Some
chest pain is present. Precautions should be individuals with this condition may also de-
taken when working with individuals who scribe weight loss, shortness of breath, or
demonstrate active pulmonary lesions to loss of appetite. A Pancoast tumor may also
prevent disease spread. cause shoulder pain. Diagnosis is made by
plain radiographs, with computed tomogra-
TUMORS phy, magnetic resonance imaging, and
biopsy used to determine extent of lesion
■ Esophageal Tumor and the occurrence of metastasis. Physical
Chief Clinical Characteristics therapists should refer individuals suspected
This presentation involves anterior chest pain of having this condition to a physician for
with possible dysphagia, painful swallowing, evaluation, because early detection can im-
and weight loss. prove odds of recovery.40
Background Information ■ Mesothelioma
Pain is caused by irritation of the esophagus
Chief Clinical Characteristics
similar to that of esophagitis. Painful
This presentation typically includes dull,
swallowing and dysphagia are caused by the
aching pain in the anterior chest unchanged
tumor blocking the path of swallowed food.
by coughing or inspiration/expiration. In
Late symptoms include hoarseness, hiccups,
some cases, pleuritic pain may be present.
pneumonia, and high blood calcium levels.
Risk factors include age greater than 70 years Background Information
old, male sex, African American race, history Pleuritic pain includes sharp and stabbing
of Barrett’s esophagus or gastroesophageal pain in the anterior or lateral chest (unilat-
reflux disease, tobacco use, long-term heavy eral or bilateral) that is made worse by deep
alcohol use, obesity, drinking very hot inspiration, by movements, and in some
liquids, a diet lacking fruits and vegetables, cases by lying down. Pain may be constant or
occupational exposure to chemicals used in intermittent, lasting 2 to 10 hours at a time.
dry cleaning, and history of lye ingestion. Individuals with this condition may also de-
Diagnosis is confirmed by barium swallow scribe joint pain, cough, and dyspnea. They
studies and endoscopy. Biopsies are taken also may display clubbing of the fingers,
during endoscopy to confirm cancer. Com- bone tenderness, and swelling. This disorder
puted tomography is sometimes used to is related to an exposure to asbestos 20 to
stage the cancer and plan for surgical inter- 50 years prior to onset of symptoms. The di-
vention. Physical therapists should refer agnosis is confirmed with plain radiographs
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Chapter 14 Costochondritis 253

that demonstrate irregular thickening of the Physical therapists should refer individuals sus-

ANTERIOR THORAX PAIN


pleura, calcium deposits on the pleura, or pected of having this condition to a physician
fluid in the pleural space.41 Computed to- (most likely a gynecologist) for evaluation.
mography or magnetic resonance imaging
may be used to determine extent of disease. ■ Candida Breast Infection
The prognosis for individuals with this type Chief Clinical Characteristics
of malignancy is poor, often resulting in This presentation can be characterized by nip-
death. ple pain, burning, itching, radiating pain toward
the chest wall, and redness around the nipple and
Local areola.42
■ Breast Abscess Background Information
Chief Clinical Characteristics Risk factors include steroid or antibiotic use,
This presentation may include nipple pain, diabetes, immune deficiency, yeast infections
burning, itching, radiating pain toward the elsewhere in body, breast-feeding, and nipple
chest wall, and redness around the nipple and trauma. Diagnosis is made based on analysis of
areola, a mass in the affected area of the breast, the history and risk factors. In some cases, milk
and fever. and skin cultures are used to determine the
presence of fungus. Treatment includes topical
Background Information medications, cleaning items that have touched
Axillary nodes may be enlarged. Abscess occurs the breast, and topical medications to baby’s
in 5% to 11% of women with mastitis.42 Most mouth if the individual is breast-feeding. Oral
cases occur within 8 weeks postpartum and are antifungal medication such as fluconazole may
more common in women over 30 years old or be given for persistent cases. Physical therapists
who give birth postmaturely.43 Breast abscess should refer individuals suspected of having
in a woman who is not lactating may be associ- this condition to a physician (most likely a gy-
ated with pituitary neoplasm. Needle aspira- necologist) for evaluation.
tion confirms the diagnosis. Initial treatment is
directed at the underlying infection. Physical ■ Costochondritis
therapists should refer individuals suspected of Chief Clinical Characteristics
having this condition to a physician (most This presentation typically includes “aching”
likely a gynecologist) for evaluation. pain in the upper anterior thorax, either cen-
trally or in the parasternal region, episodically
■ Breast Adenocarcinoma worsened to a “sharp, jabbing pain” with activ-
Chief Clinical Characteristics ity, sneezing, coughing, and deep breaths.45,46
This presentation involves localized, unilateral
Background Information
breast pain of an aching nature. Symptoms may
People over the age of 40 years and women are
be aggravated by pressure to the breast.
most commonly affected. The costal cartilage
Background Information is often tender to palpation. Pain may be re-
Individuals may experience generalized swelling produced with activities that require deep
of part of a breast, skin irritation, nipple pain, breathing and or upper extremity movement.
redness of the nipple or breast skin, or a Resisted horizontal abduction, resisted shoul-
discharge. A lump may be palpable, which is der abduction or adduction, or hugging self
significant because most breast cancer initially may also reproduce symptoms. During an-
presents as a nonpainful mass in the breast. other provocation test, the patient is instructed
This condition is rare in men but does occur. to take a deep breath, extend the arms, retract
The diagnosis is confirmed with the combina- the shoulders, and extend the neck. Then the
tion of palpation, mammogram, and biopsy; examiner applies traction to the extended
all three tests are usually performed. Ultra- arms. In Tietze’s syndrome, the costal cartilage
sound and magnetic resonance imaging may will also present as swollen and red in addition
also be used to differentiate a breast mass to being painful. Cardiac testing, radiographs,
from a cyst if the mammogram is positive.44 and echocardiograms are usually normal in
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254 Chapter 14 Cyclic Breast Pain

costochondritis. It is important for the physi- ■ Dorsal Nerve Root Irritation


ANTERIOR THORAX PAIN

cal therapist to educate the patient that all fu- Chief Clinical Characteristics
ture episodes of chest pain should be evaluated This presentation involves sharp, lancinating
as independent conditions.45 This will prevent pain that starts in the back and shoots through
future potentially life-threatening disease to the anterior chest in the midthoracic region.
processes such as cardiovascular disease from The pain is aggravated by any spinal move-
being attributed to this relatively benign con- ment, coughing, and sneezing.
dition. Physical therapists may begin treat-
ment, but should refer individuals suspected Background Information
of having this condition to a physician to rule A history of previous back pain may be present.
out comorbid cardiovascular disease. Sensory changes may be present as may be a
description of a burning sensation. This irrita-
■ Cyclic Breast Pain tion may occur because of disk herniation,
Chief Clinical Characteristics osteophyte formation, degeneration of the in-
This presentation may involve bilateral tervertebral disk space, tumor, tuberculosis, or
recurrent breast pain that may be aggravated osteomyelitis. The diagnosis is confirmed with
by movement and pressure but is not tender at magnetic resonance imaging and plain radi-
a focal point. Pain resolves after onset of ographs when they demonstrate the character-
menstruation. istic degenerative changes in the spine. Physical
therapists may begin treatment, but should refer
Background Information the patient to a physician to determine what
Diet may have an influence on the degree of other medical interventions may be available.
symptoms. Diagnosis is made by exclusion, his-
tory of recurrent nature, and blood tests that re- ■ Early Pregnancy
veal the occurrence of pain during the late luteal Chief Clinical Characteristics
phase of menstruation.46 Physical therapists This presentation includes bilateral breast ache,
should refer individuals suspected of this condi- associated with swelling, chest wall pain,
tion to a physician (most likely a gynecologist) nausea, vomiting, and light-headedness.
to verify this relatively benign cause of pain.
Background Information
■ Delayed-Onset Muscle Soreness Urine or blood pregnancy tests confirm the
Chief Clinical Characteristics diagnosis. Physical therapists should refer indi-
This presentation may include pain in the up- viduals suspected of being pregnant to a physi-
per thorax with occasional referral to the upper cian and emphasize the need for prenatal care.
extremities. Pain may be described as aching,
burning, or pulling. ■ Fibrocystic Breast Disease
Chief Clinical Characteristics
Background Information
This presentation typically includes lateral
This type of muscle soreness is often preceded
breast pain in one breast, tenderness, and pal-
by an abrupt increase in physical activity ap-
pable mass. There is a focal region of pain at the
proximately 12 to 48 hours prior to onset of
site of the mass.
pain. The symptoms usually occur in the mus-
cles that were most active during exercise. Background Information
While the pain is not usually severe enough to Diagnosis is made by mammogram, ultra-
seek medical attention, a person who is cur- sonography, and/or breast biopsy.46 Generally,
rently under treatment for another condition all three tests are done to verify that this is not
may present with these symptoms. Blood tests a manifestation of breast cancer. Needle aspi-
and imaging usually are negative. Clinical ex- ration will remove the fluid and relieve the
amination confirms the diagnosis. Physical pain, and aspirate fluid may be analyzed by a
therapists may begin treatment but should pathologist to confirm the benign nature of
revaluate the patient after 48 hours to confirm this condition. Physical therapists should refer
that the symptoms have resolved. individuals suspected of having this condition
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Chapter 14 Galactocele 255

to a physician (most likely a gynecologist) for cause more extensive, bilateral fractures that

ANTERIOR THORAX PAIN


evaluation. also may involve the sternum. Complex frac-
tures may result in a flail segment, in which a
■ Fibromyalgia portion of the chest wall paradoxically moves
Chief Clinical Characteristics inward on inspiration and compromises tidal
This presentation may be characterized by volume. Approximately 150 mL of blood loss
pain in the anterior and posterior neck, upper occurs with each rib fracture and fracture lines
chest, arms, lower back, and legs. Pain is are associated with sharp edges that may fur-
described as aching and burning, or as soreness ther damage surrounding tissues. Therefore,
and stiffness. Individuals will describe fatigue, concern for excessive blood loss increases as
difficulty sleeping, and pain increase with even the number of rib fractures increases. Metas-
mild activity. tases and other disease processes also may
predispose individuals to this condition. All
Background Information
individuals with rib fractures should be evalu-
Some patients will also present with psycho-
ated for potential pneumothorax or hemotho-
logical dysfunctions such as depression and
rax by way of plain radiographs. In individuals
anxiety disorders. Fibromyalgia is more com-
with fewer than five rib fractures, treatment
mon in women than men in a ratio of ap-
usually involves analgesics and physical therapy.
proximately 8:1. The prevalence increases
Individuals with more than five rib fractures or
with age. Because laboratory tests and imag-
a flail segment may also require surgery to fix-
ing are usually normal, patients are most
ate fracture segments and aspirate secretions.48
commonly diagnosed using the criteria es-
Physical therapists should refer individuals
tablished by the American College of
suspected of having this condition to a physi-
Rheumatology. 47 These criteria include
cian for evaluation but may begin treatment
widespread pain for at least 3 months, pain
for comfort and body mechanics training to
(not tenderness) to digital palpation in 11 of
protect the region.
18 points, fatigue, insomnia, joint pain,
headaches, and mood disorders.47 Sleep dis- ■ Galactocele
orders and deficiencies in growth hormone, Chief Clinical Characteristics
serotonin, and cortisol response have been This presentation can involve sometimes
implicated in the pathogenesis, but the dis- painful, unilateral, tender breast mass. Onset
ease is still poorly understood. Clinical ex- is gradual and the patient will usually report a
amination confirms the diagnosis. Physical recent episode of breast-feeding.
therapists may begin treatment, but should
refer patient to a physician (most likely a Background Information
rheumatologist) to rule out autoimmune Fever is not usually present. This is caused by
disease and to determine what other medical a blocked duct and usually resolves sponta-
interventions may be available. neously.42 Women who wear tight or restric-
tive clothing may be at increased risk for this
■ Fracture of the Rib or Sternum disorder. This is a diagnosis of exclusion, al-
Chief Clinical Characteristics though needle aspiration may reveal milky
This presentation involves chest wall pain fol- fluid, and mammography may identify a
lowing blunt trauma. Pain may be worsened with fluid in the lesion. Mammography is usually
trunk movements that move the fracture site or reserved for individuals with recurrent le-
when taking a deep breath. sions. Physical therapists should refer indi-
viduals suspected of having this condition to
Background Information a physician (most likely a gynecologist) for
Typically, low-velocity impacts (ie, sports par- evaluation. In addition, physical therapists
ticipation) can cause unilateral and isolated may advise regarding infant feeding posi-
rib fractures, whereas high-velocity impacts tions, avoidance of restrictive clothing, and
(ie, deceleration against a steering wheel dur- application of heat to breast prior to breast-
ing a motor vehicle accident) and crushes feeding.
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256 Chapter 14 Herpes Zoster

■ Herpes Zoster flu-like symptoms. The affected breast will have


ANTERIOR THORAX PAIN

Chief Clinical Characteristics an area of redness, swelling, tenderness, and


This presentation includes unilateral, sharp, warmth.
and shooting pain in the anterior chest similar Background Information
to that of myocardial infarction, accompanied This occurs most commonly in women who
by a nodular skin rash several days after onset. are breast-feeding and may be accompanied
Background Information by visible breaks in the skin.42 In persistent
Skin nodules are usually small, red, and oval. As cases, milk cultures will be taken to identify
the pathology progresses, the pain may change the specific bacteria to determine the most ap-
to a burning nature. Individuals with this con- propriate intravenous antibiotics. Because this
dition may have fever, malaise, or sensory condition is caused by a bacterial infection, it
changes in the region of symptoms. Wrestlers is usually treated with oral antibiotic medica-
sometimes develop infections on the thorax be- tion. Physical therapists should refer individ-
cause of skin trauma. This condition can also uals suspected of having this condition to a
cause viral esophagitis, characterized by ret- physician (most likely a gynecologist) for
rosternal chest pain. Clinical examination evaluation.
confirms the diagnosis. Treatment includes the ■ Mondor’s Disease
administration of antiviral agents as soon as the
zoster eruption is noted, ideally within 48 to Chief Clinical Characteristics
72 hours. If timing is greater than 3 days, treat- This presentation can involve pain on one side
ment is aimed at controlling pain and pruritus of the anterior chest that is made worse by deep
and minimizing the risk of secondary infec- inspiration. Individuals with this condition
tion.49 Physical therapists should refer individu- may describe a trauma to this region that causes
als suspected of having this condition to a physi- the superficial chest wall vein rupture charac-
cian for evaluation. After antiviral medications teristic of this pathology.
are initiated, the physical therapist can partici- Background Information
pate in developing pain management strategies. Palpation may reveal a tender cord-like
structure with topical redness. Symptoms
■ Iatrogenic Muscle Pain resolve spontaneously in 1 to 4 weeks. Clini-
Chief Clinical Characteristics cal examination confirms the diagnosis; im-
This presentation may involve generalized or spe- aging, blood tests, and urinalysis are usually
cific region muscle pain or “soreness” without unhelpful.
apparent cause. Symptoms do not resolve as
would be expected with delayed-onset muscle ■ Precordial Catch Syndrome
soreness. Chief Clinical Characteristics
Background Information This presentation can be characterized by
“Muscle soreness” is a predominant symptom severe, sharp, nonradiating pain in the
in certain myalgias resulting from medication central chest at rest or after mild exertion.50,51
use such as with cholesterol-lowering drugs. This pain may last only a few seconds but
Individuals using these medications who have may be followed by a short period of residual
symptoms that do not resolve or improve ache.
within a few days should consult with their Background Information
physician regarding these pains, because they There is usually no tenderness and it is not
can be a sign of a serious side effect. possible to reproduce symptoms. Radiographs
and electrocardiograms are usually normal.
■ Mastitis Clinical examination confirms the diagnosis.
Chief Clinical Characteristics Treatment consists of instructing the individ-
This presentation typically includes unilateral uals about how to alleviate symptoms by
pain in the breast, accompanied by fever and standing upright or taking a deep breath.
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Chapter 14 Polymyositis 257

RHEUMATOID ARTHRITIS–LIKE and is more common in Caucasians than

ANTERIOR THORAX PAIN


DISEASES African Americans. Dysphagia is present in
■ Dermatomyositis 60% of the patients. In this condition, unlike
polymyositis and dermatomyositis, it is com-
Chief Clinical Characteristics mon for distal extremity weakness to be
This presentation typically includes pain with more pronounced than proximal weakness.
movement in any direction and muscle ten- Extramuscular symptoms include fever,
derness, aching, and weakness. The weakness malaise, dysphagia, cardiac disturbances,
often results in increasing functional deficits pulmonary dysfunction, and subcutaneous
particularly in tasks requiring the use of prox- calcifications. Other connective tissue dis-
imal musculature. eases also may be present. Diagnosis is con-
Background Information firmed by the presence of an elevated serum
This condition affects children and adults, creatine kinase level and pathology findings
but women more commonly than men. It is on muscle biopsy. Needle electromyography
identified by a blue-purple rash on the up- may be useful to demonstrate affected mus-
per eyelids, red rash on the face and trunk, cles. Physical therapists should refer individ-
and erythema of the knuckles. Scaly erup- uals suspected of having these conditions to
tions are also common. The rash may also a physician (most likely a rheumatologist)
be present on other areas of the body and for evaluation.
may worsen with sun exposure. Muscle
weakness may be absent or may occur after ■ Polymyositis
the rash appears. Extramuscular symptoms Chief Clinical Characteristics
include fever, malaise, dysphagia, cardiac This presentation involves pain with move-
disturbances, pulmonary dysfunction, and ment in any direction and muscle tenderness,
subcutaneous calcifications. It is associated aching, and weakness. The weakness often
with the presence of a malignancy in 20% results in increasing functional deficits par-
of all cases. Other connective tissue diseases ticularly in tasks requiring the use of proxi-
may be also be present. Diagnosis is most mal musculature.
commonly confirmed by the presence of an
Background Information
elevated serum creatine kinase level and
This disease is rare and is predominantly a
pathology findings on muscle biopsy. Nee-
disease of adults. This disease is overdiag-
dle electromyography may be useful to
nosed in patients presenting with multiple
demonstrate affected muscles. Physical
regions of myalgia52,53 and is the subject of
therapists should refer individuals sus-
considerable medical debate. Generalized
pected of having these conditions to a
proximal muscle weakness in multiple re-
physician (most likely a rheumatologist) for
gions of the body is the most common
evaluation.
symptom. Dysphagia and facial weakness
■ Inclusion Body Myositis are uncommon. Extramuscular symptoms
include fever, malaise, dysphagia, cardiac
Chief Clinical Characteristics
disturbances, pulmonary dysfunction, and
This presentation includes pain with move-
subcutaneous calcifications. Other connec-
ment in any direction and muscle tenderness,
tive tissue diseases may be also be present.
aching, and weakness. The weakness often re-
Elevated serum creatine kinase level and
sults in increasing functional deficits partic-
pathology findings on muscle biopsy con-
ularly in tasks requiring the use of proximal
firm the diagnosis. Needle electromyography
musculature.
may be useful to demonstrate affected mus-
Background Information cles. Physical therapists should refer individ-
This disease is the most common myopathy uals suspected of having these conditions to
in persons over 50 years of age. It is three a physician (most likely a rheumatologist)
times more common in men than in women, for evaluation.
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258 Chapter 14 Septic Mediastinitis

■ Septic Mediastinitis Background Information


ANTERIOR THORAX PAIN

Chief Clinical Characteristics Chest pain or extremity pain is present in


This presentation may be characterized by chest about 15% of individuals with vertebral
pain and shortness of breath. Pain may radiate osteomyelitis. Percussion over the involved
to back or shoulder and become worse with segment spinous process elicits tenderness.
deep breathing, coughing, or sneezing. Muscle spasm and decreased motion in the
paraspinal region are often noted. Fever is of-
Background Information ten absent. Infections often originate from
Individuals with this condition may also have bacteremia caused by organisms in the urinary
fever or difficulty swallowing. This condition tract, contaminated intravenous lines, intra-
is usually caused by esophageal rupture or venous drug use, penetrative injuries, or spinal
infection related to recent chest or sternal surgery. The diagnosis is confirmed with plain
surgery.54 Diagnosis is confirmed with medi- radiographs that show erosions in the verte-
astinal needle aspiration and computed to- bral endplate, magnetic resonance imaging or
mography, and plain radiographs may not computed tomography that shows spinal ab-
be accurate enough for diagnosis.54 Physical scesses, and an elevated erythrocyte sedimen-
therapists should refer suspected cases to an tation rate. Magnetic resonance imaging is
emergency department immediately, because considered the best test for diagnosis. Physical
the mortality rate for individuals with this therapists should refer suspected cases to a
condition is 20% even with appropriate med- physician as soon as possible to prevent fur-
ical intervention. ther bone erosion.
■ Thoracic Disk Herniation
References
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8. Duncan M, Wong RK. Esophageal emergencies: things
■ Vertebral Osteomyelitis that will wake you from a sound sleep. Gastroenterol
Clin North Am. Dec 2003;32(4):1035–1052.
Chief Clinical Characteristics 9. Gomez JE. Typical and atypical presentations of gas-
This presentation involves pain in the neck or troesophageal reflux disease and its management. Bol
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back along with anterior chest pain. The con- 10. Wang WH, Huang JQ, Zheng GF, et al. Is proton pump
dition is aggravated by any movement of the inhibitor testing an effective approach to diagnose gas-
spine. troesophageal reflux disease in patients with noncardiac
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CHAPTER15
Case Demonstration: Chest Pain
■ Amy B. Pomrantz, PT, DPT, OCS, ATC ■ Chris A. Sebelski, PT, DPT, OCS, CSCS

NOTE: This case demonstration was developed left anterior chest pain located immediately
using the diagnostic process described in lateral to her sternum and distal to the clavi-
Chapter 4 and demonstrated in Chapter 5. The cle, deep to her breast tissue at approximately
reader is encouraged to use this diagnostic the T3–T5 region. The onset of pain was
process in order to ensure thorough clinical 12 days prior, while the patient was lying in a
reasoning. If additional elaboration is required semi-reclined position on the couch. She
on the information presented in this chapter, could not recall any specific mechanism of
please consult Chapters 4 and 5. injury or movements she made before the
pain began. At the onset of symptoms, the
THE DIAGNOSTIC PROCESS pain was described as sharp with an intensity
of 8 to 10 on the 10-point verbal numeric
Step 1 Identify the patient’s chief concern.
pain scale. At that time, the pain was con-
Step 2 Identify barriers to communication.
stant, but was aggravated by sitting in
Step 3 Identify special concerns.
slumped position, inspirations during her
Step 4 Create a symptom timeline and sketch
breathing cycle, and general movement. She
the anatomy (if needed).
did not experience shortness of breath.
Step 5 Create a diagnostic hypothesis list
No position of comfort could be found
considering all possible forms of remote and
in the supine, side-lying, or prone positions.
local pathology that could cause the
Easing factors included heat, Advil, sitting up
patient’s chief concern.
with lumbar and thoracic spine in extended
Step 6 Sort the diagnostic hypothesis list by
positions, and unloading of left shoulder.
epidemiology and specific case
When her symptoms did not change after
characteristics.
3 days, OS visited the Student Health Center.
Step 7 Ask specific questions to rule specific
Plain radiographs of the chest obtained by
conditions or pathological categories less likely.
her referring physician were unremarkable
Step 8 Re-sort the diagnostic hypothesis list
and she was referred to physical therapy. Two
based on the patient’s responses to specific
days later, the pain was significantly de-
questioning.
creased and by the morning of the initial
Step 9 Perform tests to differentiate among
physical therapy visit, symptoms at the chest
the remaining diagnostic hypotheses.
region were only aggravated with palpation
Step 10 Re-sort the diagnostic hypothesis
or weight bearing through her left upper ex-
list based on the patient’s responses to
tremity. Easing factors had not changed. De-
specific tests.
spite her symptoms, the patient was attend-
Step 11 Decide on a diagnostic impression.
ing classes.
Step 12 Determine the appropriate patient
OS denied a personal history of cardiac
disposition.
disease, respiratory pathology, and cancer. She
denied alcohol or recreational drug use and
was a nonsmoker. Current medications in-
Case Description cluded Advil and Tylenol. She had no past
surgical history and stated that she was not
OS was a 24-year-old Egyptian female who pregnant. OS reported her general health as
had been enrolled in a master’s degree com- good, with her last physical performed before
munications program in the United States starting graduate school 8 months before
for the past 8 months. Her chief concern was the onset of current symptoms. She reported
261
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262 Chapter 15 Case Demonstration: Chest Pain

experiencing symptoms of a cold approxi- STEP #3: Identify special concerns.


mately 1 month ago that were now resolved. ● Recent illness. This report was deter-
STEP #1: Identify the patient’s chief mined to require further exploration to
concern. determine its potential relationship to the
patient’s current symptoms.
● Left anterolateral chest pain
STEP #2: Identify barriers to
communication. Teaching Comments: A thorough ex-
amination should include an exploration of
● English is the patient’s second language.
the relationship of any illness with the onset
Although the patient demonstrated profi-
of pain. This will appropriately increase the
ciency in English to enter graduate school,
breadth and depth of the examination.
the therapist was sensitive to the fact that
certain terminology and colloquialisms may
have been unfamiliar to the patient, even STEP #4: Create a symptom timeline and
though an interpreter was not required. sketch the anatomy (if needed).

1 month 12 days 9 days 7 days


ago ago ago ago Today

Cold Onset of Appointment Symptoms Initial


symptoms anterior chest with Student decreased physical
pain Health, patient therapy
referred to PT evaluation

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.

Remote Remote
T Trauma T Trauma
Esophageal rupture Esophageal rupture (no trauma)
Pneumothorax Pneumothorax (no trauma, no shortness
of breath)
Thoracic disk lesion Thoracic disk lesion
I Inflammation I Inflammation
Aseptic Aseptic
Gastroesophageal reflux disease Gastroesophageal reflux disease (symptom
location, report of mechanical
aggravating factors)
Pleuritis secondary to rheumatic disease Pleuritis secondary to rheumatic disease
(plain radiographs unremarkable)
Septic Septic
• Bronchitis • Bronchitis
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Chapter 15 Case Demonstration: Chest Pain 263

Infectious esophagitis secondary to: Infectious esophagitis secondary to:


• Candida • Candida
• Cytomegalovirus • Cytomegalovirus
• Herpes simplex virus • Herpes simplex virus
Pericarditis Pericarditis
• Bacterial • Bacterial
• Idiopathic • Idiopathic
• Viral • Viral
Pleurisy/pleurodynia Pleurisy/pleurodynia (plain radiographs
unremarkable)
Pneumonia Pneumonia (plain radiographs
unremarkable)
Subdiaphragmatic abscess Subdiaphragmatic abscess (time course
of symptoms)
Tracheobronchitis Tracheobronchitis
Tuberculosis Tuberculosis (plain radiographs
unremarkable)
M Metabolic M Metabolic
Medication or stimulant use/abuse, such as: Medication or stimulant use/abuse, such as:
• Illicit substances • Illicit substances (time course of symptoms)
• Amphetamines, cocaine, or “crack” • Amphetamines, cocaine, or crack (time
course of symptoms)
• Ecstasy • Ecstasy (time course of symptoms)
• Over-the-counter substances • Over the counter substances
• Caffeine • Caffeine (time course of symptoms)
• Monosodium glutamate • Monosodium glutamate (time course
of symptoms)
• Pseudoephedrine • Pseudoephedrine (time course of
symptoms)
• Prescription drugs • Prescription drugs
• Beta agonists • Beta agonists (no medications per
report)
• Bronchodilators/nonspecific beta • Bronchodilators/nonspecific beta
agonists agonists (no medications per report)
• Withdrawal from beta blockers • Withdrawal from beta blockers (no
medications per report)
Va Vascular Va Vascular
Angina: Angina:
• Stable angina • Stable angina
• Unstable angina • Unstable angina
• Variant angina • Variant angina
Aortic dissection Aortic dissection (time course)
Cardiac disease Cardiac disease
Myocardial infarction Myocardial infarction (patient age, time
course)
Pulmonary embolism Pulmonary embolism (time course)
Sickle cell pain crisis Sickle cell pain crisis (patient age, patient
sex, time course)
De Degenerative De Degenerative
Not applicable Not applicable
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264 Chapter 15 Case Demonstration: Chest Pain

Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Esophageal tumor • Esophageal tumor (symptom behavior)
• Lung tumor • Lung tumor (plain radiographs
unremarkable)
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign: Benign:
Not applicable Not applicable
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Chest pain with panic attack Chest pain with panic attack
Chest pain without panic attack Chest pain without panic attack
Hypochondriasis Hypochondriasis
Hyperventilation Hyperventilation (symptom behavior)
Malingering Malingering (no apparent secondary gain)
Local Local
T Trauma T Trauma
Delayed-onset muscle soreness Delayed-onset muscle soreness (symptom
onset)
Fracture of costal cartilage, rib, sternum, Fracture of costal cartilage, rib, sternum,
or vertebra or vertebra (no trauma, plain radiographs
unremarkable)
Intercostal muscle strain Intercostal muscle strain
Subluxation of rib Subluxation of rib (no trauma, plain
radiographs unremarkable)
I Inflammation I Inflammation
Aseptic Aseptic
Costochondritis Costochondritis
Fibromyalgia Fibromyalgia
Galactocele Galactocele
Inflammatory muscle diseases: Inflammatory muscle diseases:
• Dermatomyositis • Dermatomyositis (patient age, symptom
location)
• Inclusion body myositis • Inclusion body myositis (patient age,
symptom location)
• Polymyositis • Polymyositis (patient age, symptom
location)
Intercostal neuritis Intercostal neuritis
Pectoral myositis Pectoral myositis
Precordial catch syndrome Precordial catch syndrome (report of
mechanical aggravating factors)
Tietze’s syndrome Tietze’s syndrome
Septic Septic
Breast abscess Breast abscess
Candida breast infection Candida breast infection
Herpes zoster Herpes zoster
Mastitis Mastitis
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Chapter 15 Case Demonstration: Chest Pain 265

Septic mediastinitis Septic mediastinitis (symptom location and


behavior)
Vertebral osteomyelitis Vertebral osteomyelitis (plain radiographs
unremarkable)
M Metabolic M Metabolic
Cyclic breast pain Cyclic breast pain
Early pregnancy Early pregnancy (patient report, which may
require additional objective confirmation)
Va Vascular Va Vascular
Mondor’s disease Mondor’s disease (symptom behavior)

De Degenerative De Degenerative
Not applicable Not applicable
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Breast adenocarcinoma • Breast adenocarcinoma
• Chondrosarcoma • Chondrosarcoma (plain radiographs
unremarkable)
• Mesothelioma • Mesothelioma (plain radiographs
unremarkable)
• Osteosarcoma of sternum or ribs • Osteosarcoma of sternum or ribs (plain
radiographs unremarkable)
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign, such as: Benign, such as:
• Fibrocystic breast disease • Fibrocystic breast disease
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Not applicable Not applicable

STEP #7: Ask specific questions to rule ● Have you noticed any rashes? Yes, approx-
specific conditions or pathological imately 1 week before the onset of pain,
categories less likely. the patient reported that she developed a
● Have you been ill recently? Yes, the pa- rash in the same location of current pain.
tient reported having nasal congestion She visited the Student Health Center at
and a cough with mucous secretions in that time and the rash was diagnosed
her throat and chest. She confirmed by the physician as an allergic reaction.
symptoms of fatigue, nausea, and loss of This finding raises the index of clinical
appetite, but denied vomiting. She also suspicion for septic and aseptic forms of
denied having a fever during this illness, inflammation.
but had no thermometer to confirm her ● Do symptoms vary at different points in
body temperature. The response to this your menstrual cycle? No, the patient
question rules more likely septic and asep- denied changes in symptoms with her
tic forms of inflammation associated with menstrual cycle, excluding pathology that
systemic illness. may be sensitive to menstruation.
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266 Chapter 15 Case Demonstration: Chest Pain

STEP #8: Re-sort the diagnostic I Inflammation


hypothesis list based on the patient’s Aseptic
responses to specific questioning. Costochondritis
Remote Fibromyalgia (onset not associated with
T Trauma illness and rash)
Galactocele (onset not associated with
Thoracic disk lesion (onset not associated
illness and rash)
with illness and rash, plain radiographs
Intercostal neuritis
unremarkable)
Pectoral myositis
I Inflammation Tietze’s syndrome
Aseptic Septic
Not applicable Breast abscess
Septic Candida breast infection
Bronchitis Herpes zoster
Infectious esophagitis secondary to: Mastitis
• Candida M Metabolic
• Cytomegalovirus
Cyclic breast pain (symptoms do not vary
• Herpes simplex virus
with menstruation)
Pericarditis
• Bacterial Va Vascular
• Idiopathic Not applicable
• Viral De Degenerative
Tracheobronchitis
Not applicable
M Metabolic
Tu Tumor
Not applicable
Malignant Primary, such as:
Va Vascular • Breast adenocarcinoma
Angina: Malignant Metastatic:
• Stable angina (onset not associated with Not applicable
illness and rash) Benign, such as:
• Unstable angina (onset not associated • Fibrocystic breast disease
with illness and rash) Co Congenital
• Variant angina (onset not associated with
Not applicable
illness and rash)
Cardiac disease (onset not associated with Ne Neurogenic/Psychogenic
illness and rash) Not applicable
De Degenerative STEP #9: Perform tests to differentiate
Not applicable among the remaining diagnostic
Tu Tumor hypotheses.
● Observation. The demeanor of the patient
Not applicable
was observed to be calm during question-
Co Congenital ing, helping to rule less likely the contribu-
Not applicable tion of panic or anxiety disorders.
Ne Neurogenic/Psychogenic ● Temperature. Normal, reducing the likeli-

Chest pain with panic attack hood of current pyrogenic conditions.


Chest pain without panic attack ● Palpation. Breast tissue, intercostal spaces

Hypochondriasis in the affected area, and the pectoral


Local muscles were unremarkable. These findings
help exclude pathology that affects these
T Trauma tissues. Palpation of the costochondral
Intercostal muscle strain joints of ribs 3, 4, and 5 reproduced
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Chapter 15 Case Demonstration: Chest Pain 267

symptoms, increasing the possibility of De Degenerative


symptoms related to these structures. Not applicable
● Anteroposterior rib spring manual tech- Tu Tumor
nique. Negative for reproduction of symp-
toms, reducing the likelihood of costochon- Not applicable
dral joint structural involvement. Co Congenital
Not applicable
Ne Neurogenic/Psychogenic
Teaching Comments: Examination of the Chest pain with panic attack (observation
indicated region in this case included the negative for anxiety)
breast tissue, because it covers the costoster- Chest pain without panic attack
nal joints and identification of the spacing be- (observation negative for anxiety)
tween the ribs. A therapist must be sensitive Hypochondriasis (observation negative for
to the cultural implications of such an exami- anxiety)
nation. Education of the patient on the impor- Local
tance of the exam procedure and enlisting the T Trauma
patient’s assistance for setting appropriate
Intercostal muscle strain (no tenderness
boundaries will ensure patient comfort with
of intercostals)
the procedure.
I Inflammation
Aseptic
Costochondritis
STEP #10: Re-sort the diagnostic Intercostal neuritis
hypothesis list based on the patient’s Pectoral myositis (no tenderness of pectoral
responses to specific tests. musculature)
Remote Tietze’s syndrome
T Trauma Septic
Not applicable Breast abscess (no tenderness of breast
I Inflammation tissue)
Candida breast infection (no tenderness
Aseptic of breast tissue)
Not applicable Herpes zoster
Septic Mastitis (no tenderness of breast tissue)
Bronchitis (no fever) M Metabolic
Infectious esophagitis secondary to:
• Candida (no fever) Not applicable
• Cytomegalovirus (no fever) Va Vascular
• Herpes simplex virus (no fever) Not applicable
Pericarditis: De Degenerative
• Bacterial (no fever, aggravating and
alleviating factors) Not applicable
• Idiopathic (no fever, aggravating and Tu Tumor
alleviating factors) Malignant Primary, such as:
• Viral (no fever, aggravating and • Breast adenocarcinoma (no tenderness
alleviating factors) of breast tissue)
Tracheobronchitis (no fever) Malignant Metastatic:
M Metabolic Not applicable
Not applicable Benign, such as:
• Fibrocystic breast disease (no tenderness
Va Vascular of breast tissue)
Not applicable
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268 Chapter 15 Case Demonstration: Chest Pain

Co Congenital STEP #12: Determine the appropriate


Not applicable patient disposition.
● Refer OS back to her physician urgently
Ne Neurogenic/Psychogenic
for additional evaluation.
Not applicable
STEP #11: Decide on a diagnostic Case Outcome
impression. OS was managed by her primary care
● Rule out herpes zoster (and potentially
provider with a combination of topical
related intercostals neuritis) vs. costochon- corticosteroid ointment and oral acy-
dritis and Tietze’s syndrome. clovir. Her symptoms subsequently re-
solved without need for physical therapy
intervention.
Teaching Comments: The patient’s pres-
entation, in conjunction with the presence of Reference
a rash (previously diagnosed as an allergic re- 1. Dworkin RH, Portenoy RK. Pain and its persistence in
action), would be consistent with herpes herpes zoster. Pain. 1996;67:241-251.
zoster and related intercostal neuritis. The ill-
ness reported by the patient approximately a
week before the onset of the rash could be
considered part of a prodrome, which often
accompanies herpes zoster. Due to the limited
time course (less than 1 month since onset of
the rash), the diagnosis would not be consid-
ered post- herpetic neuralgia.1 The current
absence of an active rash indicates the pa-
tient is no longer contagious; however, refer-
ral to a physician for a medical screening
would be appropriate for antiviral interven-
tion and application of an anti-inflammatory
cream in the region. Physical therapy inter-
vention would not be indicated.
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CHAPTER 16
Posterior Thorax Pain
■ Kathy Doubleday, PT, DPT, OCS

Description of the Symptom Special Concerns


■ Acute chest pain with nausea, pallor,
This chapter describes pathology that may anxiety, vomiting, or diaphoresis
lead to posterior thorax pain. Local causes are ■ Coughing up blood, blood in stool
defined as pathology occurring within the ■ Fainting, dizziness, or vertigo
spine and posterior chest wall structures. ■ Pain unresolved by rest or change in
Remote causes are defined as occurring position, or worse at night
outside this region, most notably deep to the ■ Pain that worsens following meals or while
parietal pleura. inhaling
■ Severe fatigue or shortness of breath

269
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270 Chapter 16 Posterior Thorax Pain

CHAPTER PREVIEW: Conditions That May Lead to Posterior Thorax Pain


POSTERIOR THORAX PAIN

T Trauma
REMOTE LOCAL
COMMON
Cervical facet joint dysfunction 274 Costovertebral or costotransverse joint
dysfunction 277
Fractures:
• Compression fracture 279
• Rib fracture 279
Thoracic facet joint dysfunction 281
UNCOMMON
Not applicable T4 syndrome 281
RARE
Pneumothorax 276 Fractures:
• Vertebral fracture/dislocation 279

I Inflammation
REMOTE LOCAL
COMMON
Aseptic Aseptic
Gastroesophageal reflux disease 274 Delayed-onset muscle soreness 278
Myofascial pain disorder 280
Septic
Peptic ulcer disease 275 Septic
Not applicable
UNCOMMON
Aseptic Aseptic
Not applicable Ankylosing spondylitis 277
Fibromyalgia 278
Septic
Gallstones and biliary disease 274 Septic
Pancreatitis 275 Discitis 278
Pneumonia 276 Herpes zoster 279
Meningitis 280
Vertebral osteomyelitis 284
RARE
Aseptic Aseptic
Not applicable Transverse myelitis 282
Septic Septic
Pleurisy/empyema 275 Epidural abscess 278
Pyelonephritis 276 Tuberculosis of the spine (Pott’s disease) 282

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Osteoporosis 280
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Chapter 16 Posterior Thorax Pain 271

Metabolic (continued)

POSTERIOR THORAX PAIN


REMOTE LOCAL
UNCOMMON
Not applicable Paget’s disease 281
RARE
Not applicable Cushing’s syndrome 277
Osteomalacia 280

Va Vascular
REMOTE LOCAL
COMMON
Angina: Not applicable
• Stable angina 273
• Unstable angina/acute coronary insufficiency 273
• Variant angina 273
UNCOMMON
Myocardial infarction/acute coronary Dissecting aortic aneurysm 278
insufficiency 275
RARE
Not applicable Not applicable

De Degenerative
REMOTE LOCAL
COMMON
Cervical degenerative disk disease 274 Not applicable
UNCOMMON
Not applicable Thoracic degenerative disk disease 281
Thoracic osteoarthrosis/osteoarthritis 282
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Malignant Primary:
Not applicable
Malignant Metastatic, such as:
• Metastases, including from primary breast,
kidney, lung, prostate, and thyroid disease 283
Benign:
Not applicable
(continued)
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272 Chapter 16 Posterior Thorax Pain

Tumor (continued)
POSTERIOR THORAX PAIN

REMOTE LOCAL
RARE
Malignant Primary, such as: Malignant Primary, such as:
• Esophageal adenocarcinoma 276 • Leukemias 282
• Gastric adenocarcinoma 277 • Multiple myeloma 283
• Pancreatic carcinoma 277 • Primary bone tumors 284
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign: Benign, such as:
Not applicable • Neurogenic tumors 283
• Osteoblastoma 283
• Osteoid osteoma 283

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Posterior Thorax Pain thoracic pain. The organs most posterior in
the cavity are most likely to refer to the poste-
Physical therapists commonly treat the tho- rior thoracic region (ie, lungs, esophagus,
racic spine as a critical mobility segment in aorta, kidneys, gallbladder, and pancreas), but
movement dysfunction. The structural archi- any of the structures in the visceral cavities
tecture of the spine and adjoining rib cage give may feature thoracic pain as one component
protection to vital organs and as a result give of their clinical presentation. This chapter dis-
stiffness to this spinal region. The central loca- cusses many diagnoses outside the scope of
tion of the thoracic spine and proximity of physical therapy, a few of them are emergent,
these organs and the adjacent cervical spine and many require referral to other specialists
require consideration as possible sources of for diagnosis.
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Chapter 16 Variant Angina 273

Description of Conditions That that the individual stop activity, sit down, and

POSTERIOR THORAX PAIN


May Lead to Posterior Thorax Pain use his or her prescribed nitroglycerin spray
or pills. If symptoms fail to resolve or this is
Remote the first presentation of symptoms, the physi-
cal therapist should activate the emergency
ANGINA medical services.
■ Stable Angina
Chief Clinical Characteristics
■ Unstable Angina/Acute
This presentation can include pain and pres-
Coronary Insufficiency
sure in the chest or between shoulder blades that Chief Clinical Characteristics
may or may not radiate to arms, neck, torso, This presentation may include pain and pres-
or jaw with symptoms lasting 2 to 10 minutes sure in the chest and intrascapular region with
(Fig. 16-1). Symptoms are aggravated by possible radiation to the arms, neck, torso, or
activity, emotional distress, or large meals. jaw with symptoms lasting 20 to 30 minutes
or occurring at rest (see Fig. 16-1).
Background Information
The diagnosis is made with echocardiogra- Background Information
phy, serial blood tests (cardiac troponin T or Individuals suspected of this condition may be
I, or CK-MB),1–3 angiogram,1 symptom unable to relieve their symptoms with nitro-
provocation during a cardiac stress test, and glycerin. Often the individual has a clot or
symptom relief following nitroglycerin ad- spasm superimposed on a region of existing
ministration. Symptoms are consistently coronary artery plaque.1,4 The diagnosis is
present at a certain rate–pressure product. If confirmed with echocardiography,5 serial
an individual with this condition develops blood tests (cardiac troponin T or I or
symptoms, a physical therapist should request CK-MB),1–3 and angiogram.1 Infarction is not
present. This condition is defined as new on-
set, angina at rest, recent increase in frequency,
Esophageal
reflux
duration, or intensity of angina.1,2,4 Initial
Angina/ Pneumonia management by physical therapists involves
Myocardial
infarction
activation of the emergency medical services.
Gallstone
biliary ■ Variant Angina
disease
Chief Clinical Characteristics
This presentation can involve pain and pres-
sure in the chest or between shoulder blades
that may or may not radiate to arms, neck,
torso, or jaw that occurs spontaneously, often
in the morning hours, causing the patient to
be awakened with pain (see Fig. 16-1).1,4
Symptoms are variable and do not always
occur at the same activity level.
Background Information
Symptoms may not be resolved with nitro-
glycerin. Diagnosis is made by echocardiog-
Peptic Pleurisy
ulcer
raphy, echocardiographic changes, or symp-
tom provocation during a cardiac stress test,
angiogram,1,3,4 serial blood tests (cardiac
troponin T or I or CK-MB),1–3 or symptom
provocation with use of medication that
Pyelonephritis Pancreatitis
produces artery spasm. Initial management
FIGURE 16-1 Visceral referral patterns to the by physical therapists involves activating the
posterior thorax. emergency medical services.
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274 Chapter 16 Cervical Degenerative Disk Disease

■ Cervical Degenerative Disk


POSTERIOR THORAX PAIN

Disease
C0-4
Chief Clinical Characteristics C2-4 C0-3
C3-5
This presentation can be characterized by uni- C4-6
lateral thoracic and posterior neck pain, limited C5-7
neck motion, or upper extremity symptoms.
Background Information
Palpation of the cervical spine may reveal
altered mobility, and pain with possible repro-
duction of upper extremity and thoracic
symptoms. Trapezius and interscapular pain
patterns exist depending on the level of in- FIGURE 16-2 Referral patterns of the cervical facet
volvement.6 Associated symptoms such as arm joints.
paresthesias, weakness, or loss of reflexes in a
radicular pattern will help to identify level of resolve symptoms; relief with facet joint injec-
involvement. This condition is associated with tions also may be pathognomonic.
a history of trauma, family history of degener-
ative changes, smoking, nutrition deficits, and ■ Gallstones and Biliary Disease
high-impact physical activities. The C5–C6 Chief Clinical Characteristics
then C6–C7 and C4–C5 disks are the most fre- This presentation involves rapid onset of severe
quently affected levels.7 Cervical radiographs pain in the right epigastrium, and referred pain
confirm the diagnosis. Advancing radiculopathy to the upper thoracic spine, shoulder, and
or myelopathy may require surgical decom- scapula. An acute attack may be associated with
pression or stabilization. fever, chills, and vomiting (see Fig. 16-1).
Background Information
■ Cervical Facet Joint Dysfunction The incidence of gallstones is common in
Chief Clinical Characteristics Western countries and increases with age.
This presentation may include unilateral and Women are diagnosed two to three times more
medial scapular border pain with or without than men of the same age, with the greatest
neck pain along with marked motion deficits in risk being in obese females over 40 years of
the neck or shoulder. age. Up to 80% of gallstones contain more
than 50% cholesterol. Two-thirds of gallstones
Background Information
are asymptomatic.13 Cholescintigraphy or
Extension or ipsilateral rotation may exacer-
ultrasonography confirms the diagnosis. This
bate symptoms. Palpation of the cervical spine
condition is managed with medication or
will reveal limited joint motion, pain and
laparoscopic cholecystectomy.13
tenderness, and occasionally reproduction of
referred symptoms. This condition is prevalent ■ Gastroesophageal Reflex Disease
during the second to fourth decade and may
Chief Clinical Characteristics
not show any signs of disk disease on imaging
This presentation can involve midthoracic and
studies but may have a history of cervical
chest pain postprandial along with difficulty
trauma. It has been suggested that cervical
swallowing, regurgitation, and a globus sensa-
facet joint pain may contribute to cervical pain
tion (see Fig. 16-1).14 Pain can be worse at night
in up to 63% of nonradicular neck pain
with a recumbent position.
patients.8 Facets C4–C5 to C7–T1 have been
shown to have pain referral patterns that ex- Background Information
tend to the upper scapula or to the lower and This pain may be mistaken for angina or may
medial scapular border (Fig. 16-2).9–12 The be missed by therapists treating for thoracic
treatment of choice for this condition is phys- dysfunction. This condition appears in about
ical therapy. However, facet joint injections 20% of the population in the United States,
may be used if treatments do not sufficiently who report episodes at least once weekly.15
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Chapter 16 Pleurisy/Empyema 275

Increasing age does not seem to lead to in- disease and 90% of chronic cases are second-

POSTERIOR THORAX PAIN


creased prevalence. Short-term utilization of ary to alcohol abuse.13 Acute pancreatitis is
proton pump inhibitor medications can be usually evaluated with contrast-enhanced
used to rule in reflux prior to undergoing an computed tomography in order to judge the
upper gastrointestinal endoscopy, which can severity of the inflammation. Treatment usu-
identify histopathological changes in the mu- ally requires hospitalization for treatment of
cosa.16 Pharmacologic management with hypovolemia with close fluid regulation and
antacids or H2 blockers and diet modification packed cells if severe anemia is present.20
is usually needed to control symptoms.
■ Peptic Ulcer Disease
■ Myocardial Infarction/Acute Chief Clinical Characteristics
Coronary Insufficiency This presentation may involve deep, gnawing,
Chief Clinical Characteristics constant pain in the midthoracic to low thoracic
This presentation typically includes an acute spine and right upper abdominal quadrant and
onset of mild to severe substernal chest, inter- epigastric regions. This pain is relieved with
scapular, and shoulder pain with possible antacids, H2 blockers, or food (see Fig. 16-1). Pain
associated atypical symptoms including nausea, is worse on an empty stomach and symptoms
vomiting, diaphoresis, dyspnea, or light- may be worse at night.
headedness (see Fig. 16-1).
Background Information
Background Information It is estimated that around 10% of the popula-
One out of every five deaths in adults can be tion of Western countries has peptic ulcer dys-
attributed to this condition. Risk factors function. Peak incidence is between the ages of
include male sex, obesity, diabetes mellitus, 30 and 60 years. The peak damage to the mu-
hypertension, heredity, and elevated fibrino- cosa of the duodenum is caused primarily by
gen, homocysteine, and C-reactive protein infection with H. pylori bacteria, whereas ulcers
levels.17,18 Individuals who are older, female, induced by nonsteroidal anti-inflammatory
hypertensive, and have diabetes are at risk for ulcers usually affect the stomach.21 Utilization
atypical symptoms.19 Patients without chest of anti-inflammatories, heavy smoking, or
pain presenting with atypical or minimal alcohol abuse increases the likelihood of devel-
symptoms are less likely to seek care or receive oping ulcerations. Endoscopy can be used to
a correct diagnosis. This condition is a medical diagnose gastrointestinal disorders, as well as
emergency. Treatment within the first 4 hours to repair ulcers that do not heal and continue
should include thrombolytic therapy, cardiac to bleed.22
drug therapy, or angioplasty to improve
myocardial perfusion. ■ Pleurisy/Empyema
Chief Clinical Characteristics
■ Pancreatitis This presentation includes sharp stabbing lat-
Chief Clinical Characteristics eral chest wall, scapular, neck, and abdominal
This presentation involves severe diffuse ab- pain and is exacerbated by deep inspiration
dominal pain in the epigastric region, upper (see Fig. 16-1). Pain will gradually increase over
quadrants, and mid to low back (see Fig. 16-1). minutes to hours, and when the condition is se-
This pain will be constant and may have an vere, breathing may become shallow or labored
abrupt onset associated with nausea, vomit- and is accompanied with cough, fever, and chills.
ing, fever, and dizziness.
Background Information
Background Information Movement may agitate the pain, but splinting
Eating and alcohol intake will exacerbate their with holding a breath and lying down may give
symptoms. There may be palpable tenderness temporary relief. Pleurisy is inflammation of
and postural guarding in the epigastric area. the pleura caused by infection or injury and
Mobility and positional change will not irritation of the parietal pleura is the pain
change symptoms. More than 50% of acute generator. When fluid collects between the
cases of pancreatitis are related to gallstone parietal and visceral pleura around the lungs
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276 Chapter 16 Pneumonia

and pus forms, this is called empyema. Pleural or no identifiable pathology. The incidence has
POSTERIOR THORAX PAIN

effusions can be diagnosed by physical exam been reported to be six times greater in males,
and subjective complaints and often a pleural 85% of whom are under the age of 40. Plain
friction rub or decreased breath sounds can chest radiographs confirm the diagnosis. Mi-
be auscultated. Children and older adults nor cases may resolve themselves in a few days,
(more than 65 years old) are at highest risk for whereas when larger regions are affected, aspi-
developing an empyema. Individuals with this ration of the air from the pleural cavity or chest
condition are hospitalized and treated with tube placement may be required. This situation
thoracocentesis and antibiotics.23,24 is considered a medical emergency.28
■ Pneumonia ■ Pyelonephritis
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation can involve sharp and stab- This presentation typically includes dull, con-
bing pain in the interscapular region that stant pain in the flank, interscapular, lumbar,
worsens with a deep breath and may be associ- or groin regions accompanied by fever, chills,
ated with a cough and fever, chills, rigors, or and dysuria (see Fig. 16-1). Tenderness and
general malaise (see Fig. 16-1). rigor can be palpated in the flank or abdomen
Background Information with percussion.
In older patients, altered mentation or confu- Background Information
sion may be the first signs of infection. There Painful urination with hematuria, frequency,
is an increased risk of developing a respiratory and urgency are common with recurrent or
infection in smokers and in patients with untreated bladder infections subsequently
organ transplants, sickle cell anemia, chronic leading to bacterial infection of the kidney.
organ diseases, or immunosuppressed condi- The incidence is highest in women under
tions.25 Symptoms frequently follow a viral 50 years old with uncomplicated cases.29 Uri-
upper respiratory tract infection in which se- nalysis confirms the diagnosis and is treated
cretions proliferate resident bacteria and with appropriate antibiotic therapy.
transport them into the alveoli. The diagnosis
can be confirmed with plain chest radi-
TUMORS
ographs. Gram stain and culture of sputum
allows for treatment of bacterial causes with ■ Esophageal Adenocarcinoma
appropriate antibiotic therapy.26,27 Approxi- Chief Clinical Characteristics
mately 20% of community-acquired pneumo- This presentation can be characterized by
nia requires hospitalization and the fatality painful dysphagia, anorexia, persistent
rate can be near 9%. thorax pain, and chronic cough. Barrett’s
esophagus (gastric epithelium) and prolonged
■ Pneumothorax persistent gastroesophageal reflux disease may
Chief Clinical Characteristics be possible precursors to this malignancy.30
This presentation may include rapid onset of
chest pain, as well as possible abrupt upper and Background Information
lateral thoracic wall pain that is aggravated by Positive risk factors are excessive alcohol
movement and coughing. consumption; cigarette smoking; diets lack-
ing fresh fruits, vegetables, and animal
Background Information proteins; and esophageal abnormalities.25
Sitting upright may be the most comfortable Diagnosis is made with endoscopic evalua-
position and will be associated with shortness tion and endoscopic ultrasonography, and
of breath and rapid breathing. Traumatic positron emission tomography is needed for
pneumothorax can be caused by multiple types appropriate staging of the lesion. Surgical re-
of trauma or penetrating injury and iatrogenic section of the tumor is the primary direction
causes like biopsy or treatment with a mechan- for treatment but palliative care to maintain
ical ventilator. Spontaneous pneumothorax the esophageal lumen may be used with
can occur with underlying pulmonary disease nonsurgical candidates.
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Chapter 16 Cushing’s Syndrome 277

■ Gastric Adenocarcinoma Background Information

POSTERIOR THORAX PAIN


Chief Clinical Characteristics This condition is more common in males, as
This presentation involves epigastric or back well as people of American indigenous de-
pain and frequently the first symptom is scent, less than 40 years of age, or who carry
weight loss associated with nausea. the human leukocyte antigen B27.33 It also
may be associated with fever, malaise, and in-
Background Information flammatory bowel disease. The diagnosis is
Early gastric cancer is usually asymptomatic confirmed with plain radiographs of the
and may already be metastatic to lymph sacroiliac joints and lumbar spine, which re-
nodes by the time it is detected, resulting in a veal characteristic findings of sacroiliitis and
poor outcome.25 Symptoms may be allevi- “bamboo spine.” Blood panels including ery-
ated with H2-receptor antagonists but they throcyte sedimentation rate are useful to track
no longer assist with dyspepsia as the disease disease activity. Treatment may involve use of
advances. Men are twice as likely as women steroidal, nonsteroidal, and biological anti-
to develop these carcinomas. Diagnosis can inflammatory agents directed to the underly-
be confirmed with endoscopy or double- ing inflammation of this disease process, as
contrast studies of the upper gastrointestinal well as physical therapy to ensure optimal
tract. Surgical treatment with a transhiatal posture and body mechanics.
approach has been shown to have the fewest
respiratory complications and least thoracic ■ Costovertebral or Costotransverse
pain.31 Stent placement can be used to de- Joint Dysfunction
crease obstruction in nonsurgical candidates. Chief Clinical Characteristics
This presentation can be characterized by
■ Pancreatic Carcinoma posterior thorax pain that may extend to the
Chief Clinical Characteristics shoulder, arm, and chest and is unilaterally
This presentation may include left epigas- worsened by respiration.
trium and left shoulder pain along with mid-
dle to low back pain. The early signs of this Background Information
tumor are weight loss, epigastric pain (usu- The upper half of the thoracic spine is affected
ally postprandial), and jaundice. most frequently and can show signs of joint
locking and may be associated with spasm of
Background Information the multisegmental paraspinal muscles. Cos-
Mean age of diagnosis was reported to be 64 tovertebral joints have innervation of the syn-
with a ratio of men to women at 1.27:1.32 ovium adequate to produce thoracic pain.34
The lack of symptoms early in the progres- Plain radiograph findings may be normal or
sion of this tumor delays diagnosis and con- may show marked joint degeneration. This
tributes to a poor prognosis. Early and more diagnosis is localized with the clinical exami-
liberal diagnostic testing may allow for diag- nation but definitive diagnosis can be made
nosis before it becomes nonoperable. The di- with a local joint nerve block.
agnosis is confirmed with ultrasound, which
has 95% specificity and 70% sensitivity for ■ Cushing’s Syndrome
detecting pancreatic carcinoma.32 Chief Clinical Characteristics
This presentation may include kyphosis, back
Local pain secondary to bone loss of the spine, along
■ Ankylosing Spondylitis with muscle wasting and weakness, slow wound
healing, easy bruising, and memory deficits.
Chief Clinical Characteristics
This presentation typically includes an insidi- Background Information
ous onset of low-back and symmetric posterior This condition is associated with thoracic hy-
hip pain associated with a slowly progressive and perkyphosis due to a fatty hump at the cervi-
significant loss of general spinal mobility. Symp- cothoracic junction. Patients often present with
toms may be worse in the morning and improve a large pendulous abdomen, thin legs, increased
with light exercise. facial hair, and purple and red striations on the
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278 Chapter 16 Delayed-Onset Muscle Soreness

skin. This condition is associated with chronic gnawing pain. This condition also may be
POSTERIOR THORAX PAIN

corticosteroid treatment such as prednisone or associated with syncope, dyspnea, aphasia,


pituitary and adrenal tumors, which lead to in- hemiparesis, and a sudden drop in blood
creased cortisol levels. Urine, blood, and saliva pressure.
tests are done to detect elevated levels of corti-
Background Information
sol. Treatment includes determining the source
One-time ultrasonography screening has been
for the increased cortisol levels and using med-
shown to be 95% sensitive and nearly 100%
ication modification or tumor management
specific in men 65 to 75 years old who have
with surgical resection and/or radiation.
smoked.38 Physical examination may reveal a
■ Delayed-Onset Muscle Soreness pulsatile abdominal mass but this exam has
low accuracy and if found is a severe medical
Chief Clinical Characteristics
emergency because it can lead to myocardial
This presentation can involve mild to severe
infarction and death.
muscle soreness in the first 12 to 48 hours after
strenuous exercise or resistance activities. It can
be felt in any muscle group. ■ Epidural Abscess
Chief Clinical Characteristics
Background Information This presentation typically includes midtho-
Eccentric loads most commonly result in this racic back pain associated with fever and
condition; self-limiting upper back and scapu- variable motor weakness. This condition is as-
lar symptoms may be reported after lifting and sociated with alcohol and intravenous drug
pulling loads.35 This diagnosis is confirmed by abuse or a concurrent nonspinal infection site.
clinical examination.36
Background Information
■ Discitis Multiple organisms can cause this condition.
Chief Clinical Characteristics Screening should occur in the presence of fever,
This presentation may include mild to excru- elevated erythrocyte sedimentation rate, and
ciating, constant low back pain, localized at the increased white blood cell count in an individ-
level of the disk involved, which is associated with ual with spinal pain. The diagnosis is con-
fever and unrelieved by rest. firmed with magnetic resonance imaging with
contrast.39,40 Individuals with progressive neu-
Background Information rological weakness require emergent surgical
This condition is associated with tenderness decompression and long-term antimicrobial
over the affected segment with associated treatment for the specific organism identified.
muscle spasm and limited motion. As symp-
toms worsen, pain may radiate to adjacent ar- ■ Fibromyalgia
eas and into the lower extremities. Adult onset
usually follows spinal surgery but may occur Chief Clinical Characteristics
following bacteremia from urinary tract infec- This presentation may involve chronic wide-
tion in older patients. Magnetic resonance spread joint and muscle pain defined as bilat-
imaging is the most sensitive and specific im- eral upper body, lower body, and spine pain,
aging modality for diagnosis of discitis. Vari- associated with tenderness to palpation of 11 of
able organisms have been responsible for these 18 specific muscle-tendon sites.
infections requiring blood and biopsy cultures Background Information
to establish appropriate antibiotic treatment.37 Individuals with this condition will demonstrate
lowered mechanical and thermal pain thresh-
■ Dissecting Aortic Aneurysm olds, high pain ratings for noxious stimuli, and
Chief Clinical Characteristics altered temporal summation of pain stimuli.41
This presentation involves excruciating, severe The etiology of this condition is unclear; multi-
radiating pain to the neck, interscapular re- ple body systems appear to be involved. Indis-
gion, shoulders, low back, or abdomen. The tinct clinical boundaries between this condition
pain is often described as hot searing or throb- and similar conditions (eg, chronic fatigue syn-
bing in nature. Aneurysms can be silent or cause drome, irritable bowel syndrome, and chronic
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Chapter 16 Herpes Zoster 279

muscular headaches) pose a diagnostic chal- and compromises tidal volume. Approxi-

POSTERIOR THORAX PAIN


lenge.41 This condition is diagnosed by exclu- mately 150 mL of blood loss occurs with
sion. Treatment may include aerobic exercise, each rib fracture, and fracture lines are asso-
manual therapy, and behavior modification for ciated with sharp edges that may further
chronic pain and sleep disorders. damage surrounding tissues. Therefore, con-
cern for excessive blood loss increases as the
FRACTURES number of rib fractures increases. Metastases
■ Compression Fracture and other disease processes also may predis-
Chief Clinical Characteristics pose individuals to this condition. All indi-
This presentation typically involves traumatic viduals with rib fractures should be evaluated
or relative insidious onset of sharp midline back for potential pneumothorax or hemothorax
pain, limited tolerance for movement, espe- by way of plain radiographs. In individuals
cially flexion, and decreased sitting tolerance. with fewer than five rib fractures, treatment
Local tenderness and muscle spasm may also usually involves analgesics and physical ther-
be present. apy. Individuals with more than five rib
fractures or a flail segment may also require
Background Information surgery to fixate fracture segments and aspi-
Percussion over the affected vertebral spin- rate secretions.43 Physical therapists should
ous processes while the spine is flexed may refer individuals suspected of having this
reproduce symptoms. Compression frac- condition to a physician for evaluation but
tures are the most common thoracic spine may begin treatment for comfort and body
fracture, usually resulting from hyperflexion mechanics training to protect the region.
injuries and, in the case of pathological frac-
tures, more moderate activities or falls. Sta- ■ Vertebral Fracture/Dislocation
bility of the rib cage causes most thoracic Chief Clinical Characteristics
compression fractures to occur in the lower This presentation can include severe trauma
thoracic and thoracolumbar regions. The di- with excruciating pain, tenderness, and severe
agnosis is confirmed with thoracic spine muscle splinting in the spinal region of injury.
plain radiographs. Depending on severity, Up to 80% of thoracic fracture dislocations re-
extension bracing may be required to limit sult in complete paralysis.44 Thoracic pain
flexion forces and allow the anterior verte- may also be associated with extremity pain and
bral body to heal. Persistent pain may be paresthesias when nerve roots or the spinal cord
treated with minimally invasive percuta- is involved.
neous spinal augmentation.42
Background Information
■ Rib Fracture Acceleration or deceleration injuries com-
Chief Clinical Characteristics monly encountered in automobile accidents
This presentation involves chest wall pain fol- and falls from height can cause a variety of
lowing blunt trauma. Pain may be worsened osseous structure injuries. Mechanisms of
with trunk movements that move the fracture injury include axial loading (resulting in
site. burst fractures) and hyperextension (result-
ing in posterior element shear injuries).45
Background Information This diagnosis is confirmed with plain radi-
Typically, low-velocity impacts (ie, sports ographs or computed tomography of the
participation) can cause unilateral and iso- thoracic spine. In cases of posterior element
lated rib fractures, whereas high-velocity im- disruption or dislocation, surgical stabiliza-
pacts (ie, deceleration against a steering tion is required.
wheel during a motor vehicle accident) and
crushes cause more extensive, bilateral frac- ■ Herpes Zoster
tures that also may involve the sternum. Chief Clinical Characteristics
Complex fractures may result in a flail seg- This presentation may involve burning inter-
ment, in which a portion of the chest wall scapular pain that wraps around one side of
paradoxically moves inward on inspiration the thorax to the anterior chest associated with
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280 Chapter 16 Meningitis

itching, hyperesthesias, paresthesia, dysesthesia, confirms the diagnosis.49 This condition is a


POSTERIOR THORAX PAIN

or a painful red vesicle. medical emergency; early diagnosis and treat-


ment are essential for optimal outcome. Treat-
Background Information
ment includes an intravenously administered
The prodromal phase of this condition involves
antibiotic or antiviral agents as appropriate,
itching and sensory changes that last 2 to 3 days,
along with other supportive medical therapies.
followed by eruption of painful red vesicles up
to 3 weeks later. Vesicles may burst, leaving ■ Myofascial Pain Disorder
brown crusts over the skin lesions. T5 and T6
Chief Clinical Characteristics
are the most common dermatomes affected.46
This presentation typically involves soreness,
Individuals with this condition demonstrate a
tightness, and tenderness in the neck and upper
previous history of varicella exposure or infec-
back musculature following chronic sustained
tion. The virus remains dormant in spinal gan-
postural positions or a single overloading episode.
glia until its reactivation during a period of
stress, infection, or physical exhaustion. Pain as- Background Information
sociated with this condition may be dispropor- These muscles are described to be abnormally
tionate to the extent of skin irritation. Risk fac- shortened and have palpable firm nodules
tors for this condition include decreased called trigger points along with hypercon-
immune function. The primary complication is tracted extrafusal muscle fibers called the taut
post-herpetic neuralgia or persistent pain for band.50 These entities are thought to be rever-
longer than 1 to 3 months following the vesi- berating spinal reflex loops of sustained neural
cles.47 This diagnosis is confirmed clinically. activity.51 This diagnosis is confirmed by clini-
Treatment typically includes oral antiviral cal examination. Physical therapy interventions
agents, which appear most effective if initiated are the treatment of choice for this condition.
within 72 hours of an outbreak. If treatment is
initiated later than 3 days after outbreak, treat- ■ Osteomalacia
ment is aimed at controlling pain and pruritus Chief Clinical Characteristics
and minimizing the risk of secondary infection. This presentation can include back pain, frac-
tures, bone tenderness to palpation, muscle
■ Meningitis weakness, kyphoscoliosis, bowing of the lower
Chief Clinical Characteristics extremities, and enlarged costochondral junc-
This presentation may include acute severe neck tions. These patients may have difficulty with
and thoracic pain that is worsened with neck flex- getting up from sitting or negotiating stairs.
ion and associated with headache, vomiting,
Background Information
fever, rash, severe fatigue, and possible altered
This condition is caused by bone weakening
consciousness. Symptoms may last several weeks
secondary to vitamin D deficiency or altered
with bacterial meningitis, whereas severity of
biotransformation of vitamin D, resulting in
symptoms will be less with viral infection and
limited calcium uptake into bone. It can be
usually will last 5 to 7 days.
caused by anticonvulsant medications, renal
Background Information disease, or intestinal disorders. Plain radi-
This condition is most commonly caused by ographs reveal the characteristic transverse
meningococcal or pneumococcal bacteria. fracture-like lines in affected bone and areas of
Fungal infection presents with more mild demineralization in the matrix. Treatment
chronic symptoms and occurs in individuals usually involves oral vitamin D and treatment
with immunosuppression. Meningitis affects of underlying disorders that may result in al-
children under the age of 5 more often than tered vitamin D metabolism.
adults. Clinical exam should include meningeal
tests such as Kernig’s or Brudzinski’s sign. ■ Osteoporosis
These tests may be remarkable,48 where active Chief Clinical Characteristics
neck flexion results in hip and knee flexion This presentation includes low thoracic or high
secondary to pain response. Lumbar puncture lumbar back pain associated with characteristic
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Chapter 16 Thoracic Facet Joint Dysfunction 281

thoracic kyphosis, loss of height, and a recent Background Information

POSTERIOR THORAX PAIN


history of fracture, which causes pain. Fracture This condition often is associated with prior
pain may be the first symptom experienced by thoracic and cervical symptoms that tend to
individuals with this condition. increase in frequency and intensity. It is most
common in women between the ages of 20 and
Background Information
50 years.55 Functional spinal units adjacent to
Porous bone develops secondary to decreased
T4 may also be involved.56 Clinical examina-
deposition or increased resorption rates, lead-
tion confirms this diagnosis, and physical ther-
ing to an imbalance and gradual loss of bone
apy interventions are the initial treatments of
mass.52 This loss is most common in post-
choice.
menopausal women who experience decreas-
ing estrogen levels, but men are at risk in the ■ Thoracic Degenerative Disk
seventh to eighth decade of life as testosterone Disease
levels decline. The diagnosis is confirmed with
Chief Clinical Characteristics
dual x-ray absorptiometry. Treatment may in-
This presentation can be characterized by tho-
clude resistance exercise, bisphosphonates, and
racic pain described as coming through to the
dietary supplements of calcium.
chest from the back and may include a dull
■ Paget’s Disease ache, spasm, or burning or may be lancinating
in nature. Compression of the T1 nerve root
Chief Clinical Characteristics
will cause symptoms in the axilla and the
This presentation may involve deep, aching
ulnar border of the arm and hand. For lesions
pain that is worse at night and associated with
below T6, patients may show signs and symp-
fatigue, headaches, hearing loss, and often a
toms in the lower extremities.
pronounced kyphosis.
Background Information
Background Information
Pain may increase with a cough or sneeze and
Men are affected more than women in a 3:2
worsen with neck flexion or rotation. This di-
ratio. Long-bone deformities occur and lead
agnosis is confirmed with magnetic resonance
to adjacent joint osteoarthrosis. Bones of the
imaging. Large lesions characterized by pro-
pelvis, lumbar spine, sacrum, thoracic spine,
gressive weakness or upper motor neuron
and ribs also are commonly affected by defor-
signs should be referred to a surgeon immedi-
mity. Vertebral collapse may occur in the tho-
ately for additional evaluation. Delay in refer-
racic region and compression neuropathies
ral may reduce the prognosis for neurological
may develop. Its incidence increases by age 50
and functional recovery.
with peak incidence after 70 years of age.53
Deformities are a result of haphazard resorp- ■ Thoracic Facet Joint Dysfunction
tion of bone that then fills in with fibrous
Chief Clinical Characteristics
tissue. Subsequent attempts to form bone
This presentation may involve a horizontal
lead to large, less compact, more vascular
band of symptoms across the posterior thorax
bone susceptible to fracture.54 Diagnosis is
at the level of the dysfunction.
confirmed with radiographs and treated with
bisphosphonates. Background Information
Symptoms can be reproduced and dysfunction
■ T4 Syndrome localized with combined thoracic motions of
Chief Clinical Characteristics side-bending, rotation, with flexion or exten-
This presentation can include some localized sion. Injection studies found the pain to be
thoracic pain and stiffness with palpation. Com- one to two segments lower than the tested level
monly the presentation also includes a glove-like and share several overlapping patches along
distribution of paresthesias in one or both of the the spine.57 Definitive diagnosis can be made
arms and is associated with headache. Symp- with a facet joint nerve block. While physical
toms usually occur at night or wake the patient therapy interventions are the primary treat-
in the early morning. ment of choice for this condition, facet joint
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282 Chapter 16 Thoracic Osteoarthrosis/Osteoarthritis

nerve blockade also may be considered in spastic paraplegia that may be associated with
POSTERIOR THORAX PAIN

individuals who do not respond favorably to fever, chills, weight loss, and fatigue during
physical therapy. active disease.
■ Thoracic Osteoarthrosis/ Background Information
Osteoarthritis Airborne transmission of Mycobacterium tu-
berculosis causes infection of the lungs that can
Chief Clinical Characteristics
spread through the circulatory system to
This presentation includes a focal region of pain
spinal structures. Lower thoracic spinal seg-
unilaterally along the thoracic spine or pain
ments are the most likely to be infected. This
centrally between the scapula that worsens with
condition is becoming more prevalent second-
prolonged positions and chronic postures of the
ary to drug-resistant strains of tuberculosis
neck and back. Symptoms are usually worse
and an increasing population of immunocom-
during the morning and warming up with
promised individuals. The diagnosis is con-
motion or a hot shower improves function.
firmed by bacteriological and histological
Background Information studies of biopsy samples and standard radi-
Postural deformity may be identified and can ographs of the spine.61 Appropriate antibiotic
lead to movement and palpation findings that treatment must be followed through a full
can identify the source of symptoms. Arthrosis course of the prescription to ensure that the
of the thoracic vertebral column is more likely organism does not become resistant. Anterior
at the lower thoracic regions.58 Lateral thoracic spinal stabilization has been recommended for
plain radiographs will show degenerative joint spinal deformities, progressive neurological
and disk changes. Physical therapy interven- compromise, or significant paravertebral or
tions are the primary treatment of choice for epidural abcess.62
this condition.
■ Transverse Myelitis TUMORS
Chief Clinical Characteristics ■ Leukemias
This presentation may be characterized by Chief Clinical Characteristics
severe sharp localized midthoracic pain that This presentation involves a variety of symp-
may be combined with a dull constant ache and toms including vague back pain, bone and
low-grade fever. Progressive motor loss and joint pains, malaise, fatigue, excessive bruis-
hypoesthesia will occur in the first 72 hours up ing or bleeding, night sweats, and weight loss.
to 1 week.59 Acute disorders have a more rapid onset with
illness, with thoracic and lumbar pain being
Background Information
one of the first symptoms experienced in chil-
Inflammation causes sensory loss at a trans-
dren, teens, and adults.63 Chronic leukemias
verse sensory level on the lower trunk and
may go undiagnosed with few symptoms
motor loss may proceed to full paraplegia.
until a routine blood test identifies them.
Lumbar puncture testing and magnetic reso-
nance imaging of the spine with contrast can Background Information
be used to differentiate this disorder from Acute myelogenous leukemias constitute 85%
multiple sclerosis, tumor, abscess, herniated of adult acute leukemias. Chronic lympho-
disk, or hematoma.60 High-dose corticos- cytic leukemia is responsible for 30% of all
teroids are used in treatment but prognosis is leukemias with the median age of onset in the
inconsistent for full recovery. seventh decade.25 A proliferation of abnormal
and dysfunctional blood cells originates in the
■ Tuberculosis of the Spine (Pott’s bone marrow and then progresses into the pe-
Disease) ripheral circulation, lymph, spleen, and liver.
Chief Clinical Characteristics Diagnosis is made with aspiration or biopsy
This presentation includes spinal pain, segmen- of bone marrow and complete blood cell
tal bone tenderness, weakness, and in rare cases examination and chemistry.
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Chapter 16 Osteoid Osteoma 283

■ Metastases, Including From ■ Neurogenic Tumors

POSTERIOR THORAX PAIN


Primary Breast, Kidney, Lung, Chief Clinical Characteristics
Prostate, and Thyroid Disease This presentation typically includes symp-
Chief Clinical Characteristics toms arising from the thoracic spine produc-
This presentation may include unremitting ing back pain, intercostal neuralgia, and
pain in individuals with these risk factors: central canal location, upper motor neuron
previous history or cancer, age 50 years or signs and gait disturbances.
older, failure to improve with conservative
Background Information
therapy, and weight change of more than
The location rather than the histology of
10 pounds in 6 months.64,65
neurogenic tumors is critical because these
Background Information lesions are space occupying. Neural fora-
The prevalence of metastatic disease in pri- men encroachment and spinal cord com-
mary care patients is less than 1%. Symp- pression is common with intrathoracic
toms also may be related to pathological and intraspinal tumors.68 Schwannomas
fracture in affected sites. Involvement of the originate from the neural sheath as benign
costovertebral joints has been shown to be encapsulated dumbbell-shaped tumors,
the greatest factor for predicting vertebral whereas neurofibromas are nonencapsu-
collapse in the thoracic spine.66 Common lated fusiform enlargements of the parent
primary sites causing metastases to bone in- peripheral nerve root. Meningiomas arise
clude breast, prostate, lung, and kidney. from arachnoid matter and have a dural
Bone scan confirms the diagnosis. Treatment attachment. Diagnosis can be confirmed
may include chemotherapy, radiation, surgi- with computed tomography and magnetic
cal resection, and/or palliative care depend- resonance imaging. Early surgical explo-
ing on the type of tumor and extent of ration with resection is optimal.
metastases.
■ Osteoblastoma
■ Multiple Myeloma Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may involve an insidious on-
This presentation can include bone pain in the set of deep and aching pain, typically in males
vertebrae and ribs. Aching pain in the spine under 30 years of age. Pain associated with this
may be the first sign of this disease following condition is not usually worse at night.
pathological vertebral fracture and vertebral Background Information
collapse. Primary osteoblastomas are rare primary
bone tumors, but they occur more com-
Background Information monly in the thoracic region than other
This condition is a destructive cancer of the spinal segments.69 This condition involves
immune system plasma cells that leads to abnormal production of osteoid and primi-
tumor formation in the bone marrow and in- tive bone, although its specific etiology re-
terference with normal blood cell formation. mains unclear. Biopsy and plain radiographs
Diagnosis requires several steps including confirm the diagnosis. Computed tomogra-
protein and immunofixation electrophoresis, phy is necessary to define the tumor margins
plain radiographs, and bone marrow aspira- when surgical resection is considered. Other
tion and biopsy. Precaution must be taken forms of treatment may include radiation
during exercise prescription with areas of and chemotherapy, although the use of these
fracture and bone demineralization for indi- interventions is considered controversial.
viduals treated while recovering from this
condition. Treatment will depend on the stage ■ Osteoid Osteoma
and severity and may include chemotherapy, Chief Clinical Characteristics
radiation, stem cell transplantation, or This presentation can be characterized by
plasmapheresis.67 focal bone pain at the site of the tumor that
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284 Chapter 16 Primary Bone Tumors

is associated with tenderness and warmth to Background Information


POSTERIOR THORAX PAIN

palpation, with significant increase in pain This condition is increasingly common in


with activity and at night, as well as substan- younger populations due to the increased
tial and immediate relief of pain with incidence of immunosuppression, and also
anti-inflammatory medication. may be associated with recent surgery,
intravenous cauterization, or drug abuse.72
Background Information
Pathogens infect the vertebrae via a penetrat-
This condition is more common in males
ing trauma or through the circulatory system
than females and in the 20- to 40-year-old
from an infection elsewhere in the body.
age groups. Its pathology includes abnormal
This condition is most common in elderly
production of osteoid and primitive bone.
patients following urinary tract infections or
Pain associated with this condition is self-
diabetes mellitus. Elevated white blood cells,
limiting, but in many cases needs surgical
increased erythrocyte sedimentation rate,
resection to relieve symptoms.69,70
and increased C-reactive protein will be
■ Primary Bone Tumors detected in blood work. Radiographs are
used to determine the level of bony destruc-
Chief Clinical Characteristics
tion, and magnetic resonance imaging or
This presentation may include severe and
computed tomography may be used to locate
abrupt or low-grade aching pain in the pos-
any paravertebral abscess. Biopsy determines
terior thorax, fatigue, malaise, and pain
the type of pathogen. Appropriate antibiotic
that can awaken individuals from a sound
therapy may be used with surgical manage-
sleep.
ment of the infected bone depending on
Background Information severity.62
Osteosarcoma and chondrosarcoma are the
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sympathetic trunk and splanchnic nerves in the thorax. cal management of thoracic and lumbar vertebral
Spine. Jul–Aug 1987;12(6):527–532. osteomyelitis and discitis in adults: a review of 43
59. Kim KK. Idiopathic recurrent transverse myelitis. Arch consecutive surgically treated patients. Neurosurgery.
Neurol. Sep 2003;60(9):1290–1294. May 1996;38(5):926–933.
60. Krishnan C, Kerr DA. Idiopathic transverse myelitis.
Arch Neurol. Jun 2005;62(6):1011–1013.
1528_Ch17_287-292 07/05/12 1:51 PM Page 287

CHAPTER17
Case Demonstration: Infrascapular Pain
■ Todd E. Davenport, PT, DPT, OCS ■ Hugh G. Watts, MD

NOTE: This case demonstration was developed infrascapular region. He is an avid weekend
using the diagnostic process described in basketball player. He reports that his symp-
Chapter 4 and demonstrated in Chapter 5. The toms began about 1 month ago when he was
reader is encouraged to use this diagnostic struck from behind while attempting to re-
process in order to ensure thorough clinical bound a missed shot. He denies falling to the
reasoning. If additional elaboration is required ground and continued to play that day. His
on the information presented in this chapter, symptoms have worsened overall since their
please consult Chapters 4 and 5. onset, which led him to seek physical therapy.
He is currently not participating in his exercise
THE DIAGNOSTIC PROCESS program due to the pain. Robert is very anx-
ious about returning to his exercise routine in
Step 1 Identify the patient’s chief concern.
order to control his work-related stress. Robert
Step 2 Identify barriers to communication.
is the chief financial officer of a local television
Step 3 Identify special concerns.
production company. He started taking 800 mg
Step 4 Create a symptom timeline and sketch
ibuprofen twice daily beginning the day after
the anatomy (if needed).
his injury, which was prescribed by his in-
Step 5 Create a diagnostic hypothesis list
ternist. However, he stopped 4 days following
considering all possible forms of remote and
his injury secondary to no effect on symptoms.
local pathology that could cause the
In general, Robert reports he has been healthy
patient’s chief concern.
without history of recent illness.
Step 6 Sort the diagnostic hypothesis list by
epidemiology and specific case STEP #1: Identify the patient’s chief
characteristics. concern.
Step 7 Ask specific questions to rule specific ● Pain in the right infrascapular region
conditions or pathological categories less
likely. STEP #2: Identify barriers to
Step 8 Re-sort the diagnostic hypothesis list communication.
based on the patient’s responses to specific ● Inability to collect diagnostically relevant

questioning. information due to the patient’s eagerness


Step 9 Perform tests to differentiate among to return to recreational basketball. The
the remaining diagnostic hypotheses. patient may be less willing to share the full
Step 10 Re-sort the diagnostic hypothesis list extent of symptoms or demonstrate a bias
based on the patient’s responses to specific against musculoskeletal causes of pain
tests. secondary to the level of his desire to return
Step 11 Decide on a diagnostic impression. to activity.
Step 12 Determine the appropriate patient ● Presence of trauma. May lead the physical

disposition. therapist to deprioritize atraumatic causes


of symptoms in this patient too early in the
process.
Case Description STEP #3: Identify special concerns.
None identified.
Robert is a 41-year-old male, tall and slender,
who presents without notable physical dis-
tress. His chief concern is of pain in the right
287
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288 Chapter 17 Case Demonstration: Infrascapular Pain

STEP #4: Create a symptom timeline and


sketch the anatomy (if needed).

1 month
ago Today

Onset Initial physical


of chief therapy
concern evaluation
Trial of
Advil

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.

Remote Remote
T Trauma T Trauma
Cervical facet joint dysfunction (C6–T1) Cervical facet joint dysfunction (C6–T1)
Pneumothorax Pneumothorax (time course)
I Inflammation I Inflammation
Aseptic Aseptic
Gastroesophageal reflux disease Gastroesophageal reflux disease
Septic Septic
Gallstones (biliary disease, cholecystitis) Gallstones (biliary disease, cholecystitis)
Pancreatitis Pancreatitis
Peptic ulcer Peptic ulcer
Pleurisy/empyema Pleurisy/empyema (time course)
Pneumonia Pneumonia (no recent illness)
Pyelonephritis Pyelonephritis
M Metabolic M Metabolic
Cushing’s syndrome Cushing’s syndrome
Renal calculus Renal calculus
Va Vascular Va Vascular
Angina: Angina:
• Stable angina • Stable angina
• Unstable angina • Unstable angina
• Variant angina • Variant angina
Myocardial infarction/acute coronary Myocardial infarction, acute coronary
insufficiency insufficiency (time course)
De Degenerative De Degenerative
Cervical degenerative disk disease Cervical degenerative disk disease
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Esophageal adenocarcinoma • Esophageal adenocarcinoma (no recent
illness)
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Chapter 17 Case Demonstration: Infrascapular Pain 289

• Gastric adenocarcinoma • Gastric adenocarcinoma (no recent


illness)
• Pancreatic carcinoma • Pancreatic carcinoma (no recent illness)
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign: Benign:
Not applicable Not applicable
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Anxiety disorder Anxiety disorder
Hypochondriasis Hypochondriasis
Local Local
T Trauma T Trauma
Compression fracture of rib or scapula Compression fracture of rib or scapula
Costovertebral or costotransverse joint Costovertebral or costotransverse joint
dysfunction dysfunction
T4 syndrome T4 syndrome (distribution of symptoms)
Thoracic facet joint dysfunction Thoracic facet joint dysfunction
Vertebral fracture/dislocation Vertebral fracture/dislocation
I Inflammation I Inflammation
Aseptic Aseptic
Ankylosing spondylitis Ankylosing spondylitis (no relief with
ibuprofen)
Costochondritis Costochondritis (no relief with ibuprofen)
Delayed-onset muscle soreness Delayed-onset muscle soreness (time
course of symptoms)
Fibromyalgia Fibromyalgia (pattern of symptoms)
Hepatitis Hepatitis (no recent illness)
Myofascial pain disorder Myofascial pain disorder (pattern of
symptoms)
Transverse myelitis Transverse myelitis (time course)
Septic Septic
Discitis Discitis
Epidural abscess Epidural abscess
Herpes zoster Herpes zoster (distribution of symptoms
across multiple dermatomes)
Meningitis Meningitis (no recent illness)
Tuberculosis of the spine (Pott’s disease) Tuberculosis of the spine (Pott’s disease)
(no recent illness)
Vertebral osteomyelitis Vertebral osteomyelitis (no recent illness)
M Metabolic M Metabolic
Osteomalacia Osteomalacia
Osteoporosis Osteoporosis (age)
Paget’s disease Paget’s disease (location of symptoms)
Va Vascular Va Vascular
Dissecting aortic aneurysm Dissecting aortic aneurysm (time course)
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290 Chapter 17 Case Demonstration: Infrascapular Pain

De Degenerative De Degenerative
Thoracic degenerative disk disease Thoracic degenerative disk disease
Thoracic osteoarthrosis Thoracic osteoarthrosis
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Leukemias • Leukemias
• Multiple myeloma • Multiple myeloma
• Primary bone tumors • Primary bone tumors
Malignant Metastatic, such as: Malignant Metastatic, such as:
• Metastatic tumors • Metastatic tumors
Benign, such as: Benign, such as:
• Neurogenic tumors • Neurogenic tumors (pattern of
symptoms)
• Osteoblastoma • Osteoblastoma (age)
• Osteoid osteoma • Osteoid osteoma (no ready relief with
Advil)
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Not applicable Not applicable

STEP #7: Ask specific questions to rule I Inflammation


specific conditions or pathological Aseptic
categories less likely. Gastroesophageal reflux disease (pain
● Do you have a fever? No. unrelated to eating)
● Is your pain worsened with moving or
Septic
exercising? No. Gallstones (biliary disease, cholecystitis)
● Do you have a pinkish tinge to your urine? (blood in urine)
Yes. Pancreatitis (blood in urine)
Peptic ulcer (blood in urine)
Pleurisy/empyema (blood in urine)
Teaching Comments: Questioning of the Pneumonia (blood in urine)
effect of the gastric system on symptoms may Pyelonephritis
require several follow-up or clarification ques-
M Metabolic
tions to ensure that if a pattern exists, it can
be explored. Clarifying questions may include Cushing’s syndrome (blood in urine)
24-hour behavior of pain, association with Renal calculus
meals and/or mealtimes, alcohol consump- Va Vascular
tion, and diet. Angina:
• Stable angina (pain not associated with
exercising)
STEP #8: Re-sort the diagnostic • Unstable angina (pain not associated
hypothesis list based on the patient’s with exercising)
responses to specific questioning. • Variant angina (pain not associated with
Remote exercising)
T Trauma De Degenerative
Cervical facet joint dysfunction (C6–T1) Cervical degenerative disk disease (pain
(pain unrelated to movement) unrelated to movement)
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Chapter 17 Case Demonstration: Infrascapular Pain 291

Tu Tumor Co Congenital
Malignant Primary: Not applicable
Not applicable Ne Neurogenic/Psychogenic
Malignant Metastatic:
Not applicable
Not applicable
Benign: STEP #9: Perform tests to differentiate
Not applicable among the remaining diagnostic
Co Congenital hypotheses.
● Percussion over the costovertebral angle.
Not applicable
Positive for reproduction of symptoms,
Ne Neurogenic/Psychogenic making more likely primary renal pathology.
Not applicable ● Urinalysis with culture. Requested from

Local the physician; positive for blood in urine,


T Trauma but negative for bacteria. This finding
makes pyelonephritis less likely.
Compression fracture of rib or scapula
(pain unrelated to movement) STEP #10: Re-sort the diagnostic
Costovertebral or costotransverse joint- hypothesis list based on the patient’s
dysfunction (pain unrelated to responses to specific tests.
movement) I Inflammation
T4 syndrome (pain unrelated to movement)
Septic
Thoracic facet joint dysfunction (pain
Pyelonephritis
unrelated to movement)
Vertebral fracture/dislocation (pain M Metabolic
unrelated to movement) Renal calculus
I Inflammation STEP #11: Decide on a diagnostic
Aseptic impression.
Not applicable A thorough history combined with physical
Septic exam techniques such as percussion over the
Discitis (no fevers) costovertebral angle accurately reflects renal
Epidural abscess (blood in urine) involvement in the presenting symptoms.1
From the physical therapist’s perspective,
M Metabolic
this cluster of findings is sufficient to suggest
Osteomalacia (blood in urine) that referral to a physician is warranted.
Va Vascular Given the range of pathology remaining
Not applicable most likely after history and physical exami-
nation, the definitive test to differentiate the
De Degenerative
remaining pathologies is urinalysis with cul-
Thoracic degenerative disk disease (less ture. This test will help differentiate between
likely as it is not related to motion) kidney infection and renal calculus, which
Thoracic osteoarthrosis (less likely as it is then can be appropriately managed by the
not related to motion) physician.
Tu Tumor
STEP #12: Determine the appropriate
Malignant Primary, such as: patient disposition.
• Leukemias (no fever) ● Refer patient to a physician for consultation.
• Multiple myeloma (no fever)
• Primary bone tumors (no fever) Case Outcome
Malignant Metastatic, such as:
• Metastatic tumors (no fever) After excluding infection as a cause of the
Benign: patient’s symptoms, the patient was to follow
Not applicable up with his primary care physician at an
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292 Chapter 17 Case Demonstration: Infrascapular Pain

appointment 36 hours after intake to physical Reference


therapy. While awaiting his appointment, the 1. Eskelinen M, Ikonen J, Lipponen P. Usefulness of
history-taking, physical examination and diagnostic scor-
patient passed the kidney stone and experi- ing in acute renal colic. Eur Urol. 1998;34(6):467–473.
enced resolution of infrascapular symptoms.
1528_Ch18_293-315 11/05/12 3:58 PM Page 293

CHAPTER18
Lumbar Pain
■ Michael A. Andersen, PT, DPT, OCS ■ J. Raul Lona, DPT, OCS, ATC

Description of the Symptom Special Concerns


■ Abdominal pulsating mass
This chapter describes pathology that may lead ■ Bilateral lower extremity pain/numbness or
to lumbar pain, including thoracolumbar weakness
pain. Local causes of lumbar pain are defined ■ Bowel and bladder changes
as pathology occurring within anatomical ■ First episode of back pain in individuals
structures posterior to the abdominal aorta. younger than age 20 or older than age 55
Remote causes are defined as originating from ■ Pain at rest or at night
structures anterior to and including the ab- ■ Previous history of cancer
dominal aorta. ■ Previous history of prolonged use of
steroidal medication, diabetes, human im-
mune deficiency virus, or organ transplant
■ Rapid progression of neurological symptoms
■ Recent significant trauma
■ Saddle anesthesia

293
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294 Chapter 18 Lumbar Pain

CHAPTER PREVIEW: Conditions That May Lead to Lumbar Pain


LUMBAR PAIN

T Trauma
REMOTE LOCAL
COMMON
Not applicable Acute lumbar sprain/strain 305
Disk disruption (with or without disk herniation) 306
Facet syndrome 307
Fractures:
• Burst fracture 307
• Compression fracture 307
• Pars interarticularis fracture (spondylolysis) 308
• Traumatic spondylolisthesis 308
Myofascial pain (quadratus lumborum syndrome,
piriformis syndrome) 308
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

I Inflammation
REMOTE LOCAL
COMMON
Aseptic Aseptic
Not applicable Fibromyalgia 307
Septic Septic
Acute appendicitis 297 Not applicable
Acute cholecystitis 298
Acute pelvic inflammatory disease 298
Acute renal or urinary tract disease 298
Diverticulitis of the colon 299
Urinary tract infection 303
UNCOMMON
Aseptic Aseptic
Not applicable Abscesses:
• Epidural abscess 303
Septic • Paraspinal muscle abscess 304
Acute prostatitis 298 • Psoas muscle abscess 304
Crohn’s disease 299 • Subdural abscess 304
Duodenal ulcer 300 Ankylosing spondylitis 305
Herpes zoster 300 Complex regional pain syndrome 306
Reiter’s syndrome 309
Rheumatoid arthritis of the lumbar spine 310
Septic
Meningitis 308
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Chapter 18 Lumbar Pain 295

Inflammation (continued)

LUMBAR PAIN
REMOTE LOCAL
RARE
Aseptic Aseptic
Not applicable Polymyalgia rheumatica 309
Psoriatic arthritis 309
Septic
Bacterial endocarditis 299 Septic
Pancreatitis 301 Arachnoiditis 305
Pleuritis 301 Septic discitis 310
Splenic abscess 302 Spinal osteomyelitis 310
Transverse myelitis 311
Tuberculosis of the spine (Pott’s disease) 311

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Ectopic pregnancy 300 Osteoporosis 308
Endometriosis 300 Paget’s disease 309
RARE
Not applicable Not applicable

Va Vascular
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Aortic or iliac aneurysm 298 Not applicable
Aortic or iliac arteriosclerosis 299
RARE
Infarctions: Arteriovenous malformation of spinal cord 305
• Kidney 301 Epidural hematoma 306
• Spinal cord/conus medullaris 301
• Spleen 301

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Degenerative spondylolisthesis 306
Disk degeneration 306
Spinal stenosis 310
UNCOMMON
Not applicable Not applicable
(continued)
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296 Chapter 18 Lumbar Pain

Degenerative (continued)
LUMBAR PAIN

REMOTE LOCAL
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Malignant Primary: Not applicable
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Uterine fibroids 303

UNCOMMON
Malignant Primary: Malignant Primary:
Not applicable Not applicable
Malignant Metastatic: Malignant Metastatic, such as:
Not applicable • Metastases, including from primary breast, kidney, lung,
Benign, such as: prostate, and thyroid disease 311
• Ovarian cysts 303 Benign:
Not applicable
RARE
Malignant Primary, such as: Malignant Primary, such as:
• Carcinoma of the colon 302 • Primary bone tumor (eg, osteosarcomas, Ewing’s
• Multiple myeloma 302 sarcoma, fibrosarcoma, and chondrosarcoma) 312
• Retroperitoneal tumor 303 • Spinal cord tumor 312
Malignant Metastatic, such as: Malignant Metastatic:
• Metastases, including from primary Not applicable
breast, kidney, lung, prostate, Benign, such as:
and thyroid disease 302 • Intraspinal lipoma 311
Benign: • Osteoblastoma 312
Not applicable

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable

RARE
Not applicable Tethered spinal cord 311
(continued)
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Chapter 18 Acute Appendicitis 297

LUMBAR PAIN
Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Lumbar Pain


Lower back and thoracolumbar pain is a major
public health concern because it causes signifi-
cant functional disability, emotional distress,
and annual treatment costs. The causes of
lower back pain are often difficult to identify.
As a result, referral diagnoses from physicians
describing lumbar and thoracolumbar pain are
often nonspecific. Physical therapists’ expertise
and the large amount of time they spend with
each patient place them in a unique position to
provide information to a physician that may Kidney Liver
lead to a more specific diagnosis. Pertinent in-
formation for appropriate medical manage- Ureters Gall-
ment may include additional details about the bladder
patient’s presentation or changes in the patient’s
status. The purpose of this chapter is to serve as
a building block to help clinicians develop a sys- Ovaries
tem of differential diagnosis in patients with FIGURE 18-1 Referred pain patterns from abdom-
lumbar and/or thoracolumbar pain. inal and pelvic viscera.

Description of Conditions That


May Lead to Lumbar Pain
Background Information
Remote Other physical findings include mild tachy-
cardia, low-grade fever, hypoactive bowel
■ Acute Appendicitis sounds, and tenderness in the right lower
Chief Clinical Characteristics quadrant at McBurney’s point.1 The duration
This presentation can involve pain in the right of pain is shorter in individuals with appen-
lower abdominal and lumbar regions with lo- dicitis than with other disorders. The diagno-
cal right iliac and/or lumbar direct tenderness. sis is confirmed with clinical examination
Reports of vague periumbilical pain followed by or computed tomography scanning. This
anorexia, nausea, and vomiting are common condition is a medical emergency. Patients
(Fig. 18-1).1 presenting to physical therapy with signs and
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298 Chapter 18 Acute Cholecystitis

symptoms of this condition should be hospitalization. If left untreated, this condi-


LUMBAR PAIN

referred to the emergency department for tion often leads to infertility, ectopic preg-
immediate treatment. nancy, and chronic pelvic pain.5

■ Acute Cholecystitis ■ Acute Prostatitis


Chief Clinical Characteristics Chief Clinical Characteristics
This presentation typically includes back pain This presentation may be characterized by pain
associated with abdominal right upper in the central lower back, rectal, and/or per-
quadrant and/or right infrascapular pain, ineal region associated with fever, chills, and
fever, and signs of local peritoneal irritation painful urination. Other initial symptoms may
including right upper quadrant pain and include malaise, arthralgia, and myalgia. As
tenderness (see Fig. 18-1). The characteristic the disease progresses, prostatic inflammation
abdominal pain commonly begins after eat- produces symptoms of dysuria, frequency,
ing a large or fatty meal. Constant pain in urgency, and urine retention.
the right upper quadrant (>12 hours) and
Background Information
Murphy’s sign (inspiration is inhibited by pain
Prostatitis is usually caused by a bacterial
on palpation) are used to help diagnose acute
infection in the prostate gland. Prostatitis
cholecystitis.2 The pain may radiate to the
commonly accompanies or follows a urinary
upper and lower back, as well as the right tract infection, urethritis, or epididymitis.6
shoulder.3 Some patients with acute cholecys-
The diagnosis is confirmed by urinalysis.
titis may also report nausea vomiting, fever, Treatment usually includes intravenous antibi-
and chills. otics, which are specific to the infective agent.
Background Information
Most patients with chronic cholecystitis will ■ Acute Renal or Urinary Tract
also report a similar previous episode that re- Disease
solved spontaneously. In 90% of cases, chole- Chief Clinical Characteristics
cystitis is caused by gallstones that obstruct the This presentation includes pain in the cen-
neck of the gallbladder or the cystic duct. tral lower back (see Fig. 18-1), flank, and
Other less common causes of cholecystitis in- groin, associated with fever, nausea, and vom-
clude alcoholism, severe illness, and tumors.4 iting. Other common symptoms include urgent,
The diagnosis is confirmed with ultrasound frequent, and painful urination, abdominal
scanning. Individuals with signs of this condi- bloating, and pain upon palpation of the
tion should be referred to the emergency kidney.6
department immediately.
Background Information
■ Acute Pelvic Inflammatory Renal or urinary tract disease may follow
Disease streptococcal infection of the upper respira-
tory tract or skin within 8 to 21 days. Blood
Chief Clinical Characteristics
and urine tests are necessary for a definitive
This presentation may include lumbosacral
diagnosis. Treatment typically includes an
pain associated with dull, constant, and poorly
appropriate antibiotic, and hospitalization for
localized abdominal pain (see Fig. 18-1), abnor-
monitoring.
mal vaginal discharge, fever, painful intercourse,
and irregular menstrual bleeding. ■ Aortic or Iliac Aneurysm
Background Information Chief Clinical Characteristics
Acute pelvic inflammatory disease is most This presentation typically involves vague
prevalent in women between 15 and 23 years abdominal pain, usually located in the
of age.5 Bacterial infection in the upper epigastrium that may radiate to the low
genital tract causes this condition, most com- back, flank, or groin. 7 Other symptoms
monly associated with gonorrhea and chlamy- include early satiety, nausea, vomiting, gas-
dia. Urinalysis confirms the diagnosis. Treat- trointestinal bleeding, and lower extremity
ment includes aggressive antibiotics and ischemia.7
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Chapter 18 Diverticulitis of the Colon 299

Background Information cardiac sequelae that occur. Treatment may in-

LUMBAR PAIN
Sixty-six to 75% of abdominal aortic aneurysms clude hospitalization with specific intravenous
are asymptomatic. This condition can be diag- bactericidal antibiotics.6
nosed during a routine physical examination
by palpation of a pulsatile abdominal mass ■ Crohn’s Disease
above the umbilicus in the epigastrium.7 Chief Clinical Characteristics
Patients are considered for elective repair of This presentation can involve abdominal pain,
aneurysm when the risk of rupture exceeds bowel obstruction, weight loss, fever, chronic
the risk of surgery, which is typically an open or nocturnal diarrhea, or night sweats.12
repair for the aneurysm.7
Background Information
■ Aortic or Iliac Arteriosclerosis Symptoms of back pain occur in up to 20% of
bowel-related spondyloarthropathies.13 Crampy,
Chief Clinical Characteristics
intermittent abdominal right lower quadrant
This presentation may involve generalized
pain is common in patients with ileal Crohn’s
low back pain and symptoms consistent with
disease and is exacerbated by eating. Arthralgia
lumbar disk degeneration.8
and arthritis may also be associated with
Background Information this condition. The arthritis usually affects
Studies have correlated the occurrence of aor- the lower extremities in an asymmetrical
tic arteriosclerosis and disk degeneration.8,9 fashion and has a migratory pattern.13 The di-
The first to fourth lumbar arteries leave the agnosis is confirmed with endoscopy. Depend-
aorta in front of the corresponding vertebral ing on the severity of the pathology, medical
body supplying that vertebra and the adjacent management includes use of salicylates, corticos-
intervertebral disk via diffusion through the teroids, antibiotics, and immunosuppressants.12
vertebral end plate.8 Findings suggest that cal-
cific lesions in the upper part of the abdominal ■ Diverticulitis of the Colon
aorta predict disk deterioration at any lumbar Chief Clinical Characteristics
level.8 The link between arteriosclerosis and This presentation typically includes left lower
low back pain can be explained by the com- quadrant tenderness.14,15 Back pain can be a clin-
mon risk factors between the two pathologies, ical symptom, representing the varied disposi-
including age and smoking. The diagnosis is tion of the sigmoid colon. Signs of localized
confirmed with radiographs or computed peritoneal inflammation including involun-
tomography. Surgical techniques for treatment tary guarding, localized percussion tenderness,
of occlusive disease of the artery include en- and fever may also be present.14 Anorexia, nau-
dovascular stented-graft treatment.10 sea, and vomiting may occur as well as dysuria
and urinary frequency due to an irritated
■ Bacterial Endocarditis bladder from the sigmoid colon.15
Chief Clinical Characteristics
This presentation may include generalized Background Information
arthralgia in large proximal joints, low back A palpable, tender cylindrical mass may
pain, neck pain, and myalgia in the presence be present with guarding and rebound
of fever, chills, weight loss, and weakness.11 tenderness.15 This condition is caused by fecal
Musculoskeletal symptoms present early during material that becomes lodged in the diverticu-
the course of the disease. lum.14,15 The diagnosis is confirmed with
colonoscopy. Minimal symptoms are treated
Background Information by placing the patient on a clear liquid diet for
This condition is an infectious disease of the 7 to 10 days. Patients with signs of more signif-
endothelial lining of the heart and/or valves. icant diverticulitis are typically hospitalized
As the pathology progresses clinical symptoms for bowel rest, intravenous fluids, and broad-
will include chest pain, dyspnea, and edema, spectrum intravenous antibiotics.14,15 Elective
which if left untreated will lead to heart fail- surgery is recommended following a recurrent
ure.6 Early diagnosis is confirmed by blood episode because the chance for a third episode
culture and is crucial in order to prevent the is greater than 50%.14
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300 Chapter 18 Duodenal Ulcer

■ Duodenal Ulcer intervention, treatment may include medica-


LUMBAR PAIN

Chief Clinical Characteristics tion and surgical intervention if necessary.19


This presentation involves midline thoracolum- ■ Endometriosis
bar pain with or without abdominal epigastric
pain (see Fig. 18-1). Symptoms are typically Chief Clinical Characteristics
episodic, lasting for weeks or months at a time This presentation may include dysmenorrhea,
before resolution.16 Pain associated with this dyspareunia, and low back pain that worsens
condition is usually absent in the morning, but with menses.20 Rectal pain and painful defeca-
occurs 1 to 3 hours after meals. This symptom tion can also be present.20
is usually relieved by the ingestion of food or Background Information
antacids. This condition is the presence of tissues that
Background Information are histologically similar to endometrium at
There are no specific physical findings but an sites outside the endometrial cavity. Causes
individual with a duodenal ulcer may present include retrograde menstruation and peri-
with epigastric tenderness or fecal occult toneal epithelium transformation to endome-
blood.16 Helicobacter pylori and use of nons- trial tissue, as well as possible genetic causes.20
teroidal anti-inflammatory medications are Pelvic ultrasonography, computed tomography,
two major causes of ulcers.16,17 The diagnosis magnetic resonance imaging, and laparoscopy
is confirmed by endoscopy. Treatment of duo- are commonly used to confirm the diagnosis.20
denal ulcers includes the use of medications to Treatment in cases of women with few symp-
neutralize gastric acids resulting in up to 80% toms can include oral contraceptives or
to 90% ulcer healing.18 Standard medical ther- progestins for pain relief.20 Surgery may be
apy also includes the use of antibiotics de- required in more advanced stages to remove
signed to eradicate H. pylori colonization.18 endometrial lesions.20
Patients who exhibit acute duodenal ulcer per-
foration or other complication may require ■ Herpes Zoster
surgical management. Chief Clinical Characteristics
This presentation may involve an exquisitely
■ Ectopic Pregnancy painful rash or blisters along a specific
Chief Clinical Characteristics dermatomal pattern of the lumbar region
This presentation can be characterized by accompanied by flu-like symptoms.
pelvic pain and abdominal pain with spotting
Background Information
that occurs 6 to 8 weeks following last normal
Individuals with this condition also demon-
menstrual period. Sharp, stabbing low-back
strate a previous history of varicella exposure
pain with possible pain referral to the shoul-
or infection. The virus remains dormant in the
der or neck may be another indication of this
spinal ganglia until its reactivation during a
condition.
period of stress, infection, or physical exhaus-
Background Information tion. Pain associated with this condition may
Other physical findings can include an en- be disproportionate to the extent of skin irri-
larged uterus, a palpable adnexal mass, abdom- tation. The initial presentation of this condi-
inal tenderness with guarding, and rebound tion may be confused with radiculopathy due
tenderness. There are numerous risk factors for to the distribution of symptoms. The presence
this condition, including pelvic inflammatory of the rash, extreme pain, general malaise, and
disease, previous ectopic pregnancy, and en- an unclear association with spinal movement
dometriosis. The reported incidence of this aid in differential diagnosis. Treatment in-
condition is approximately 19.7 cases per 1,000 cludes the administration of antiviral agents as
pregnancies, and it is detected more often in soon as the zoster eruption is noted, ideally
females over 35 years of age.19 The diagnosis is within 48 to 72 hours. If timing is greater
confirmed with abdominal or transvaginal than 3 days, treatment is aimed at controlling
ultrasonography. Although most ectopic preg- pain and pruritus and minimizing the risk of
nancies (68% to 77%) will resolve without secondary infection.21
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Chapter 18 Pleuritis 301

INFARCTIONS due to torsion, tumor invasion, compression,

LUMBAR PAIN
■ Kidney thrombosis, or thromboembolism causes
this condition.24 Infarction of the spleen
Chief Clinical Characteristics frequently occurs in patients with myelo-
This presentation typically involves persistent proliferative disorders, endocarditis, and
low back, abdominal, and flank pain (see sickle cell anemia.24 The diagnosis is con-
Fig. 18-1).22 Accompanying nausea and vom- firmed with ultrasonography. There is a
iting are common as well. high self-healing tendency in acute splenic
Background Information infarction.24
Diagnosis is often delayed or missed due to its
nonspecific clinical presentation.22 Clinical ■ Pancreatitis
diagnosis is typically made based on these Chief Clinical Characteristics
nonspecific clinical findings and patients with This presentation may include central low back
an increased risk for thromboembolism.22 pain occurring with pain in the middle to left
Risk factors for renal infarction include atrial upper abdominal quadrant that is worse after
fibrillation, previous embolism, and valvular eating (see Fig. 18-1).
or ischemic heart disease.22 The diagnosis is
confirmed with contrast-enhanced computed Background Information
tomography. Treatment involves thromboly- Pain associated with pancreatitis also may be
sis, anticoagulation, or embolectomy.22 exacerbated by supine positions and alleviated
by flexing forward or assuming a fetal posi-
tion.6 Nausea and vomiting may also be asso-
■ Spinal Cord/Conus Medullaris ciated with this condition. This condition may
Chief Clinical Characteristics be painless. It commonly is associated with
This presentation can involve neurological diabetes mellitus, weight loss and nutritional
symptoms depending on the artery involved and insufficiencies, anemia, and jaundice. Major
the level of the infarction.23 Initial symptoms causes include chronic alcohol abuse and
can include lower thoracic and lumbar pain. cholelithiasis. The diagnosis is confirmed with
pancreas function lab tests followed by com-
Background Information puted tomography if necessary. Treatment is
Spinal infarction is typically located in the usually supportive and includes fluid resusci-
anterior spinal artery area.23 Neurological tation, oxygen supplementation, and pain
symptoms can include para- or tetraparesis, control.
bladder dysfunction, and pain and tempera-
ture loss below the level of infarction in ■ Pleuritis
cases of anterior spinal artery involve- Chief Clinical Characteristics
ment.23 The diagnosis is confirmed with This presentation can be characterized by bilat-
magnetic resonance imaging. Treatment is eral lower back pain in association with chest
usually palliative, with rehabilitation neces- or bilateral shoulder pain (see Fig. 18-1). It can
sary to recover function and strength after be worsened by deep inspiration.
paralysis.
Background Information
■ Spleen This condition arises from multiple etiolo-
gies. Fever in the presence of chest pain is
Chief Clinical Characteristics
suggestive of active pneumonia. Weight loss
This presentation typically includes a sudden
and malaise in the presence of chest pain
onset of pain in the left upper abdomen, flank,
may be suggestive of a malignancy or tuber-
and shoulder (see Fig. 18-1).24 Pain is typically
culosis. Severe cases also may lead to pul-
sharp and increased by deep respiration or
monary compromise, resulting in a cough or
jarring.
dyspnea.6 Auscultation of the chest wall will
Background Information reveal a pleural friction rub, which is a con-
Common physical findings include fever and stant grating sound during inspiration and
left pleural effusion. Splenic artery occlusion expiration. The diagnosis is confirmed with
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302 Chapter 18 Splenic Abscess

chest plain radiographs or ultrasonography. sigmoidoscopy or colonoscopy. Treatment


LUMBAR PAIN

Treatment is directed toward the underlying depends on the stage of the cancer and may
cause. include surgical resection and colostomy,
chemotherapy, and radiation.6
■ Splenic Abscess
Chief Clinical Characteristics ■ Metastases, Including From
This presentation can include generalized ab- Primary Breast, Kidney, Lung,
dominal pain, left upper quadrant tenderness, Prostate, and Thyroid Disease
and fever.25,26 Other less common symptoms Chief Clinical Characteristics
include left-sided chest pain, left shoulder pain, This presentation can include unremitting
and generalized weakness.26 low back pain due to referral from abdomi-
nal sites of metastases. The four clinical
Background Information findings with the highest likelihood ratios
Pain can also be reported at the base of the left for predicting cancer are a previous history
thorax.26 This condition usually is observed in of cancer, age 50 years or older, failure to
individuals with underlying disorders includ- improve with conservative therapy, and un-
ing infection, emboli, trauma, recent surgery, explained weight loss of more than 10 pounds
malignant hematologic conditions, and im- in 6 months.
munosuppression. The diagnosis is confirmed
with abdominal ultrasound and computed Background Information
tomography. Treatment may involve the use Radiotherapy is the most common treat-
of appropriate antibiotics followed by splenec- ment for metastases, with surgical decom-
tomy in all patients who are reasonable anes- pression and stabilization required if
thetic risks.25 Percutaneous drainage guided by spinal instability or neurological compro-
computed tomography also has gained favor as mise exists28,29 or if the tumor is relatively
a viable first-line treatment option with suc- radioresistant.29
cess rates ranging between 51% and 72%.27
■ Multiple Myeloma
TUMORS Chief Clinical Characteristics
■ Carcinoma of the Colon This presentation typically involves low
Chief Clinical Characteristics back pain, dermatomal sensation loss, and
This presentation includes abdominal pain, possible weakness and numbness of the lower
as well as tenderness and infrequent disten- extremities.30
tion of the lower abdomen. Leakage from a
perforated carcinoma can cause pain in the Background Information
left lumbar and iliac region (see Fig. 18-1).6 Presentation is typically insidious and non-
Most cases of early colon cancer are asymp- specific. Approximately two-thirds of patients
tomatic; however, patients displaying the present with back30–32 and/or rib pain that is
following symptoms may lead the clinician to exacerbated by movement.30 Weakness and
suspect colon cancer: change in bowel habits fatigue are also common.30 Pathological
(diarrhea or constipation), rectal bleeding fracture is the presenting feature in approx-
or blood in the stool, unexplained anemia, un- imately 30% of cases.31 A high index of
explained weight loss, and stools narrower suspicion should be assigned to individuals
than usual. with low back pain who are over 50 years
of age and have pain that is worse in the
Background Information supine position, occurs at night or awakens
Digital rectal examination may reveal a mass patient, is located in a band-like distribution
if the malignancy is in the rectum. Carcino- around the body, is unrelieved with conven-
mas of the colon arise from the lining of the tional methods, and is associated with con-
large intestine as polyps, which over a period stitutional symptoms or progressive neuro-
of several years develop into cancer. Defini- logical deficits.31 Diagnostic tests include
tive diagnosis of colon cancer is made with a serum and urine immunoelectrophoresis.30
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Chapter 18 Epidural Abscess 303

Radiographic findings include classic in the thighs and lower legs due to impingement

LUMBAR PAIN
punched-out lesions of the skull and long of the lumbosacral plexus (see Fig. 18-1). Symp-
bones with diffuse osteopenia and vertebral toms and signs also may include abdominal
compression fractures.30,31 distention, palpable evidence of a pelvic mass,
heavy prolonged menstrual cycles, bladder
■ Ovarian Cysts pressure leading to constant urinary urgency,
Chief Clinical Characteristics and constipation or bloating due to pressure
This presentation includes ipsilateral pain in on the bowels.36
the lumbar and thigh regions and lower quad-
rant of the abdomen, with possible nausea and Background Information
vomiting, abnormal vaginal bleeding, and dif- These are common benign tumors that
ficulty urinating completely (see Fig. 18-1). develop in the muscular walls of the uterus.
First detection usually occurs during routine
Background Information gynecological examination because most are
Ovarian cysts are fluid-filled sacs that develop asymptomatic. Abdominal ultrasound con-
adjacent to the ovary in the fallopian tube. firms the diagnosis. If the fibroids are large
Most cysts are asymptomatic remnants of enough to cause significant symptoms, treat-
normal ovulation.33 Enlarged cysts may lead ment options will include surgical emboliza-
to torsion or distention of the fallopian tube. tion or resection (myomectomy), as well as
The diagnosis is confirmed with clinical pelvic hysterectomy in severe cases.36
examination and pelvic ultrasound. Pre-
scribed hormones may be utilized to shrink ■ Urinary Tract Infection
the cyst. If the cysts persists or symptoms Chief Clinical Characteristics
progress, laparoscopic examination is required This presentation can involve dysuria,
and surgical removal may be indicated.6 frequency, hematuria, and back pain (see
Fig. 18-1). These symptoms significantly in-
■ Retroperitoneal Tumor crease the probability of an individual having
Chief Clinical Characteristics this condition.37
This presentation typically includes abdom-
Background Information
inal pain with radiation into the flank or
Acute cases account for 7 million office visits
lumbar region, groin, and anterior thighs.34
annually in the United States and can
If the tumor extends into the spinal canal,
affect half of all women at least once during
radiating pain and numbness into the lower
their lifetime.37 Three well-established risk
extremities may also be present.35
factors for this condition in young women
Background Information are recent sexual intercourse, use of spermi-
Retroperitoneal masses also may present as cide during sexual intercourse, and previous
cysts, or benign versus malignant tumors. If the history.37 A urine culture is the most com-
tumor is malignant, symptoms may be associ- mon diagnostic tool for all types of this
ated with malaise, weight loss, and night pain. condition. Treatment typically involves an
Retroperitoneal tumors often reach a large size oral antibiotic medication directed against
and may be palpable over the abdominal cavity the infective agent.
or compress other vital structures. The diagno-
sis is confirmed with abdominal computed Local
tomography or magnetic resonance imaging. ABSCESSES
Treatment includes aggressive and complete
laparoscopic removal of the tumor. ■ Epidural Abscess
Chief Clinical Characteristics
■ Uterine Fibroids This presentation typically includes fever and
Chief Clinical Characteristics malaise and is associated with local low back
This presentation may include pelvic pain pain and tenderness, with or without neuro-
and pressure, with possible neurogenic pain logical deficits.38,39
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304 Chapter 18 Paraspinal Muscle Abscess

Background Information ■ Psoas Muscle Abscess


LUMBAR PAIN

Pain is the most consistent symptom and Chief Clinical Characteristics


occurs in virtually all patients presenting This presentation involves fever, lateral lum-
with a spinal epidural abscess. Neurological bar or abdominal pain, and limp. The classi-
deficits may be due to cord and nerve root cal triad of symptoms (ie, fever, back pain, and
compression from the extradural mass limping) is present in approximately 30% of
within the spinal canal.38 Compromise of patients with a psoas abscess.42 Other symp-
the spinal cord vasculature is thought to be toms include malaise, weight loss, or presen-
the cause of neurological compromise tation with a mass. Physical findings include
rather than direct compression. Posterior an externally rotated hip, flank tenderness, and
abscesses originate from a distant area, such fullness in the lateral lumbar region.
as a skin infection, pharyngitis, or dental
abscess.38,40 Anterior epidural abscesses Background Information
are associated with discitis or vertebral os- Psoas abscesses may be primary, due to
teomyelitis.38 Predisposing factors include a hematogenous spread, or secondary due to
compromised immune system as in patients such pathologies as Crohn’s disease, appen-
with diabetes mellitus, acquired immune dicitis, diverticulitis, ulcerative colitis, os-
deficiency syndrome, chronic renal failure, teomyelitis, neoplasm, disk infection, renal
alcoholism, or cancer, or following epidural infections, and trauma.42,43 Computed to-
anesthesia, spinal surgery, or trauma.38 mography of the abdomen with contrast is
Leukocytosis may be the only abnormal lab- the most effective imaging study to confirm
oratory value.38,39 The diagnosis is con- the diagnosis. Treatment usually begins with
firmed with magnetic resonance imaging. a course of antibiotics, with surgical or radi-
Surgical decompression with a follow-on ologically guided percutaneous drainage
course of antibiotic medication comprises essential to effective treatment.42,43
the primary treatment for spinal epidural
abscess. ■ Subdural Abscess
Chief Clinical Characteristics
■ Paraspinal Muscle Abscess This presentation may be characterized by
Chief Clinical Characteristics fever, pain over the involved segment(s),
This presentation can involve severe progres- progressive neurological weakness, and
sive back pain, hip pain, and possible neuro- numbness in the lower extremities. Depend-
logical symptoms following progress of ing on the severity of neural compression,
the infecting mass causing spinal cord fecal and urinary incontinence may be
compromise.41 present.44
Background Information Background Information
Regional lumbar soft tissue swelling, or These symptoms appear in stages with an un-
fluctuance, may be palpated in association predictable rate of progression. The first stage
with increased regional temperature. is fever with or without spinal pain; the sec-
Case reports in the literature of paraspinal ond stage consists of neurological symptoms
abscesses have been shown following lum- such as motor deficits, sensory loss, and
bar injections, such as epidural anesthesia sphincter dysfunction; and the third is paral-
for women in labor.41 This condition is ysis and complete sensory loss.44 This condi-
associated with the possibility of systemic tion results from the spread of a distant or
infection, so urgent referral to a physician local infection, most commonly Staphylococcus
is necessary if this condition is suspected. aureus, following a neurosurgical procedure or
Magnetic resonance imaging has been spinal puncture. Diagnosis is confirmed with
supported as the method of choice for magnetic resonance imaging.3 Due to its
confirming the diagnosis.41 Treatment potentially devastating consequences, this
includes surgically guided drainage and ir- condition is treated with immediate surgical
rigation followed by appropriate antibiotic decompression, drainage, and follow-up with
treatment. the appropriate antibiotics.3
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Chapter 18 Arteriovenous Malformation of Spinal Cord 305

■ Acute Lumbar Sprain/Strain location of the spine that is affected. Pain usu-

LUMBAR PAIN
Chief Clinical Characteristics ally is described as burning and stinging,
This presentation involves pain, muscle spasm, and can be very debilitating in nature. Symp-
edema, and increased temperature of the local toms of this condition also may include bilat-
tissues. Pain may radiate into the buttocks and eral asymmetric radiating lower leg pain and
rarely into the thighs or lower legs. accompanying neurological symptoms such as
numbness and tingling (sciatica).
Background Information
Pain may increase with extension (contrac- Background Information
tion) or flexion (stretch). Sprains are limited to This condition is caused by an inflammation
ligaments, and strains affect muscle-tendon of the arachnoid layer of the central nervous
units. This condition often is caused by exces- system due to trauma during surgery, lumbar
sive physical demands on the back, including punctures during epidural anesthesia, chemi-
repetitive lifting, excessive flexion, extension, cals such as myelographic contrast dye, and in-
or rotation movements with or without fection.46 This inflammation causes constant
load.45 The diagnosis is confirmed with clini- irritation, scarring, and binding of nerve roots
cal examination. Treatment generally involves and blood vessels.3 The disease can progress to
nonsurgical interventions, such as lumbar cause bowel/bladder dysfunctions, sexual dys-
mobilization and manipulation, trunk and function, and spinal cord cysts. The diagnosis
proximal lower extremity exercises, activity is confirmed with computed tomography,
modification, and physical modalities. magnetic resonance imaging, and/or myelo-
grams. There is no cure for this condition.
■ Ankylosing Spondylitis Treatments are often aimed at controlling the
Chief Clinical Characteristics severe pain that results and include transcuta-
This presentation may involve an insidious neous electrical nerve stimulation, steroidal/
onset of low back and symmetric posterior hip nonsteroidal pain medications, antispasmodic
pain associated with a slowly progressive and medications, and spinal cord stimulators.
significant loss of general spinal mobility.
■ Arteriovenous Malformation of
Background Information Spinal Cord
Symptoms may be worse in the morning and Chief Clinical Characteristics
improve with light exercise. This condition is This presentation involves back pain, progres-
more common in males, as well as people of sive paraparesis, paresthesias of the lower extrem-
American indigenous descent, less than 40 years ities, and possible urinary incontinence.
of age, or who carry the human leukocyte anti-
gen B27. It also may be associated with fever, Background Information
malaise, and inflammatory bowel disease. The This condition is divided into dural arteriove-
diagnosis is confirmed with plain radiographs nous fistulas and intradural medullary spinal
of the sacroiliac joints and lumbar spine, which cord angiomas.47 Intradural arteriovenous
reveal characteristic findings of sacroiliitis and malformations are seen in patients less than
“bamboo spine.” Blood panels including ery- 30 years old with acute onset of symptoms,
throcyte sedimentation rate are useful to track subarachnoid hemorrhage, a spinal bruit, and
disease activity. Treatment typically includes a symptoms affecting the arms. Patients with
combination of steroidal, nonsteroidal, and bi- dural arteriovenous malformations are usually
ological anti-inflammatory medications com- greater than 40 years of age, have gradual on-
bined with physical therapy to address postural set and progressive worsening of symptoms,
and movement considerations associated with experience exacerbation of symptoms by a
changing spinal position. change in posture or activity, and the lesions
are always in the lower half of the spinal cord,
■ Arachnoiditis affecting the legs.48 Magnetic resonance imag-
Chief Clinical Characteristics ing is typically used to search for vascular ab-
This presentation typically includes severe normalities of the spinal cord with gadolinium
lower back or thoracic pain, depending on the increasing the sensitivity of the detection of
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306 Chapter 18 Complex Regional Pain Syndrome

arteriovenous malformation.47 Embolization ■ Disk Degeneration


LUMBAR PAIN

using microcatheters has been suggested Chief Clinical Characteristics


as a primary treatment especially for dural This presentation can be characterized by an
fistulas.47,49 individual’s report of low back pain with
possible radiation to the hips, with or without
■ Complex Regional Pain Syndrome
neurological symptoms.
Chief Clinical Characteristics
This presentation may include a traumatic on- Background Information
set of severe chronic lower back pain accompa- Disk degeneration can alter disk height leading
nied by allodynia, hyperalgesia, as well as to symptoms of sciatica or neurological com-
trophic, vasomotor, and sudomotor changes in pression, change the mechanics of the spinal
later stages. column affecting spinal muscles and liga-
ments, and limit lumbar range of motion. The
Background Information incidence of disk degeneration increases
This condition is characterized by dispropor- sharply with age. Failure of the nutrient supply
tionate responses to painful stimuli. It is a re- to the disk, abnormal mechanical loading of
gional neuropathic pain disorder that presents the disks, and genetic factors have been impli-
either without direct nerve trauma (Type I) cated.52 Magnetic resonance imaging confirms
or with direct nerve trauma (Type II) in the diagnosis. Nonsurgical treatment is a com-
any region of the body.50 This condition may mon initial approach. Surgical treatment typi-
precipitate due to an event distant to the af- cally includes discectomy or spinal fusion for
fected area. Thermography may confirm asso- individuals with progressive neurological
ciated sympathetic dysfunction. Treatment symptoms.52
may include physical therapy interventions
to improve patient and client functioning, ■ Disk Disruption (With or Without
biofeedback, analgesic or anti-inflammatory Disk Herniation)
medication, transcutaneous or spinal electrical Chief Clinical Characteristics
nerve stimulation, and surgical or pharmaco- This presentation involves an individual’s reports
logic sympathectomy. of pain deep in the lumbar region with occasional
buttock pain. Usually symptoms are preceded
■ Degenerative Spondylolisthesis by a traumatic event such as lifting a heavy
Chief Clinical Characteristics object or other forceful movement. Pain is
This presentation involves low back pain with aggravated with lumbar rotation, flexion, and
concurrent symptoms of spinal stenosis, involv- side-bending, and sitting tolerance is typically
ing unilateral or bilateral hip or lower diminished.
extremity pain, and chronic nerve root com-
pression. Background Information
Individuals with true internal disk disruption
Background Information lack symptoms of radiculopathy; when
Degenerative spondylolisthesis is the result of present, leg pain follows a nondermatomal
prolonged instability of the spinal motion pattern.53 Magnetic resonance imaging and
segment. Spondylolisthesis can be caused by discography confirm the diagnosis. Nonsurgi-
facet joint dysplasia, elongation or fracture of cal treatment is a common first approach.
the pars interarticularis, degeneration and Surgical treatment typically includes discec-
secondary instability of the facet joints, tomy or spinal fusion for individuals with
trauma, or pathologies including bone tu- progressive neurological symptoms.52
mor, osteogenesis imperfecta, or primary
bone disorders.51 Plain radiographs confirm ■ Epidural Hematoma
the diagnosis. Nonsurgical treatment is a Chief Clinical Characteristics
common first approach. Surgical treatment This presentation may involve the acute onset
typically includes discectomy or spinal fusion of back (or neck) pain followed by rapidly
for individuals with progressive neurological progressive sensory and/or motor deficits. Less
symptoms.52 frequently, this condition is associated with
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Chapter 18 Compression Fracture 307

slowly progressive, chronic or relapsing symp- pain stimuli.57 The etiology of this condition

LUMBAR PAIN
toms or with neurological signs and symptoms is unclear; multiple body systems appear to be
that mimic an acute intervertebral disk involved. Indistinct clinical boundaries be-
prolapse.54 tween this condition and similar conditions
(eg, chronic fatigue syndrome, irritable bowel
Background Information
syndrome, and chronic muscular headaches)
Epidural bleeding could be the result of a rup-
pose a diagnostic challenge.57 This condition
tured epidural vein caused by either a sudden
is diagnosed by exclusion. Treatment will
increase in intra-abdominal pressure or by
often include polypharmacy and elements to
mild trauma. This condition is confirmed with
improve self-efficacy, physical training, and
magnetic resonance imaging. It is a medical
cognitive-behavioral techniques.58
emergency, so individuals suspected of this
condition should be referred to an emergency FRACTURES
department immediately.54
■ Burst Fracture
■ Facet Syndrome Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically involves severe de-
This presentation includes pain in the lumbar bilitating low back pain and, depending on the
region with referral to the buttocks and upper extent of the injury, neurological symptoms,
thigh. The majority of pain referral for the including complete paralysis and loss of bowel
L1–L2 through L4–L5 joints includes the lum- and bladder control.
bar spinal region. Other referral areas include Background Information
the gluteal region (L3–L4 to L5–S1), the Most burst fractures result from high-
lateral upper thigh (L2–L3 to L5–S1), and the energy axial trauma (eg, motor vehicle acci-
posterior thigh (L2–L3 to L5–S1).55 dents and falls), although pathological burst
Background Information fractures could result from bone tumors.59
Causes of facet-mediated pain include sys- Burst fractures are most common in the
temic inflammatory arthritidies, micro- thoracolumbar region of the spine and in-
trauma, and osteoarthritis. The etiology of this volve damage to the anterior and middle
condition may include meniscoid entrapment, columns of the body of a spinal vertebra.
synovial impingement, joint subluxation, This injury is confirmed with plain radi-
chondromalacia facetiae, capsular and syn- ographs of the spine, and is considered a
ovial inflammation, mechanical injury to the medical emergency.
joint capsule, and restriction of normal articu-
lar motion.56 This diagnosis is confirmed by ■ Compression Fracture
controlled diagnostic blocks of the joint or its Chief Clinical Characteristics
nerve supply.56 Nonsurgical treatment is a This presentation of can involve severe debil-
common initial approach to management of itating low back pain, which usually worsens
this condition with walking, as well as other potential seque-
lae of secondary symptoms.60
■ Fibromyalgia
Background Information
Chief Clinical Characteristics
Radiating pain into the leg is highly unlikely
This presentation involves chronic widespread
with vertebral compression fractures.3 This
joint and muscle pain defined as bilateral up-
condition usually is associated with osteo-
per body, lower body, and spine pain, associated
porotic changes within the vertebral body,
with tenderness to palpating 11 of 18 specific
usually in the lower thoracic and upper lum-
muscle-tendon sites.
bar spine. The loss of vertebral height may
Background Information lead to reduction in abdominal space with
Individuals with this condition will demon- associated loss of appetite and secondary
strate lowered mechanical and thermal pain sequelae related to poor nutrition. Most no-
thresholds, high pain ratings for noxious tably, this condition leads to chronic pain
stimuli, and altered temporal summation of with an associated loss of sleep, decreased
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308 Chapter 18 Pars Interarticularis Fracture (Spondylolysis)

mobility, and depression.60 The diagnosis is ■ Meningitis


LUMBAR PAIN

confirmed with plain radiographs of the Chief Clinical Characteristics


spine. Treatment usually includes rest, nar- This presentation may involve high body tem-
cotic analgesics, and spinal orthoses.60 perature, poor feeding, vomiting, lethargy, and
■ Pars Interarticularis Fracture irritability. The infection can also present as a
(Spondylolysis) meningoradiculitis with its initial presentation
as low back pain.64 More classical signs also can
Chief Clinical Characteristics include neck stiffness, headache, and photo-
This presentation may involve low back pain phobia as well as signs of nuchal stiffness.
following severe trauma with possible radia-
tion to the thigh depending on affected level, Background Information
neurological deficits, and cauda equina syn- Progression of this presentation can include
drome.61 Symptoms may begin following an neurological signs such as weakness and loss of
acute injury or gradually after an initiating reflexes.64 This condition occurs when an or-
event. The neurological examination should ganism crosses the blood-brain barrier and be-
be normal. comes isolated from the immune system. It
can occur following lumbar punctures as a di-
Background Information agnostic tool or for spinal anesthesia for pelvic
This condition may lead to spondylolisthe- and intra-abdominal surgery,65 following
sis, which involves local tenderness, swelling, corticosteroid injections, and after diagnostic
and a palpable step-off between L5 and S1. myelograms.65 The diagnosis is confirmed
Radiographic findings can include dislo- with magnetic resonance imaging, blood
cated and locked facets with the inferior serum level, and cerebrospinal fluid culture.
facets of L5 anterior to the S1 facets. This Treatment of meningitis includes antibiotics
condition is considered rare and may occur directed against the causative agent with treat-
following extreme hyperflexion, axial rota- ment time depending on the offending agent
tion, and application of compression forces. as well (10 to 21 days).66
Indications for surgical interventions, in-
cluding decompressive laminectomy and ■ Myofascial Pain (Quadratus
fusion, include progressive segmental insta- Lumborum Syndrome, Piriformis
bility, intractable pain, and development of Syndrome)
neurological deficits.62,63 Chief Clinical Characteristics
This presentation typically includes low back
■ Traumatic Spondylolisthesis pain with reports of a regional, persistent pain
Chief Clinical Characteristics with the presence of one of more trigger points.
This presentation can be characterized by low
back pain following severe trauma with pos- Background Information
sible radiation to the thigh depending on af- Trigger points within the iliocostalis lumbo-
fected level, neurological deficits, and cauda rum, longissimus thoracis, multifidus, quadra-
equina syndrome.61 tus lumborum, gluteus medius, and piriformis
have been implicated. Trigger points typically
Background Information include localized tenderness, referred pain, a
Local tenderness, swelling, and a palpable taut, palpable band in the muscle, and limited
step-off between L5 and S1 are also reported. stretch range of motion.67 Clinical examina-
Radiographic findings for diagnosis can in- tion confirms the diagnosis. Common initial
clude dislocated and locked facets with the nonsurgical interventions include physical
inferior facets of L5 anterior to the S1 facets. therapy to improve patient functioning and
Traumatic spondylolisthesis is considered also injections.
rare and may occur following extreme hy-
perflexion, axial rotation, and application of ■ Osteoporosis
compression forces. Surgical treatment may Chief Clinical Characteristics
be warranted due to the frank instability of This presentation includes midline spinal pain
the injury.61 and deformity over the involved thoracolumbar
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Chapter 18 Reiter’s Syndrome 309

spinal segments. Radiating leg pain or radicu- associated with fatigue, weight loss, fever, and

LUMBAR PAIN
lar symptoms are very uncommon. sweats.
Background Information Background Information
Symptoms relate to fractures of the hip and Pelvic girdle involvement usually causes pain
vertebrae. Vertebral compression fractures re- that radiates to the knee.70 Diagnosis is con-
sult from the collapse of columns within the firmed with blood panels that demonstrate an
vertebral bodies. This condition is a disease of elevated erythrocyte sedimentation rate.70
decreased bone mass density, in which bone This condition is associated with temporal ar-
resorption exceeds bone formation. Dual- teritis, which can cause blindness if untreated,
energy x-ray absorptiometry confirms the di- so urgent referral to a physician is necessary if
agnosis.3 Treatment of osteoporosis is geared this condition is suspected. Treatment of this
toward prevention of future bone loss in condition mainly includes the use of low-dose
younger women. Women postmenopause can oral prednisone in the morning.70,71
be treated with hormone replacement therapy
to slow the rate of bone loss.68 ■ Psoriatic Arthritis
Chief Clinical Characteristics
■ Paget’s Disease This presentation can be characterized by an
Chief Clinical Characteristics insidious onset of spinal pain associated with
This presentation is characterized by deep, psoriasis. Pitting nail lesions occur in 80% of
aching pain that is worse at night and associ- individuals with this condition. Dactylitis,
ated with fatigue, headaches, hearing loss, and tenosynovitis, and peripheral arthritis also are
likely a pronounced kyphosis. common.
Background Information Background Information
Men are affected more than women in a 3:2 The severity of arthritis is uncorrelated with
ratio. Long bone deformities occur and lead to the extent of skin involvement.13 Radiographs
adjacent joint osteoarthrosis. Bones of the of the distal phalanges may reveal a character-
pelvis, lumbar spine, sacrum, thoracic spine, istic “pencil and cup” deformity. Blood panels
and ribs also are commonly affected by defor- including erythrocyte sedimentation rate are
mity. Vertebral collapse may occur in the tho- useful to track disease activity. Psoriatic arthri-
racic region and compression neuropathies tis is treated using patient education, nons-
may be identified. Deformities are a result of teroidal anti-inflammatory medications, and
haphazard resorption of bone that then fills in disease-modifying drugs.13,72
with fibrous tissue. Subsequent attempts to
form bone lead to large, less compact, more ■ Reiter’s Syndrome
vascular bone susceptible to fracture. This Chief Clinical Characteristics
condition is the second-most common skeletal This presentation typically includes pain and
disorder. Its incidence increases by age 50 with stiffness in the low back, sacroiliac, and poste-
peak incidence after 70 years of age. The diag- rior hip regions in the presence of conjunctivi-
nosis of this condition is based on an elevated tis and urethritis.73 Dactylitis may be present.72
serum alkaline phosphatase in patients with Weight loss and fever are common in the acute
x-ray findings of osteosclerosis, osteolysis, and phase as well.13
bone expansion.69 Treatment includes the use
of osteoclast-inhibiting and antiresorptive Background Information
medications.69 This condition is a reactive arthritis that in-
cludes the clinical triad of nongonococcal
■ Polymyalgia Rheumatica urethritis, conjunctivitis, and arthritis.13,72 It
Chief Clinical Characteristics typically begins after an infection of the
This presentation typically includes the acute genitourinary or gastrointestinal tract72 and
onset of proximal myalgias and stiffness involv- usually involves more than one joint, preferen-
ing the neck, shoulders, pelvic girdle, and hips tially affecting the joints of the lower extremi-
that is worse at night and with movement and ties.13 Onset of symptoms is acute with two to
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310 Chapter 18 Rheumatoid Arthritis of the Lumbar Spine

four joints becoming painful within a few antibiotics and external mobilization; surgery
LUMBAR PAIN

days.13 There is usually an asymmetrical in- is rarely needed.76


volvement of the distal interphalangeal joint
and asymmetrical sacroiliitis with skin lesions ■ Spinal Osteomyelitis
frequently seen on the palms and soles of the Chief Clinical Characteristics
feet.72 The diagnosis is confirmed by blood This presentation can include low back pain
panel and radiographs. Symptomatic treat- and tenderness that is not relieved by rest and
ment is used including nonsteroidal anti- tends to be the primary complaint.77–79 Various
inflammatory medications, sulfasalazine, and neurological impairments may be associated
intra-articular corticosteroid injections.13 with this condition, including lower extrem-
ity weakness, bowel/bladder incontinence, or
■ Rheumatoid Arthritis of the even paraplegia. Approximately one-third of
Lumbar Spine patients also may present with fever.78
Chief Clinical Characteristics
Background Information
This presentation involves low back pain that
This condition can be caused by direct exten-
radiates to the hips and lower extremities with
sion of an infection or through hematogenous
positions that involve spinal loading, as well as
seeding from a distant sight of infection.78
possible cauda equina symptoms. Leg pain
Risk factors for osteomyelitis include the use of
(18%) and leg numbness (14%) have been
central venous catheters, immunosuppression,
reported in people with this condition.74
surgery, and placement of a urinary catheter.78
Background Information The diagnosis is confirmed with magnetic res-
This condition affects women twice as often as onance imaging followed by microbiological
men. Symptoms associated with this progres- tests. Treatment generally includes medication
sive inflammatory joint disease are caused by for the particular infecting agent. Some indi-
synovial membrane thickening and cytokine viduals with this condition will undergo com-
production in synovial fluid. Blood tests con- bined medical and surgical treatment, which
firm the diagnosis if rheumatoid factor is de- consists of extensive debridement, bone graft-
tected. Typical radiographic findings include ing, decompression, and stabilization of the
disk space narrowing, facet erosion, and end- vertebral column.77–79
plate erosion.74 Various steroidal, nonsteroidal,
and biological anti-inflammatory medications ■ Spinal Stenosis
have been widely used in the treatment of this Chief Clinical Characteristics
condition.74 This presentation typically involves a prolonged
history of low back pain in older individuals that
■ Septic Discitis is aggravated with standing, walking, or other
Chief Clinical Characteristics positions of lumbar spinal extension and alle-
This presentation may include fever, chills, viated with spinal flexion or no weight bearing.
sweats, and intractable lower back pain with pos- Chronic nerve root compression can lead to
sible radiation to the hip or groin, worsened by radicular pain and sensory, motor, and reflex
movement and not easily alleviated even with changes in one or both lower extremities, most
narcotic analgesia. commonly affecting the L3–L4 and L4–L5
segments.80 Unilateral or bilateral leg pain is
Background Information
reported in up to 90% of cases with a more
From most to least common etiology, discitis
recent onset than low back pain, and walking
may occur following spinal surgery during or
tolerance is often diminished due to neurogenic
after an infection, following spine or skin
claudication.81
punctures, or spontaneously.75 The diagnosis
is confirmed by aspiration of the affected disk Background Information
guided by computed tomography. Early diag- Causes of symptoms involve spinal cord
nosis is critical to prevent infection of the sur- and nerve root impingement due to spinal
rounding tissues. Treatment of septic discitis is canal narrowing. Plain radiographs and mag-
usually successful with specific intravenous netic resonance imaging confirm the diagnosis.
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Chapter 18 Metastases, Including from Primary Disease 311

Indications for surgery have not been clearly ■ Tuberculosis of the Spine

LUMBAR PAIN
defined and serve as elective procedures to (Pott’s Disease)
improve quality of life in persons with dis- Chief Clinical Characteristics
abling low back pain.81 Therefore, nonsurgical This presentation typically includes insidious
options such as physical therapy to improve onset of stiffness with pain over the involved
patient functioning and injections are the vertebrae radiating into the buttock or lower
initial treatments of choice. extremity, low-grade fever, chills, weight loss, and
■ Tethered Spinal Cord nonspecific constitutional symptoms of varying
duration.87 Weakness, nerve root compression,
Chief Clinical Characteristics and sensory involvement can be present to vary-
This presentation typically includes back pain, ing degrees.
associated with neurological deficits, and bowel
and bladder dysfunction.82 The most common Background Information
manifestations of this condition are reduced This condition usually results from spread of
motor function of the lower extremities (and up- pulmonary or other primary infection involv-
per extremities, although less likely), changes in ing Mycobacterium tuberculosis. If the disease
muscle tone and deep tendon reflexes, progres- affects one vertebral body, the intervertebral
sive loss of articular dexterity, progressively disk may be spared. However, collapse of the
worsening scoliosis or kyphosis, and back or affected segment could occur due to impaired
leg pain.83 disk nutrition. Magnetic resonance imaging
confirms the diagnosis. Treatment consists of
Background Information antituberculosis medications, which are effec-
This condition occurs commonly in children, tive 90% of the time, with surgery in more
but can present in undiagnosed adults as well. advanced cases.88
Magnetic resonance imaging confirms the di-
agnosis, with a low-lying (caudally positioned) TUMORS
conus medullaris present. Surgical resection ■ Intraspinal Lipoma
of a thickened filum terminale is a common
treatment. Chief Clinical Characteristics
This presentation includes lumbosacral pain
■ Transverse Myelitis and skin stigmata.89 Symptoms depend on
Chief Clinical Characteristics the location of the lipoma and proximity to
This presentation typically includes low back neurological tissue; this condition may be
pain associated with acute or subacute motor, associated with spinal cord tethering and
sensory, and autonomic neurological deficits.84,85 progressive neurological symptoms.
At maximal level of deficits (within 4 hours to Background Information
21 days), approximately 50% of individuals If the lipoma is impinging on neurological
with this condition lose all movements of their tissue, sphincter disturbance and inconti-
legs, virtually all have bladder dysfunction, and nence will occur.89 This condition is charac-
80% to 94% have numbness, paresthesias, or terized by slow-growing, fatty cells.89 This
band-like dysesthesias.84 condition often is associated with spina
Background Information bifida. Computed tomography and magnetic
This condition is associated with various viral resonance imaging confirm the diagnosis.
and bacterial infections as well as systemic au- Treatment consists of surgical exploration
toimmune diseases. The etiology of this condi- and resection of the tumor.
tion is unclear. Magnetic resonance imaging
combined with lumbar puncture confirms ■ Metastases, Including from
the diagnosis. Treatment includes the use of Primary Breast, Kidney, Lung,
multiple medications typically directed against Prostate, and Thyroid Disease
an autoimmune response even when this Chief Clinical Characteristics
condition cannot be attributed to a particular This presentation involves unremitting
etiology.86 pain in individuals with these risk factors:
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312 Chapter 18 Osteoblastoma

previous history of cancer, age 50 years or therapy, and the presence of anorexia, malaise,
LUMBAR PAIN

older, failure to improve with conservative or night pain.92


therapy, and unexplained weight change of
Background Information
more than 10 pounds in 6 months.90
Diagnosis of a malignant bone tumor may
Background Information be delayed due to the similarity in clinical
Cauda equina symptoms or nerve root com- presentation to other musculoskeletal disor-
pression of the spinal canal can be caused by ders. Definitive diagnosis is made using
vertebral collapse or infiltration of the tu- appropriate imaging, blood tests (elevated
mor.28 The skeletal system is the third most alkaline phosphatase), and tumor biopsy.
common site of metastatic disease; lumbar Depending on the location and stage, treat-
metastases account for 20% of cases.29 ment of this condition may include surgical
Symptoms also may be related to pathologi- resection, radiation therapy, or chemotherapy.
cal fracture in affected sites. Common pri-
mary sites causing metastases to bone in- ■ Spinal Cord Tumor
clude breast, prostate, lung, and kidney. Chief Clinical Characteristics
Bone scan confirms the diagnosis. Common This presentation may involve low back pain,
treatments for metastases include surgical sciatica, saddle and perianal hypesthesia or
resection, chemotherapy, radiation treat- analgesia, decreased rectal tone, absent patel-
ment, and palliation, depending on the lar and Achilles reflexes, bowel and bladder dys-
tumor type and extent of metastasis. function, and lower extremity weakness.
Other symptoms may include unexplained
■ Osteoblastoma weight loss, spontaneous onset of symptoms,
Chief Clinical Characteristics failure to improve with prior medical care, and
This presentation can involve an insidious duration of symptoms longer than 1 month.93
onset of deep and aching pain, typically A long history of back pain and paresthesias
in males under 30 years of age. Pain as well as occasional urinary difficulties is
associated with this condition is not usu- common in patients with this condition.94
ally worse at night. This condition may
Background Information
rarely affect the pelvic bones, and the prox-
Primary tumors common to the lumbar
imal femoral epiphysis is even less com-
spine include myxopapillary ependymomas,
monly affected.
schwannomas, paragangliomas, astrocy-
Background Information tomas, and chordomas.94 Common metas-
This condition involves abnormal produc- tases to the lumbar spine occur in lung,
tion of osteoid and primitive bone, although breast, renal cell, and colorectal carcinomas
its specific etiology remains unclear. Biopsy and lymphoma.93,94 Magnetic resonance im-
and plain radiographs confirm the diagno- aging confirms the diagnosis.93 Primary
sis. Computed tomography is necessary to treatment includes surgical excision with
define the tumor margins if surgical resec- possible radiotherapy and/or chemother-
tion is considered. apy.95 Treatment of spinal cord tumors
causing cauda equina includes immediate
■ Primary Bone Tumor surgical decompression.94
(eg, Osteosarcomas, Ewing’s
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nosis and treatment of primary bone sarcoma of the 95. Guerrero D. The management of primary spinal cord
pelvis. J Bone Joint Surg Am. 1999;81(3):317–325. tumours. Nurs Times. Oct 21–27 2003;99(42):28–31.
1528_Ch19_316-352 07/05/12 1:52 PM Page 316

CHAPTER 19
Hip Pain
■ Kyle F. Baldwin, PT, DPT ■ Todd E. Davenport, PT, DPT, OCS
■ Michael A. Andersen, PT, DPT, OCS

Description of the Symptom Special Concerns


■ Acute inability to bear weight through the
This chapter describes pathology that may lead affected lower extremity or pelvis second-
to hip pain, including anterior, buttock, inguinal/ ary to pain
medial thigh, and lateral pain. Local causes of ■ Decreased pulses in the lower extremities
hip pain are defined as pathology occurring ■ Skin break and fever
within the intra- and extra-articular structures ■ Sudden loss of motor or sensory function
of the hip joint. Remote causes are defined associated with trauma
as originating from sites external to this ■ Warmth and swelling in the legs associated
region. with a fever

CHAPTER PREVIEW: Conditions That May Lead to Hip Pain

T Trauma
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Acute lumbar sprain/strain 324 Hernias:
Disk disruption (with or without herniation) 326 • Femoral hernia 340
Facet syndrome 327 • Inguinal hernia 340
Lumbar compression fracture 328 Labral tear 343
Lumbar radiculopathies: Muscle strains:
• L1–L3 radiculopathy 328 • Adductor strain 343
• L4 radiculopathy 328 • Iliopsoas strain 343
• L5 radiculopathy 330
• S1 radiculopathy 330
UNCOMMON
Not applicable Fractures:
• Intracapsular femur fracture 339
• Stress fracture of the femoral neck 339
Hernias:
• Sports hernia 340
Nerve entrapments/neuropathy:
• Femoral neuropathy 344

316
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HIP PAIN
Buttock
Anterior Lateral
hip and
thigh
Anterior Anterior
hip and Medial hip and
thigh thigh/ thigh
inguinal
Lateral region Medial

LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Iliotibial band friction Fractures: Labral tear 343


syndrome 343 • Avulsion fractures: Muscle strains:
Trochanteric contusion (hip • Hamstrings off ischial • Iliopsoas strain 343
pointer) 350 tuberosity 338 • Quadriceps muscle strain 344
Gluteal contusion 339
Muscle strains:
• Hamstring strain 343
Sacroiliac joint dysfunction 349

Iliac crest apophysitis 342 Bursitis: Fractures:


Nerve entrapments/neuropathy: • Ischiogluteal bursitis 336 • Extracapsular femur
• Meralgia paresthetica 345 Nerve entrapments/neuropathy: fracture 338
• Piriformis syndrome 345 • Femoral shaft fracture 339
Hernias:
• Sports hernia 340

(continued)

317
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318 Chapter 19 Hip Pain

Trauma (continued)
HIP PAIN

LOCAL INGUINAL/
REMOTE MEDIAL THIGH
UNCOMMON
• Genitofemoral nerve entrapment 344
• Iliohypogastric nerve entrapment 344
• Ilioinguinal nerve entrapment 345
• Obturator nerve entrapment 345
Sacroiliac joint dysfunction 349
RARE
Traumatic spondylolisthesis 335 Fractures:
• Acetabular fracture 337
• Avulsion fractures:
• Hamstrings off ischial tuberosity 338
• Rectus femoris off anterior inferior iliac
spine 338
• Sartorius off anterior superior iliac spine 338
Hip dislocation 341

I Inflammation
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Aseptic Not applicable
Not applicable
Septic
Appendicitis 326

UNCOMMON
Aseptic Aseptic
Rheumatoid arthritis–like diseases of the Complex regional pain syndrome 337
lumbar spine: Iliopsoas tendinitis 342
• Scleroderma 333 Osteitis pubis 345
• Systemic lupus erythematosus 333 Reiter’s syndrome 347
Rheumatoid arthritis of the hip 348
Septic Rheumatoid arthritis–like diseases:
Brucellosis of the lumbar spine 326 • Scleroderma 348
Pelvic inflammatory disease 331 • Systemic lupus erythematosus 348
Prostatitis 331
Renal or urinary tract infection 332 Septic
Septic arthritis of the sacroiliac joint 334 Herpes zoster 340
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Chapter 19 Hip Pain 319

HIP PAIN
LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Nerve entrapments/
neuropathy:
• Femoral neuropathy 344
• Meralgia paresthetica 345

Hip dislocation 341 Hip dislocation 341 Hip dislocation 341

LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Aseptic Aseptic Not applicable


Bursitis: Fibromyalgia 337
• Ischiogluteal bursitis 336
• Trochanteric bursitis 336 Septic
Fibromyalgia 337 Not applicable

Septic
Not applicable

Aseptic Aseptic Aseptic


Complex regional pain Ankylosing spondylitis 336 Complex regional pain
syndrome 337 Complex regional pain syndrome 337
Polymyalgia rheumatica 347 syndrome 337 Iliopsoas tendinitis 342
Reiter’s syndrome 347 Polymyalgia rheumatica 347 Rheumatoid arthritis of the
Rheumatoid arthritis of the hip 348
hip 348 Septic Rheumatoid arthritis–like
Rheumatoid arthritis–like Herpes zoster 340 diseases:
diseases: • Scleroderma 348
• Scleroderma 348 • Systemic lupus
• Systemic lupus erythematosus 348
erythematosus 348
Septic
Septic Herpes zoster 340
Herpes zoster 340
(continued)
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320 Chapter 19 Hip Pain

Inflammation (continued)
HIP PAIN

LOCAL INGUINAL/
REMOTE MEDIAL THIGH
RARE
Aseptic Aseptic
Reiter’s syndrome of the lumbar spine 331 Rheumatoid arthritis–like diseases:
Rheumatoid arthritis of the lumbar spine 332 • Inflammatory muscle diseases 348
Rheumatoid arthritis–like diseases of the spine: • Psoriatic arthritis 348
• Inflammatory bowel disease 332
• Inflammatory muscle diseases 333 Septic
• Psoriatic arthritis 333 Brucellosis of the hip 336
Iliopsoas abscess 342
Septic Septic arthritis of the hip 349
Psoas muscle abscess 331 Systemic fungal infection 349
Retrocecal appendicitis 332 Tuberculosis of the hip 350
Retroperitoneal abscess 332
Septic discitis 334
Systemic fungal infection of the lumbar spine 335
Tuberculosis of the lumbar spine (Pott’s disease) 335

M Metabolic
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Not applicable Not applicable
UNCOMMON
Endometriosis 327 Heterotopic ossification 341
Myositis ossificans 344
Osteomalacia 346
Transient osteoporosis of the hip 350
RARE
Not applicable Gout 339
Pseudogout 347

Va Vascular
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Aortic artery aneurysm 325 Avascular necrosis of the hip 336
Aortic or iliac arteriosclerosis 325 Greater saphenous vein thrombophlebitis 340
Epidural hematoma 327 Iliac artery aneurysm 341
Iliofemoral venous thrombosis 342
Sickle cell crisis 349
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Chapter 19 Hip Pain 321

HIP PAIN
LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Aseptic Aseptic Aseptic


Herpes zoster 340 Reiter’s syndrome 347 Gout 339
Rheumatoid arthritis–like Rheumatoid arthritis–like Pseudogout 347
diseases: diseases: Reiter’s syndrome 347
• Inflammatory muscle • Inflammatory muscle Rheumatoid arthritis–like
diseases 348 diseases 348 diseases:
• Psoriatic arthritis 348 • Psoriatic arthritis 348 • Inflammatory muscle
diseases 348
Septic Septic • Psoriatic arthritis 348
Cellulitis 337 Brucellosis of the hip 336
Systemic fungal infection 349 Cellulitis 337 Septic
Systemic fungal infection 349 Brucellosis of the hip 336
Tuberculosis of the hip 350 Iliopsoas abscess 342
Tuberculosis of the hip 350

LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Not applicable Not applicable Not applicable

Not applicable Transient osteoporosis of Transient osteoporosis of the


the hip 350 hip 350

Not applicable Not applicable Not applicable

LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Iliac artery aneurysm 341

(continued)
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322 Chapter 19 Hip Pain


HIP PAIN

De Degenerative
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Degenerative spondylolisthesis 326 Hip osteoarthrosis/
Disk degeneration 326 osteoarthritis 341
Spinal stenosis 334
Spondylolysis 334
UNCOMMON
Not applicable Iliopsoas tendinosis 342
RARE
Not applicable Not applicable

Tu Tumor
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Malignant Primary: Not applicable
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Ovarian cysts 331
• Uterine fibroids 335
UNCOMMON
Not applicable Malignant Primary, such as:
• Primary bone tumor of the acetabulum or
proximal femur:
• Ewing’s sarcoma 347
• Osteosarcoma 347
Malignant Metastatic, such as:
• Metastases, including from primary breast,
kidney, lung, prostate, and thyroid disease 343
Benign:
Not applicable

RARE
Malignant Primary, such as: Malignant Primary, such as:
• Spinal cord tumor 334 • Primary bone tumor of the acetabulum or
Malignant Metastatic, such as: proximal femur:
• Leukemia 327 • Ewing’s sarcoma 347
Benign, such as: • Osteosarcoma 347
• Osteoblastoma of the spine 331 Malignant Metastatic:
Not applicable
Benign, such as:
• Osteoblastoma of the hip 346
• Osteochondroma 346
• Osteoid osteoma 346
• Pigmented villonodular synovitis 346
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Chapter 19 Hip Pain 323

HIP PAIN
LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Hip osteoarthrosis/ Hip osteoarthrosis/ Not applicable


osteoarthritis 341 osteoarthritis 341

Not applicable Not applicable Iliopsoas tendinosis 342

Not applicable Not applicable Not applicable

LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Not applicable Not applicable Not applicable

Malignant Primary, such as: Malignant Primary, such as: Malignant Primary, such as:
• Primary bone tumor of the • Primary bone tumor of the • Primary bone tumor of the
acetabulum or proximal femur: acetabulum or proximal femur: acetabulum or proximal femur:
• Ewing’s sarcoma 347 • Ewing’s sarcoma 347 • Ewing’s sarcoma 347
• Osteosarcoma 347 • Osteosarcoma 347 • Osteosarcoma 347
Malignant Metastatic, such as: Malignant Metastatic, such as: Malignant Metastatic, such as:
• Metastases, including from • Metastases, including from • Metastases, including from
primary breast, kidney, lung, primary breast, kidney, lung, primary breast, kidney, lung,
prostate, and thyroid disease 343 prostate, and thyroid disease 343 prostate, and thyroid disease 343
Benign: Benign: Benign:
Not applicable Not applicable Not applicable

Malignant Primary, such as: Not applicable Malignant Primary, such as:
• Primary bone tumor of the • Primary bone tumor of the
acetabulum or proximal femur: acetabulum or proximal femur:
• Ewing’s sarcoma 347 • Ewing’s sarcoma 347
• Osteosarcoma 347 • Osteosarcoma 347
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign: Benign, such as:
Not applicable • Pigmented villonodular
synovitis 346

(continued)
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324 Chapter 19 Acute Lumbar Sprain/Strain


HIP PAIN

Co Congenital
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Ne Neurogenic/Psychogenic
LOCAL INGUINAL/
REMOTE MEDIAL THIGH
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Hip Pain Description of Conditions That


May Lead to Hip Pain
The diagnosis of hip pain is complex because
precise symptom localization often is confus- Remote
ing for the patient and clinician. Patients may ■ Acute Lumbar Sprain/Strain
associate their hip within the region extend-
ing anywhere between the lateral midlumbar Chief Clinical Characteristics
spine and lateral thigh. Pain referral patterns This presentation may involve pain, muscle
also may serve as diagnostic pitfalls in the hip spasm, edema, and increased temperature with
region. Lumbar, sacroiliac, and hip causes radiation into the buttocks and rarely into
of pain may be difficult to differentiate, par- the thighs or lower legs. Pain may increase
ticularly in individuals with long-standing with contraction or stretching of affected
pain. In addition, primary hip pathology musculature.
may present as inguinal, medial thigh, or me- Background Information
dial knee pain. A careful history should elicit Sprains and strains involve ligamentous and
the patient’s own anatomical definition of the musculotendinous damage, respectively. This
hip region in order to clarify the location condition often results from excessive physical
of pain, due to the importance of this infor- demands on the low back, such as repetitive
mation to accurate diagnosis in the hip lifting, excessive flexion, extension, or rotation
region. movements with or without load.1 Treatment
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Chapter 19 Aortic or Iliac Arteriosclerosis 325

HIP PAIN
LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL ANTERIOR
LOCAL LATERAL LOCAL BUTTOCK HIP/THIGH

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

typically includes lumbar mobilization/ nodosum. This condition occurs when the
manipulation, activity modification, exercise tunica intima, tunica media, and tunica adven-
for the trunk and proximal lower extremities, titia become structurally compromised due to
and physical modalities. various acquired factors (eg, hypertension, cig-
arette smoking) and congenital factors (eg,
■ Aortic Artery Aneurysm
Marfan’s syndrome). Most often, this condi-
Chief Clinical Characteristics tion is asymptomatic prior to rupture. The
This presentation typically includes vague abdominal aorta is most commonly involved
abdominal pain, usually located in the epigas- due to its morphology. Elective open surgical
trium, that may radiate to the low back, flank, repair may be considered when the risk of
or groin. Other symptoms include early satiety, rupture exceeds that of surgery.2
nausea, vomiting, gastrointestinal bleeding,
and lower extremity ischemia.2 The problem ■ Aortic or Iliac Arteriosclerosis
arises in that 66% to 75% of abdominal aortic Chief Clinical Characteristics
aneurysms are asymptomatic.2 This presentation may be characterized by gen-
Background Information eralized low back and hip pain, and symptoms
Aneurysms can be diagnosed during a routine consistent with lumbar disk degeneration.3
physical examination by palpation of a pul- Background Information
satile abdominal mass above the umbilicus, Studies have correlated the occurrence of aor-
which may be associated with erythema tic arteriosclerosis and disk degeneration.3,4
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326 Chapter 19 Appendicitis

The first to fourth lumbar arteries leave the ■ Degenerative Spondylolisthesis


HIP PAIN

aorta in front of the corresponding vertebral Chief Clinical Characteristics


body supplying that vertebra and the adjacent This presentation may involve low back pain
intervertebral disk via diffusion through the with concurrent symptoms of spinal stenosis,
vertebral end plate.3 Findings suggest that cal- involving unilateral or bilateral hip or lower
cific lesions in the upper part of the abdominal extremity pain, and chronic nerve root
aorta predict disk deterioration at any lumbar compression.
level.3 The link between arteriosclerosis and
low back/hip pain can be explained by the Background Information
common risk factors between the two patholo- Degenerative spondylolisthesis is the result of
gies, including age and smoking. The diagno- prolonged instability of the spinal motion seg-
sis is confirmed with radiographs or computed ment. Spondylolisthesis can be caused by facet
tomography. Surgical techniques for treatment joint dysplasia, elongation or fracture of the
of occlusive disease of the artery include pars interarticularis, degeneration and second-
endovascular stented-graft treatment.5 ary instability of the facet joints, trauma, or
pathologies including bone tumor, osteogene-
■ Appendicitis sis imperfecta, or primary bone disorders.7
Chief Clinical Characteristics Plain radiographs confirm the diagnosis. Non-
This presentation typically includes pain in the surgical intervention may be considered for
right lower abdominal and lumbar regions with individuals without progressive neurological
local right iliac and/or lumbar direct tenderness. deficits. Surgical treatment typically includes
Reports of vague periumbilical pain followed by discectomy or spinal fusion for individuals
anorexia, nausea, and vomiting are common. with neurological symptoms.8
Other physical findings include tachycardia, ■ Disk Degeneration
low-grade fever, hypoactive bowel sounds, and
tenderness in the right lower quadrant at Chief Clinical Characteristics
McBurney’s point.6 This presentation typically includes reports of
low back pain with possible radiation to the
Background Information hips, with or without neurological symptoms.
This condition involves septic inflammation of
the appendix, involving obstruction of the ap- Background Information
pendix lumen. The end result of this disease The incidence of disk degeneration increases
process is rupture of contents into the abdominal sharply with age. Disk degeneration can alter
cavity and the corresponding potential for sys- disk height leading to symptoms of sciatica or
temic infection. Therefore, individuals suspected neurological compression, change the mechan-
of this condition should be referred to an emer- ics of the spinal column affecting spinal muscles
gency department for immediate treatment. and ligaments, and limit lumbar range of
motion. Failure of the nutrient supply to the
■ Brucellosis of the Lumbar Spine disk, abnormal mechanical loading of the disks,
Chief Clinical Characteristics and genetic factors have been implicated.8 Plain
This presentation can be characterized by a radiographs and magnetic resonance imaging
gradual onset of nonspecific myalgias, fever, confirm the diagnosis. Surgical treatment typi-
malaise, and generalized lumbopelvic, hip, or cally includes discectomy or spinal fusion for
sacroiliac joint pain. individuals with neurological symptoms.8

Background Information ■ Disk Disruption (With or Without


This uncommon condition in the United Disk Herniation)
States results from ingestion of bacteria that Chief Clinical Characteristics
are found in nonpasteurized milk or other in- This presentation involves an individual’s reports
fected animal products. It is associated with of pain deep in the lumbar region with occasional
sacroiliac joint effusions that produce some buttock pain. Usually symptoms are preceded
arthritic changes over time if left untreated. by a traumatic event such as lifting a heavy
Treatment includes antibiotics. object or other forceful movement. Pain is
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Chapter 19 Leukemia 327

aggravated with lumbar rotation, flexion, and mild trauma.11 Magnetic resonance imaging

HIP PAIN
side-bending, and sitting tolerance is typically confirms the diagnosis. An epidural hematoma
diminished. is a medical emergency that requires surgical
management.
Background Information
Individuals with true internal disk disruption ■ Facet Syndrome
lack symptoms of radiculopathy; when present, Chief Clinical Characteristics
leg pain follows a nondermatomal pattern.9 This presentation includes pain in the lumbar
Magnetic resonance imaging and discography region with referral to the buttocks and upper
confirm the diagnosis. Nonsurgical treatment thigh. The majority of pain referral for the
is a common first approach. Surgical treatment L1–L2 through L4–L5 joints includes the lum-
typically includes discectomy or spinal fusion bar spinal region. Other referral areas include
for individuals with progressive neurological the gluteal region (L3–L4 to L5–S1), the lat-
symptoms.8 eral upper thigh (L2–L3 to L5–S1), and the pos-
terior thigh (L2–L3 to L5–S1).12
■ Endometriosis
Chief Clinical Characteristics Background Information
This presentation may include dysmenorrhea, Causes of facet-mediated pain include systemic
dyspareunia, and low back pain that worsens inflammatory arthritides, microtrauma, and
with menses.10 Rectal pain and painful defeca- osteoarthritis. The etiology of this condition
tion can also be present.10 may include meniscoid entrapment, synovial
impingement, joint subluxation, chondromala-
Background Information cia facetiae, capsular and synovial inflamma-
This condition is the presence of tissues that tion, mechanical injury to the joint capsule,
are histologically similar to endometrium at and restriction of normal articular motion.13
sites outside the endometrial cavity. Causes in- This diagnosis is confirmed by controlled diag-
clude retrograde menstruation and peritoneal nostic blocks of the joint or its nerve supply.13
epithelium transformation to endometrial Nonsurgical treatment is a common initial
tissue, as well as possible genetic causes.10 approach to management of this condition.
Pelvic ultrasonography, computed tomogra-
phy, magnetic resonance imaging, and la- ■ Leukemia
paroscopy are commonly used to confirm the Chief Clinical Characteristics
diagnosis.10 Treatment in cases of women with This presentation typically includes lumbopelvic
few symptoms can include oral contraceptives and hip pain, malaise, fatigue, excessive bruising
or progestins for pain relief.10 Surgery may be or bleeding, night sweats, and weight loss. Acute
required in more advanced stages to remove disorders have a more rapid onset with illness.14
endometrial lesions.10
Background Information
■ Epidural Hematoma Leukemic cells will accumulate in the bone mar-
row and replace the normal hematopoietic cells,
Chief Clinical Characteristics
which may then infiltrate any other organ. Acute
This presentation typically includes the acute
myelogenous leukemias constitute 85% of adult
onset of back (or neck) pain with possible refer-
acute leukemias. Chronic lymphocytic leukemia
ral to the hip, followed by rapidly progressive
is responsible for 30% of all leukemias in
sensory and/or motor deficits. Less frequently,
Western countries and the median age of onset
patients may present with slowly progressive,
in the seventh decade, whereas acute lym-
chronic or relapsing symptoms or with neuro-
phoblastic leukemia is responsible for 80% of
logical signs and symptoms that mimic an acute
childhood cases.15 Lower back pain may result
intervertebral disk prolapse.
from reactive arthritis, as well as metastatic dis-
Background Information ease. The diagnosis is confirmed with the pres-
Epidural bleeding could be the result of a rup- ence of disabling bone pain, night pain, hemato-
tured epidural vein caused by either a sudden logic findings in the blood tests, leukopenia, or
increase in intra-abdominal pressure or by positive findings in a bone marrow biopsy.16–18
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328 Chapter 19 Lumbar Compression Fracture

■ Lumbar Compression Fracture radiating from the anterior aspect of the hip,
HIP PAIN

Chief Clinical Characteristics thigh, and knee, sometimes extending an-


This presentation can involve severe debilitat- teromedially from the knee to the foot. De-
ing low back pain that is usually worse with pending on the severity, the presentation
walking, as well as other potential sequelae of may also include a decreased or absent patel-
secondary symptoms.19 lar tendon reflex and motor loss in the mus-
cles innervated by the L4 nerve. The prone
Background Information knee bend test may reproduce symptoms.
Radiating pain into the leg is highly unlikely
with vertebral compression fractures.20 This Background Information
condition usually is associated with osteo- A lumbar disk herniation is the most com-
porotic changes within the vertebral body; mon cause for this condition. The diagnosis
usually in the lower thoracic and upper lum- is confirmed with magnetic resonance imag-
bar spine. The loss of vertebral height may ing. Surgical intervention may be indicated
lead to reduction in abdominal space with as- in severe cases of lower extremity pain
sociated loss of appetite and secondary seque- accompanied by neurological signs.
lae related to poor nutrition. Most notably,
this condition leads to chronic pain with an
associated loss of sleep, decreased mobility,
and depression.19 The diagnosis is confirmed
with plain radiographs of the spine. Treat-
ment usually includes rest, narcotic analgesics,
and spinal orthoses.19
L1
LUMBAR RADICULOPATHIES
(Fig. 19-1A to E) L2
■ L1–L3 Radiculopathy
L3
Chief Clinical Characteristics
This presentation typically includes pain in the L4
lumbar spine and paresthesias in the antero-
L5
medial aspect of the hip and knee. Depend-
ing on severity, the presentation may also in-
Motor
clude a decreased or absent patellar tendon S1
reflex and motor loss in the muscles innervated
by the L2 or L3 nerve. The prone knee bend
test may reproduce symptoms.
Background Information
A lumbar disk herniation is the most com-
mon cause for this condition; however, com-
pression of the L2 and L3 nerve roots is
relatively uncommon. The diagnosis is con-
firmed with magnetic resonance imaging.
Surgical intervention may be indicated in
severe cases of lower extremity pain accompa-
nied by neurological signs.
Anterior Posterior
■ L4 Radiculopathy A Sensory
Chief Clinical Characteristics FIGURE 19-1 Lumbar radiculopathy creates charac-
This presentation typically includes pain teristic reflex, motor, and sensory findings including
in the lumbar spine and paresthesias (A) L1–L2 radiculopathy;
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Chapter 19 L4 Radiculopathy 329

HIP PAIN
Reflex

L1

L2
L1

L2 L3

Reflex L4
L3

L4 L5
S1
L5
S1

Motor Motor

Posterior Anterior Posterior


B Sensory C Sensory
FIGURE 19-1 cont’d (B) L3 radiculopathy; (C) L4 radiculopathy;
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330 Chapter 19 L5 Radiculopathy


HIP PAIN

L1 L1

L2 L2

L3 L3

L4 Reflex
L4 Reflex

L5 L5
S1 S1

Motor Motor

Anterior Posterior Anterior Posterior


D Sensory E Sensory
FIGURE 19-1 cont’d (D) L5 radiculopathy; and (E) S1 radiculopathy.

■ L5 Radiculopathy ■ S1 Radiculopathy
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation typically includes pain in the This presentation typically includes pain in the
lumbar spine and paresthesias radiating from lumbar spine and paresthesias radiating from
the lateral aspect of the hip and buttock to the the buttock to the posterior aspect of the knee
lateral aspect of the knee, extending antero- and extending posterolaterally from the knee
laterally down to the foot. Depending on the to the foot. Depending on the severity, the
severity, the presentation may also include presentation may also include a decreased or
motor loss in the muscles innervated by the absent Achilles tendon reflex and motor loss
L5 nerve root. in the muscles innervated by the S1 nerve.
Background Information Background Information
A lumbar disk herniation is the most com- A lumbar disk herniation is a common cause
mon cause for this condition. The diagnosis for this condition. The diagnosis is con-
is confirmed with magnetic resonance imag- firmed with magnetic resonance imaging.
ing. Surgical intervention may be indicated Surgical intervention may be indicated in
in severe cases of lower extremity pain severe cases of lower extremity pain accom-
accompanied by neurological signs. panied by neurological signs.
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Chapter 19 Reiter’s Syndrome of the Lumbar Spine 331

■ Osteoblastoma of the Spine diagnosis. Treatment typically includes an

HIP PAIN
Chief Clinical Characteristics aggressive course of antibiotic medication.
This presentation typically includes deep and ■ Prostatitis
aching pain of insidious onset that is not worse
at night, most commonly in males under Chief Clinical Characteristics
30 years of age. Approximately 40% of these This presentation involves pain in the central
tumors are found in the spine, so a number of lower back, hip, rectal, or perineal region asso-
these lesions may refer pain into the hip region ciated with fever, chills, and painful urination.
depending on their exact location around the Other initial symptoms may include malaise,
spinal column. arthralgia, and myalgia. As this condition
progresses, prostatic inflammation produces
Background Information dysuria, frequency, urgency, and urine retention.
Biopsy is necessary to determine the nature of
the tumor. Surgical excision is required, partic- Background Information
ularly in cases that involve neurological signs. Prostatitis is usually caused by a bacterial infec-
tion of the prostate gland, commonly associ-
■ Ovarian Cysts ated with urinary tract infection, urethritis, or
Chief Clinical Characteristics epididymitis.22 Blood and urine tests confirm
This presentation includes ipsilateral pain in the the diagnosis. Treatment for this condition
lumbar and thigh region, and the lower quad- includes intravenous antibiotics, which are
rant of the abdomen, with possible nausea and specific to the infecting organism.
vomiting, abnormal vaginal bleeding, and dif- ■ Psoas Muscle Abscess
ficulty urinating completely.
Chief Clinical Characteristics
Background Information This presentation involves fever, lateral lumbar
Ovarian cysts are fluid-filled sacs that develop or abdominal pain, and limp. The classical triad
adjacent to the ovary in the fallopian tube. of symptoms (ie, fever, back pain, and limp-
Most cysts are asymptomatic remnants of ing) is present in approximately 30% of pa-
normal ovulation.21 Enlarged cysts may lead to tients with a psoas abscess.24 Other symptoms
torsion or distention of the fallopian tube. The include malaise, weight loss, or presentation
diagnosis is confirmed with clinical examina- with a mass. Physical findings include an exter-
tion and pelvic ultrasonography. Prescribed nally rotated hip, flank tenderness, and full-
hormones may be utilized to shrink the cyst. If ness in the lateral lumbar region.
the cysts persist or symptoms progress, laparo-
scopic examination is required and surgical Background Information
removal may be indicated.22 Psoas abscesses may be primary, due to
hematogenous spread, or secondary due to such
■ Pelvic Inflammatory Disease pathologies as Crohn’s disease, appendicitis,
diverticulitis, ulcerative colitis, osteomyelitis,
Chief Clinical Characteristics
neoplasm, disk infection, renal infections, and
This presentation may involve lumbosacral and
trauma.24,25 Computed tomography of the
hip pain associated with dull, constant, and
abdomen with contrast is the most effective
poorly localized abdominal pain, abnormal
imaging study to confirm the diagnosis. Treat-
vaginal discharge, fever, painful intercourse,
ment usually begins with a course of antibi-
and irregular menstrual bleeding.
otics, with surgical or radiologically guided per-
Background Information cutaneous drainage essential to effective
Pelvic inflammatory disease is most prevalent treatment.24,25
in women between 15 and 23 years of age.
Gonorrhea and chlamydia infections of the ■ Reiter’s Syndrome of the Lumbar
upper genital tract are common causes. This Spine
condition may lead to infertility, ectopic Chief Clinical Characteristics
pregnancy, and chronic pelvic pain if left This presentation can include pain and stiffness
untreated.23 Blood and urine tests confirm the in the low back, sacroiliac, and posterior hip
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332 Chapter 19 Renal or Urinary Tract Infection

regions in the presence of conjunctivitis and ■ Retroperitoneal Abscess


HIP PAIN

urethritis.26 These patients will have asymmet- Chief Clinical Characteristics


ric distribution of pain between the hips and will This presentation can include constant groin and
most certainly have some form of spinal discom- low back pain associated with a fever. The patient
fort associated with this condition. may choose to rest with the hip in 20 to 30 degrees
Background Information of flexion to slacken the psoas muscle. Individu-
This condition also has been called a reactive als with this condition also may present with
arthritis because it may present after other infec- quadriceps weakness and decreased knee jerk sec-
tions. Clinical examination confirms the diag- ondary to compression on the femoral nerve as it
nosis. Symptomatic treatment is used including courses through the iliopsoas muscle.
nonsteroidal anti-inflammatory medications, Background Information
sulfasalazine, and intra-articular corticosteroid Magnetic resonance imaging or abdominal com-
injections.27 puted tomography confirms the diagnosis. Sur-
■ Renal or Urinary Tract Infection gical management involving percutaneous
drainage may be indicated, and individuals sus-
Chief Clinical Characteristics pected of having this condition should be re-
This presentation may involve pain in the cen- ferred for emergent medical attention due to the
tral lower back, flank, and groin, associated high risk for mortality related to systemic infec-
with fever, nausea, and vomiting. Other com- tion in individuals with a retroperitoneal abscess.
mon symptoms include urgent, frequent, and
painful urination, abdominal bloating, and ■ Rheumatoid Arthritis of the
pain upon palpation of the kidney.22 Renal or Lumbar Spine
urinary tract disease may follow streptococcal Chief Clinical Characteristics
infection of the upper respiratory tract or skin This presentation may involve low back pain that
within 8 to 21 days.28 radiates to the hips and lower extremities
Background Information with positions that involve spinal loading, as
Blood and urine tests confirm the diagnosis. well as possible cauda equina symptoms. Leg
Treatment typically includes an appropriate pain (18%) and leg numbness (14%) have been
antibiotic medication and possible hospital- reported in people with this condition.29
ization for monitoring. Background Information
■ Retrocecal Appendicitis This condition affects women twice as often as
men. Symptoms associated with this progres-
Chief Clinical Characteristics sive inflammatory joint disease are caused by
This presentation can be characterized by groin, synovial membrane thickening and cytokine
anterior thigh, or lower back pain, associated production in synovial fluid. Blood tests con-
with fever, nausea, and vomiting. Only mild firm the diagnosis if rheumatoid factor is
abdominal symptoms are usually present with detected. Typical radiographic findings in-
this condition because of the retrocecal orien- clude disk space narrowing, facet erosion, and
tation of the infected appendix. end-plate erosion.29 A variety of steroidal,
Background Information nonsteroidal, and biological anti-inflamma-
Abdominal computed tomography confirms tory medications have been widely used in the
the diagnosis. This condition involves septic treatment of this condition.29
inflammation of the appendix, involving RHEUMATOID ARTHRITIS–LIKE
obstruction of the appendix lumen. The end DISEASES OF THE LUMBAR SPINE
result of this disease process is rupture of con-
tents into the abdominal cavity and the corre- ■ Inflammatory Bowel Disease
sponding potential for systemic infection. Chief Clinical Characteristics
Therefore, individuals suspected of this condi- This presentation may involve abdominal
tion should be referred to an emergency pain, bowel obstruction, weight loss, fever,
department for immediate treatment. chronic or nocturnal diarrhea, night sweats,
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Chapter 19 Systemic Lupus Erythematosus 333

and rectal bleeding, as well as asymmetric Dactylitis, tenosynovitis, and peripheral

HIP PAIN
and migratory arthralgia/arthritis that may arthritis also are common.
affect the hip. Crampy, intermittent abdom-
Background Information
inal right lower quadrant pain is common in
Radiographs of the distal phalanges may
patients with ileal disease and is exacerbated
reveal a characteristic “pencil in cup” defor-
by eating.30
mity. Blood panels including erythrocyte
Background Information sedimentation rate are useful to track disease
Inflammatory bowel disease refers to Crohn’s activity. Management usually includes use of
disease and ulcerative colitis.31 The etiology salicylates, corticosteroids, antibiotics, and
of these conditions is unclear, but immune immunosuppressants depending on the sever-
system abnormalities seem to cause inflam- ity of the pathology.30
mation of the small intestine. Colonoscopy
confirms the diagnosis. Management usually ■ Scleroderma
includes use of salicylates, corticosteroids, Chief Clinical Characteristics
antibiotics, and immunosuppressants de- This presentation typically includes myal-
pending on the severity of the pathology.30 gia, arthralgia, fatigue, weight loss, limited
mobility, and hardened skin about the hands,
■ Inflammatory Muscle Diseases knees, or elbows. This condition occurs in in-
Chief Clinical Characteristics dividuals between 25 and 55 years of age,
This presentation includes a gradual onset and is four to five times more likely in women
of mild muscle pain associated with proxi- than men. Additional symptoms may include
mal muscle weakness that causes difficulty dry mouth and eyes, as well as Raynaud’s
with daily activities such as walking, ascend- phenomenon.
ing and descending stairs, and rising from Background Information
chairs. This rare and progressive autoimmune disor-
Background Information der affects blood vessels and many internal
This condition describes a group of patho- organs, including the lungs and the gastroin-
logically, histologically, and clinically distinct testinal system. Overproduction of collagen in
disorders: polymyositis, dermatomyositis, this condition eventually leads to poor blood
and inclusion body myositis. They may be flow in the extremities, which can cause ulcers
associated with other collagen, vascular, and in the fingers, changes in skin color, and a dis-
immune disorders. Although proximal ex- appearance of creases in the skin. Continued
tremity weakness is a classic finding, up to damage to small-diameter vasculature leads
50% also demonstrate distal weakness that to scar tissue production that impairs joint
may be equally as severe.32 Blood panels help range of motion. Blood tests confirm the
confirm the diagnosis and track disease ac- diagnosis. Treatment includes both steroidal
tivity, revealing elevated serum levels of cre- and nonsteroidal anti-inflammatory medica-
atine phosphokinase. Management usually tions and gentle exercise.
includes use of salicylates, corticosteroids,
■ Systemic Lupus Erythematosus
antibiotics, and immunosuppressants de-
pending on the severity of the pathology.30 Chief Clinical Characteristics
This presentation may involve low back pain
■ Psoriatic Arthritis radiating to the hip and groin pain, associated
with fatigue and joint pain/swelling affecting
Chief Clinical Characteristics
the hands, feet, knees, and shoulders.
This presentation can be characterized by an
insidious onset of lumbopelvic and hip pain Background Information
associated with psoriasis. The severity of This condition affects mostly women of
arthritis is uncorrelated with the extent of childbearing age. It is a chronic autoimmune
skin involvement.27 Pitting nail lesions occur disorder that can affect any organ system, in-
in 80% of individuals with this condition. cluding skin, joints, kidneys, brain, heart,
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334 Chapter 19 Septic Arthritis of the Sacroiliac Joint

lungs, and blood. The diagnosis is confirmed that may involve the hip region. Symptoms may
HIP PAIN

by the presence of skin lesions; heart, lung, include slowly progressive paresis, sensory
or kidney involvement; and laboratory ab- deficits, and difficulty walking.
normalities including low red or white cell
Background Information
counts, low platelet counts, or positive ANA
This condition is rare. However, spinal paragan-
and anti-DNA antibody tests.33
gliomas demonstrate a particular affinity for
■ Septic Arthritis of the Sacroiliac the cauda equina and filum terminale, so they
Joint may cause sciatic symptoms.36 Biopsy and mag-
netic resonance imaging confirm the diagnosis.
Chief Clinical Characteristics
Primary treatment of spinal cord neoplasms
This presentation can include an insidious
includes surgical excision with possible radio-
onset of buttock, hip, and groin pain with weight
therapy and/or chemotherapy.37 Treatment of
bearing and movement, accompanied by
spinal cord tumors causing cauda equina in-
possible fever and malaise. Overlying tissue is
cludes immediate surgical decompression.38
usually swollen and inflamed, and this may
extend to the anterior and lateral thigh. ■ Spinal Stenosis
Background Information Chief Clinical Characteristics
Septic arthritis of the sacroiliac joint occurs by This presentation typically involves a prolonged
way of bacterial, yeast, fungal, or viral infec- history of low back pain, typically in older in-
tion of the hip joint. The infection also can dividuals, that is aggravated with standing,
damage articular cartilage, leading to os- walking, or other positions of lumbar spinal
teoarthritis later in life. Culture of aspirated extension and alleviated with spinal flexion or
synovial fluid confirms the diagnosis and di- no weight bearing. Chronic nerve root com-
rects medical management, which consists of a pression can lead to radicular pain and sen-
regimen of antibiotic medication directed to sory, motor, and reflex changes in one or both
the infective agent. lower extremities, most commonly affecting the
L3–L4 and L4–L5 segments.39 Unilateral or
■ Septic Discitis bilateral leg pain is reported in up to 90% of cases
Chief Clinical Characteristics with a more recent onset than low back pain,
This presentation may include fever, chills, and walking tolerance is often diminished due
sweats, and intractable lower back pain with pos- to neurogenic claudication.40
sible radiation to the hip or groin, worsened by
Background Information
movement and not easily alleviated even with
Causes of symptoms involve spinal cord and
narcotic analgesia.
nerve root impingement due to spinal canal
Background Information narrowing. Plain radiographs and magnetic
From most to least common etiology, discitis resonance imaging confirm the diagnosis.
may occur following spinal surgery during or Indications for surgery have not been clearly
after an infection, following spine or skin defined and serve as elective procedures to
punctures, or spontaneously.34 The diagnosis improve quality of life in persons with dis-
is confirmed by aspiration of the affected disk abling low back pain.40 Therefore, nonsurgical
guided by computed tomography. Early diag- options such as physical therapy to improve
nosis is critical to prevent infection of the sur- patient functioning and injections are the
rounding tissues. Treatment of septic discitis is initial treatments of choice.
usually successful with specific intravenous
antibiotics and external mobilization; surgery ■ Spondylolysis
is rarely needed.35 Chief Clinical Characteristics
This presentation typically includes low back
■ Spinal Cord Tumor pain with possible radiation to the buttock
Chief Clinical Characteristics or proximal lower extremity that is worsened
This presentation typically includes a long his- with positions and activities involving spinal
tory of insidious back and lower extremity pain extension and rotation. Symptoms may begin
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Chapter 19 Uterine Fibroids 335

following an acute injury or gradually prior to Radiographic findings for diagnosis can in-

HIP PAIN
an initiating event. The neurological exami- clude dislocated and locked facets with the in-
nation should be normal. ferior facets of L5 anterior to the S1 facets.
Traumatic spondylolisthesis is considered rare
Background Information
and may occur following extreme hyperflex-
This condition is a defect in the pars interartic-
ion, axial rotation, and compression forces.
ularis due to repetitive loading. It is most com-
Surgical treatment may be warranted due to
mon in young athletes.41 This condition can
the frank instability of the injury.44
progress to spondylolisthesis, particularly be-
fore skeletal maturity.41 Plain radiographs ■ Tuberculosis of the Lumbar Spine
confirm the diagnosis; computed tomography (Pott’s Disease)
and magnetic resonance imaging demonstrate
Chief Clinical Characteristics
higher diagnostic accuracy. Indications for
This presentation involves insidious onset of
surgical interventions, such as decompressive
stiffness with pain over the involved vertebrae
laminectomy and fusion, include progressive
radiating into the buttock or lower extremity,
segmental instability, intractable pain, and de-
low-grade fever, chills, weight loss, and non-
velopment of neurological deficits.41,42
specific constitutional symptoms of varying
■ Systemic Fungal Infection of the duration.45 Weakness, nerve root compression,
Lumbar Spine and sensory involvement can be present to
varying degrees.
Chief Clinical Characteristics
This presentation can be characterized by lum- Background Information
bopelvic and hip pain associated with signs of This condition usually results from the spread
systemic infection, such as fever, anorexia, and of a pulmonary or other primary infection in-
malaise. Individuals with this condition may volving Mycobacterium tuberculosis. If the
present after a prolonged and varied course of disease affects one vertebral body, the interver-
antibiotics to address their symptoms. tebral disk may be spared. However, collapse of
the affected segment could occur due to
Background Information
impaired disk nutrition. Magnetic resonance
This condition is most common in immune-
imaging confirms the diagnosis. Treatment
compromised individuals, such as patients in
consists of antituberculosis medications,
intensive care, patients receiving chemotherapy,
which are effective 90% of the time, or surgery
patients with acquired immunodeficiency syn-
in more advanced cases.46
drome, and transplant recipients. This condi-
tion is most commonly due to Candida species ■ Uterine Fibroids
infection, although Aspergillus also may be a
Chief Clinical Characteristics
culprit organism. The infection is transferred
This presentation typically includes pelvic pain
from health care worker to patient by way of
and pressure, with possible neurogenic pain in
hand contact. Serum pathology and molecular
the thighs and lower legs due to impingement
techniques confirm the diagnosis and direct ap-
of the lumbosacral plexus. Symptoms and signs
propriate pharmacologic management.43
also may include abdominal distention, palpa-
■ Traumatic Spondylolisthesis ble evidence of a pelvic mass, heavy prolonged
menstrual cycles, bladder pressure leading to
Chief Clinical Characteristics constant urinary urgency, and constipation or
This presentation can be characterized by low back bloating due to pressure on the bowels.47
pain following severe trauma with possible
radiation to the thigh depending on affected Background Information
level, neurological deficits, and cauda equina These are common benign tumors that de-
syndrome.44 velop in the muscular walls of the uterus. First
detection usually occurs during routine gyne-
Background Information cological examination since most are asymp-
Local tenderness, swelling, and a palpable tomatic. Abdominal ultrasound confirms the
step-off between L5 and S1 are reported. diagnosis. If the fibroids are large enough to
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336 Chapter 19 Ankylosing Spondylitis

cause significant symptoms, treatment options considered if subchondral bone loss is exten-
HIP PAIN

will include surgical embolization or resection sive and the articular collapse is significant.
(myomectomy), as well as hysterectomy in
severe cases.47 ■ Brucellosis of the Hip
Chief Clinical Characteristics
Local This presentation can be characterized by a
■ Ankylosing Spondylitis gradual onset of nonspecific myalgias, fever,
malaise, and generalized hip or sacroiliac joint
Chief Clinical Characteristics pain.
This presentation may involve an insidious on-
set of low back and symmetric posterior hip Background Information
pain associated with a slowly progressive and This uncommon condition in the United
significant loss of general spinal mobility. States results from ingestion of bacteria that is
found in nonpasteurized milk or other in-
Background Information fected animal products. It is associated with
Symptoms may be worse in the morning and sacroiliac joint effusions that produce some
improve with light exercise. This condition is arthritic changes over time if left untreated.
more common in males, as well as people of Treatment includes antibiotics.
American indigenous descent, less than 40 years
of age, or who carry the human leukocyte anti- BURSITIS
gen B27.51 It also may be associated with fever,
■ Ischiogluteal Bursitis
malaise, and inflammatory bowel disease. The
diagnosis is confirmed with plain radiographs Chief Clinical Characteristics
of the sacroiliac joints and lumbar spine, which This presentation typically includes traumatic
reveal characteristic findings of sacroiliitis and or insidious onset of pain over the ischial
“bamboo spine.” Blood panels including ery- tuberosity with possible radiation to the pos-
throcyte sedimentation rate are useful to track terior thigh, worsened with palpation, active
disease activity. Treatment typically includes a hip extension, and sitting. This uncommon
combination of steroidal, nonsteroidal, and condition may be worsened with hip motion,
biological anti-inflammatory medications com- so the hip joint is sometimes erroneously
bined with physical therapy to address postural implicated as the source of symptoms.
and movement considerations associated with Background Information
changing spinal position. Ischiogluteal bursitis can result from a direct
■ Avascular Necrosis of the Hip injury to the bursa or from sitting for
prolonged periods on hard surfaces. Inflam-
Chief Clinical Characteristics mation of the bursa between the ischial
This presentation typically includes insidious tuberosity and proximal hamstrings causes
onset of groin and anterolateral thigh pain. this condition. Clinical examination con-
Background Information firms the diagnosis with additional certainty
This condition may be associated with sickle provided by magnetic resonance imaging.
cell anemia, femoral head or neck trauma, This condition is treated nonsurgically.
Gaucher’s disease, alcoholism, Caisson disease,
prolonged steroid use, irradiation, or preg- ■ Trochanteric Bursitis
nancy.48 It is characterized by articular collapse Chief Clinical Characteristics
of subchondral bone due to a lack of blood This presentation typically involves an insid-
supply. The femoral head is most likely to be ious onset of lateral hip, thigh, and buttock pain
involved. Up to 20% of individuals with this that is worsened with palpation, weight-bearing
condition have bilateral disease. Plain radi- activities, laying on the involved side, and
ographs confirm the diagnosis, but early detec- active/resisted hip abduction. Individuals
tion is difficult. Surgical decompression is typ- with this condition may note improvement ini-
ically performed to facilitate revascularization tially with walking, but return as they continue
of the femoral head. Hemiarthroplasty may be to walk. Symptoms from this condition may
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Chapter 19 Acetabular Fracture 337

radiate as far distally as the knee, and may be ■ Fibromyalgia

HIP PAIN
associated with crepitus over the greater Chief Clinical Characteristics
trochanter with hip motion. This presentation involves chronic widespread
Background Information joint and muscle pain defined as bilateral up-
Clinical examination confirms the diagnosis. per body, lower body, and spine pain, associated
This condition is treated nonsurgically. with tenderness to palpating 11 of 18 specific
muscle-tendon sites.
■ Cellulitis
Background Information
Chief Clinical Characteristics
Individuals with this condition will demon-
This presentation typically includes lateral hip
strate lowered mechanical and thermal pain
and thigh pain, associated with pain during
thresholds, high pain ratings for noxious stim-
activities involving compression of the superfi-
uli, and altered temporal summation of pain
cial tissues, as well as redness and warmth of the
stimuli.51 The etiology of this condition is un-
affected region.
clear; multiple body systems appear to be in-
Background Information volved. Indistinct clinical boundaries between
Individuals with this condition may have a low- this condition and similar conditions (eg,
grade fever, and complete blood count may chronic fatigue syndrome, irritable bowel syn-
show an elevated white blood cell count. Indi- drome, and chronic muscular headaches) pose
viduals following hip surgery are at particular a diagnostic challenge.51 This condition is
risk of developing this condition. Symptoms diagnosed by exclusion. Treatment will often
related to this condition may cause increasing include polypharmacy and elements to im-
difficulty with muscle activation about the prove self-efficacy, physical training, and cog-
hip, resulting in limited functional activities.49 nitive-behavioral techniques.52
Clinical examination confirms the diagnosis.
Treatment involves oral antibiotics and obser- FRACTURES (Fig. 19-2)
vation to ensure that the underlying infection
■ Acetabular Fracture
does not spread to adjacent tissues.
Chief Clinical Characteristics
■ Complex Regional Pain Syndrome This presentation can be characterized by
Chief Clinical Characteristics constant pain in the groin and thigh region
This presentation may include a traumatic with an acute inability to weight bear through
onset of severe chronic lower back pain accom- the pelvis or affected limb.
panied by allodynia and hyperalgesia, as well Background Information
as trophic, vasomotor, and sudomotor changes This condition results from acute, high-
in later stages. energy trauma involving axial loading of the
Background Information femur. The direction and magnitude of the
This condition is characterized by dispropor- force and position of the femoral head at im-
tionate responses to painful stimuli. It is a re- pact determine the pattern of injury. Ure-
gional neuropathic pain disorder that presents thral, uterine, and vaginal ruptures should
either without direct nerve trauma (Type I) or be considered, although bladder injury and
with direct nerve trauma (Type II) in any region clinically significant pelvic hemorrhage are
of the body.50 This condition may precipitate not routinely observed in individuals with
due to an event distant to the affected area. acetabular fracture unless a concomitant
Thermography may confirm associated sympa- pelvic ring injury is present. Concomitant
thetic dysfunction. Treatment may include phys- pelvic ring and extremity fractures are com-
ical therapy interventions to improve patient mon. Plain radiographs confirm the diagno-
and client functioning, biofeedback, analgesic or sis. The treatments for these fractures are ini-
anti-inflammatory medication, transcutaneous tially directed at stabilization of fracture
or spinal electrical nerve stimulation, and surgi- segments, eventually leading to restoration
cal or pharmacologic sympathectomy. of normal movement.53
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338 Chapter 19 Hamstrings Off Ischial Tuberosity

Extracapsular Intracapsular Avulsion


HIP PAIN

A B C D E F
FIGURE 19-2 Fractures of the proximal femur and pelvis include (A) intertrochanteric fracture; (B) subtrochanteric
fracture; (C) femoral head fracture; (D) femoral neck fracture; (E) avulsion of sartorius or rectis femoris off the
anterior superior iliac spine; and (F) avulsion of hamstrings off the ischial tuberosity.

AVULSION FRACTURES ■ Sartorius Off Anterior Superior


■ Hamstrings Off Ischial Iliac Spine
Tuberosity Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may include groin and
This presentation can involve a traumatic proximal medial thigh pain, swelling, and
onset of pain in the buttock with possible tenderness following acute trauma.
radiation to the posterior thigh. Pain is wors- Background Information
ened with sitting and resisted hip extension. These fractures are more common in
Background Information younger individuals participating in high-
This condition is more common in younger speed activities due to the relative strength of
individuals, resulting from a sudden applica- tendons with respect to their attachment
tion of great muscular force to the ischial sites in this age group. Radiographs confirm
apophysis. The diagnosis is confirmed with the diagnosis, usually revealing minimal dis-
plain radiographs that also are used to assess placement of fracture fragments due to the
the displacement of fracture segments. strength of the surrounding periosteum. Sta-
ble injuries may be managed nonoperatively,
■ Rectus Femoris Off Anterior although surgical treatment may be indi-
Inferior Iliac Spine cated to repair the avulsed segments if there
Chief Clinical Characteristics is significant displacement.
This presentation involves groin and proximal
medial thigh pain, swelling, and tenderness ■ Extracapsular Femur Fracture
following acute trauma. Chief Clinical Characteristics
This presentation can be characterized by a
Background Information
traumatic onset of constant anterior, anterior
These fractures are more common in
medial, or anterior lateral thigh region pain
younger individuals participating in high-
associated with an acute inability to bear
speed activities due to the relative strength of
weight or move the affected limb. Extracap-
tendons with respect to their attachment
sular fractures include intertrochanteric and
sites in this age group. Radiographs confirm
femoral shaft fractures.
the diagnosis, usually revealing minimal dis-
placement of fracture fragments due to the Background Information
strength of the surrounding periosteum. Sta- This condition is often associated with
ble injuries may be managed nonoperatively, bleeding into the soft tissues surrounding the
although surgical treatment may be indi- fracture site. This blood accumulation is a
cated to repair the avulsed segments if there potential source of infection.54 Individuals
is significant displacement. suspected of having this condition should
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Chapter 19 Gout 339

receive a clinical neurovascular examination, usually are present over an extended period

HIP PAIN
because the associated swelling may cause of time. Early stress fractures may cause only
compartment syndrome. Plain radiographs a minimal loss of hip range of motion and
confirm the diagnosis. Surgical stabilization limping often is absent.
of fracture segments may be necessary.
Background Information
■ Femoral Shaft Fracture Diagnostic delay may lead to fracture dis-
placement. Therefore, individuals who report
Chief Clinical Characteristics
acute changes in training intensity and vol-
This presentation typically includes a trau-
ume or pathology that weakens bone should
matic onset of severe circumferential pain
be suspected of this condition. Bone scan and
and swelling about the femur over the fracture
plain radiographs confirm the diagnosis.
site. Characteristic symptoms include dull
This condition is managed nonsurgically.
pain at rest, worsening with bearing weight,
active motion, and passive motion. Hip and ■ Gluteal Contusion
knee range of motion also may be limited as
a protective response. Chief Clinical Characteristics
This presentation includes a traumatic onset of
Background Information ecchymosis and pain that is worsened with sit-
Plain radiographs confirm the diagnosis. ting, resisted hip extension, and activities that
Immediate treatment for this condition in- engage the gluteal musculature.
cludes immobilization of the extremity by a
fracture brace or cast. Background Information
Individuals competing in sports and working
■ Intracapsular Femur Fracture industrial jobs are at elevated risk. This condi-
Chief Clinical Characteristics tion occurs when capillaries in the affected
This presentation may involve constant pain musculature rupture due to blunt trauma.
in the hip region following acute trauma. Pain Clinical examination confirms the diagnosis.
is worsened with weight bearing or hip range Treatment for this condition is usually nonsur-
of motion in any direction (see Fig. 19-2). gical, involving relative rest, protective
The femur is often positioned in excessive in- padding or bracing, gentle exercise, and physi-
ternal or external rotation. There is also short- cal modalities.
ening of the limb.
■ Gout
Background Information
Chief Clinical Characteristics
These fractures are usually associated with
This presentation involves a sudden onset of
high-energy forces in normal bone (eg, a fall
deep stabbing pain in the hip joint. Pain is wors-
from height) or low-energy forces in patho-
ened with weight bearing and is associated with
logical bone (eg, osteoporosis). Displaced
tenderness, warmth, and erythema of the super-
fractures may disturb the nearby neurovas-
ficial tissues.
cular bundle; patients suspected of this
condition should be screened for distal neu- Background Information
rological signs. Surgical treatment is aimed Increased prevalence is associated with ad-
at anatomical reduction and ensuring stabil- vanced age, male sex, and alcohol consumption
ity of the fracture segment, as well as the on a regular basis. This condition is less com-
decompression of the nerve if necessary. mon in the hip. It is caused by a metabolic defect
that results in overproduction of uric acid or a
■ Stress Fracture of the Femoral reduced ability to eliminate uric acid, allowing
Neck urate crystal deposition in affected joints. Blood
Chief Clinical Characteristics tests or microscopic examination of aspirated
This presentation typically includes an insid- synovial fluid confirms the diagnosis. Treatment
ious onset of deep groin pain that may radi- usually involves anti-inflammatory medication
ate to the knee. Symptoms are worsened with and diet changes to address this condition’s un-
standing, walking, running, and at night; they derlying metabolic dysfunction.
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340 Chapter 19 Greater Saphenous Vein Thrombophlebitis

■ Greater Saphenous Vein extend into the scrotum in males. This


HIP PAIN

Thrombophlebitis condition is a herniation of a portion of the


Chief Clinical Characteristics intestines through a weakening in the ab-
This presentation may be characterized by pain dominal wall at the site of the inguinal canal.
in the medial thigh along the course of the Risk factors for developing this disorder in-
greater saphenous vein, which also may be as- clude a parent or sibling with a hernia,
sociated with palpation of a tender cord-like chronic cough, and developmental dysplasia
band along its length. Generally, the range of mo- of the hip. Treatment involves surgical repair
tion of the hip joint or knee is not affected. of the contributing abdominal wall defect.
Background Information ■ Sports Hernia
The etiology of this condition is associated Chief Clinical Characteristics
with Virchow’s triad of intimal damage, This presentation can involve an insidious on-
stasis, or changes in blood composition.55 set of deep chronic groin pain that radiates
Genetic predisposition also may cause elevated along the inguinal ligament, perineum, rectus
risk. Computed tomography, magnetic reso- abdominis, and adductors. Symptoms are
nance angiography, and Doppler ultrasonog- worsened with endurance running, kicking,
raphy confirm the diagnosis. This condition is coughing, sneezing, and the Valsalva maneuver.56
a medical emergency because the underlying Physical findings include tenderness along
thrombus may propagate proximally and the external ring and posterior wall of the in-
cause pulmonary embolism. guinal canal, as well as spasm of the surround-
ing musculature. Testicular pain may be pres-
HERNIAS
ent in males, making testicular pathology an
■ Femoral Hernia important differential diagnosis.
Chief Clinical Characteristics Background Information
This presentation includes groin pain associ- This condition is caused by weakness of the
ated with a grape-sized lump, frequent con- posterior inguinal wall. The diagnosis is con-
stipation, and discomfort with rising to a firmed with herniography due to the absence
standing position, coughing, and pushing of classic palpation findings for hernia.
heavy loads. Treatment involves surgical repair of the
Background Information contributing abdominal wall defect.
This condition is more common in women
than men. It is characterized by escape of the ■ Herpes Zoster
large bowel through a weakened portion of Chief Clinical Characteristics
abdominal wall at the femoral canal. Dis- This presentation typically includes an exquis-
comfort also may be exacerbated with end itely painful rash or blisters along a specific der-
range motion of the hip into full flexion or matomal pattern accompanied by flu-like
extension. For this reason, patients may re- symptoms. Individuals with this condition also
late their discomfort to a musculoskeletal demonstrate a previous history of varicella
problem. Treatment involves surgical repair exposure or infection. Pain associated with this
of the contributing abdominal wall defect. condition may be disproportionate to the extent
of skin irritation. The initial presentation of
■ Inguinal Hernia this condition may be confused with radicu-
Chief Clinical Characteristics lopathy due to the distribution of symptoms. The
This presentation typically may involve groin presence of the rash, extreme pain, general
pain with a reducible lump, possibly wors- malaise, and an unclear association with spinal
ened by activities that involve sprinting and movement aids in differential diagnosis.
direction change.
Background Information
Background Information The virus remains dormant in the spinal gan-
Inguinal hernias are more common in men glia until its reactivation during a period of
than women, and this herniation can also stress, infection, or physical exhaustion.
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Chapter 19 Iliac Artery Aneurysm 341

Treatment includes the administration of an- affected limb’s shortened appearance is due to

HIP PAIN
tiviral agents as soon as the zoster eruption is the proximal migration of the dislocated
noted, ideally within 48 to 72 hours. If timing femoral head. Individuals suspected of this
is greater than 3 days, treatment is aimed at condition should be screened for neurovascu-
controlling pain and pruritus and minimiz- lar involvement, because the dislocated
ing the risk of secondary infection.57 femoral head often compresses the sciatic
nerve. This condition is a medical emergency.
■ Heterotopic Ossification
Chief Clinical Characteristics ■ Hip Osteoarthrosis/Osteoarthritis
This presentation can be characterized by groin Chief Clinical Characteristics
and lateral hip pain with active and passive This presentation typically includes medial, an-
movement, associated with decreased hip range terior, or lateral hip pain that may refer to the
of motion, reduced flexibility of the associated anteromedial thigh, characterized by increased
musculature, and progressively firmer palpable pain at end ranges of passive hip abduction and
mass. extension, and limping. This condition is the
most common cause of hip pain in people older
Background Information
than 50 years of age.59 It may be related to a prior
The palpable mass associated with this condi-
injury or begin insidiously over the course of
tion gradually becomes less tender and
many months or years.
smaller, but its density increases. It develops as
a result of direct soft tissue trauma (eg, blunt Background Information
force or total hip arthroplasty) or in associa- This condition involves degeneration of the
tion with various central nervous system dis- articular cartilage of the femoral head and ac-
orders (eg, traumatic brain injury, spinal cord etabulum. Radiographs confirm the diagnosis,
injury, poliomyelitis, or Guillain-Barré syn- typically revealing a loss of joint space, osteo-
drome). A previous history of this condition phytes, and subchondral bone cysts. Treatment
predisposes individuals to future occurrence. in mild to moderate stages involves lifestyle
This condition involves abnormal bone for- modification, exercises to maintain joint flexi-
mation within extraskeletal tissues.58 Radi- bility and strength, oral anti-inflammatory
ographs confirm the diagnosis. Surgical resec- medication, and corticosteroid injections. In
tion of heterotopic bone is associated with a cases of severe pain and functional limitation,
high recurrence rate for this condition, but the total joint arthroplasty is considered.
surgery results in reestablishing joint range of
motion and soft tissue flexibility. ■ Iliac Artery Aneurysm
Chief Clinical Characteristics
■ Hip Dislocation This presentation typically includes lower back,
Chief Clinical Characteristics abdominal, or groin pain with a pulsatile mass.
This presentation typically includes extreme Other symptoms may include lower extremity
pain with weight bearing and range of motion ischemia and erythema nodosum.
of the affected hip, as well as shortening of the
Background Information
affected lower extremity following a traumatic
This rare condition is more common in men
event and extreme pain. Individuals with hip
than women.60 It usually involves a defect in
dislocation will present with an acute inability
the wall of the common iliac artery. This con-
to walk or weight bear due to debilitating pain.
dition occurs when the tunica intima, tunica
They also may demonstrate external rotation
media, or tunica adventitia becomes struc-
of the affected lower extremity.
turally compromised due to various acquired
Background Information factors (eg, hypertension, cigarette smoking)
This condition involves escape of the femoral or congenital factors (eg, Marfan’s syn-
head from the acetabulum, usually in a superior- drome). Risk factors include arteriosclerosis,
posterior direction, due to a variety of acquired infection, lumbar or hip surgical trauma, and
factors (eg, trauma) or congenital factors (eg, pregnancy. Computed tomography or mag-
developmental dysplasia of the hip). The netic resonance angiography confirm the
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342 Chapter 19 Iliac Crest Apophysitis

diagnosis. Elective open repair of the aneurysm the lateral lumbar region. Other symptoms
HIP PAIN

is considered when the risk of rupture ex- may include malaise, weight loss, or palpable
ceeds that of surgery.2 mass.

■ Iliac Crest Apophysitis Background Information


Primary iliopsoas abscesses mainly result
Chief Clinical Characteristics from staphylococcal infections. Secondary
This presentation typically includes pain over abscesses may result from Crohn’s disease,
the lateral ilium with active movement and re- appendicitis, diverticulitis, ulcerative colitis,
sisted abduction of the affected hip, as well as osteomyelitis, neoplasm, disk infection, renal
tenderness to palpation. infections, and trauma.25,62 Abdominal com-
Background Information puted tomography confirms the diagnosis.
This condition is more common in active, Treatment involves eliminating the underly-
skeletally immature individuals.61 It occurs af- ing infection through medication or surgical
ter a sudden contraction of the abdominal debridement.
muscles is opposed by simultaneous contrac-
■ Iliopsoas Tendinitis
tion of the gluteus medius and tensor fascia
latae. Constant traction on the insertion site Chief Clinical Characteristics
can lead to a chronic soreness of the lateral il- This presentation includes deep groin or an-
ium. This condition is managed nonsurgically teromedial thigh pain, worsened with resisted
with relative rest, gentle exercise, and physical hip flexion, passive internal rotation during
modalities. extension of the affected hip, and palpation of
the iliopsoas muscle. The tenderness of the
■ Iliofemoral Venous Thrombosis iliopsoas tendon may be palpated lateral to the
Chief Clinical Characteristics femoral artery, inferior to the inguinal liga-
This presentation typically includes an insidi- ment in the femoral triangle.
ous onset of pain extending distal to the lesion, Background Information
accompanied by diminished pulses and swelling Both acute and repetitive trauma may con-
in the affected lower extremity. tribute; overuse is the classic cause. Acute
Background Information tendon inflammation may cause snapping or
Genetic predisposition may cause elevated clicking during hip motion.63 Clinical exami-
risk. This condition also may manifest itself in nation confirms the diagnosis. This condition
pregnant women due to compression of the is typically managed nonsurgically.
iliac vein. In addition, a history of malignancy ■ Iliopsoas Tendinosis
or prior thrombosis increases the risk for
this condition. The etiology of this condition Chief Clinical Characteristics
is associated with Virchow’s triad of intimal This presentation includes deep groin or an-
damage, stasis, and changes in blood compo- teromedial thigh pain, worsened with resisted
sition.55 Computed tomography, magnetic hip flexion, passive internal rotation during
resonance angiography, and Doppler ultra- extension of the affected hip, and palpation of
sonography confirm the diagnosis. It is a the iliopsoas muscle. The tenderness of the
medical emergency because of the risk for iliopsoas tendon may be palpated lateral to the
propagation of the thrombus to the lungs. femoral artery, inferior to the inguinal liga-
ment in the femoral triangle.
■ Iliopsoas Abscess Background Information
Chief Clinical Characteristics Both acute and repetitive trauma may con-
This presentation typically includes hip, lateral tribute; overuse is the classic cause. Tendinosis
lumbar, or abdominal pain with fever and involves disorganization of the intratendinous
limping.25 Hip pain is present in approxi- collagen matrix and angiofibroblastic hyper-
mately 29% of individuals with this condi- plasia.64 Clinical examination confirms the di-
tion.62 Physical findings include an externally agnosis. This condition is typically managed
rotated hip, flank tenderness, and fullness in nonsurgically.
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Chapter 19 Iliopsoas Strain 343

■ Iliotibial Band Friction Syndrome common site of metastatic disease; lumbar

HIP PAIN
Chief Clinical Characteristics metastases account for 20% of cases.68 Symp-
This presentation may involve an insidious on- toms also may be related to pathological frac-
set of sharp, burning lateral hip and thigh pain ture in affected sites. Common primary sites
extending from the greater trochanter to the causing metastases to bone include breast,
lateral femoral condyle. prostate, lung, and kidney. Bone scan confirms
the diagnosis. Common treatments for metas-
Background Information tases include surgical resection, chemotherapy,
This condition may be associated with snap- radiation treatment, and palliation, depending
ping during hip motion and significant ten- on the tumor type and extent of metastasis.
derness to palpation of the iliotibial band. The
etiology of this condition involves chronic MUSCLE STRAINS
overuse. It may be associated with a positive ■ Adductor Strain
Ober test, although this finding itself is not Chief Clinical Characteristics
pathognomic. Clinical examination confirms This presentation may include pain in the
the diagnosis. This condition is typically medial thigh and proximally to the area of the
managed nonsurgically. pubic symphysis. This condition is common
among athletes who participate in sports re-
■ Labral Tear quiring high-speed changes in direction. Ad-
Chief Clinical Characteristics ductor strains are characterized by pain with
This presentation involves either insidious or palpation of the affected muscle and pain
traumatic onset of stabbing hip and groin pain, with resisted adduction.
worsened with weight bearing and associated
with a global decrease in hip range of motion. Background Information
This condition may be associated with a painful Radiographs can exclude fractures or avul-
clunk or snapping with hip passive range of sions. The usual treatment for this condition
motion. is nonsurgical.

Background Information ■ Hamstring Strain


Labral tears may lead to joint instability, lead- Chief Clinical Characteristics
ing some patients to report insecurity with This presentation can include an acute onset
their hip. Magnetic resonance imaging and of posterior thigh pain that is worsened
therapeutic cortisone injections may confirm with resisted action and stretch of the affected
the diagnosis. Treatment includes both surgi- muscle.
cal65 and nonoperative strategies, such as
corticosteroid injections and physical therapy. Background Information
This condition is often associated with active
■ Metastases, Including From overlengthening or eccentric contraction,
Primary Breast, Kidney, Lung, as in acceleration and deceleration while
Prostate, and Thyroid Disease sprinting. Palpation reproduces symptoms.
Tissue edema may cause associated fullness
Chief Clinical Characteristics
and ecchymosis may be present in the poste-
This presentation can be characterized by un-
rior thigh. Radiographs may help exclude
remitting pain in individuals with these risk
avulsion fractures. Usual treatment for this
factors: previous history of cancer, age 50 years
condition is nonsurgical.
or older, failure to improve with conservative
therapy, and unexplained weight change of ■ Iliopsoas Strain
more than 10 pounds in 6 months.66
Chief Clinical Characteristics
Background Information This presentation may involve aching, throb-
Cauda equina symptoms or nerve root com- bing groin pain with active movement and a
pression of the spinal canal can be caused preference toward passive positioning of the
by vertebral collapse or infiltration of the hip in flexion to slacken the iliopsoas. Deep
tumor.67 The skeletal system is the third most palpation of the femoral triangle and passive
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344 Chapter 19 Quadriceps Muscle Strain

hip extension stretching reproduce symptoms. weakness, limited knee jerk, and intact sen-
HIP PAIN

A “snapping” sensation is associated with sation in the affected region.


pain in 30% of cases.
Background Information
Background Information This condition may be associated with prox-
Iliopsoas strains may occur due to acute or imal compression of the femoral nerve due
repetitive trauma.69 Usual treatment for this to contact with surgical instruments or de-
condition is nonsurgical. velopment of a retroperitoneal hematoma
due to intra-abdominal or lumbar surgery.
■ Quadriceps Muscle Strain Electromyographic studies may confirm the
Chief Clinical Characteristics diagnosis. This condition is typically man-
This presentation typically includes quadri- aged nonsurgically, and surgical intervention
ceps muscle pain that is worsened with is reserved for cases in which the exact
weight-bearing activities, palpation, acute anatomical location of the entrapment has
stretch, and repeated eccentric muscular con- been identified.
tractions. Symptoms are often reproduced
with active knee extension and active or ■ Genitofemoral Nerve
passive knee flexion. Entrapment
Chief Clinical Characteristics
Background Information
This presentation typically involves burning
This condition is often associated with active
medial thigh pain that radiates to the scrotum
overlengthening or eccentric contraction, as
or labia majora and is worsened with hip
in acceleration and deceleration while sprint-
extension and associated with tenderness over
ing. Tissue edema may cause associated full-
the inguinal canal. This condition most com-
ness, and ecchymosis may be present. Clinical
monly presents after lower abdominal surgery.
examination confirms the diagnosis. Usual
It is commonly mistaken for hernia, upper
treatment for this condition is nonsurgical.
lumbar radiculopathy, or ilioinguinal nerve
■ Myositis Ossificans entrapment. The cremasteric reflex and perineal
sensation may be absent.
Chief Clinical Characteristics
This presentation involves thigh or gluteal pain at Background Information
the specific site of a prior trauma, associated with Selective nerve blocks confirm this diagno-
reduced hip flexibility and a tender, firm, some- sis.70 This condition is typically managed
what mobile mass within the affected muscle belly. nonsurgically, and surgical intervention is
reserved for cases in which the exact
Background Information
anatomical location of the entrapment has
This condition results from abnormal bone
been identified.
growth within a hematoma in the soft tissues.
Radiographs confirm the diagnosis 6 weeks ■ Iliohypogastric Nerve
after onset by showing centripetal ossification Entrapment
of a hematoma. Surgical resection of hetero-
topic bone is associated with a high recurrence Chief Clinical Characteristics
rate for this condition, but the surgery results This presentation involves onset of burning
in reestablishing joint range of motion and inguinal and suprapubic pain with radiation
soft tissue flexibility. to the genitals following lower abdominal
surgery. Palpation of the surgical scar repro-
NERVE ENTRAPMENTS/ duces symptoms. Sensation loss is usually
NEUROPATHY minimal.
■ Femoral Neuropathy Background Information
Chief Clinical Characteristics Selective nerve blocks confirm this diagno-
This presentation typically includes a trau- sis.71 This condition is typically managed
matic or insidious onset of anterior thigh nonoperatively, and surgical intervention is
or groin pain associated with quadriceps reserved for cases in which the exact
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Chapter 19 Osteitis Pubis 345

anatomical location of the entrapment has ■ Obturator Nerve Entrapment

HIP PAIN
been identified. Chief Clinical Characteristics
This presentation may include unilateral me-
■ Ilioinguinal Nerve Entrapment dial thigh pain and paresthesias, worsened
Chief Clinical Characteristics with hip abduction or extension, and is asso-
This presentation may be characterized by ciated with hip adductor weakness. Individ-
an insidious onset of burning pain in the uals with this condition may walk with a
medial thigh that radiates to the scrotum or wide-based gait. Sensory loss may extend from
labia majora and is worsened with standing the midportion of the medial thigh to the re-
erect and hip motion. Abdominal surgery, gion distal to the knee. Medial thigh wasting
pregnancy, and asphericity of the femoral may be present, and the hip adductor tendon
head are risk factors for developing this reflex may be absent.
condition. Palpation medial to the anterior
superior iliac spine reproduces symptoms. Background Information
Transversalis and internal oblique paralysis Electromyography confirms the diagnosis.73
may be present, causing a small abdominal This condition is typically managed nonop-
bulge that may be confused with a hernia. eratively, and surgical intervention is re-
Perineal sensory loss also may be present. served for cases in which the exact anatomi-
cal location of the entrapment has been
Background Information identified.
Selective nerve blocks confirm this diag-
nosis.70 This condition is typically managed ■ Piriformis Syndrome
nonoperatively, and surgical intervention is
reserved for cases in which the exact Chief Clinical Characteristics
anatomical location of the entrapment has This presentation can include traumatic or in-
been identified. sidious onset of dull buttock pain with radia-
tion to the posterior thigh. This condition may
■ Meralgia Paresthetica be associated with limping and painful weak-
ness of hip musculature during resisted testing.
Chief Clinical Characteristics Palpation over the muscle and sciatic notch
This presentation can involve unilateral prox- usually results in symptom reproduction, as
imal lateral thigh pain and numbness, which well as active and passive hip external rotation.
is relieved with sitting and worsened with
standing and walking. Tapping over the Background Information
inguinal ligament or moving the hip into Radiating pain is caused by compression of
extension may also provoke the symptoms. the sciatic nerve by the inflamed piriformis.
Clinical examination confirms the diagnosis.
Background Information This condition is typically managed nonsur-
Focal compression of the lateral femoral gically, and surgical intervention is reserved
cutaneous nerve as it passes beneath the in- for severe cases in which certainty regarding
guinal ligament most commonly causes this the role of the piriformis as a site of com-
condition. Pregnancy, obesity, diabetes, and pression has been determined to be high.
tight-fitting clothes are among risk factors.
Less frequently, this condition may be ■ Osteitis Pubis
caused by blunt trauma, ischemia, or
Chief Clinical Characteristics
traction.72 Clinical examination and elec-
This presentation may involve a traumatic or
trodiagnostic studies confirm the diagnosis.
insidious onset of lower abdominal and supra-
Treatment for this condition includes re-
pubic pain that is worsened with walking and
moving the cause of the compression, which
hip abduction.
can include alteration of clothing as well as
weight loss. In more severe cases, nerve Background Information
blocks or surgical decompression may be Individuals presenting with this condition
warranted. may demonstrate a wide-based gait. This
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346 Chapter 19 Osteoblastoma of the Hip

condition involves inflammation of the pubic tenderness and warmth to palpation, with sig-
HIP PAIN

symphysis following trauma, pelvic surgery, nificant increase in pain with activity and at
childbirth, or athletic overuse injuries. Its etiol- night, and with substantial and immediate
ogy is unclear. The diagnosis is confirmed with relief of pain with anti-inflammatory medica-
plain radiographs and technetium scan.74 This tion. This condition is more common in males
condition is managed nonsurgically with activ- than females, and it rarely presents in people
ity modification and gentle exercise. Surgical younger than 5 or older than 40 years of age.
intervention, including open reduction and in-
Background Information
ternal fixation of the pubic symphysis, are typ-
The pathology of osteoid osteomas includes
ically reserved for more active individuals and
abnormal production of osteoid and primitive
individuals with recalcitrant symptoms that
bone. The proximal femur is the most com-
fail to respond to nonsurgical interventions.
mon site for this tumor. Pain associated with
■ Osteoblastoma of the Hip this condition is self-limiting and may resolve
spontaneously over the course of 2 to 4 years.75
Chief Clinical Characteristics
This presentation can involve an insidious on-
set of deep and aching pain, typically in males ■ Osteomalacia
under 30 years of age. Pain associated with this Chief Clinical Characteristics
condition is not usually worse at night. This This presentation can involve diffuse bone pain
condition may rarely affect the pelvic bones, about the hip joint, associated with fatigue,
and the proximal femoral epiphysis is even less malaise, generalized bone pain, fractures due to
commonly affected. minor trauma, and possible joint deformity.
Background Information Background Information
This condition involves abnormal production This condition is analogous to rickets in chil-
of osteoid and primitive bone, although its spe- dren. It involves bone demineralization due to
cific etiology remains unclear. Biopsy and plain inadequate dietary intake of vitamin D, malab-
radiographs confirm the diagnosis. Computed sorption of vitamin D by the intestines, inade-
tomography is necessary to define the tumor quate exposure to the sunlight, renal deficien-
margins if surgical resection is considered. cies, and chronic use of anticonvulsant
medications. Radiographs and laboratory tests
■ Osteochondroma help confirm the diagnosis, revealing de-
Chief Clinical Characteristics creased mineralization of bone and low phos-
This presentation can be characterized by bone phorous level, respectively. Treatment typically
and joint pain that is usually only present when includes amelioration of contributing factors,
the lesion is mechanically stressed, typically in including diet modification and vitamin D
males less than 20 years of age. supplementation.
Background Information ■ Pigmented Villonodular Synovitis
This condition is asymptomatic in most cases. It
Chief Clinical Characteristics
can arise in any bone that undergoes enchon-
This presentation can include an insidious on-
dral ossification, but is most common around
set of mild discomfort and stiffness of the affected
the knee. Plain radiographs typically demon-
hip that is relieved by positioning the hip in
strate bony projections from the areas adjacent
passive hip flexion and external rotation. This
to growth plates. Treatment includes surgical
condition usually presents unilaterally.
excision only with significant and persistent soft
tissue irritation. If left untreated, lesions usually Background Information
do not metastasize but can continue to grow. Pigmented villonodular synovitis is a rare, be-
nign, idiopathic disorder of the synovium that
■ Osteoid Osteoma results in villous (nodular) formation in
Chief Clinical Characteristics joints, tendon sheaths, and bursae. This condi-
This presentation may include focal bone pain tion progresses slowly, although its long-term
at the site of the tumor that is associated with presence in the hip can result in femoral head
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Chapter 19 Reiter’s Syndrome 347

erosions that lead to a degenerative condi- Pain due to this condition may be aggravated

HIP PAIN
tion.76 Clinical imaging confirms the diagno- with activity, causing limping.
sis. Treatment includes synovectomy and sub-
sequent radiation therapy. Background Information
This condition is frequently found in adoles-
■ Polymyalgia Rheumatica cents because of the active bone growth in
Chief Clinical Characteristics this age group. African American individuals
This presentation typically includes the acute on- are affected slightly more often than Cau-
set of proximal myalgias and stiffness involving casian individuals. Plain films confirm the
the neck, shoulders, pelvic girdle, and hips that diagnosis, typically revealing tumors near
is worse at night and with movement and asso- metaphyseal growth plates of the femur. This
ciated with fatigue, weight loss, fever, and sweats. condition can be especially fatal if metasta-
sized to the lungs. Treatment for this condi-
Background Information tion involves extensive surgical resection of
Pelvic girdle involvement usually causes pain the involved bone along with orthopedic
that radiates to the knee.77 Diagnosis is con- reconstruction.
firmed with blood panels that demonstrate an
elevated erythrocyte sedimentation rate.77 ■ Pseudogout
This condition is associated with temporal Chief Clinical Characteristics
arteritis, which can cause blindness if un- This presentation includes a sudden onset of
treated, so urgent referral to a physician is nec- deep stabbing hip pain, worsened with weight
essary if this condition is suspected. Treatment bearing and hip passive range of motion, and
of this condition mainly includes the use of is associated with tenderness, warmth, and red-
low-dose oral prednisone in the morning.77,78 ness of overlying soft tissues.
PRIMARY BONE TUMOR OF THE Background Information
ACETABULUM OR PROXIMAL This condition is more common in older
FEMUR males than females. It is less common in the
■ Ewing’s Sarcoma hip joint. This condition’s presentation mimics
gout; however, calcium pyrophosphate dehy-
Chief Clinical Characteristics drate crystal deposits mediate its characteristic
This presentation includes anterior or lateral joint pain and articular cartilage destruction.
hip pain and swelling that persists for weeks Blood tests and microscopic examination of
or months, accompanied by intermittent aspirated synovial fluid confirm the diagnosis.
fevers. Individuals with this condition may Treatment usually involves medication to
report resting and night pain, and their symp- address this condition’s underlying metabolic
toms may be unchanged with activity. dysfunction.
Background Information
This condition is prevalent throughout the ■ Reiter’s Syndrome
life span, and is most common during the Chief Clinical Characteristics
first and second decades of life.79 It affects This presentation can involve pain and stiff-
Caucasian individuals more frequently than ness in the low back, sacroiliac, and poste-
African American and/or Asian American rior hip regions in the presence of conjunc-
individuals. Males are affected more often tivitis and urethritis.26 These patients will
than females. Treatment includes surgical re- have asymmetric distribution of pain be-
section of the involved bone. Nonsurgical in- tween the hips and will most certainly have
terventions usually fail to change symptoms some form of spinal discomfort associated
in individuals with this condition. with this condition.
■ Osteosarcoma Background Information
Chief Clinical Characteristics This condition also has been called a reactive
This presentation may involve an insidious on- arthritis because it may present after other
set of pain that persists for weeks or months. infections. Clinical examination confirms the
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348 Chapter 19 Rheumatoid Arthritis of the Hip

diagnosis. Treatment includes anti-inflammatory ■ Psoriatic Arthritis


HIP PAIN

medication to control inflammation, as well as Chief Clinical Characteristics


antibiotics to prevent the development of This presentation involves an insidious onset
chronic disease. of asymmetric posterior hip pain associated
■ Rheumatoid Arthritis of the Hip with psoriasis. Pitting nail lesions occur
in 80% of individuals with this condition.
Chief Clinical Characteristics Dactylitis, tenosynovitis, and peripheral
This presentation may involve morning stiffness arthritis also are common.
and generalized pain throughout multiple joints
in a symmetric distribution, with possible Background Information
tenderness and swelling of affected joints. The severity of arthritis is uncorrelated with
the extent of skin involvement.27 Radi-
Background Information ographs of the distal phalanges may reveal a
Women are twice as likely to be affected as characteristic “pencil in cup” deformity.
men. Symptoms associated with this progres- Blood tests including erythrocyte sedimen-
sive inflammatory joint disease are caused by tation rate are useful to track disease
synovial membrane thickening and cytokine activity. Usual treatment includes a variety of
production in synovial fluid. Articular cartilage steroidal, nonsteroidal, and biological anti-
erosion, synovial hypertrophy, and constant inflammatory medications.
joint effusion80 eventually cause bony erosions
and joint deformities that have a significant ■ Scleroderma
impact on daily function. Younger age of onset Chief Clinical Characteristics
is associated with a greater extent of disability This presentation typically includes myal-
later. Plain radiographs and blood tests con- gia, arthralgia, fatigue, weight loss, limited
firm the diagnosis. Treatment typically in- mobility, and hardened skin about the hands,
cludes a variety of steroidal, nonsteroidal, and knees, or elbows. This condition occurs in in-
biological anti-inflammatory medications. dividuals between 25 and 55 years of age,
RHEUMATOID ARTHRITIS–LIKE and is four to five times more likely in women
DISEASES than men. Additional symptoms may include
dry mouth and eyes, as well as Raynaud’s
■ Inflammatory Muscle Diseases phenomenon.
Chief Clinical Characteristics
Background Information
This presentation includes a gradual onset of
This rare and progressive autoimmune dis-
mild muscle pain associated with proximal
order affects blood vessels and many inter-
muscle weakness that causes difficulty with
nal organs, including the lungs and the gas-
daily activities such as walking, ascending
trointestinal system. Overproduction of
and descending stairs, and rising from chairs.
collagen in this condition eventually leads to
Background Information poor blood flow in the extremities, which
This condition describes a group of patho- can cause ulcers in the fingers, changes in
logically, histologically, and clinically distinct skin color, and a disappearance of creases
disorders: polymyositis, dermatomyositis, in the skin. Continued damage to small-
and inclusion body myositis. They may be diameter vasculature leads to scar tissue
associated with other collagen, vascular, production that impairs joint range of
and immune disorders. Although proximal motion. Blood tests confirm the diagnosis.
extremity weakness is a classic finding, Treatment includes both steroidal and
up to 50% also demonstrate distal weakness nonsteroidal anti-inflammatory medica-
that may be equally as severe.32 Blood tests tions and gentle exercise.
help confirm the diagnosis and track disease
activity, revealing elevated serum levels of ■ Systemic Lupus Erythematosus
creatine phosphokinase. Treatment typically Chief Clinical Characteristics
involves steroidal, nonsteroidal, and biologi- This presentation can be characterized by hip
cal anti-inflammatory medications. and groin pain associated with fatigue and
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Chapter 19 Systemic Fungal Infection 349

joint pain/swelling affecting the hands, feet, the diagnosis and directs medical manage-

HIP PAIN
knees, and shoulders. ment, which involves a regimen of antibiotic
medication directed to the infective agent.
Background Information
This condition affects mostly women of ■ Sickle Cell Crisis
childbearing age. It is a chronic autoimmune
Chief Clinical Characteristics
disorder that can affect any organ system, in-
This presentation may involve bone pain about
cluding skin, joints, kidneys, brain, heart,
the hip joint, worsened by cold weather, overex-
lungs, and blood. The diagnosis is confirmed
ertion, dehydration, and being overly fatigued.
by the presence of skin lesions; heart, lung,
or kidney involvement; and laboratory Background Information
abnormalities including low red or white cell This condition involves abnormal red blood cell
counts, low platelet counts, or positive ANA morphology that causes them to become rigid
and anti-DNA antibody tests.33 Usual treat- and sticky, disrupting blood flow to bones, and
ment includes a variety of steroidal, nons- resulting in painful bone infarcts. This condi-
teroidal, and biological anti-inflammatory tion may cause complications in multiple organ
medications. systems, including stroke, skin ulcers, and
blindness. There is no cure for this condition;
■ Sacroiliac Joint Dysfunction treatment is palliative and preventive.
Chief Clinical Characteristics
This presentation typically includes either an ■ Synovial Sarcoma
acute or insidious onset of buttock pain with Chief Clinical Characteristics
possible radiation to the groin, posterior thigh, This presentation can be characterized by pain
and lower leg. Sacral sulcus tenderness shows and tenderness in a long-standing soft tissue
high sensitivity but poor specificity for detect- nodule that has rapidly increased in size over a
ing individuals with this condition.81 short period of time. This uncommon condition
is typically found within 5 cm of the knee joints
Background Information
of people younger than 30 years of age.82
It is associated with articular and periarticular
nociception due to pathological joint mechan- Background Information
ics, although this theory remains contentious. Synovial sarcoma spreads along fascial planes,
A high index of suspicion for this condition is so the disease may be more widespread
warranted for individuals with excessive liga- than apparent on initial evaluation. Biopsy
mentous laxity (eg, pregnant women). Clinical confirms the diagnosis, and magnetic reso-
examination and selective joint blockade con- nance imaging shows the extent of spread.
firm the diagnosis. Imaging usually is unhelp- This condition is slow growing and locally
ful to confirm the diagnosis. This condition is aggressive. Its recurrence rate within 5 years is
managed nonsurgically. significant. Surgical resection of the tumor
and surrounding soft tissues is indicated.
■ Septic Arthritis of the Hip
Chief Clinical Characteristics ■ Systemic Fungal Infection
This presentation involves an insidious onset of Chief Clinical Characteristics
hip and groin pain with weight bearing and This presentation includes hip pain associated
movement, accompanied by possible fever and with signs of systemic infection, such as fever,
malaise. Overlying tissue is usually swollen anorexia, and malaise. Individuals with this
and inflamed, and this may extend to the an- condition may present after a prolonged and
terior and lateral thigh. varied course of antibiotics to address their
symptoms.
Background Information
Septic arthritis of the hip occurs by way of Background Information
bacterial, yeast, fungal, or viral infection of the This condition is most common in immune-
hip joint. Sepsis also can damage articular compromised individuals, such as patients in
cartilage, leading to osteoarthritis later in life. intensive care, patients receiving chemother-
Culture of aspirated synovial fluid confirms apy, patients with acquired immunodeficiency
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350 Chapter 19 Transient Osteoporosis of the Hip

syndrome, and transplant recipients. This con- range of motion of affected joints due to artic-
HIP PAIN

dition is most commonly due to Candida ular cartilage erosion.


species infection, although aspergillus also
Background Information
may be a culprit organism. The infection is
This condition results from the hematogenous
transferred from health care worker to patient
spread of a primary respiratory infection.
by way of hand contact. Serum pathology
Chest radiographs and culture of aspirated
and molecular techniques confirm the diagno-
synovial fluid confirm the diagnosis. Magnetic
sis and direct appropriate pharmacologic
resonance imaging confirms the diagnosis.
management.
Treatment consists of antituberculosis medica-
■ Transient Osteoporosis of the Hip tions, which are effective 90% of the time, with
surgery in more advanced cases.46
Chief Clinical Characteristics
This presentation involves an acute or gradual
onset of severe hip and groin pain that is wors- References
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CHAPTER20
Case Demonstration: Hip Pain
■ Alison R. Scheid, PT, DPT, OCS, NCS ■ Elizabeth M. Poppert, PT, DPT, MS, OCS

NOTE: This case demonstration was developed tendinosis.” He presented with a 1-year history
using the diagnostic process described in of hip pain that was originally diagnosed as
Chapter 4 and demonstrated in Chapter 5. The avascular necrosis of the femoral head of
reader is encouraged to use this diagnostic unknown etiology with subsequent surgical
process in order to ensure thorough clinical rea- replacement of the femoral head. Postsurgical
soning. If additional elaboration is required on therapy was discontinued after 1 month
the information presented in this chapter, please (patient self-discharged). However, the patient
consult Chapters 4 and 5. returned to therapy now due to his reports of
an inability to return to prior level of function,
THE DIAGNOSTIC PROCESS discomfort with simple daily activities, and,
although working, he was unable to resume
Step 1 Identify the patient’s chief concern.
full duties independently (climbing ladders
Step 2 Identify barriers to communication.
and on his feet more than 8 hours per day).
Step 3 Identify special concerns.
He described the pain as feeling “stiff and
Step 4 Create a symptom timeline and sketch
achy,” located mostly on the anterior and lateral
the anatomy (if needed).
area surrounding the right hip (not including
Step 5 Create a diagnostic hypothesis list
groin) and denied any pain or pathology of the
considering all possible forms of remote and
left lower extremity. He rated his minimum
local pathology that could cause the
pain as 0/10 and his maximal pain during the
patient’s chief concern.
last month at about 7/10 to 8/10. The morning
Step 6 Sort the diagnostic hypothesis list by
and evenings were the worst because he felt the
epidemiology and specific case
stiffest at these times. Pain did not usually wake
characteristics.
him up at night. His pain and stiffness started
Step 7 Ask specific questions to rule specific
immediately upon moving and took about 3 to
conditions or pathological categories less
4 minutes to decrease while weight bearing.
likely.
However, if he sat or laid down, the pain imme-
Step 8 Re-sort the diagnostic hypothesis list
diately decreased. He reported Advil had a mild
based on the patient’s responses to specific
effect on pain relief. Walking (especially trying
questioning.
to take “longer/faster” steps and pain with the
Step 9 Perform tests to differentiate among
first several minutes of walking were the worst),
the remaining diagnostic hypotheses.
squatting, using the elliptical, and going up-
Step 10 Re-sort the diagnostic hypothesis list
stairs (more than down) all aggravated his pain.
based on the patient’s responses to specific
Recent radiographs were taken 1 month
tests.
prior to check placement of the femoral com-
Step 11 Decide on a diagnostic impression.
ponent and revealed mild degeneration at the
Step 12 Determine the appropriate patient
acetabulum, with stable componentry. Due to
disposition.
a medical history of testicular cancer several
years ago, he received a computed tomography
scan of his abdomen and pelvis every
Case Description 6 months. The most recent was 4 months ago
and was clear for signs of cancerous changes.
CD was a 43-year-old male who was referred CD fractured his right tibial plateau 6 years
to physical therapy by an orthopedic surgeon ago, which was treated surgically, and he had
with a diagnosis of “right hip rectus femoris arthroscopic surgery 3 to 4 years ago to clean
353
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354 Chapter 20 Case Demonstration: Hip Pain

out the knee after the original surgery. The only neglect of a pathology not appropriate for
medication he took was Advil 3 to 4 times a day physical therapy intervention.
at the time of physical therapy evaluation. ● Report of limited physical therapy follow-
STEP #1: Identify the patient’s chief ing hip replacement. This report from the
concern. patient could have led to the therapist’s as-
sumption of unresolved mechanical factors
● Right hip pain
for chronic pain. His pain did not appear to
STEP #2: Identify barriers to be “new” and his limited function brought
communication. him back to physical therapy.
● Gender difference between patient and
therapist. When a male patient is evaluated STEP #3: Identify special concerns.
by a female physical therapist, the type of in- ● Relatively recent history of cancer
formation elicited from the history could be ● Avascular necrosis of unknown etiology
affected, both from the perspectives of the These factors raise the suspicion of cancer or
therapist’s questions and the type of infor- other pathology that would require additional
mation the patient may be willing to share. consultation with other health care providers.
● Negative tests and relatively young patient
age. Recent, negative imaging results STEP #4: Create a symptom timeline and
and relatively young age could have led to sketch the anatomy (if needed).

4 years 10 months 9 months 4 months 1 month


ago ago ago ago ago Present

Testicular Hip AVN and Discharge CT pelvis Negative Physical


cancer partial hip from post-op negative radiographic Therapy
replacement. Physical findings findings Evaluation
post-op Therapy for loose
Physical Therapy component

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.

Remote Remote
T Trauma T Trauma
Acute lumbar sprain/strain Acute lumbar sprain/strain (time course,
presentation)
Disk disruption (with or without herniation) Disk disruption (with or without herniation)
(time course, presentation)
Facet syndrome Facet syndrome
Lumbar compression fracture Lumbar compression fracture (presentation,
age, no mechanism of injury)
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Chapter 20 Case Demonstration: Hip Pain 355

Lumbar radiculopathy Lumbar radiculopathy


Traumatic spondylolisthesis Traumatic spondylolisthesis (presentation,
no mechanism of injury)
I Inflammation I Inflammation
Aseptic Aseptic
Complex regional pain syndrome Complex regional pain syndrome
Crohn’s disease Crohn’s disease
Fibromyalgia Fibromyalgia
Reiter’s syndrome of the lumbar spine Reiter’s syndrome of the lumbar spine
Rheumatoid arthritis of the lumbar spine Rheumatoid arthritis of the lumbar spine
Septic Septic
Appendicitis Appendicitis (time course)
Herpes zoster Herpes zoster
Iliopsoas abscess Iliopsoas abscess (negative abdominal
computed tomography scan)
Pelvic inflammatory disease Pelvic inflammatory disease (uncommon in
patient’s sex)
Prostatitis Prostatitis (time course)
Renal or urinary tract infection Renal or urinary tract infection (time
course)
Retrocecal appendicitis Retrocecal appendicitis (negative
abdominal computed tomography
scan)
Retroperitoneal abscess Retroperitoneal abscess (negative
abdominal computed tomography scan)
Septic discitis Septic discitis
Tuberculosis of the lumbar spine (Pott’s Tuberculosis of the lumbar spine (Pott’s
disease) disease)
M Metabolic M Metabolic
Endometriosis Endometriosis (patient’s sex)
Va Vascular Va Vascular
Aortic arteriosclerosis Aortic arteriosclerosis
Aortic artery aneurysm Aortic artery aneurysm
Epidural hematoma Epidural hematoma
De Degenerative De Degenerative
Degenerative spondylolisthesis Degenerative spondylolisthesis
Disk degeneration Disk degeneration
Spinal stenosis Spinal stenosis
Spondylolysis Spondylolysis
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Bone tumor of the lumbar spine • Bone tumor of the lumbar spine
• Spinal cord tumor • Spinal cord tumor
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign, such as: Benign, such as:
• Osteoblastoma • Osteoblastoma (patient age)
• Ovarian cysts • Ovarian cysts (patient’s sex)
• Uterine fibroids • Uterine fibroids (patient’s sex)
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356 Chapter 20 Case Demonstration: Hip Pain

Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Anxiety Anxiety (time course)
Depression Depression
Malingering Malingering (presentation, no apparent
external motives, return to work)
Munchausen’s syndrome Munchausen’s syndrome (presentation of
symptoms, medical attention not sought
for months)
Secondary gain Secondary gain (presentation, no external
motives)
Somatoform disorder Somatoform disorder
Local Local
T Trauma T Trauma
Fractures: Fractures:
• Extracapsular femur fracture • Extracapsular femur fracture (no
mechanism of injury; negative hip
radiographs)
• Fracture of femoral shaft • Fracture of femoral shaft (no mechanism
of injury; negative hip radiographs)
Hip dislocation Hip dislocation (no mechanism of injury;
negative hip radiographs)
Labral tear Labral tear (presentation: lack of groin pain
and clicking)
Iliac crest apophysitis Iliac crest apophysitis (location of
symptoms, patient age)
Iliotibial band friction syndrome Iliotibial band friction syndrome
Muscle strains: Muscle strains:
• Iliopsoas strain • Iliopsoas strain
• Quadriceps muscle strain • Quadriceps muscle strain
Nerve entrapments/neuropathy: Nerve entrapments/neuropathy:
• Femoral neuropathy • Femoral neuropathy
• Meralgia paresthetica • Meralgia paresthetica
Sports hernia Sports hernia
Trochanteric contusion Trochanteric contusion (no mechanism of
injury)
I Inflammation I Inflammation
Aseptic Aseptic
Complex regional pain syndrome Complex regional pain syndrome
Fibromyalgia Fibromyalgia
Gout Gout (time course)
Herpes zoster Herpes zoster
Polymyalgia rheumatica Polymyalgia rheumatica (age)
Pseudogout Pseudogout (time course, age)
Reiter’s syndrome of the hip Reiter’s syndrome of the hip
Rheumatoid arthritis of the hip Rheumatoid arthritis of the hip
Trochanteric bursitis Trochanteric bursitis
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Chapter 20 Case Demonstration: Hip Pain 357

Tendinitis: Tendinitis:
• Iliopsoas • Iliopsoas (time course)
• Rectus femoris • Rectus femoris (time course)
• Sartorius • Sartorius (time course)
• Tensor fasciae latae • Tensor fasciae latae (time course)
Septic Septic
Cellulitis Cellulitis
Herpes zoster Herpes zoster
Iliopsoas abscess Iliopsoas abscess (negative abdominal
computed tomographic scan)
Sepsis Sepsis
Tuberculosis of the hip Tuberculosis of the hip
M Metabolic M Metabolic
Transient osteoporosis of the hip Transient osteoporosis of the hip (proximal
femur replaced)
Va Vascular Va Vascular
Iliac artery aneurysm Iliac artery aneurysm (negative pelvic
computed tomography scan)
De Degenerative De Degenerative
Failure of arthroplasty component Failure of arthroplasty component (negative
radiographic findings)
Hip osteoarthrosis (osteoarthritis) Hip osteoarthrosis (osteoarthritis) (mild
radiographic changes)
Tendinoses: Tendinoses:
• Iliopsoas • Iliopsoas
• Rectus femoris • Rectus femoris
• Sartorius • Sartorius
• Tensor fasciae latae • Tensor fasciae latae
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Bone tumor of the acetabulum or femur • Bone tumor of the acetabulum or femur
(negative radiographic findings)
Malignant Metastatic, such as: Malignant Metastatic, such as:
• Metastases, including from primary • Metastases, including from primary
breast, kidney, lung, prostate, thyroid breast, kidney, lung, prostate, thyroid
disease, and testicular cancer disease, and testicular cancer
Benign, such as: Benign, such as:
• Pigmented villonodular synovitis • Pigmented villonodular synovitis
(negative radiographs; no femoral joint
surface)
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Not applicable Not applicable
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358 Chapter 20 Case Demonstration: Hip Pain

STEP #7: Ask specific questions to rule Fibromyalgia (no pain in multiple joints)
specific conditions or pathological Reiter’s syndrome of the lumbar spine (no
categories less likely. back pain)
● Have you been ill? No, ruling less likely Rheumatoid arthritis of the lumbar spine
forms of septic inflammation. (no back pain, patient sex, radiographic
● Do you have pain in the pelvis or groin?
findings)
No, decreasing likelihood of referral from Septic
primary abdominal or pelvic pathology, in- Herpes zoster (no rash)
cluding hernia. Septic discitis (no back pain)
● Have you gained or lost weight that you Tuberculosis of the lumbar spine (Pott’s
didn’t intend to gain or lose? No, ruling less disease) (no back pain)
likely metastatic disease. M Metabolic
● Do you have numbness, tingling, or weak- Not applicable
ness in your legs? No, which makes pathol-
Va Vascular
ogy affecting the lumbar spine and periph-
eral nervous system of the lower extremities Aortic arteriosclerosis (no abdominal pain)
less likely. Aortic artery aneurysm (no abdominal pain)
● Do you have pain in other joints? No, de-
Epidural hematoma (no back pain)
creasing the likelihood of lumbar involve- De Degenerative
ment and rheumatic disease. Degenerative spondylolisthesis (no back
STEP #8: Re-sort the diagnostic pain, no lower extremity neurological
hypothesis list based on the patient’s symptoms)
responses to specific questioning. Disk degeneration (no back pain, no lower
extremity neurological symptoms)
Spinal stenosis (no back pain, patient age,
Teaching Comment: Despite CD’s his- no lower extremity neurological
tory of cancer, it appeared he did not present symptoms)
with cardinal signs or symptoms of metasta- Spondylolysis (no back pain)
tic cancer or other serious pathology. CD had Tu Tumor
regularly scheduled oncological follow-up ap- Malignant Primary, such as:
pointments that included hip and pelvic im- • Bone tumor of the lumbar spine (no back
aging studies during the time symptoms pain)
were present, and his chronic symptoms re- • Spinal cord tumor (no lower extremity
mained consistent. Most lumbar and vascular neurological symptoms, no back pain)
pathologies were significantly less likely in the Malignant Metastatic, such as:
absence of low back pain during the months • Metastases, including from primary
of hip pain. His chronic pain appeared to be breast, kidney, lung, prostate, thyroid
consistent with local hip pathology. disease, and testicular cancer (no weight
change)
Benign:
Remote Not applicable
T Trauma Co Congenital
Facet syndrome (no back pain) Not applicable
Lumbar radiculopathy Ne Neurogenic/Psychogenic
I Inflammation Depression (absence of generalized
Aseptic symptoms)
Crohn’s disease (no abdominal pain) Somatoform disorder (absence of
Complex regional pain syndrome generalized symptoms)
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Chapter 20 Case Demonstration: Hip Pain 359

Local Co Congenital
T Trauma Not applicable
Iliotibial band friction syndrome
Muscle strains:
• Iliopsoas strain Teaching Comment: CD’s young age,
• Quadriceps muscle strain good overall health, and decreased likeli-
Nerve entrapments/neuropathy: hood of metastases from testicular cancer
• Femoral neuropathy with recent negative imaging results all con-
• Meralgia paresthetica (no lower extremity tributed to a continued search for other local
sensory symptoms) causes of hip pain. The rate of metastases
Sports hernia from testicular cancer to the hip/pelvic re-
gions is small, with rates of inguinal metas-
I Inflammation tases as low as 2%.1
Aseptic
Complex regional pain syndrome
Fibromyalgia (no pain in multiple body Ne Neurogenic/Psychogenic
regions) Not applicable
Herpes zoster
Reiter’s syndrome of the hip (no illness, no STEP #9: Perform tests to differentiate
pain in multiple joints) among the remaining diagnostic
Rheumatoid arthritis of the hip (no pain in hypotheses.
multiple joints, patient sex) ● Inspection. No signs of rash.
Trochanteric bursitis ● Lower extremity reflexes. 2+, decreasing

Septic likelihood of projected pain from lumbar


Cellulitis spine pathology.
Herpes zoster ● Prone knee bending. Negative for repro-
Sepsis (no illness) duction of symptoms, but positive for lim-
Tuberculosis of the hip (no illness) ited muscle length.
M Metabolic ● Palpation. Unremarkable for reproduction

Not applicable of symptoms (except for tenderness to pal-


pation of hip flexor muscles).
Va Vascular
Not applicable STEP #10: Re-sort the diagnostic
hypothesis list based on the patient’s
De Degenerative responses to specific tests.
Tendinoses: Remote
• Iliopsoas
• Rectus femoris T Trauma
• Sartorius Lumbar radiculopathy (normal lower
• Tensor fasciae latae extremity deep tendon reflexes)
Tu Tumor I Inflammation
Malignant Primary: Aseptic
Not applicable Complex regional pain syndrome
Malignant Metastatic, such as: (inspection unremarkable)
• Metastases, including from primary Septic
breast, kidney, lung, prostate, thyroid Not applicable
disease, and testicular cancer (no weight M Metabolic
change)
Benign: Not applicable
Not applicable Va Vascular
Not applicable
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360 Chapter 20 Case Demonstration: Hip Pain

De Degenerative
Teaching Comment: Many of the local
Not applicable pathologies specific to hip arthroplasty were
Tu Tumor difficult to rule out based on the initial
Not applicable exam, but their prevalence has been re-
ported. In a study by Bhave and colleagues,2
Co Congenital
the rate of component malalignment was re-
Not applicable ported as 13%. Muscle contracture and
Ne Neurogenic/Psychogenic weakness was much more common, 28%
Not applicable and 47% respectively.
Local
T Trauma STEP #11: Decide on a diagnostic
Iliotibial band friction syndrome (negative impression.
palpation) ● Tendinosis of the hip musculature vs. failure

Muscle strains: of arthroplasty component.


• Iliopsoas strain (negative palpation)
• Quadriceps muscle strain (negative
palpation) Teaching Comment: Upon objective
Nerve entrapments/neuropathy: examination, a 3-cm right leg length discrep-
• Femoral neuropathy (negative prone knee ancy was found, as was weakness of the
bend) proximal right lower extremity muscles, lim-
Sports hernia (negative palpation) ited hip active and passive range of motion,
I Inflammation right more than left, with pain only associ-
ated with the right passive flexion and rota-
Aseptic tion movements; some pain reproduction
Complex regional pain syndrome with palpation of hip flexor tendons; pain
(inspection unremarkable) with quadriceps activation; gait and postural
Trochanteric bursitis (negative palpation) deviations. The local diagnoses remaining on
Septic the list most likely exist in combination and
Cellulitis (inspection unremarkable) are not exclusive diagnoses.
Herpes zoster (inspection unremarkable)
M Metabolic
STEP #12: Determine the appropriate
Not applicable
patient disposition.
Va Vascular ● Physical therapy was initiated with the
Not applicable caveat that failure of an arthroplasty com-
De Degenerative ponent could not be completely excluded,
Tendinoses:
but results of plain radiographs were en-
• Iliopsoas
couraging. The patient would be monitored
• Rectus femoris
throughout intervention for signs of intol-
• Sartorius
erance to treatment.
• Tensor fasciae latae
Case Outcome
Tu Tumor
Not applicable
CD was treated for the known impairments.
Treatment included a shoe lift, strengthening
Co Congenital exercises for hip/pelvic stabilization and lower
Not applicable extremity strength/endurance, and stretching
Ne Neurogenic/Psychogenic exercises. He made measureable gains during
Not applicable
the next 8 weeks for muscle strength, en-
durance, and flexibility. Upon resuming full
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Chapter 20 Case Demonstration: Hip Pain 361

work responsibilities, he developed increased References


anterior and lateral hip pain with distal symp- 1. Daugaard G, Karas V, Sommer P. Inguinal metastases
from testicular cancer. BJU Int. Apr 2006;97(4): 724–726.
toms into the knee. CD was referred to his 2. Bhave A, Marker DR, Seyler TM, Ulrich SD, Plate JF,
physician. Follow-up radiographs and MRI Mont MA. Functional problems and treatment
confirmed loosening of the hip component. solutions after total hip arthroplasty. J Arthroplasty.
The hip joint was aspirated. CD had an allergic Sep 2007;22(6 suppl 2):116–124.
3. Ortiguera CJ, Pulliam IT, Cabanela ME. Total hip
reaction to one of the metals contained in the arthroplasty for osteonecrosis: matched-pair analysis
femoral component and a revision of his of 188 hips with long-term follow-up. J Arthroplasty.
arthroplasty was performed. Following revi- Jan 1999;14(1):21–28.
sion surgery, CD quickly returned to full work
responsibilities.

Teaching Comment: In comparing out-


comes in individuals with a primary diagnosis of
avascular necrosis and osteoarthritis, a higher
rate of mechanical failure and revision was re-
ported in patients younger than 50 years old
with avascular necrosis.3
1528_Ch21_362-391 07/05/12 1:53 PM Page 362

CHAPTER21
Knee Pain
■ Della Lee, PT, DPT, OCS, ATC ■ Daniel Farwell, PT, DPT

Description of the Symptom Special Concerns


■ Decreased distal pulses
This chapter describes pathology that may lead ■ Inability to bear weight
to knee pain, including general, anterior, lat- ■ Skin break and fever
eral, medial, and posterior knee pain. Local ■ Warmth and swelling associated with
causes of knee pain are defined as pathology a fever
occurring in the musculoskeletal, nervous, and
vascular structures of the knee joint, between
the midthigh and proximal one-third of
the lower leg. Remote causes occur outside
this area.

CHAPTER PREVIEW: Conditions That May Lead to Knee Pain

T Trauma
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Hip pathologies: Acute patellar dislocation 372 Not applicable
• Avascular necrosis 370 Patellofemoral pain
• Hip osteoarthrosis/ syndrome 380
osteoarthritis 370

UNCOMMON
Lumbar radiculopathies: Fractures: Fractures:
• L4 radiculopathy 370 • Intercondylar eminence fracture • Osteochondral fracture of the
• L5 radiculopathy 370 of the tibia 375 patella 376
• S1 radiculopathy 371 • Patellar fracture 376 • Patellar fracture 376
• Supracondylar fracture of the
femur 376
Patellar instability 380

362
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KNEE PAIN
Anterior

Medial

Lateral
Posterior

Anterior

Medial

Lateral

Anterior Anterior Posterior Posterior


A lateral view medial view B medial view lateral view

LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR

Iliotibial band friction Ruptures and tears: Baker’s cyst 372


syndrome 377 • Medial collateral Muscle strains:
Muscle strains: ligament sprain/ • Biceps femoris muscle strain 378
• Biceps femoris muscle strain 378 rupture 382 • Gastrocnemius muscle strain 378
Nerve entrapments: • Medial meniscus Ruptures and tears:
• Common peroneal nerve tear 383 • Posterior cruciate ligament sprain/
entrapment at the fibular rupture 383
head 379
Ruptures and tears:
• Lateral collateral ligament
sprain/rupture 382
• Lateral meniscus tear 382

Not applicable Muscle strains: Muscle strains:


• Semimembranosus • Semimembranosus muscle
muscle strain 379 strain 379

(continued)

363
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364 Chapter 21 Knee Pain

Trauma (continued)
KNEE PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


RARE
Not applicable Not applicable Ruptures and tears:
• Quadriceps tendon rupture 384

I Inflammation
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Aseptic
Bursitis:
• Infrapatellar bursitis 372
• Prepatellar bursitis 374
Tendinitis:
• Patellar tendinitis 385
• Quadriceps tendinitis 385
Septic
Not applicable
UNCOMMON
Not applicable Aseptic Aseptic
Fibromyalgia 375 Infrapatellar fat pad hypertrophy
Reiter’s syndrome 381 and inflammation (Hoffa’s
Rheumatoid arthritis of the disease) 377
knee 382 Plica syndrome 380
Septic Septic
Osteomyelitis of the distal femur, Not applicable
proximal tibia, or proximal
fibula 380
Septic arthritis 384

RARE
Not applicable Aseptic Not applicable
Complex regional pain
syndrome 374
Septic
Not applicable

M Metabolic
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Gout 376 Not applicable
Pseudogout 381
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Chapter 21 Knee Pain 365

KNEE PAIN
LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR

Ruptures and tears: Nerve entrapments: Ruptures and tears:


• Popliteus tendon rupture 383 • Saphenous nerve • Plantaris muscle rupture 383
entrapment 379 • Popliteus tendon rupture 383

LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR

Not applicable Aseptic Aseptic


Bursitis: Tendonitis:
• Pes anserine • Hamstring tendinitis 385
bursitis 374
Tendinitis: Septic
• Pes anserine Not applicable
tendinitis 385
Septic
Not applicable

Aseptic Aseptic Aseptic


Osteomyelitis of the distal Infrapatellar fat pad Tendinitis:
femur, proximal tibia, or hypertrophy and • Popliteus tendinitis 385
proximal fibula 380 inflammation
(Hoffa’s disease) 377 Septic
Septic Plica syndrome 380 Not applicable
Not applicable
Septic
Osteomyelitis of the
distal femur, proximal
tibia, or proximal
fibula 380

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable


(continued)
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366 Chapter 21 Knee Pain

Metabolic (continued)
KNEE PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


RARE
Not applicable Not applicable Not applicable

Va Vascular
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable

UNCOMMON
Not applicable Osteochondritis dissecans 379 Not applicable

RARE
Not applicable Arteriovenous malformation 372 Deep venous thrombosis 374
Hemarthrosis 377
Sickle cell crisis 384

De Degenerative
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Primary osteoarthrosis/ Tendinoses:
osteoarthritis 381 • Patellar tendinosis (“jumper’s
knee”) 386
• Quadriceps tendinosis 387

UNCOMMON
Not applicable Not applicable Not applicable

RARE
Not applicable Not applicable Not applicable

Tu Tumor
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable

UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Malignant Primary, such as: Not applicable
• Chondrosarcoma 387
• Osteosarcoma 388
• Parosteal osteosarcoma 388
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Chapter 21 Knee Pain 367

KNEE PAIN
LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR RARE

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Not applicable Spontaneous avascular Deep venous thrombosis 374


necrosis of the medial
femoral condyle/
proximal tibia 384

Not applicable Not applicable Arteriovenous malformation 372


Popliteal artery occlusion 381

LOCAL LATERAL LOCAL MEDIAL LOCAL POSTERIOR

Lateral meniscus Medial meniscus Tendinoses:


degeneration 377 degeneration 378 • Hamstring tendinosis 386
Tendinoses:
• Pes anserine tendinosis
386

Not applicable Not applicable Tendinoses:


• Popliteus tendinosis 386

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL MEDIAL LOCAL SUPERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Malignant Primary, such as:


• Parosteal osteosarcoma 388
Malignant Metastatic:
Not applicable
(continued)
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368 Chapter 21 Knee Pain

Tumor (continued)
KNEE PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


RARE
• Synovial sarcoma 389
Malignant Metastatic, such as:
• Metastases to the knee, including
from primary breast, kidney, lung,
prostate, and thyroid disease 378
Benign, such as:
• Chondroblastoma 387
• Ganglion cysts 387
• Giant cell tumor 387
• Osteochondroma 388
• Osteoid osteoma 388
• Pigmented villonodular
synovitis 388
• Synovial chondromatosis 389

Co Congenital
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Knee Pain connected, injury oftentimes results in pathol-


ogy to multiple structures in multiple loca-
The intent of this chapter is to serve as a tions. Thus, during an examination of the
guide to differential diagnosis of knee pain knee, precise palpation in conjunction with
based on the anatomical location of pain. the history of the pain will facilitate quickly
However, because the knee joint itself has ruling in or out common diagnoses. During a
numerous structures that lie in proximity to physical therapy evaluation of the knee, typi-
each other and, in some cases, are physically cally the most common causes of knee pain,
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Chapter 21 Knee Pain 369

KNEE PAIN
LOCAL LATERAL LOCAL MEDIAL LOCAL SUPERIOR

Benign:
Not applicable

LOCAL LATERAL LOCAL MEDIAL LOCAL SUPERIOR

Not applicable Not applicable Not applicable

Discoid meniscus 375 Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL MEDIAL LOCAL SUPERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

such as ligamentous sprains, meniscal lesions, Occasionally, remote causes of knee pain that
and patellofemoral pain syndrome, are con- commonly occur may also be initially over-
sidered before the uncommon and rare looked by clinicians during an evaluation, such
causes of knee pain. as pathologies arising from the lumbar spine
One of the primary purposes of this and hip. Therefore, a secondary purpose of
chapter is to introduce diagnoses that may not this chapter is to reinforce a clinician’s knowl-
routinely be a part of the initial thought edge of the common causes of knee pain
process during an evaluation of knee pain. and to serve as a reminder to be cognizant of
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370 Chapter 21 Avascular Necrosis

diagnoses that may also originate from remote Background Information


KNEE PAIN

sources as well. This condition involves degeneration of


the articular cartilage of the femoral head
Description of Conditions That and acetabulum. Radiographs confirm
May Lead to Knee Pain the diagnosis, typically revealing a loss of
joint space, osteophytes, and subchondral
Remote bone cysts. Treatment in mild to moderate
stages involves lifestyle modification, exer-
HIP PATHOLOGIES
cises to maintain joint flexibility and
■ Avascular Necrosis strength, oral anti-inflammatory medica-
Chief Clinical Characteristics tion, and corticosteroid injections. In cases
This presentation may involve insidious of severe pain and functional limitation,
onset of groin and anterolateral thigh pain that total joint arthroplasty is considered.
radiates into the anteromedial aspect of the
knee. An antalgic gait or a slight limp accom- LUMBAR RADICULOPATHIES
panied by hip pain may be observed. Unilat- ■ L4 Radiculopathy
eral, intermittent pain that radiates from Chief Clinical Characteristics
the groin down the anterior thigh and knee This presentation can be characterized
typically indicates an intra-articular hip by pain in the lumbar spine and paresthesias
pathology.1 radiating from the anterior aspect of the
Background Information hip, thigh, and knee, sometimes ending antero-
This condition also may be associated with medially from the knee to the foot (see
sickle cell anemia, femoral head or neck Fig. 21-1). Depending on the severity, the
trauma, Gaucher’s disease, alcoholism, Cais- presentation may also include a decreased or
son disease, prolonged steroid use, irradia- absent patellar tendon reflex and motor loss
tion, or pregnancy.2 It is characterized by in the muscles innervated by the L4 nerve.
articular collapse of subchondral bone due Prone knee bend may reproduce symptoms.
to a lack of blood supply. The femoral head Background Information
is most likely to be involved. Up to 20% of A lumbar disk herniation is the most com-
individuals with this condition have bilateral mon cause of this condition. The diagnosis is
disease. Plain radiographs confirm the diag- confirmed with magnetic resonance imag-
nosis, but early detection is difficult. Surgical ing. Surgical intervention may be indicated in
decompression is typically performed to severe cases of lower extremity pain accompa-
facilitate revascularization of the femoral nied by neurological signs.
head. Hemiarthroplasty may be considered if
subchondral bone loss is extensive and the ■ L5 Radiculopathy
articular collapse is significant. Chief Clinical Characteristics
■ Hip Osteoarthrosis/ This presentation includes pain in the
Osteoarthritis lumbar spine and paresthesias radiating from
the lateral aspect of the hip and buttock to the
Chief Clinical Characteristics lateral aspect of the knee, extending antero-
This presentation may include medial, ante- laterally down to the foot (see Fig. 21-1).
rior, or lateral hip pain that may refer to the Depending on the severity, the presentation
anteromedial thigh, characterized by in- may also include motor loss in the muscles
creased pain at the end ranges of passive hip innervated by the L5 nerve root.
abduction and extension and by limping
(Fig. 21-1). This condition is the most com- Background Information
mon cause of hip pain in people older than A lumbar disk herniation is the most com-
50 years of age.3 It may be related to a prior mon cause for this condition. The diagnosis
injury or begin insidiously over the course of is confirmed with magnetic resonance imag-
many months or years. ing. Surgical intervention may be indicated
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Chapter 21 S1 Radiculopathy 371

KNEE PAIN
L2 L2 L2

S1 S1
L3 L3
L5 L5
L4 L4 L3

S2 L4 S2

L5 L5

A Anterior view
Posterior view

Femoral nerve
Obturator nerve (anterior cutaneous
nerves)
Femoral nerve
(anterior cutaneous Obturator nerve
branches)
Lateral sural
Lateral sural cutaneous nerves
cutaneous nerves Saphenous nerve

Saphenous nerve Medial sural


cutaneous nerves
B Anterior view Posterior view

Primary hip Primary hip


symptoms symptoms
Secondary hip Secondary hip
referral referral

C Anterior view Posterior view


FIGURE 21-1 Referred and projected sources of knee pain, including (A) dermatomal patterns, (B) periph-
eral nerve patterns, and (C) hip joint referral patterns.

in severe cases of lower extremity pain the buttock to the posterior aspect of the knee
accompanied by neurological signs. and extending posterolaterally from the knee
to the foot (see Fig. 21-1). Depending on the
■ S1 Radiculopathy severity, the presentation may also include a
Chief Clinical Characteristics decreased or absent Achilles tendon reflex and
This presentation typically includes pain in the motor loss in the muscles innervated by the
lumbar spine and paresthesias radiating from S1 nerve.
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372 Chapter 21 Acute Patellar Dislocation

Background Information Individuals with this condition often are


KNEE PAIN

A lumbar disk herniation is a common cause asymptomatic, so the condition is commonly


for this condition. The diagnosis is con- misdiagnosed as juvenile rheumatoid arthritis,
firmed with magnetic resonance imaging. hemophilic arthropathy, tuberculous arthritis,
Surgical intervention may be indicated in or pigmented villonodular synovitis.4 The
severe cases of lower extremity pain accom- malformation typically develops during in-
panied by neurological signs. fancy; however, symptoms may present in
adolescence if not treated. Diagnosis is con-
Local firmed with magnetic resonance imaging,
angiography, and ultrasonography. Treatment
■ Acute Patellar Dislocation
includes nonsurgical management in mildly
Chief Clinical Characteristics symptomatic or asymptomatic patients, or
This presentation includes an acute and severe surgical excision of localized lesions.
onset of pain with the patella positioned
laterally over the femoral trochlear groove. ■ Baker’s Cyst
Chief Clinical Characteristics
Background Information
This presentation can involve posterior
The definition of a complete dislocation
knee pain, stiffness, tenderness, edema,
is the absence of contact between the
and a palpable mass located posterior to the
patella and the trochlear groove. This pathol-
medial femoral condyle between the tendons
ogy presents as a severe disruption of the
of the medial head of the gastrocnemius and
extensor mechanism as a result of the patella
semimembranosus muscles. The mass will be
sliding over the lateral portion of the
rounded, smooth, supple, and transilluminating.
trochlear groove. Acute dislocations are most
often associated with trauma, but they may Background Information
also be atraumatic. When associated with This condition typically results from fluid
trauma, a major risk factor for acute patellar distention from the gastrocnemius-semi-
dislocations is frequent exposure to the membranosus bursae that communicates
primary mechanism of injury, oftentimes a with the posterior aspect of the joint capsule.
noncontact lower extremity internal rotation In adults, it may result from an intra-
and knee valgus stress on a fixed distal ex- articular pathology that leads to posterior
tremity. In nontraumatic dislocations, some effusion. Intra-articular knee disorders such
form of patellar malalignment or abnormal- as meniscal tears, primary osteoarthritis, and
ity such as patellar alta, ligamentous laxity, or rheumatoid arthritis are frequently associated
increased Q angle is frequently observed. with this condition.5,6 A common complica-
Rate of recurrence is high in children tion is rupture or dissection of fluid into the
and preadolescents. Manual reduction is gastrocnemius muscle, which may mimic
required, followed by conservative rehabili- symptoms of a deep venous thrombosis.
tation, emphasizing strengthening of the Diagnosis is confirmed by imaging studies in-
quadriceps and gluteal muscles, orthotic cluding ultrasound and magnetic resonance
therapy, and flexibility of the lateral soft imaging. Treatment typically consists of aspi-
tissue structures of the knee. Surgical inter- rating the knee effusion, although there is a
vention is indicated when patellar instability high rate of recurrence when the underlying
continues after rehabilitation. cause is unaddressed.
■ Arteriovenous Malformation BURSITIS
Chief Clinical Characteristics ■ Infrapatellar Bursitis
This presentation may involve mild pain,
Chief Clinical Characteristics
edema, and disfigurement.
This presentation includes localized edema
Background Information over the inferior aspect of the patellofemoral
This condition is a rare vascular anomaly joint and pain in the infrapatellar region with
that most frequently affects the knee joint. palpation (Fig. 21-2).
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Chapter 21 Infrapatellar Bursitis 373

Anterior view

KNEE PAIN
Patellar and prepatellar region Suprapatellar region
•Anterior cruciate ligament • Osteosarcoma
tear/rupture • Osteoid osteoma
• Osteoarthrosis/ • Quadriceps tendon rupture
osteoarthritis • Quadriceps tendinopathy
• Patellar dislocation (tendonitis/tendinosis)
• Patellar fractures
• Patellofemoral Medial joint line
pain syndrome • Avascular necrosis
• Prepatellar bursitis of the tibial condyle
• Medial collateral
ligament tear
Lateral joint line • Medial meniscus tear
• Discoid meniscus and degeneration
• Iliotibial band • Osteoarthrosis/
friction syndrome osteoarthritis
• Lateral collateral • Plica syndrome
ligament tear
and degeneration Pes anserinus
• Lateral meniscus • Pes anserine bursitis
tear • Pes anserine
• Osteoarthrosis/ tendonitis/tendinosis
osteoarthritis Gerdy’s tubercle Infrapatellar region
• Iliotibial band • Infrapatellar bursitis
friction syndrome • Infrapatellar fat pad
hypertrophy and
A inflammation
Posterior view

Hamstring
tendinopathy
Muscle strains:
• Gastrocnemius
(medial head)

Arteriovenous
malformation
Baker’s cyst
Deep vein
thrombosis
Muscle ruptures:
• Plantaris
• Popliteus
Popliteal artery
occlusion
Hamstring tendinopathy Popliteus
Muscle strains: tendinopathy
• Gastrocnemius Posterior cruciate
(lateral head) ligament tear/
B • Biceps femoris rupture
FIGURE 21-2 Surface anatomy of selected local sources of knee pain, causing either (A) anterior knee
pain or (B) posterior knee pain.
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374 Chapter 21 Pes Anserine Bursitis

Background Information Background Information


KNEE PAIN

Bursae are structures lined by synovial tissue Bursae are structures lined by synovial
that produce a small amount of fluid that is tissue that produce a small amount of
essential in decreasing friction between fluid that is essential in decreasing friction
ligaments and tendons as they stretch over between ligaments and tendons as they
bony structures. With movement dysfunction stretch over bony structures. This condi-
involving the extensor mechanism or irrita- tion involves inflammation of the prepatel-
tion to the infrapatellar fat pad, the infrapatel- lar bursa, located just superficial to the
lar bursa may become enlarged, inflamed, anterior surface of the patella. Clinical
and painful. Pain is often associated with examination confirms the diagnosis. Treat-
hyperextension or extension overpressure. ment includes avoidance of kneeling and
Clinical examination confirms the diagnosis. inflammation control.
Treatment includes relative rest and inflam-
■ Complex Regional Pain Syndrome
mation control.
Chief Clinical Characteristics
■ Pes Anserine Bursitis This presentation may include a traumatic
Chief Clinical Characteristics onset of severe knee pain accompanied by
This presentation typically includes pain, allodynia, hyperalgesia, as well as trophic,
tenderness, and localized edema at the antero- vasomotor, and sudomotor changes in later
medial aspect of the knee (see Fig. 21-2). Pa- stages.
tients may report pain when ascending stairs Background Information
and tenderness to palpation at the insertion of This condition is characterized by dispropor-
site of the semitendinosus, gracilis, and sarto- tionate responses to painful stimuli. It is a
rius insertions. Activities that involve repeti- regional neuropathic pain disorder that
tive cutting or side-to-side stepping may also presents either without direct nerve trauma
result in pes anserine bursitis. (Type I) or with direct nerve trauma
(Type II) in any region of the body.10 This
Background Information
condition may precipitate due to an event
Bursae are structures lined by synovial tissue
distant to the affected area. Thermography
that produce a small amount of fluid that is
may confirm associated sympathetic dysfunc-
essential in decreasing friction between liga-
tion. Treatment may include physical therapy
ments and tendons as they stretch over bony
interventions to improve patient and client
structures. This condition involves inflam-
functioning, biofeedback, analgesic or anti-
mation of the pes anserine bursa, which typ-
inflammatory medication, transcutaneous or
ically is located approximately 4 cm below
spinal electrical nerve stimulation, and surgical
the joint line at the anteromedial aspect of
or pharmacologic sympathectomy.
the knee.7 Chronic bursitis has been associ-
ated with degenerative joint disease of the ■ Deep Venous Thrombosis
knee or rheumatoid arthritis.8,9 Clinical
examination confirms the diagnosis. Treat- Chief Clinical Characteristics
ment includes relative rest and inflammation This presentation involves unilateral edema,
control. pain, warmth, erythema, and tenderness in the
posterior knee and calf region. Reproduction of
■ Prepatellar Bursitis pain occurs with passive dorsiflexion of the foot
in full knee extension (Homan’s sign).
Chief Clinical Characteristics
This presentation may be characterized Background Information
by superficial edema and diffuse pain over the Primary risk factors associated with a deep
anterior aspect of the knee with palpation venous thrombosis include age, prolonged
(see Fig. 21-2). The mechanism of injury immobilization, childbirth within the last
typically involves repeated minor trauma or 6 months, surgery in the last 4 weeks, major
kneeling, inciting inflammation of the subcu- trauma, cancer treatment, hormone replace-
taneous bursa over the patella. ment therapy, and long car or airplane travel
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Chapter 21 Intercondylar Eminence Fracture of the Tibia 375

in the last 4 weeks (Table 21-1). This condi- ■ Discoid Meniscus

KNEE PAIN
tion involves a blood clot (thrombus) that Chief Clinical Characteristics
develops in a deep vein, usually in the lower This presentation typically includes pain,
leg and thigh. A thrombus could interfere edema, locking, and catching. This condition
with circulation of the region, break off, and may involve a “snapping knee” or an audible,
embolize to the brain, lungs, and heart. palpable, or visible pop near terminal knee
Doppler ultrasound confirms the diagnosis. extension that most commonly presents in
Complications include severe tissue damage children.
and death, making this condition a medical
emergency. Background Information
A discoid meniscus is an anatomical variant
with a propensity for tearing and is most
TABLE 21-1 commonly present in the lateral meniscus.11,12
Does My Patient With Knee Pain Have Deep Magnetic resonance imaging confirms the
Venous Thrombosis? A Clinical Decision Rule for diagnosis. Surgical intervention usually is
Diagnosis required. The type of surgical intervention
depends on the nature of this condition.
CLINICAL FINDING SCORE*
Treatment options include partial or total
Activity cancer (treatment ongoing, 1 meniscectomies, meniscal stabilization, and
within previous 6 months, or palliative) saucerization.
Paralysis, paresis, or recent plaster 1
immobilization of the lower extremities ■ Fibromyalgia
Recently bedridden for > 3 days or 1 Chief Clinical Characteristics
major surgery within 4 weeks This presentation typically includes chronic
widespread joint and muscle pain defined as
Localized tenderness along the 1 bilateral upper body, lower body, and spine
distribution of the deep venous
system†
pain, associated with tenderness to palpation
at 11 of 18 specific muscle-tendon sites. Individ-
Entire leg swelling 1 uals with this condition will demonstrate low-
Calf swelling by > 3 cm when 1 ered mechanical and thermal pain thresholds,
compared with the asymptomatic leg‡ high pain ratings for noxious stimuli, and
Pitting edema (greater in the 1 altered temporal summation of pain stimuli.13
symptomatic leg) Background Information
Collateral superficial veins 1 The etiology of this condition is unclear;
(nonvaricose) multiple body systems appear to be involved.
Alternative diagnosis as likely or -2 Indistinct clinical boundaries between this
greater than that of PDVT§ condition and similar conditions (eg, chronic
fatigue syndrome, irritable bowel syndrome,
*A score is obtained by summing all items that are judged and chronic muscular headaches) pose a diag-
to be present; score of ≤ 0 = low probability of PDVT;
score of 1 or 2 = moderate probability of PDVT; score of nostic challenge.13 This condition is diagnosed
≥ 3 = high probability of PDVT. by exclusion. Treatment will often include
†Tenderness along the deep venous system is assessed by polypharmacy and elements to improve
firm palpation in the center of the posterior calf, the self-efficacy, physical training, and cognitive-
popliteal space, and along the area of the femoral vein behavioral techniques.14
in the anterior thigh and groin.
‡Measured with a tape measure 10 cm below tibial
tuberosity.
FRACTURES
§
More common alternative diagnoses are cellulitis, calf ■ Intercondylar Eminence
strain, Baker’s cyst, or postoperative swelling. Fracture of the Tibia
Reprinted with permission from Wells PS, Anderson DR,
Bormanis J, et al. Value of assessment of pretest
Chief Clinical Characteristics
probability of deep-vein thrombosis in clinical This presentation typically includes pain,
management. Lancet. 1997;350[9094]:1795–1798. joint effusion, and the inability to bear weight.
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376 Chapter 21 Osteochondral Fracture of the Patella

Background Information (especially in any angle of knee flexion) is


KNEE PAIN

Fracture has been reported to occur most often significantly painful.


commonly after a fall from a bicycle and the
mechanism of injury is usually a hyperexten- Background Information
sion force with or without a valgus or rota- Patellar fractures most commonly occur in
tional moment about the knee.15 This condi- relatively young patients due to a direct force
tion often is observed in patients prior to to the knee.16 In young patients, patellar frac-
skeletal maturity, resulting from injuries that tures are most often transverse due to the
would ordinarily cause anterior cruciate lig- mechanism of injury being from a distraction
ament tears in adults. When the tibia is or “pulling apart” of the patella by a forceful
forcefully anteriorly displaced on the femur, active contraction of the quadriceps.17 Com-
the incompletely ossified tibial eminence will minuted fractures result from a direct force to
fail before the anterior cruciate ligament. the patella while the quadriceps is under
The diagnosis is confirmed with plain radi- tension. Differential diagnosis includes patel-
ographs. Depending on the extent of the lar bursitis, dislocation, and bipartite patella
fracture, treatment typically includes immo- (typically asymptomatic). The diagnosis is
bilization with protected weight bearing, confirmed with plain radiographs. Usual
surgical fixation, or reduction. treatment involves immobilization, although
surgical fixation of fracture segments may be
■ Osteochondral Fracture of indicated if the fracture is displaced.
the Patella
■ Supracondylar Fracture of
Chief Clinical Characteristics
the Femur
This presentation includes patellar tender-
ness, edema, and a history of direct or indi- Chief Clinical Characteristics
rect injury. Injuries that damage articular This presentation includes pain, edema, limb
cartilage and adversely affect the subchon- deformity, and an inability to bear weight. In
dral bone are often referred to as osteochon- the absence of osteoporosis, the patient will
dral fractures. often have some history of trauma.

Background Information Background Information


This condition most commonly occurs as a Supracondylar femur fractures typically
result of a direct or indirect blow and from result from low-energy trauma to osteoporotic
patellar dislocations. Compared to adults, bone in elderly persons or high-energy
children are more susceptible to this type of trauma in young patients. These fractures
fracture because they have greater patellar are most commonly observed in elderly pa-
mobility. Diagnosis may be confirmed by tients with multiple medical comorbidities
plain radiographs. The fracture occurs at the and osteoporosis. Surgical treatment almost
point of contact, with the separate fracture always is indicated, involving reduction fol-
fragment containing articular cartilage, lowed by external or internal fixation to
subchondral bone, and supporting trabecu- maintain alignment.
lar bone. These fragments may be displaced
intra-articularly and become loose bodies, or ■ Gout
they may be in place and heal. Surgical inter- Chief Clinical Characteristics
vention for the removal of loose bodies may This presentation includes severe pain, edema,
be indicated if pain persists. warmth, erythema, and localized tenderness.
Systemic findings may include fever, chills,
■ Patellar Fracture malaise, and sweating. Patients typically re-
Chief Clinical Characteristics port an acute onset of symptoms within several
This presentation ranges from mild concerns hours. Pain may be triggered by low alcohol
of instability in the knee, to severe pain and abuse, dehydration, trauma, surgery, septic
complete inability to actively flex or extend arthritis, protein fasting, excessive purine in-
the knee. Bearing weight on the affected limb gestion, and allopurinol or uricosuric agents.
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Chapter 21 Lateral Meniscus Degeneration 377

Background Information runners, cyclists, downhill skiers, and military

KNEE PAIN
This condition is a peripheral arthritis that recruits. In runners, the pain often begins at a
results from the deposition of sodium urate predictable distance, is relieved when the knee
crystals in one or more joints. A variety of is maintained in full extension, and is aggra-
conditions, including renal disease, have been vated by repetitive knee flexion, specifically at
implicated as contributory, but most cases are 30 degrees of knee flexion.19 Factors that may
idiopathic. Gout most commonly affects the contribute to the development of this syn-
feet, ankles, hands, wrists, elbows, and knees. drome include sudden changes in training
This diagnosis is confirmed with microscopic volume, genu varus, decreased flexibility of the
study of synovial fluid aspirated from affected iliotibial band, hip abduction weakness, rear-
joints. Treatment involves medication to ame- foot varus, and pes cavus.19 Clinical examina-
liorate the underlying metabolic dysfunction. tion confirms the diagnosis. Treatment in-
volves nonsurgical interventions, such as
■ Hemarthrosis relative rest, exercise, and physical modalities
Chief Clinical Characteristics for symptom and inflammation control.
This presentation may involve joint effusion, pain
with motion and weight bearing, and a fixed ■ Infrapatellar Fat Pad
flexion deformity. The knee, ankle, and elbow Hypertrophy and Inflammation
are most susceptible to this condition.18 (Hoffa’s Disease)
Chief Clinical Characteristics
Background Information
This presentation includes pain, edema, and
Hemarthrosis is commonly associated with
tenderness over the anterior or medial aspect of
hemophilia, an X-linked hematologic disorder
the knee in the region of the patellar tendon.
characterized by a propensity to hemorrhage
Patients will report pain with end-range knee
due to the inability to produce clotting factors
extension. Palpation may reveal local tender-
VIII and IX. Acute bleeding increases the
ness and a hypertrophied infrapatellar fat pad.
pressure in the synovial cavity and bone mar-
row, which leads to severe pain and possible Background Information
avascular necrosis or pseudotumoral mass. Inflammation of the infrapatellar fat pad
Intra-articular hemorrhage may occur sponta- may result from the impingement of the fat
neously or result from insignificant trauma. pad in the tibiofemoral joint during knee
Diagnosis is confirmed with ultrasound, plain extension or from direct trauma.20 This diag-
radiographs, and magnetic resonance imaging. nosis may present as an acute or chronic con-
Treatment involves a combination of factor dition. Clinical examination confirms the di-
replacement, joint aspiration, rest (with or agnosis. Acute management includes rest and
without splinting), joint injections of radioac- inflammation control. In chronic conditions,
tive substances to control hemorrhage, and restoration of extension range of motion,
surgical joint replacements for end-stage strength, and quadriceps muscle flexibility
disease. should be emphasized.
■ Iliotibial Band Friction Syndrome ■ Lateral Meniscus Degeneration
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation typically includes a gradual This presentation can involve localized pain in
and progressive onset of pain and localized ten- the region of the lateral joint line. Pain is wors-
derness over the lateral aspect of the knee that ened with squatting activities and ascending/
increases with activities involving repetitive descending stairs (see Fig. 21-2). Reports of
knee flexion (see Fig. 21-2). locking and catching are less common with
degenerative tears as opposed to acute trau-
Background Information
matic tears.
This condition is an inflammatory injury that
results from friction between the iliotibial Background Information
band and the lateral femoral condyle. It is This condition occurs when the collagen fibers
commonly observed among long-distance within the meniscus start to break down and
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378 Chapter 21 Medial Meniscus Degeneration

lend less support to the structure of the menis- Background Information


KNEE PAIN

cus. Age is a risk factor; 60% of individuals The skeletal system is the third most common
over the age of 65 present with this condi- site of metastatic disease.23 Symptoms also
tion.21 Degenerative tears may result from may be related to pathological fracture in
repetitive activities over time such as squatting affected sites. Common primary sites causing
and kneeling, but may also occur over time metastases to bone include breast, prostate,
secondary to trauma or previous history of lung, and kidney. Bone scan confirms the diag-
surgery. Magnetic resonance imaging confirms nosis. Common treatments for metastases in-
the diagnosis. Nonsurgical intervention is clude surgical resection, chemotherapy, radia-
commonly attempted first, and severe cases tion treatment, and palliation, depending on
may require partial or total knee replacements. the tumor type and extent of metastasis.
■ Medial Meniscus Degeneration MUSCLE STRAINS
Chief Clinical Characteristics ■ Biceps Femoris Muscle Strain
This presentation may include localized pain Chief Clinical Characteristics
in the region of the medial joint line. Patient This presentation can involve pain and ten-
reports pain with squatting activities and derness over the posterolateral thigh and knee,
ascending/descending stairs (see Fig. 21-2). with a history of sudden onset during activ-
Background Information ity. Symptoms include pain with resisted knee
Degenerative meniscal tears occur as part of flexion and passive knee extension. The biceps
the aging process when the collagen fibers femoris muscle is the most commonly injured
within the meniscus start to break down and muscle of the hamstring complex,24–26 often
lend less support to the structure of the menis- occurring in athletes who run, kick, and jump.
cus. Age is a risk factor; 60% of individuals Background Information
over the age of 65 have degenerative meniscal Injury is most likely to occur while the muscu-
tears.21 Degenerative tears may result from lotendinous junction undergoes maximum
repetitive activities over time such as squatting strain during an eccentric contraction of the
and kneeling, but may also occur over time hamstrings. A hamstring strain can occur dur-
secondary to trauma or previous history of ing an isolated event or result from persistent
surgery. Patients are less likely to report lock- repetitive stress. Ecchymosis and edema are
ing and catching with degenerative tears as typically present in second- and third-degree
opposed to acute traumatic tears. Diagnosis is strains. The diagnosis is confirmed on the
confirmed with magnetic resonance imaging. basis of clinical examination. Treatment in-
Degenerative tears are typically associated with cludes inflammation control and strength and
articular cartilage degeneration; therefore, flexibility exercises. Caution should be taken to
arthroscopic surgical outcomes may not be as avoid early aggressive stretching. Surgical inter-
successful as those for acute traumatic tears. vention is required only in the case of complete
Nonsurgical intervention is commonly at- rupture of the proximal or distal attachment.
tempted first. Severe cases may require partial
or total knee replacements. ■ Gastrocnemius Muscle Strain
■ Metastases to the Knee, Including Chief Clinical Characteristics
From Primary Breast, Kidney, This presentation may involve pain and ten-
Lung, Prostate, and Thyroid derness to palpation at the origin of the gas-
Disease trocnemius muscle and posterior calf. De-
pending on the severity of the injury, pain
Chief Clinical Characteristics
may radiate into the ankle. Symptoms are
This presentation typically includes unremitting
aggravated by passive ankle dorsiflexion and
pain in individuals with these risk factors: pre-
active ankle plantarflexion.
vious history of cancer, age 50 years or older, fail-
ure to improve with conservative therapy, and Background Information
unexplained weight change of more than This condition results from a forceful
10 pounds in 6 months.22 push-off with the foot. Tennis, jumping, hill
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Chapter 21 Osteochondritis Dissecans 379

running, and sprinting are commonly asso- crossed-legged, prolonged immobility in bed

KNEE PAIN
ciated with this injury. The medial head of against bedrails or firm mattresses, trauma,
the gastrocnemius is most frequently in- squatting, crouching, kneeling, and idio-
volved. Clinical examination confirms the pathic origins. Dynamic entrapments also
diagnosis. Treatment includes rest, inflam- may occur during activities such as running.
mation control, gentle, pain-free ankle range The common peroneal nerve may be injured
of motion, use of a heel lift, and strength and at any location along the nerve; however,
flexibility exercises. Caution should be taken entrapment most frequently occurs at the
to avoid early, aggressive stretching. fibular head. The nerve may become com-
pressed under the fibrous arch in the region
■ Semimembranosus Muscle where the bifurcation of the nerve into its
Strain deep and superficial branches occurs.27,28 An
Chief Clinical Characteristics electrodiagnostic evaluation, including a
This presentation includes a sudden onset of nerve conduction velocity test and needle
pain and tenderness over the posteromedial electromyography, may confirm the diagno-
thigh and knee. Ecchymosis and edema are sis. Treatment is generally conservative;
more commonly present in second- and third- however, surgical decompression may be
degree strains. Symptoms include pain with indicated in recalcitrant cases in which
resisted knee flexion and passive knee exten- the anatomical site of entrapment is well
sion. Strains most commonly occur in ath- characterized.
letes who run, kick, and jump.
■ Saphenous Nerve Entrapment
Background Information Chief Clinical Characteristics
Injury is most likely to occur while the mus- This presentation involves pain and/or pares-
culotendinous junction undergoes maxi- thesias in the medial thigh and knee, tender-
mum strain during eccentric contraction of ness to palpation over the adductor canal,
the hamstrings. This condition can occur fol- and normal motor function of the affected
lowing an isolated event or persistent repeti- extremity. Symptoms include a deep ache that
tive stress. Clinical examination confirms the may radiate into the foot along the saphe-
diagnosis. Treatment usually includes inflam- nous nerve distribution. Symptoms are exac-
mation control, strength, and flexibility. erbated by prolonged walking or standing.
Caution should be taken to avoid early,
aggressive stretching. Surgical intervention is Background Information
required only in the case of complete rupture Entrapment typically occurs where the
of the proximal or distal attachment. saphenous nerve pierces the fascia of the
adductor canal, resulting in inflammation.
NERVE ENTRAPMENTS Mechanisms for saphenous nerve entrap-
■ Common Peroneal Nerve ment may be traumatic, nontraumatic, or
Entrapment at the Fibular Head iatrogenic (eg, following knee surgery or
Chief Clinical Characteristics saphenous vein harvest). Diagnosis may be
This presentation may be characterized by a confirmed with injection of local anesthetic.
partial or total loss of sensation in the distri- Symptoms typically improve following an
bution of the peroneal nerve. Weakness with injection with a local anesthetic and steroids
ankle dorsiflexion and extension of the toes and and avoiding aggravating activities. Neuroly-
a positive Tinel’s sign at the fibular head may sis or neurectomy may be performed if non-
also be present. surgical treatment fails in recalcitrant cases
in which the anatomical site of entrapment
Background Information is well characterized.29
Pain is an uncommon feature unless it is
related to the specific cause of the nerve ■ Osteochondritis Dissecans
entrapment, such as entrapment secondary Chief Clinical Characteristics
to soft tissue swelling and inflammation This presentation typically includes vague knee
from direct trauma. Causes include sitting pain and effusion without a history of recent
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380 Chapter 21 Osteomyelitis of the Distal Femur, Proximal Tibia, or Proximal Fibula

trauma. Pain increases with activity and patient knee may be present. Symptoms occur with
KNEE PAIN

may report a history of locking or catching if a jumping, running, or quick changes in direction.
loose body is present.
Background Information
Background Information The most common mechanism of patellar dis-
This condition is a partial or total separation location is lower extremity internal rotation
of intra-articular bone fragment and/or artic- with combined knee valgus on a planted foot
ular cartilage without a history of specific (noncontact). Younger children and adoles-
trauma. It is associated with acute bone necro- cents are at greater risk for instability. A num-
sis. In adults and adolescents, these lesions are ber of risk factors are associated with patellar
classically located on the lateral aspect of the instability, including ligament laxity, decreased
medial femoral condyle.30 This diagnosis is strength and muscle mass, patella alta, in-
confirmed with plain radiographs, computed creased Q angle, increased femoral antever-
tomography, or magnetic resonance imaging. sion, iliotibial band tightness, and excessive
Treatment includes immobilization and rest; midfoot pronation. Treatment typically in-
however, surgical treatment may be indicated volves rehabilitation, although surgical inter-
if no progression toward healing is seen on vention is indicated if patellar instability con-
radiographs or if the lesion becomes unstable. tinues after rehabilitation, with concomitant
osteochondral lesions, if palpable disruption
■ Osteomyelitis of the Distal Femur, of the medial patellofemoral ligament-vastus
Proximal Tibia, or Proximal medialis obliquus-adductor mechanism
Fibula occurs, or if participation in high-level athlet-
Chief Clinical Characteristics ics is required.
This presentation involves local pain, edema, and
erythema with associated systemic findings such ■ Patellofemoral Pain Syndrome
as malaise, chills, night sweats, and an abrupt Chief Clinical Characteristics
onset of fever. This presentation involves pain in the anterior
aspect or deep inside the knee joint (see
Background Information
Fig. 21-2). Symptoms may begin insidiously or
This condition is an acute or chronic infection
following trauma. Patients report pain with
of bone secondary to infection with pyogenic
squatting, prolonged sitting, and ascending or
organisms. The two primary types of acute
descending stairs.
osteomyelitis are direct inoculation and
hematogenous. Direct inoculation osteomyelitis Background Information
primarily occurs in adults and is the result In the absence of trauma or acute inflamma-
of direct contact of tissue and bacteria tion, minimal effusion will be present and pain
during trauma or surgery. Hematogenous os- with palpation is often absent. The mechanism
teomyelitis is an infection caused by bacterial of injury can present acutely following an
seeding from the blood and most commonly episode of trauma to the knee joint (falling or
occurs in children. The diagnosis is confirmed any compressive force directly on the knee) or
with blood panels demonstrating elevated symptoms may develop over a prolonged
white blood cell count and plain radiographs. period of time (overuse from repeated run-
Treatment involves intravenous antibiotic ning or squatting activities). Treatment in-
therapy and surgical treatment of the lesion. volves relative rest, proximal lower extremity
muscle strengthening and flexibility exercises,
■ Patellar Instability and physical modalities for symptom control.
Chief Clinical Characteristics
This presentation includes vague anterior knee ■ Plica Syndrome
pain and swelling with a history of patellar Chief Clinical Characteristics
subluxations or reports of the knee “giving way.” This presentation typically includes an acute
A positive patellar apprehension test and onset of pain and tenderness just anterior and
palpable tenderness over the lateral condyle, medial to the joint line. Localized tenderness over
medial soft tissue, or the anterior aspect of the the medial femoral condyle and medial patella
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Chapter 21 Reiter’s Syndrome 381

may be present. Patient may report snapping is the most common joint disease for middle-aged

KNEE PAIN
between 50 and 70 degrees of knee flexion.20 and older individuals.32
Symptoms may mimic loose bodies, a meniscal
Background Information
lesion, or patellofemoral pain syndrome.
Primary osteoarthrosis/osteoarthritis involves
Background Information a progressive loss of articular cartilage, remod-
Plicae are redundant folds of synovium, eling and sclerosis of subchondral bone, and
located in the knee, that may become inflamed osteophyte formation. Risk factors include age,
and symptomatic as a result of direct trauma joint injury, excessive repetitive joint loading,
or overuse. There are three major types of and joint dysplasia.32 Plain radiographs con-
plica: the infrapatellar, suprapatellar, and firm the diagnosis. Nonoperative treatment
mediopatellar plicae. The mediopatellar plica may include inflammation control, patient
is most often implicated. Arthroscopy is the education for weight control, exercise (avoid-
gold standard for diagnosis of plica syn- ing high-impact activities), and foot orthoses.
drome.31 Nonsurgical intervention is typically In severe cases, osteotomy, arthroscopy,
initiated first, which includes patellar mobi- chondroplasty, or joint replacement may be
lization, stretching, and anti-inflammatory indicated.
medication. Surgical intervention to excise
the pathological plica is rare, but may be ■ Pseudogout
successful. Chief Clinical Characteristics
■ Popliteal Artery Occlusion This presentation includes warmth and ery-
thematous, tender, and asymmetrical edema
Chief Clinical Characteristics of the knee. This condition is characterized by
This presentation includes an acute onset of an insidious onset of symptoms over several
severe claudication, dependent rubor, absent days. The most commonly affected joints are
ankle pulses, and decreased temperature to the knees, wrists, and shoulders.
palpation distally.
Background Information
Background Information Many cases are idiopathic; however, pseudo-
In severe chronic conditions, pain at rest, gout also has been associated with aging,
cyanosis, or a nonhealing ischemic ulcer may trauma, and metabolic abnormalities such as
be present. This condition is caused by hyperparathyroidism and hemochromatosis.
trauma, atherosclerosis, emboli, popliteal This condition involves joint inflammation
artery aneurysm, cystic adventitial disease, and caused by calcium pyrophosphate crystals and
popliteal entrapment syndrome. Patients at is often referred to as calcium pyrophosphate
risk include elderly patients and patients with disease. This diagnosis is confirmed with
diabetes mellitus and cardiovascular disease. microscopic study of synovial fluid aspirated
Diagnosis is confirmed with angiography, from affected joints. Treatment involves med-
ultrasonography, and the ankle brachial index. ication to ameliorate the underlying metabolic
Surgical and nonsurgical treatment of an arte- dysfunction.
rial occlusion depends on the cause of the
occlusion, with interventions ranging from ■ Reiter’s Syndrome
drug therapy to bypass surgery. Chief Clinical Characteristics
■ Primary Osteoarthrosis/ This presentation may be characterized by joint
Osteoarthritis pain and stiffness, involved with a classic triad
of arthritis, urethritis, and conjunctivitis.
Chief Clinical Characteristics
Disease incidence peaks during the third decade
This presentation typically includes joint pain,
of life, with a male-to-female ratio of 5:1.33
stiffness, and radiographic evidence of articu-
lar cartilage degeneration. Other signs and Background Information
symptoms may include limited range of This condition is typically preceded by either
motion, intermittent aching associated with an episode of dysentery or infectious arthritis,
activity, or constant deep pain. This condition and individuals with the HLA-B27 genetic
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382 Chapter 21 Rheumatoid Arthritis of the Knee

makeup are at greater risk. Asymmetric During varus stress testing, pain with no
KNEE PAIN

arthropathy involving the knee, ankle, foot, joint laxity is a Grade I (stretch) injury.
and sacroiliac joint is common. It is generally a Laxity with a firm end-feel is a Grade II
self-limiting disease that typically resolves in (partial tear) injury and no firm end-feel is a
3 to 4 months. However, it is common for Grade III (complete tear) injury.34 Clinical
approximately half of all patients to have examination and magnetic resonance imag-
recurring symptoms. Medications are used in ing confirm the diagnosis. Grade I, II, and III
the treatment of the disease and physical ther- injuries are managed nonsurgically.34,35
apy intervention should be targeted toward Surgical repair may be necessary to address
restoration of range of motion, flexibility, and associated meniscal or combined ligament
strength. tears.
■ Rheumatoid Arthritis of the Knee ■ Lateral Meniscus Tear
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes morning stiffness This presentation may involve pain, lateral
and generalized pain throughout multiple joints joint line tenderness, and reports of catching,
in a symmetric distribution, with possible ten- clicking, and locking (see Fig. 21-2). Mild joint
derness and swelling of affected joints. Women line effusion is present and pain or a palpa-
are twice as likely to be affected as men. ble click may be provoked with McMurray’s
and Apley’s compression tests.
Background Information
Symptoms associated with this progressive Background Information
inflammatory joint disease are caused by syn- Mechanism of injury involves an acute, non-
ovial membrane thickening and cytokine pro- contact rotatory force with the knee flexed
duction in synovial fluid. Articular cartilage and the foot planted. Meniscal compromise
erosion, synovial hypertrophy, and constant leads to increased stress on the articular
joint effusion eventually cause bony erosions cartilage and early degenerative changes.
and joint deformities that have a significant Magnetic resonance imaging confirms this
impact on daily function.50 Younger age of diagnosis.36–38 Tears located in the periph-
onset is associated with a greater extent of dis- eral one-third of the meniscus respond well
ability later. Plain radiographs and blood tests to surgical intervention. Some researchers
confirm the diagnosis. Treatment typically in- advocate that tears in the middle one-third
cludes a variety of steroidal, nonsteroidal, and zone also be repaired.39,40
biological anti-inflammatory medications.
■ Medial Collateral Ligament
RUPTURES AND TEARS Sprain/Rupture
■ Lateral Collateral Ligament Chief Clinical Characteristics
Sprain/Rupture This presentation includes pain, localized
Chief Clinical Characteristics edema along the medial aspect of the knee, and
This presentation involves pain, localized medial joint line tenderness (see Fig. 21-2).
edema along the lateral aspect of the knee, and Pain and/or laxity is present with valgus stress
lateral joint line tenderness (see Fig. 21-2). Pain testing at 30 degrees of knee flexion. This in-
and/or laxity is present with varus stress jury generally results in minimal effusion and
testing at 30 degrees of knee flexion. This in- pain with walking; however, it also may cause
jury generally results in minimal effusion and difficulty with running and cutting.
pain with walking; however, the patient may
report difficulty with running and cutting Background Information
activities. Mechanism of injury involves a valgus stress
applied to the knee, such as a direct blow to
Background Information the lateral aspect of the knee. The incidence
Mechanism of injury is typically from a of medial meniscus tears increases with
varus stress applied to the knee, such as a increased severity of the sprain because of its
direct blow to the medial aspect of the knee. attachment to the medial collateral ligament.
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Chapter 21 Posterior Cruciate Ligament Sprain/Rupture 383

With valgus stress testing, pain with no joint Rupture of the plantaris muscle rarely occurs

KNEE PAIN
laxity is a Grade I injury (stretch). Laxity in isolation and is most commonly associ-
with a firm end-feel is a Grade II injury ated with strain of the medial head of the
(partial tear) and no firm end-feel is a gastrocnemius.41,42 The diagnosis is con-
Grade III injury (complete tear).34 Clinical firmed by magnetic resonance imaging. This
examination and magnetic resonance imag- condition is typically treated nonsurgically.
ing confirm the diagnosis. Tears located in When surgical intervention is indicated, the
the peripheral one-third of the meniscus retracted muscle is resected.41
respond well to surgical intervention. Some
researchers advocate that tears in the middle ■ Popliteus Tendon Rupture
one-third zone also be repaired.39,40 Chief Clinical Characteristics
This presentation involves acute, lateral joint
■ Medial Meniscus Tear line and posterolateral knee pain and
Chief Clinical Characteristics decreased range of motion. Acute symptoms
This presentation can be characterized by may be associated with a crack or pop at the
pain, medial joint line tenderness, and time of the injury and difficulty bearing
reports of catching, clicking, and locking (see weight on the affected limb.
Fig. 21-2). Mild joint line effusion is present
Background Information
and pain or a palpable click may be provoked
Mechanism of injury involves a rapid exter-
with McMurray’s and Apley’s compression
nal rotation of the tibia with the knee in a
tests. Mechanism of injury involves an acute,
flexed and fixed position; however, rupture
noncontact rotatory force with the knee flexed
may also occur without a history of
and the foot planted.
trauma.43 Isolated ruptures of the popliteus
Background Information tendon are rare. This condition occurs more
The medial meniscus is more commonly in- frequently with posterolateral corner injuries
jured than the lateral meniscus because it is in which there is concomitant disruption of
less mobile.39 This condition often is associ- the arcuate ligament complex, the lateral
ated with medial collateral ligament injuries collateral ligament, the anterior/posterior
because of its rigid attachment to the liga- cruciate ligaments, or the menisci.44 The
ment and the joint capsule. Meniscal diagnosis is confirmed with magnetic reso-
compromise leads to increased stress on the nance imaging. Intervention requires surgi-
articular cartilage and early degenerative cal reattachment of the ruptured tendon.
changes. Magnetic resonance imaging con-
firms the diagnosis.36–38 Typically, tears ■ Posterior Cruciate Ligament
located in the peripheral one-third of the Sprain/Rupture
meniscus respond well with surgical inter- Chief Clinical Characteristics
vention. Some researchers advocate that This presentation typically includes pain,
tears in the middle one-third zone also be edema, and tenderness in the region of the
repaired.39,40 popliteal fossa. Positive posterior sag sign
of the tibia with the hip and knee flexed to
■ Plantaris Muscle Rupture 90 degrees and a positive posterior drawer
Chief Clinical Characteristics test may be present.
This presentation may include pain, tender-
Background Information
ness, the presence of a hematoma, and retrac-
Mechanism of injury is typically from a
tion of the muscle over the proximal, postero-
hyperextension force or a direct anterior
medial aspect of the calf. A “pop” in the calf
blow to the knee in a flexed position. This
is commonly experienced. This condition
condition occurs with falls onto a flexed
results from a forceful push-off with the foot.
knee with the foot in plantarflexion, causing
Background Information the tibial tubercle to contact the ground first,
Tennis, jumping, hill running, and sprinting and in motor vehicle accidents resulting
are commonly associated with this injury. from contact with the dashboard. Magnetic
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384 Chapter 21 Quadriceps Tendon Rupture

resonance imaging confirms the diagnosis. Treatment includes antibiotic therapy and
KNEE PAIN

Even complete ruptures are not usually aspiration. Surgical intervention may be
repaired surgically. necessary in cases that do not respond to
aspiration.
■ Quadriceps Tendon Rupture
Chief Clinical Characteristics ■ Sickle Cell Crisis
This presentation typically involves acute, Chief Clinical Characteristics
severe pain over the anterior aspect of the This presentation includes an acute, severe on-
knee and the inability to actively extend the set of pain and limited range of motion that may
knee. Superior dislocation of the patella and be associated with fever, cold weather, dehy-
hemarthrosis often result immediately. A dration, infection, and physical/psychological
palpable gap in the suprapatellar region may stress in individuals with sickle cell anemia.46
be present, although this is often masked by
Background Information
hemarthrosis. This condition is frequently
An acute exacerbation of the signs and symp-
observed in patients older than 40 years of
toms associated with sickle cell disease is
age and in patients with comorbid medical
known as a crisis. There are four patterns of an
conditions, such as metabolic disease, obesity,
acute crisis based on their location: bone crisis,
and long-term steroid use.45
acute chest syndrome, abdominal crisis, and
Background Information joint crisis. During a bone crisis, the tibia,
Mechanism of injury is often associated with femur, and humerus are commonly involved
a strong concentric contraction of the and single or multiple joints may be affected.
quadriceps in association with forced flexion This condition is a group of inherited disor-
of the knee. Diagnosis is confirmed with ders with abnormalities caused by hemoglobin
magnetic resonance imaging. Incomplete S. This condition typically is associated with
ruptures are typically managed nonsurgi- pain due to tissue infarction and/or worsening
cally with the knee immobilized in full ex- anemia. Diagnosis of a crisis is confirmed
tension for 6 weeks followed by protected with plain radiographs and magnetic reso-
range of motion and strengthening once the nance imaging. This condition is a medical
patient is able to exhibit good quadriceps emergency.
control and perform a straight leg raise with
minimal discomfort. Surgical repair is indi- ■ Spontaneous Avascular Necrosis
cated for complete ruptures. of the Medial Femoral
Condyle/Proximal Tibia
■ Septic Arthritis Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can be characterized by a
This presentation may involve severe aching sudden onset of severe medial knee pain that is
pain, edema, erythema, stiffness, general exacerbated by weight-bearing activities and fre-
malaise, and fever. The fever may be low grade quently present at rest and worst at night. This
and there also may be a restriction to active condition most commonly occurs after the sixth
and passive range of motion. decade of life, and women are affected three
times more frequently than men.47–51
Background Information
Common risk factors include rheumatoid Background Information
arthritis, immunodeficiency, intravenous drug This condition is the result of circulatory
use, and joint replacement. This condition impairments to an area of bone that result in
involves inflammation of the synovial mem- infarct due to either primary vascular insuffi-
brane with purulent effusion into the joint ciency, minor trauma, or repetitive insults,
capsule, usually due to bacterial infection. leading to microfractures in the subchondral
In adults, septic arthritis most commonly bone. Unilateral involvement dominates and
affects the knee. The diagnosis is confirmed by lesions most frequently are observed at either
elevated white blood cell count and erythro- the medial femoral condyle or the medial
cyte sedimentation rate and needle aspiration. tibial plateau.47–49,51,52 The diagnosis is
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Chapter 21 Quadriceps Tendinitis 385

confirmed with magnetic resonance imaging. ■ Pes Anserine Tendinitis

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This diagnosis may be managed nonsurgically Chief Clinical Characteristics
with protective weight bearing, inflammation This presentation typically includes acute
control, and strengthening of the quadriceps pain, tenderness, and edema localized an-
and hamstring muscles. When surgical inter- teromedially (see Fig. 21-2). Patient reports
vention is required, treatment options are pain with ascending stairs and tenderness to
arthroscopy, tibial osteotomy, osteochondral palpation at the insertion of site of the three
grafts, core decompression, unicondylar knee tendons that comprise the pes anserine group
arthroplasty, and total knee arthroplasty. (semitendinosus, gracilis, and sartorius).
TENDINITIS Background Information
■ Hamstring Tendinitis Pain is typically located approximately 4 cm
below the joint line at the anteromedial
Chief Clinical Characteristics
aspect of the knee.7 This diagnosis is often
This presentation involves acute, localized
observed in long-distance runners.53 Symp-
pain, tenderness, and edema over the involved
toms may mimic or be associated with pes
tendon. Symptoms include pain with resisted
anserine bursitis. Treatment includes rest,
knee flexion and passive knee extension. His-
control of inflammation, orthotic therapy,
tory is usually significant for repetitive ham-
and strengthening to reduce stress on the
string muscle strains.
medial structures of the knee.
Background Information
This condition most commonly occurs in ■ Popliteus Tendinitis
athletes who run, kick, and jump. Clinical Chief Clinical Characteristics
examination and magnetic resonance imag- This presentation involves acute pain, ten-
ing confirm an acute inflammation of the derness, and edema over the posterolateral
hamstring tendon. Treatment includes rest, aspect of the knee and along the proximal
inflammation control, and improving mus- course of the popliteal tendon. Activities that
cle flexibility of the quadriceps and ham- require frequent deceleration, such as down-
string muscles. hill running or hiking, may produce and
exacerbate symptoms. Reproduction of symp-
■ Patellar Tendinitis toms occurs with resisted tibial external rota-
Chief Clinical Characteristics tion at 90 degrees of knee flexion.
This presentation may be characterized by Background Information
acute, localized pain and edema at the infe- Overuse or fatigue of the quadriceps may
rior pole of the patella (origin), the tibial lead to inflammation of the popliteus mus-
tubercle (insertion), or the patellar tendon culotendinous unit, causing overuse of the
itself (see Fig. 21-2). Pain is most often local- popliteus musculotendinous unit.54 Mag-
ized at the insertion of the patellar tendon and netic resonance imaging confirms the diag-
onset is typically insidious; however, most nosis. Treatment includes rest and control of
patients may relate a period of onset with inflammation.
increased activity or sport. Patient reports
pain with squatting and jumping activities. ■ Quadriceps Tendinitis
Chief Clinical Characteristics
Background Information
This presentation may involve acute pain, ten-
Mechanism of injury is most often from
derness, and edema on the anterior aspect of the
repetitive eccentric overload of the knee in
thigh, just along the superior border of the
flexion ranges, resulting in microscopic
patella (see Fig. 21-2). Patient reports pain with
destruction of the patellar tendon. Magnetic
eccentric or concentric quadriceps contractions.
resonance imaging confirms the diagnosis.
Treatment includes rest, inflammation con- Background Information
trol, and improving muscle flexibility of the This condition typically results from repetitive
quadriceps and hamstring muscles. hyperextension of the hip or a combination of
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386 Chapter 21 Hamstring Tendinosis

hip extension and knee flexion. Injury is degeneration and the disordered, haphazard
KNEE PAIN

most often associated with athletic activity, healing of collagen with vascular ingrowth.55
such as running and jumping. Diagnosis is Clinical examination, ultrasonography, and
confirmed with magnetic resonance imag- magnetic resonance imaging confirm this
ing. Treatment includes rest, inflammation diagnosis. Present research supports the use
control, and improving muscle flexibility of of eccentric exercise to reduce pain and facil-
the quadriceps and hamstring muscles. itate return to sport in chronic cases.57,58
TENDINOSES ■ Pes Anserine Tendinosis
■ Hamstring Tendinosis Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically includes a history
This presentation includes a history of chronic of chronic pain and tenderness localized an-
pain and tenderness over the involved ten- teromedially (see Fig. 21-2). Patient reports
don with the absence of inflammation. Symp- pain when ascending stairs and tenderness
toms include pain with resisted knee flexion to palpation at the insertion of site of the
and passive knee extension. History is usually three tendons that comprise the pes anserine
significant for repetitive hamstring muscle group (semitendinosus, gracilis, and sarto-
strains. rius). Pain is typically located approximately
4 cm below the joint line at the anteromedial
Background Information aspect of the knee.7 This diagnosis is often
This condition most commonly occurs in observed in long-distance runners.53
athletes who run, kick, and jump. Tendinosis
is characterized as noninflammatory intra- Background Information
tendinous collagen degeneration and the Tendinosis is characterized as noninflamma-
disordered, haphazard healing of collagen tory intratendinous collagen degeneration and
with vascular ingrowth.55 Clinical examina- the disordered, haphazard healing of collagen
tion, ultrasonography, and magnetic reso- with vascular ingrowth.55 Clinical examina-
nance imaging confirm this diagnosis. A tion, ultrasonography, and magnetic reso-
combination of patient/client education, nance imaging confirm this diagnosis. Treat-
unloading the affected musculotendinous ment includes rest, control of inflammation,
unit, controlled mechanical reloading, and orthotic therapy, and strengthening to reduce
preventive measures appear effective to stress on the medial structures of the knee.
manage this condition.56
■ Popliteus Tendinosis
■ Patellar Tendinosis (“Jumper’s Chief Clinical Characteristics
Knee”) This presentation can be characterized by a
Chief Clinical Characteristics chronic history of pain over the posterolateral
This presentation may include a history of aspect of the knee and tenderness along the
chronic pain at the inferior pole of the patella proximal course of the popliteal tendon. Re-
(origin), the tibial tubercle (insertion), or the production of symptoms occurs with resisted
patellar tendon itself (see Fig. 21-2). Pain is tibial external rotation at 90 degrees of knee
most often localized at the insertion of the flexion.
patellar tendon and onset is typically insidi-
Background Information
ous; however, most patients may relate a
Overuse or fatigue of the quadriceps may
period of onset with increased activity or
lead to inflammation of the popliteus mus-
sport. Patient reports pain with squatting and
cle, causing overuse of the popliteus muscu-
jumping activities.
lotendinous unit.54 Thus, activities that
Background Information require frequent deceleration, such as down-
Mechanism of injury is most often from hill running or hiking, may produce and
repetitive eccentric overload of the knee in exacerbate symptoms. Tendinosis is charac-
flexion ranges. Tendinosis is characterized as terized as noninflammatory intratendinous
noninflammatory intratendinous collagen collagen degeneration and the disordered,
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Chapter 21 Giant Cell Tumor 387

haphazard healing of collagen with vascular imaging studies including plain radiographs,

KNEE PAIN
ingrowth.55 Clinical examination, ultra- bone scan, and magnetic resonance imaging.
sonography, and magnetic resonance imag- Treatment consists of curettage, en bloc
ing confirm this diagnosis. A combination resection, and radiation therapy.60
of patient/client education, unloading the
affected musculotendinous unit, controlled ■ Chondrosarcoma
mechanical reloading, and preventive meas- Chief Clinical Characteristics
ures appears to be effective for managing this This presentation includes pain that is dull in
condition.56 character, has been present for months, and
may be worst at night. When the tumor is
■ Quadriceps Tendinosis
located near a joint, effusion and limited
Chief Clinical Characteristics range of motion may be present.
This presentation includes a history of chronic
pain on the anterior aspect of the thigh, just Background Information
along the superior border of the patella, with- This condition is a malignant tumor of carti-
out signs of inflammation (see Fig. 21-2). Pa- laginous origin, in which the tumor matrix
tient reports pain with eccentric or concentric formation is entirely chondroid in nature
quadriceps contractions. and may arise within the medullary canal or
in the periphery as either a primary or sec-
Background Information ondary lesion. Tumors are most commonly
Quadriceps tendinosis typically results from found in the pelvis, femur, humerus, ribs,
repetitive hyperextension of the hip or a scapula, sternum, and spine. The proximal
combination of hip extension and knee flex- metaphysis is more frequently involved than
ion. Injury is most often associated with ath- the distal end of the bone. This type of tumor
letic activity, such as running and jumping. is generally unresponsive to chemotherapy,
Tendinosis is characterized as noninflamma- thus treatment involves surgical resection.
tory intratendinous collagen degeneration
and the disordered, haphazard healing of ■ Ganglion Cysts
collagen with vascular ingrowth.55 Clinical Chief Clinical Characteristics
examination, ultrasonography, and magnetic This presentation involves a nonpainful or
resonance imaging confirm this diagnosis. minimally painful soft tissue mass. Pain, click-
A combination of patient/client education, ing and locking, decreased range of motion,
unloading the affected musculotendinous and effusion are commonly present with
unit, controlled mechanical reloading, and intra-articular cysts.
preventive measures appears to be effective
for managing this condition.56 Background Information
This condition frequently is found at the in-
TUMORS sertion sites of ligaments, near the region of
■ Chondroblastoma the epiphysis, and may result from minor
trauma or be congenital in origin. Intra-
Chief Clinical Characteristics articular cysts typically present with greater
This presentation typically includes nonspe- losses of range of motion compared to in-
cific pain, edema, local tenderness to palpa- traosseous cysts.61 Diagnosis is confirmed by
tion, decreased range of motion, and joint plain radiographs and magnetic resonance
stiffness. This condition most commonly pres- imaging. Aspiration or surgical excision may
ents between the ages of 10 and 20 years, with be necessary in some cases; however, most
a male-to-female ratio of 2:1.59 cysts do not require surgical intervention.
Background Information
This condition arises from the development ■ Giant Cell Tumor
of a benign neoplasm of cartilaginous ori- Chief Clinical Characteristics
gin. It is typically found in the epiphysis or This presentation can be characterized by
apophysis of long bones in younger individ- pain and tenderness but the tumor is typically
uals. Diagnosis is confirmed with biopsy and asymptomatic.
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388 Chapter 21 Osteochondroma

Background Information plain radiographs. Pain associated with this


KNEE PAIN

If pain is present, it may be the result of an condition is self-limiting and may resolve
associated inflammatory response. This con- spontaneously over the course of 2 to
dition involves benign, locally aggressive 4 years, but surgical excision also may be
bone tumors that occur between the ages of effective.66
20 and 40 years and affects females more
often than males.62 They are frequently ■ Osteosarcoma
found in the epiphysis of long bones, with Chief Clinical Characteristics
the majority located at the distal femur and This presentation may involve localized pain,
proximal tibia. Giant cell tumors are charac- soft tissue swelling or mass, muscle atrophy,
terized by their locally aggressive behavior. and decreased knee range of motion.
Diagnosis is confirmed by plain radiographs
Background Information
and computed tomography. Treatment may
This condition is the most common bone
include radiation therapy, intralesional
malignancy. It is more common in males
curettage with and without bone graft or in-
than females, with a peak incidence in the
sertion of polymethylmethacrylate, cryother-
second decade of life.67 The most com-
apy, and surgical resection.
monly affected sites are the distal femur,
■ Osteochondroma proximal tibia, and proximal humerus.
Diagnosis is confirmed with a biopsy. This
Chief Clinical Characteristics
type of tumor is generally unresponsive
This presentation involves a palpable mass
to chemotherapy, thus treatment involves
that is typically painless. This condition is
surgical resection.
typically asymptomatic, but a decrease in
range of motion may present depending on the ■ Parosteal Osteosarcoma
location of the lesion.
Chief Clinical Characteristics
Background Information This presentation can be characterized by an
This condition is the most common benign insidious onset of pain, edema, decreased
tumor of bone that originates near the ends range of motion, and a palpable mass.
of long bones and typically grows away
Background Information
from the joint. They generally occur as single
This condition is a variant of osteosarcoma
lesions, with most occurring at the knee.63,64
and is often found in the metaphysis of long
Plain radiographs confirm the diagnosis; this
bones; it is present on the posterior aspect of
condition often is incidentally detected
the distal femur in 75% of cases.68 The lesion
while examining films obtained for another
arises from the surface of the bone and has a
reason. Treatment generally involves surgical
tendency to encircle the bone. Radiographi-
resection of the lesion.
cally, parosteal osteosarcoma is characterized
■ Osteoid Osteoma by a large, dense, lobulated mass broadly
attached to the underlying bone without
Chief Clinical Characteristics
involvement of the medullary canal. Diagno-
This presentation may include localized pain,
sis is confirmed with biopsy and imaging
often greatest at night, and adjacent soft tis-
studies, including plain radiographs, mag-
sue edema. Classically, pain is readily relieved
netic resonance imaging, and computed
by aspirin.
tomography. Wide excision of the lesion
Background Information is the preferred treatment and there is nor-
This condition is a benign skeletal neoplasm mally no role for chemotherapy.
of unknown etiology that is composed of
osteoid and woven bone and is most fre- ■ Pigmented Villonodular
quently found in young children and adoles- Synovitis
cents.65 It is usually found in the long bones Chief Clinical Characteristics
of the lower extremity, particularly the prox- This presentation may involve diffuse, recur-
imal femur. The diagnosis is confirmed with rent edema that is initially pain free, repeated
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Chapter 21 Synovial Sarcoma 389

hemarthrosis in the absence of trauma, ■ Synovial Sarcoma

KNEE PAIN
progressive and insidious onset of pain, Chief Clinical Characteristics
palpable nodules, and decreased range of This presentation may include a dull, deep pain
motion. Symptoms may include locking and that is accompanied by a slowly enlarging
catching. mass. Vague pain may occur for months with-
Background Information out a mass being appreciated. Tumors located
This condition is a benign, proliferative near the joint may also result in decreased
pathology of unknown etiology that affects range of motion.
synovial tissue. It results in various degrees Background Information
of villous and/or nodular changes in the This condition is a malignant mesenchymal
joint, with the knee being the most com- neoplasm that arises from soft tissue, repre-
monly involved.69–72 The two forms of the senting 10% of all soft tissue sarcomas and
disorder are diffuse and focal. The diffuse most commonly affecting the lower extrem-
form involves large joints and the entire syn- ities of young adults.75 Diagnosis is con-
ovial lining, resulting in destructive changes firmed with biopsy and imaging studies
to the joint. The focal form involves small including plain radiographs, computed
joints, such as the hands and feet, and results tomography scan, and magnetic resonance
in mechanical symptoms such as locking and imaging. Treatment includes a combination
catching. Diagnosis is confirmed with plain of chemotherapy and surgical resection.
radiographs and magnetic resonance imag-
ing. Treatment includes synovectomy and
subsequent radiation therapy. References
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■ Synovial Chondromatosis pain: diagnosis and medical treatment, circa 2004. Clin
Orthop Relat Res. Jan 2004(418):9–17.
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ovial membrane proliferation and metapla- 368–372.
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CHAPTER22
Lower Leg Pain
■ Jason R. Cozby, PT, DPT, OCS

Description of the Symptom Special Concerns


■ Decreased sensation
This chapter describes pathology that may lead ■ Diminished femoral, popliteal, tibial, or
to lower leg pain, including generalized, an- dorsalis pedis pulses
teromedial, anterolateral, and posterior lower ■ Fever or signs of systemic disease
leg pain. Local causes of lower leg pain are de- ■ Inability to bear weight on the affected
fined as pathology occurring between the lower extremity
proximal one-third of the tibia and the area ■ Pain increased at night and/or rest
just proximal to the medial and lateral malleoli ■ Palpable warmth and swelling
of the tibia. Remote causes are defined as ■ Skin breakdown
occurring outside this region.

Anterior Lateral
lower Medial Anterior
lower
leg lower lower
leg
leg leg

Anterior Anterior

CHAPTER PREVIEW: Conditions That May Lead to Lower Leg Pain

T Trauma
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Lumbar radiculopathies: Not applicable Fractures:
• L4 radiculopathy 400 • Stress fracture of the tibia 405
• L5 radiculopathy 400 • Tibia 405
• S1 radiculopathy 400 Shin splints:
• Posteromedial shin splints 411

392
1528_Ch22_392-416 07/05/12 1:53 PM Page 393

LOWER LEG PAIN


Posterior
lower Posterior
leg lower
leg

Anterior Anterior
Medial lower lower
lower leg leg
leg

Posterior Posterior

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Muscle strains: Muscle strains:


• Anterior compartment muscle strain 407 • Gastrocnemius muscle strain 407
• Lateral compartment muscle strain 407 • Soleus muscle strain 407
Shin splints: Rupture of the Achilles tendon 411
• Anterolateral shin splints 411
(continued)

393
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394 Chapter 22 Lower Leg Pain

Trauma (continued)
LOWER LEG PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL


UNCOMMON
Not applicable Not applicable Nerve entrapments:
• Saphenous nerve 408

RARE
Not applicable Not applicable Not applicable

I Inflammation
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Not applicable Aseptic Aseptic
Delayed onset muscle soreness Tendinitis:
secondary to exercise 403 • Tibialis posterior tendinitis 413
Septic Septic
Cellulitis 402 Infectious periostitis 406
Erythema nodosum 404
UNCOMMON
Not applicable Aseptic Aseptic
Complex regional pain Tendinitis:
syndrome 403 • Tibialis anterior tendinitis 413
Gas gangrene 405
Nodular panniculitis 409 Septic
Osteomyelitis of the tibia 410
Septic
Erysipelas 404

RARE
Not applicable Aseptic Not applicable
Erythema induratum 404
Septic
Necrotizing fasciitis 408
Nonclostridial myositis 409

M Metabolic
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Not applicable Distal symmetric polyneuropathy, Not applicable
including from acquired
immunodeficiency syndrome,
alcohol/drug abuse,
carcinoma, diabetes mellitus,
and kidney failure 404
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Chapter 22 Lower Leg Pain 395

LOWER LEG PAIN


LOCAL ANTEROLATERAL LOCAL POSTERIOR

Fractures: Hematoma of the calf 406


• Fibula 405 Nerve entrapments:
Nerve entrapments: • Sciatic nerve 408
• Superficial peroneal nerve 408 • Sural nerve 408
• Tibial nerve 409

Not applicable Not applicable

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Aseptic Aseptic
Tendinitis: Tendinitis:
• Peroneal tendinitis 413 • Achilles tendinitis 412
Septic Septic
Not applicable Not applicable

Aseptic Aseptic
Paratenonitis of the extensor tendons of the Achilles paratenonitis 401
foot and toes 410
Tendinitis: Septic
• Extensor digitorum longus tendinitis 412 Not applicable
• Extensor hallucis longus tendinitis 413
Septic
Not applicable

Aseptic Aseptic
Not applicable Inflammatory muscle disease 406
Septic Septic
Pyomyositis 410 Pyomyositis 410

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Not applicable Benign muscle cramps 402

(continued)
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396 Chapter 22 Lower Leg Pain

Metabolic (continued)
LOWER LEG PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL


UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Malnutrition 406 Not applicable

Va Vascular
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Acute femoropopliteal arterial Compartment syndromes:
occlusion 401 • Distal deep posterior
Sickle cell crisis 411 compartment syndrome 402
Skin ulcers: Greater saphenous vein
• Arterial or hypertensive thrombophlebitis 405
ulcers 412
• Systemic vasculitis with
ulceration 412
RARE
Not applicable Not applicable Not applicable

De Degenerative
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Baker’s cyst 399 Not applicable Tendinoses:
• Tibialis posterior tendinosis 415
UNCOMMON
Not applicable Not applicable Tendinoses:
• Tibialis anterior tendinosis 415

RARE
Not applicable Not applicable Not applicable

Tu Tumor
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
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Chapter 22 Lower Leg Pain 397

LOWER LEG PAIN


LOCAL ANTEROLATERAL LOCAL POSTERIOR

Not applicable Not applicable

Not applicable Acute rhabdomyolysis 401

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Not applicable Posterior tibial vein thrombosis 410

Anterior tibial vein thrombosis 401 Compartment syndromes:


Compartment syndromes: • Posterior compartment syndrome 403
• Anterior compartment syndrome 402

Compartment syndromes: Arteriovenous malformation 402


• Lateral compartment syndrome 403

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Tendinoses: Tendinoses:
• Peroneal tendinosis 414 • Achilles tendinosis 413

Tendinoses: Not applicable


• Extensor digitorum longus tendinosis 414
• Extensor hallucis longus tendinosis 414

Not applicable Not applicable

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Not applicable Not applicable

Not applicable Not applicable


(continued)
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398 Chapter 22 Lower Leg Pain

Tumor (continued)
LOWER LEG PAIN

REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL


RARE
Not applicable Malignant Primary, such as: Malignant Primary:
• Osteosarcoma 410 Not applicable
Malignant Metastatic, such as: Malignant Metastatic:
• Metastases to the lower leg, Not applicable
including from primary breast, Benign, such as:
kidney, lung, prostate, and • Osteoid osteoma 409
thyroid disease 406
• Multiple myeloma 407
Benign, such as:
• Osteoid osteoma 409

Co Congenital
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL GENERALIZED LOCAL ANTEROMEDIAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Fear and avoidance 400 Not applicable Not applicable
RARE
Psychological effect of Not applicable Not applicable
complex regional
pain syndrome 400

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Lower Leg Pain trauma accounts for many of the potential di-
agnoses, multiple peripheral nerves and blood
The lower leg is subject to internal trauma as vessels traverse the region and therefore diag-
a result of ground reaction forces, stress noses may also be related to vascular compro-
from the powerful muscle attachments in the mise, a peripheral nerve compression, or sys-
region, or forces that may occur to correct de- temic disease. The lower leg is often an end
ficiencies at the foot, ankle, or hip. These defi- target site for multiple disease processes, so
ciencies may be weakness, congenital anom- even if mechanical trauma is associated with
alies, limitations in flexibility, or decreased the presentation, diagnosis should consider all
range of motion, to name a few. Although potential possibilities.
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Chapter 22 Baker’s Cyst 399

LOWER LEG PAIN


LOCAL ANTEROLATERAL LOCAL POSTERIOR

Malignant Primary: Malignant Primary:


Not applicable Not applicable
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign, such as: Benign, such as:
• Osteoid osteoma 409 • Osteoid osteoma 409

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Not applicable Not applicable

Not applicable Not applicable

Not applicable Not applicable

LOCAL ANTEROLATERAL LOCAL POSTERIOR

Not applicable Not applicable

Not applicable Not applicable

Not applicable Not applicable

condyle between the tendons of the medial head


Description of Conditions That
of the gastrocnemius and semimembranosus
May Lead to Lower Leg Pain
muscles. The mass will be rounded, smooth,
Remote supple, and transilluminating.
■ Baker’s Cyst Background Information
Chief Clinical Characteristics This condition typically results from fluid disten-
This presentation involves posterior knee pain, tion from the gastrocnemius-semimembranosus
stiffness, tenderness, edema, and a palpable bursae that communicates with the posterior
mass located posterior to the medial femoral aspect of the joint capsule. In adults, it also
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400 Chapter 22 Fear and Avoidance

may result from an intra-articular pathology ■ L5 Radiculopathy


LOWER LEG PAIN

that leads to posterior effusion. Intra-articular Chief Clinical Characteristics


knee disorders such as meniscal tears, primary This presentation includes pain in the
osteoarthritis, and rheumatoid arthritis are lumbar spine and paresthesias radiating
frequently associated with this condition.1,2 from the lateral aspect of the hip and
A common complication is rupture or dissec- buttock to the lateral aspect of the knee,
tion of fluid into the gastrocnemius muscle, extending anterolaterally down to the foot.
which may mimic symptoms of a deep venous Depending on the severity, the presentation
thrombosis. Diagnosis is confirmed by imaging may also include motor loss in the muscles
studies including ultrasound and magnetic res- innervated by the L5 nerve root.
onance imaging. Treatment typically consists
of aspirating the knee effusion, although there Background Information
is a high rate of recurrence when the underly- A lumbar disk herniation is the most com-
ing cause is unaddressed. mon cause for this condition. The diagnosis
is confirmed with magnetic resonance imag-
■ Fear and Avoidance ing. Surgical intervention may be indicated
Chief Clinical Characteristics in severe cases of lower extremity pain
This presentation may include avoiding the use accompanied by neurological signs.
of an extremity as a result of apprehension
■ S1 Radiculopathy
about onset of pain.
Chief Clinical Characteristics
Background Information This presentation typically includes pain in the
Injury to the foot and ankle may contribute to lumbar spine and paresthesias radiating from
the disuse of the lower extremity even with the buttock to the posterior aspect of the knee
minimal injury. Fear of reinjury, pain, or other and extending posterolaterally from the knee
factors will need to be explored. In all cases to the foot. Depending on the severity, the
the basis of pathology must be thoroughly ex- presentation may also include a decreased or
plored before this presentation is considered. absent Achilles tendon reflex and motor loss
If this condition is present, a team approach in the muscles innervated by the S1 nerve.
ensures optimal treatment.
Background Information
LUMBAR RADICULOPATHIES A lumbar disk herniation is a common cause
■ L4 Radiculopathy for this condition. The diagnosis is con-
firmed with magnetic resonance imaging.
Chief Clinical Characteristics Surgical intervention may be indicated in
This presentation can be characterized by severe cases of lower extremity pain accom-
pain in the lumbar spine and paresthesias panied by neurological signs.
radiating from the anterior aspect of the
hip, thigh, and knee, sometimes extending ■ Psychological Effect of Complex
anteromedially from the knee to the foot. Regional Pain Syndrome
Depending on the severity, the presentation Chief Clinical Characteristics
may also include a decreased or absent patel- This presentation typically includes a traumatic
lar tendon reflex and motor loss in the onset of severe chronic ankle and foot pain ac-
muscles innervated by the L4 nerve. Prone companied by allodynia, hyperalgesia, and, in
knee bend may reproduce symptoms. later stages, trophic, vasomotor, and sudomotor
changes. This condition is characterized by
Background Information
disproportionate responses to painful stimuli.
A lumbar disk herniation is the most com-
mon cause for this condition. The diagnosis Background Information
is confirmed with magnetic resonance imag- This regional neuropathic pain disorder
ing. Surgical intervention may be indicated presents either without direct nerve trauma
in severe cases of lower extremity pain (Type I) or with direct nerve trauma (Type II)
accompanied by neurological signs. in any region of the body.3 This condition
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Chapter 22 Anterior Tibial Vein Thrombosis 401

may precipitate due to an event distant to and/or burning pain as a result of an occlusion

LOWER LEG PAIN


the affected area. Thermography may con- at the bifurcation just distal to the last detectable
firm associated sympathetic dysfunction. pulse.
This condition has been shown to have a
psychological component and may be influ- Background Information
enced by cortical mechanisms.4 Treatment Common causes include trauma, embolus,
may include physical therapy interventions or thrombus.5 Symptoms often will reverse if
to improve patient/client functioning, biofeed- the occlusion is treated and relieved within
back, analgesic or anti-inflammatory medica- 6 hours; otherwise, complications include
tion, transcutaneous or spinal electrical nerve gangrene and residual weakness or sensory
stimulation, and surgical or pharmacologic impairment. Diagnosis is most often con-
sympathectomy. However, a team approach firmed with Doppler ultrasonography; how-
ensures optimal treatment for individuals with ever, angiography, assessment of systolic blood
this condition. pressure, and magnetic resonance angiography
also may be utilized. This condition is a med-
Local ical emergency in its acute form due to the risk
for compromise of the distal tissues. Treat-
■ Achilles Paratenonitis ment usually involves pharmacologic or surgi-
Chief Clinical Characteristics cal thrombolysis.
This presentation typically includes diffuse pain,
■ Acute Rhabdomyolysis
tenderness, and thickening and swelling along
the Achilles tendon, especially 2 to 6 cm from the Chief Clinical Characteristics
calcaneal insertion. However, the painful pal- This presentation includes calf pain, generalized
pable thickening does not move with dorsiflex- tenderness, swelling, darkened urine, and aggra-
ion or plantarflexion because the tendon moves vation of symptoms with bearing weight on the
with this action but the paratenon does not. affected lower extremity and muscle contraction.
Pain is greatest in the mornings or at the start Background Information
of a physical activity, but the pain will often Toxic syndrome from alcoholic binge drinking
decrease with continued walking or activity as is the most common etiology, followed by
the tendon begins to move more freely inside the trauma and infection.6 Urine darkening associ-
paratenon. ated with this condition is caused by the excre-
Background Information tion of myoglobin. The diagnosis is confirmed
The paratenon is usually the site of inflamma- with histopathological testing that reveals mus-
tory injury related to overuse, but it may also cle fiber necrosis and myophagocytosis. This
be damaged from direct traumatic pressure. condition requires urgent referral for evaluation
The diagnosis is determined clinically, but by a physician.
magnetic resonance or ultrasound imaging
■ Anterior Tibial Vein Thrombosis
may be necessary for confirmation if the clin-
ical diagnosis is unclear. Treatment includes Chief Clinical Characteristics
rest, inflammation control, improving flexi- This presentation can be characterized by
bility of the calf muscle group, and improving anterolateral lower leg pain, tenderness, lower
strength of the antipronator (supinator) muscle extremity swelling, cramping, erythema, palpa-
groups. ble warmth, engorged veins, and potentially a
low-grade fever and pain along the course of the
■ Acute Femoropopliteal Arterial vein. Individuals with this condition usually
Occlusion demonstrate thromboses at other sites within the
lower leg. This condition may be effort induced
Chief Clinical Characteristics
or spontaneous.
This presentation usually involves diffuse lower
leg pain, coldness, pallor, paresthesias, muscle Background Information
weakness, and diminished or absent distal The etiology of this condition is associated
pulses. The onset may be abrupt with deep achy with Virchow’s triad of intimal damage, stasis,
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402 Chapter 22 Arteriovenous Malformation

or changes in blood composition.7 Computed ■ Cellulitis


LOWER LEG PAIN

tomography, magnetic resonance angiography, Chief Clinical Characteristics


and Doppler ultrasonography confirm the This presentation may involve pain, erythema,
diagnosis. It is a medical emergency because of edema, warmth, and tenderness spreading
the risk for propagation of the thrombus to from the region of the initial infection. Vesi-
the lungs. cles, bullae, and necrosis may be present with
occasional petechiae and ecchymosis. Fever,
■ Arteriovenous Malformation chills, headaches, malaise, hypotension, tachy-
Chief Clinical Characteristics cardia, and delirium are common signs of
This presentation includes lower leg pain and systemic involvement.
fatigue in the presence of possible limb hyper-
trophy, hypotrophy, or length discrepancy. Nu- Background Information
merous clinical constellations of skin, muscle, This condition usually results from a wound
and skeletal complications are common, in- infection by staphylococcal and streptococcal
cluding skin ulceration secondary to impaired species. Edema is a significant risk factor. Lab-
peripheral circulation.8 These changes are usu- oratory tests help determine the causative
ally present in early childhood, although they pathogen. Most cases are self-limiting. Severe
may remain unaddressed until they cause cases involve necrosis and may require surgical
disability in adulthood. debridement and antibiotic therapy.

Background Information COMPARTMENT SYNDROMES


Arteriovenous malformations include vascular ■ Anterior Compartment
tumors (eg, hemangiomas) and vascular mal- Syndrome
formations. Vascular malformations may in- Chief Clinical Characteristics
clude capillary, lymphatic, venous, and arterial This presentation typically includes early signs
deformities. The specific etiology of arteriove- of paresthesia and hypesthesia in the anterior
nous malformation is suggested by magnetic lower leg and dorsal foot, followed by signif-
resonance imaging. Treatment may involve icant pain, massive edema, ecchymoses, soft
surgical resection and thrombosis treatment tissue tenderness, erythema, palpable warmth,
techniques. and weakness of the foot and toe dorsiflexor
muscle group. Pulses and capillary refill often
■ Benign Muscle Cramps
remain normal until the pressure increases
Chief Clinical Characteristics to the level of severe injury.
This presentation involves painful muscle
cramps, palpable tightness, and induration that Background Information
is often correlated with a sustained contraction This condition can occur spontaneously in
of the affected muscle.9 This condition may last patients with diabetes mellitus.10 Altered
several minutes to approximately one-half hour, vessel perfusion and edema result in in-
with tenderness for several hours following an creased intercompartmental pressure that
acute episode. disrupts the oxygen diffusion and results in
ischemia.11 This condition often is the result
Background Information of trauma. Clinical presentation and inter-
This condition is associated with an insidious compartmental pressure measurements con-
onset following muscle overuse. Other poten- firm the diagnosis.12 This condition is a
tial causes include dehydration, hypona- medical emergency and requires urgent sur-
tremia, hypomagnesemia, geriatric nocturnal gical decompression.
leg cramps, or association with pregnancy.9
Treatment typically involves addressing the ■ Distal Deep Posterior
underlying cause of muscle cramps. Quinine Compartment Syndrome
sulfate is an established pharmacologic treat- Chief Clinical Characteristics
ment that also has potential adverse cardiac This presentation may be characterized by
side effects. early signs of paresthesia and hypesthesia in
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Chapter 22 Delayed Onset Muscle Soreness Secondary to Exercise 403

the lower leg and foot, followed by signifi- lower leg and foot, followed by significant

LOWER LEG PAIN


cant pain, massive edema, ecchymosis, pain, massive edema, ecchymosis, soft tissue
soft tissue tenderness, erythema, palpable tenderness, erythema, palpable warmth, and
warmth, and weakness of the foot plan- weakness of the plantarflexors or toe flexors.
tarflexor-invertors and toe flexor muscle Pulses and capillary refill often remain nor-
groups. Pulses and capillary refill often re- mal until the pressure increases to the level
main normal until the pressure increases to of severe injury.
the level of severe injury.
Background Information
Background Information This condition can occur spontaneously in
This condition can occur spontaneously in patients with diabetes mellitus.10 Altered
patients with diabetes mellitus.10 Altered vessel perfusion and edema result in in-
vessel perfusion and edema result in in- creased intercompartmental pressure that
creased intercompartmental pressure that disrupts the oxygen diffusion and results
disrupts the oxygen diffusion and results in in ischemia.11 This condition often is the
ischemia.11 This condition often is the result result of trauma. Clinical presentation and
of trauma. Clinical presentation and inter- intercompartmental pressure measurements
compartmental pressure measurements con- confirm the diagnosis.12 This condition is
firm the diagnosis.12 This condition is a a medical emergency and requires urgent
medical emergency and requires urgent sur- surgical decompression.
gical decompression.
■ Complex Regional Pain Syndrome
■ Lateral Compartment Syndrome Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may include a traumatic
This presentation can involve early signs of onset of severe chronic ankle and foot pain ac-
paresthesia and hypesthesia, followed by companied by allodynia, hyperalgesia, and,
significant pain, massive edema, ecchymo- in later stages, trophic, vasomotor, and sudo-
sis, soft tissue tenderness, erythema, palpa- motor changes. This condition is character-
ble warmth, and weakness of the foot ized by disproportionate responses to painful
plantarflexor-evertor muscle group. Pulses stimuli.
and capillary refill often remain normal
Background Information
until the pressure increases to the level of
This regional neuropathic pain disorder pres-
severe injury.
ents either without direct nerve trauma (Type I)
Background Information or with direct nerve trauma (Type II) in any re-
This condition can occur spontaneously in gion of the body.3 This condition may precipi-
patients with diabetes mellitus.10 Altered tate due to an event distant to the affected area.
vessel perfusion and edema result in in- Thermography may confirm associated sympa-
creased intercompartmental pressure that thetic dysfunction. Treatment may include
disrupts the oxygen diffusion and results in physical therapy interventions to improve pa-
ischemia.11 This condition often is the result tient/client functioning, biofeedback, analgesic
of trauma. Clinical presentation and inter- or anti-inflammatory medication, transcuta-
compartmental pressure measurements con- neous or spinal electrical nerve stimulation, and
firm the diagnosis.12 This condition is a surgical or pharmacologic sympathectomy.
medical emergency and requires urgent sur-
gical decompression. ■ Delayed Onset Muscle Soreness
Secondary to Exercise
■ Posterior Compartment Chief Clinical Characteristics
Syndrome This presentation may be characterized by pain,
Chief Clinical Characteristics soreness, swelling, restricted range of motion, and
This presentation typically includes early tenderness to palpation and stretching. Self-
signs of paresthesia and hypesthesia in the limiting symptoms are usually aggravated with
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404 Chapter 22 Distal Symmetric Polyneuropathy

bearing weight on the affected lower extremity The lower legs and feet are primarily
LOWER LEG PAIN

or muscle contraction, but also may be present affected, but involvement of the arms and face
at rest. also is common.13
Background Information Background Information
This condition is caused by muscle damage This condition results from a superficial inter-
and immune response following exercise at an digital fungal infection with lymphatic in-
unaccustomed intensity and duration. Clinical volvement. Blood tests and pathological stain-
examination confirms the diagnosis. Blood ing techniques confirm the diagnosis and
panels may reveal increased serum creatine ki- guide medical management. Antibiotic med-
nase levels, which usually begin to increase 24 ications directed toward the underlying
to 48 hours following exercise. Rare complica- infective agent comprise the typical treatment
tions may include rhabdomyolysis or myoglo- for this condition.
binuria. This condition is usually self-limiting, ■ Erythema Induratum
but can respond favorably to gentle flexibility
exercises. Chief Clinical Characteristics
This presentation can involve brown or blue
■ Distal Symmetric Polyneuropathy, subcutaneous nodules on the lower leg that may
Including From Acquired be accompanied by painful ulcerations that are
Immunodeficiency Syndrome, present for a significant length of time. Necro-
Alcohol/Drug Abuse, Carcinoma, sis or plaques also may be present.
Diabetes Mellitus, and Kidney Background Information
Failure This condition is a dermatological disorder
Chief Clinical Characteristics that is most common to the calf. Diagnosis is
This presentation may be characterized by de- confirmed with biopsy of the nodules or ulcer-
creased distal deep tendon reflexes, as well as pain, ations. This condition may be treated with rest
dysesthesia, or paresthesia in the feet and toes. in combination with a polypharmaceutical an-
Background Information tibiotic approach. Intralesional corticosteroids
Peripheral neuropathy is one of the more com- to address inflammation also may be necessary.
mon complications in patients presenting with ■ Erythema Nodosum
acquired immunodeficiency syndrome, dia-
betes, renal failure, hypothyroidism, toxins, Chief Clinical Characteristics
malignancy, and malnutrition. It is character- This presentation can include pretibial pain,
ized by retrograde axonal degeneration, so this edema, and arthralgia that may be associated
condition commonly progresses from distal to with knee or ankle pain. Characteristic findings
proximal. Electromyographic studies and include bilateral red subcutaneous nodules that
biopsy confirm the diagnosis. Vibration and may resemble bruises in the early stages, later
light touch sensation (10-g Semmes Weinstein changing to a darker brown. Systemic signs of
monofilament) tests are useful to assess protec- infection often include fever and malaise.
tive sensation. Curative treatment of this con- Background Information
dition remains controversial, so the focus of in- This condition most commonly appears in in-
tervention typically includes patient education dividuals ranging from 20 to 40 years of age.
to maintain blood glucose control and care for Upper respiratory infections are the common
the insensate regions to prevent progression source of infection in children, while strepto-
and complications secondary to this condition. coccal and sarcoidal infections are the com-
mon causes in adults. Diagnosis is often one of
■ Erysipelas exclusion. Diagnosis may be assisted with
Chief Clinical Characteristics determination of the causative agent. Also,
This presentation may include tenderness, shiny many individuals presenting with this condi-
and red erythema, induration, vesicles, bullae, and tion often have an elevated erythrocyte
possibly lymphadenopathy. Fever, chills, and sedimentation rate. This condition often
malaise are common signs of systemic involvement. resolves spontaneously along with treatment of
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Chapter 22 Greater Saphenous Vein Thrombophlebitis 405

the underlying infection, although anti- swelling, ecchymosis, crepitus, and pain with

LOWER LEG PAIN


inflammatory and antibiotic medications also vibration through the tibia.
may be used.
Background Information
FRACTURES The pain is usually severe when the fracture
is located in the tibial shaft. Limb shortening
■ Fibula
may be seen in the involved extremity. Frac-
Chief Clinical Characteristics tures of the tibia tend to be of either trau-
This presentation typically includes pain, poor matic or pathological etiology. Traumatic
tolerance to bearing weight on the affected fractures are more common and related to
lower extremity, tenderness to palpation, direct trauma. Pathological fractures are
swelling, and ecchymosis, with possible crepi- associated with comorbidities such as metas-
tus and pain with vibration. tases, leukemia, bone lesions, or osteoporo-
Background Information sis. Plain radiographs confirm the diagnosis
This condition has either traumatic or and guide medical management. Depending
pathological etiology. It is more commonly on fracture stability, treatment options
traumatic and may be associated with frac- range from immobilization to reduction and
tures of the tibia. Isolated fibula fractures are fixation.
more commonly associated with ankle in- ■ Gas Gangrene
juries. Pathological fractures may have an
unknown etiology, but are often associated Chief Clinical Characteristics
with comorbidities of metastases, leukemia, This presentation may involve severe local
bone lesions, or osteoporosis. The diagnosis wound pain, edema, tenderness to palpation,
is confirmed with plain radiographs. Depend- pallor, a malodorous brown and serous dis-
ing on fracture stability, treatment options charge, and, in the later stages, gas crepitation.
range from immobilization to reduction and Skin may appear pale at the early stages and later
fixation. turn a red or bronze color. Systemic signs of
infection may include pallor, diaphoresis, fever,
■ Stress Fracture of the Tibia hypotension, apathy, drowsiness, agitation,
Chief Clinical Characteristics stupor, or coma.
This presentation includes pain and palpable Background Information
tenderness over the posteromedial border of This condition is caused by a clostridial infec-
the tibia. Pain is aggravated with bearing tion that usually follows trauma to deep tissues
weight on the affected lower extremity, such or infection of postoperative wounds. This
as with running; it is alleviated with rest. condition can progress within several hours to
Background Information several days. It is a medical emergency. Diag-
This condition usually occurs in runners and nosis is confirmed with Gram staining. Surgi-
is associated with an abrupt increase in cal exploration may be necessary to reveal the
training. The clinical examination, plain extent of myonecrosis.14
radiographs, and bone scan confirm the
■ Greater Saphenous Vein
diagnosis. The fracture usually heals in
Thrombophlebitis
approximately 6 to 8 weeks as a result of no
weight bearing or weight-bearing restrictions. Chief Clinical Characteristics
In the meantime, continued activity in a re- This presentation includes anteromedial lower
duced-impact environment may be considered leg pain, tenderness, edema, erythema, palpa-
to maintain general strength and conditioning. ble warmth, engorged veins, and possibly a low-
grade fever and pain along the course of the
■ Tibia vein. An indurated cord may be palpated along
Chief Clinical Characteristics the course of the vein.
This presentation may involve pain, poor Background Information
tolerance to bearing weight on the affected This condition involves thrombosis and in-
lower extremity, tenderness to palpation, flammation of the involved vein. The etiology
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406 Chapter 22 Hematoma of the Calf

of this condition is associated with Virchow’s with antibiotic medication appropriately


LOWER LEG PAIN

triad of intimal damage, stasis, and changes in directed to the infective agent.
blood composition7; it usually occurs due to
trauma to the vein or surrounding tissue, but ■ Inflammatory Muscle Disease
also may present spontaneously. Saphenous Chief Clinical Characteristics
venous thrombosis is correlated with hyperco- This presentation includes a gradual onset of
agulability.15 Computed tomography, magnetic mild muscle pain associated with proximal
resonance angiography, and Doppler ultra- muscle weakness that causes difficulty with
sonography confirm the diagnosis. It is a med- daily activities such as walking, ascending
ical emergency because of the risk for propaga- and descending stairs, and rising from chairs.
tion of the thrombus to the lungs. Background Information
■ Hematoma of the Calf This condition describes a group of patholog-
ically, histologically, and clinically distinct dis-
Chief Clinical Characteristics orders: polymyositis, dermatomyositis, and in-
This presentation can involve pain, swelling, clusion body myositis. They may be associated
tenderness to palpation, and occasional ecchy- with other collagen, vascular, and immune dis-
mosis with symptoms very similar to throm- orders. Although proximal extremity weakness
bophlebitis. Pain is aggravated with bearing is a classic finding, up to 50% also demonstrate
weight on the affected lower extremity, muscle distal weakness that may be equally as severe.16
contraction, and passive ankle dorsiflexion. Blood panels help confirm the diagnosis and
Symptoms may have an abrupt or gradual on- track disease activity, revealing elevated serum
set, depending on the nature of the bleed. levels of creatine phosphokinase. Treatment
Background Information typically involves steroidal, nonsteroidal, and
Intercompartmental pressure may elevate to biological anti-inflammatory medications.
the level of a compartment syndrome, causing
neuralgia or ischemia. This condition is caused ■ Malnutrition
by muscle tear, fracture, direct blunt trauma, Chief Clinical Characteristics
or rupture of the lower extremity vasculature. This presentation typically includes muscle
The clinical examination in combination with cramps and muscular pain that more often
magnetic resonance or ultrasound imaging affect the calf musculature. Systemic signs may
confirms the diagnosis. This condition may be include lethargy, mental and physical fatigue,
managed with evacuation of the hematoma apathy, impaired learning ability, diminished
and rest. immune system function, and delayed healing
response.
■ Infectious Periostitis
Background Information
Chief Clinical Characteristics
Morphological changes are observed in the
This presentation may be characterized by
later stages. Etiology includes starvation, alco-
aching to severe pain with tenderness and
holic malnutrition, inadequate diet, eating dis-
edema of the infected bone. Suppuration may
orders, or imbalanced diet. Hypomagnesemia
be present and an individual with this condi-
and hyponatremia are common causes of the
tion will often exhibit systemic signs of infection,
muscular symptoms. Clinical and biochemical
such as fever, chills, and fatigue.
tests confirm the diagnosis. Treatment in-
Background Information cludes amelioration of diet.
This condition involves a chronic infection of
the periosteum from a hematogenous source ■ Metastases to the Lower Leg,
or trauma to the affected region. Not all cases Including From Primary Breast,
of periostitis are the result of an infection; Kidney, Lung, Prostate, and
therefore, the diagnosis is confirmed by radi- Thyroid Disease
ographic imaging revealing necrosis of the af- Chief Clinical Characteristics
fected bone and bone scan revealing increased This presentation typically includes unremitting
uptake. This condition is usually managed pain in individuals with these risk factors:
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Chapter 22 Soleus Muscle Strain 407

previous history of cancer, age 50 years or older, Background Information

LOWER LEG PAIN


failure to improve with conservative therapy, The three dorsiflexor muscles in the antero-
and unexplained weight change of more than lateral compartment may be injured individ-
10 pounds in 6 months.17 ually or in combination. This condition often
occurs during activities that place excessive
Background Information
eccentric demand on the anterior compart-
The skeletal system is the third most common
ment muscles.19 The diagnosis is confirmed
site of metastatic disease.18 Symptoms also
by magnetic resonance imaging or computed
may be related to pathological fracture in
tomography. Usual treatment is nonsurgical.
affected sites. Common primary sites causing
metastases to bone include breast, prostate, ■ Gastrocnemius Muscle Strain
lung, and kidney. Bone scan confirms the Chief Clinical Characteristics
diagnosis. Common treatments for metastases This presentation may involve pain, tenderness,
include surgical resection, chemotherapy, ra- pain with contraction, difficulty weight bear-
diation treatment, and palliation, depending ing and sometimes swelling or ecchymoses in
on the tumor type and extent of metastasis. the region of the gastrocnemius muscle.
■ Multiple Myeloma Background Information
Chief Clinical Characteristics Injury to the muscle often occurs during
This presentation involves unexplained skele- activities that place excessive eccentric de-
tal pain in the feet, lower legs, arms, hands, mand on the gastrocnemius, usually on an
back, and thorax. This may be associated with extended knee.19 Diagnosis may be aided by
renal failure, recurrent bacterial infections, magnetic resonance imaging. Treatment is
pathological fractures, and anemia. often nonsurgical and healing usually takes 6
to 10 weeks.
Background Information
This condition is an insidious plasma cell can- ■ Lateral Compartment Muscle
cer that originates in the bone marrow and can Strain
affect multiple areas with multiple lytic and Chief Clinical Characteristics
osteosclerotic affects. Pain results from either This presentation includes pain, tenderness,
neuropathy or bone pain. Radiographically, difficulty bearing weight on the affected
the bones will have lesions of a punched-out extremity and walking, and possible edema
appearance with generalized osteoporosis. or ecchymosis in the region of the peroneal
Diagnosis may be confirmed with blood muscles. Symptoms may be reproduced by
tests, urinalysis, serum protein electrophore- either muscle action or stretching.
sis, bone scan, magnetic resonance imaging, Background Information
and bone marrow aspiration and biopsy. Injury to the muscle often occurs during
Depending on the aggressiveness of this con- activities that place excessive eccentric de-
dition, treatment may range from “watchful mand on the peroneal muscles.19 The diag-
waiting” to chemotherapy and bone marrow nosis is confirmed by magnetic resonance
transplantation. imaging or computed tomography. Usual
treatment is nonsurgical.
MUSCLE STRAINS
■ Anterior Compartment Muscle
■ Soleus Muscle Strain
Strain Chief Clinical Characteristics
Chief Clinical Characteristics This presentation may involve pain, tenderness,
This presentation typically includes pain and pain with contraction, difficulty bearing weight
tenderness, difficulty bearing weight on the on the affected lower extremity, and possibly
affected lower extremity, and possible edema edema or ecchymosis over the soleus muscle.
and ecchymosis in the anterolateral lower leg. Background Information
Symptoms may be reproduced with either The injured area is often more distal and
muscle action or stretching. deep to the gastrocnemius; this may be
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408 Chapter 22 Necrotizing Fasciitis

assessed through an individual’s history and sites reserved for cases in which the site of
LOWER LEG PAIN

palpation. This condition often occurs dur- entrapment is well characterized.


ing activities that place excessive eccentric
demand on the soleus, more often with a ■ Sciatic Nerve
flexed knee.19 Magnetic resonance imaging Chief Clinical Characteristics
confirms the diagnosis. Usual treatment is This presentation may include diminished
nonsurgical. Achilles deep tendon reflex, weakness of the
hamstrings or ankle plantarflexors and dor-
■ Necrotizing Fasciitis siflexors, pain, and buttock, posterior thigh,
Chief Clinical Characteristics posterior lower leg, and foot paresthesias or
This presentation involves pain with erythema, hypesthesias.
warmth, swelling, possible skin discoloration,
blisters, gangrene, and joint crepitus. Systemic Background Information
signs of an infection may include fever, tachy- Common etiologies include entrapment at
cardia, hypotension, diaphoresis, chills, malaise, the sciatic notch or the hamstrings. This con-
and altered mental status. The wound may be dition is a diagnosis of exclusion after lumbar
odorous and sometimes subcutaneous gas is radiculopathy is ruled less likely. The clinical
observed. examination, including the straight leg raise
test and a standard neurological examina-
Background Information tion, and electrodiagnostic tests confirm the
The underlying infection often is caused by a diagnosis. Typical treatment is nonsurgical,
bacterium following trauma or infection of an with surgical exploration and release of en-
open wound or ulcer. This condition often is trapment sites reserved for cases in which the
mistaken for cellulitis in its early stages. Diag- site of entrapment is well-characterized.
nosis may be confirmed with radiographs,
abnormal blood serum tests, and a Gram stain ■ Superficial Peroneal Nerve
of the infected tissue. This condition is a med- Chief Clinical Characteristics
ical emergency. Treatment includes debride- This presentation can involve pain, paresthe-
ment of necrotic tissue and an aggressive regi- sias, dysesthesias, or numbness on the antero-
men of antibiotic medication. lateral lower leg and dorsum of the foot.
Plantarflexion and foot inversion are often
NERVE ENTRAPMENTS
aggravating factors, and Tinel’s sign over the
■ Saphenous Nerve superficial peroneal nerve also may reproduce
Chief Clinical Characteristics pain. Associated motor deficits may include
This presentation involves pain, paresthesias, weakness of foot eversion.
dysesthesias, or numbness on the medial Background Information
aspect of the lower leg and distal to the me- Etiology includes compression due to
dial ankle and foot. This condition also may trauma or idiopathic entrapment.22 This
be associated with medial knee pain.20 condition is a diagnosis of exclusion after
Background Information lumbar radiculopathy is ruled out. The clin-
The saphenous nerve is a cutaneous afferent, ical examination, including the straight leg
so entrapment produces no motor deficits. raise test and a standard neurological exam-
Potential entrapment sites include the ination, and electrodiagnostic tests confirm
adductor hiatus and the distal sartorius mus- the diagnosis. Typical treatment is nonsurgi-
cle. Knee surgery, surgical excision of the cal, with surgical exploration and release of
saphenous vein, femoral neuropathy, or knee entrapment sites reserved for cases in which
trauma may cause this condition.21 The the site of entrapment is well characterized.
diagnosis is confirmed with the clinical ex-
amination, with additional studies poten- ■ Sural Nerve
tially useful to detect contributory condi- Chief Clinical Characteristics
tions. Typical treatment is nonsurgical, with This presentation can be characterized by
surgical exploration and release of entrapment hypesthesias or paresthesias and pain along
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Chapter 22 Osteoid Osteoma 409

the posterior calf, posterolateral ankle, or the drainage that can be stained as free fat. Skin le-

LOWER LEG PAIN


dorsolateral foot. Tinel’s sign also may repro- sions are the primary sign; fever, leukocytosis,
duce pain. This condition does not include ecchymoses, pulmonary lesions, and elevated
motor involvement because the sural nerve is serum amylase and lipase levels also may be
a sensory afferent. present.
Background Information Background Information
Common etiologies include ankle sprains, This condition often has a systemic cause such
stretch injury, Achilles injury, trauma, nerve as connective tissue disorders, lymphoprolifer-
compression due to ganglion cyst, or cal- ative disorders, alpha1-antitrypsin deficiency,
caneal fracture. This condition is a diagnosis or lipodystrophy. Histological examination of
of exclusion after lumbar radiculopathy the affected tissue confirms the diagnosis. In-
has been ruled out. The clinical examination, tralesional corticosteroids to address inflam-
including the straight leg raise test and a mation also may be necessary.
standard neurological examination, and
electrodiagnostic tests confirm the diagno- ■ Nonclostridial Myositis
sis. Typical treatment is nonsurgical, with Chief Clinical Characteristics
surgical exploration and release of entrap- This presentation involves pain, erythema,
ment sites reserved for cases in which the site edema, malodorous discharge, and possibly tis-
of entrapment is well characterized. sue crepitus. Symptoms may vary depending
on the nature of the infection. In advanced cases,
■ Tibial Nerve dark areas of gangrene are present.
Chief Clinical Characteristics
Background Information
This presentation may involve hypesthesias or
This condition usually results from penetrat-
paresthesias and pain along the posterior calf,
ing trauma, complications of diabetes melli-
medial ankle, and plantar surface of the foot.
tus, ischemia, or direct infection from an out-
Motor symptoms are less common. Pain may
side source. Laboratory tests determine the
be aggravated with bearing weight on the af-
pathogen, plain radiographs confirm tissue
fected lower extremity and walking, manual
gas, and surgical exploration may confirm the
compression over the nerve, or Tinel’s sign.
myonecrosis or myositis as well as guide med-
Background Information ical management. Typical treatment involves
Common etiologies include space-occupying an aggressive regimen of antibiotic medication
lesions, fractures, calluses, arthritis and and surgical drainage.
osteophytes, edema, bursitis, tendonitis or ■ Osteoid Osteoma
tenosynovitis, accessory bones, or other pro-
longed pressure. This condition is a diagno- Chief Clinical Characteristics
sis of exclusion after lumbar radiculopathy This presentation may involve focal bone pain
has been ruled out. The clinical examination, at the site of the tumor that is associated with
including the straight leg raise test and a tenderness and warmth to palpation, with
standard neurological examination, and significant increase in pain with activity and
electrodiagnostic tests confirm the diagno- at night, as well as substantial and immedi-
sis. Typical treatment is nonsurgical, with ate relief of pain with anti-inflammatory
surgical exploration and release of entrap- medication.
ment sites reserved for cases in which the site Background Information
of entrapment is well characterized. This condition is more common in males than
females, and it rarely presents in people younger
■ Nodular Panniculitis than age 5 or older than 40 years of age. Its
Chief Clinical Characteristics pathology includes abnormal production of os-
This presentation may be characterized by teoid and primitive bone. The proximal femur
painfully tender plaques and nodules that are is the most common site for this tumor. Pain as-
often reddish in color on the lower extremities. sociated with this condition is self-limiting and
The nodules may necrose with a “pus-like” may resolve spontaneously over the course of
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410 Chapter 22 Osteomyelitis of the Tibia

2 to 4 years.23 Pain relief also may be achieved or distance, change of shoe, tightness in the
LOWER LEG PAIN

by surgical removal of the tumor. shoe laces over the tendons, midtarsal joint hy-
perostosis, or tightness of the posterior calf
■ Osteomyelitis of the Tibia muscles. Differentiation among extensor ten-
Chief Clinical Characteristics dons can be achieved by passive stretching and
This presentation typically involves localized active contraction of the specific muscles.
tenderness, warmth, erythema, swelling, and Clinical examination confirms the diagnosis.
systemic signs of infection such as weight loss and Usual treatment is nonsurgical.
fatigue. The toes may present with a “sausage
toe” deformity.
■ Posterior Tibial Vein Thrombosis
Chief Clinical Characteristics
Background Information This presentation may include calf pain, tender-
This condition is an inflammation and necrosis ness, lower extremity swelling, cramping,
of bone as a result of an infection. The calcaneus erythema, palpable warmth, engorged veins,
is the most common site of infection in the sometimes a low-grade fever, and pain poten-
foot, followed by the metatarsals, tarsals, and tially along the course of the vein. The throm-
then the phalangeal bones. Biopsy, magnetic bosis can affect superficial or deep veins, but the
resonance imaging, and histology confirm the popliteal and calf veins are more commonly
diagnosis and guide medical intervention.24 affected.
Treatment may involve an aggressive regimen of
antibiotic medications, with surgical resection Background Information
of affected areas potentially necessary. Venous thrombosis may be effort induced or
spontaneous. This condition involves throm-
■ Osteosarcoma bosis and inflammation of the involved vein.
Chief Clinical Characteristics The etiology of this condition is associated
This presentation may be characterized by an with Virchow’s triad of intimal damage,
insidious onset of pain that persists for weeks or stasis, and changes in blood composition7; it
months. Pain due to this condition may be usually occurs due to trauma to the vein or
aggravated with activity, causing limping. surrounding tissue, but also may present
spontaneously. Homan’s sign, D-dimer assay
Background Information measurement, computed tomography, mag-
This condition is frequently found in adoles- netic resonance angiography, and Doppler ul-
cents because of the active bone growth in this trasonography confirm the diagnosis.25,26
age group. African American individuals are This condition is a medical emergency be-
affected slightly more often than Caucasian cause of the risk for propagation of the
individuals. This condition can be especially thrombus to the lungs.
fatal if metastasized to the lungs. Plain films
confirm the diagnosis. Treatment includes ■ Pyomyositis
chemotherapy and surgery to remove the Chief Clinical Characteristics
cancerous cells or tumors. This presentation often involves cramping pain
and edema with early-stage induration, fol-
■ Paratenonitis of Extensor lowed by increased pain and edema in the later
Tendons of the Foot and Toes stages. Systemic signs of infection may include
Chief Clinical Characteristics fever, chills, and sometimes diaphoresis. Leuko-
This presentation includes pain, possible cytosis is common. Abscess formation due to
swelling, erythema, and tenderness to palpation Staphylococcus aureus infection often follows
over the anterior ankle and may include crepi- muscle tissue trauma.
tus with active or passive movements if the
Background Information
tendon sheaths are inflamed.
Risk factors include poorly controlled diabetes
Background Information mellitus, presence of a contiguous bone or soft
This condition usually occurs with a sudden tissue infection, malnutrition, or a compro-
onset following increases in walking intensity mised immune system. Diagnosis is confirmed
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Chapter 22 Sickle Cell Crisis 411

by the clinical examination, computed tomogra- tibia. The pain usually lessens following

LOWER LEG PAIN


phy or magnetic resonance imaging, and Gram- warm-up exercises of the involved muscles, but
stained culture of the aspirated pus.27 Treatment is often present during activity with soreness
is directed at the underlying infection. following the activity. Pain usually is absent
at night.
■ Rupture of the Achilles Tendon
Background Information
Chief Clinical Characteristics Shin splints are the result of eccentric
This presentation may include a sudden onset overuse, leading to an inflammatory mus-
of pain, palpable tenderness, significant weak- culotendinous injury along the lateral
ness, and sometimes a palpable sulcus, usually aspect of the tibia, with potential muscular
2 to 6 cm proximal to the distal insertion of the avulsion from the tibia.28 Diagnosis is
Achilles tendon (Fig. 22-1).
usually based on the clinical presentation.
Background Information Imaging is usually unhelpful, although
This condition often occurs spontaneously fat-suppressed magnetic resonance imag-
during an activity that places loading on the ing may allow differentiation between
plantarflexors. It is associated with age over stress fractures and shin splints.29 Usual
40 years and a history of Achilles tendinopathy. treatment is nonsurgical.
The Thompson test and magnetic resonance
or ultrasound imaging confirm the diagnosis. ■ Posteromedial Shin Splints
This condition is treated with surgical repair of Chief Clinical Characteristics
the affected tendon. This presentation involves pain and palpa-
ble tenderness along the muscles attached to
SHIN SPLINTS the posteromedial aspect of the tibia within
■ Anterolateral Shin Splints the deep posterior compartment. Pain is
Chief Clinical Characteristics usually less often located directly over the
This presentation includes pain and palpa- tibia. Pain is aggravated with stretching
ble tenderness along the involved anterior into foot dorsiflexion and eversion, and with
tibial muscles immediately adjacent to the active contraction of the involved muscles.
Pain usually lessens following warm-up
of the muscles, there is usually pain
during activity with soreness following the
activity, and there is usually no pain during
the night.
Background Information
Shin splints are due to an eccentric over-
use, leading to musculotendinous injury
along the posteromedial attachment of the
tibia, resulting in a periostitis.30,31 Diagno-
sis is based on the clinical presentation.
Imaging is usually unhelpful, although
fat-suppressed magnetic resonance imag-
Ruptured ing may help differentiate between stress
Achilles fractures and shin splints.29 Typical treat-
tendon ment is nonsurgical.
■ Sickle Cell Crisis
FIGURE 22-1 Achilles tendon rupture may be de- Chief Clinical Characteristics
tected by the Thompson test (inset), in which manual This presentation may be characterized by bone
circumferential pressure is exerted against the calf. A pain about the hip joint, worsened by cold
normal response includes passive ankle plantarflex- weather, overexertion, dehydration, and being
ion. A positive test is absence of ankle movement. overly fatigued.
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412 Chapter 22 Arterial or Hypertensive Ulcers

Background Information TENDINITIS


LOWER LEG PAIN

Sickle cell crisis involves abnormal red blood ■ Achilles Tendinitis


cell morphology that causes the cells to be-
come rigid and sticky, disrupting blood flow Chief Clinical Characteristics
to bones and resulting in painful bone in- This presentation can involve a sharp,
farcts. This condition is a group of inherited aching, or burning pain with point tender-
disorders with abnormalities caused by he- ness 2 to 6 cm proximal to the calcaneal in-
moglobin S. This condition typically is asso- sertion. Insertional tendinopathy may be
ciated with pain due to tissue infarction located at the distal insertion at the calcaneus.
and/or worsening anemia. Diagnosis of a cri- Symptoms are reproduced with palpation of
sis is confirmed with plain radiographs and the tendon, passive dorsiflexion, and active
magnetic resonance imaging. This condition resisted plantarflexion. Palpable crepitus may
is a medical emergency. be present with movement, and an increased
thickening of the tendon that moves with ten-
SKIN ULCERS don excursion may be palpable in compari-
■ Arterial or Hypertensive Ulcers son to the asymptomatic side. Calf atrophy
may be observed, the patient may have
Chief Clinical Characteristics limitations in ankle dorsiflexion, and the
This presentation involves small, painful, patient may report increased stiffness in the
and tender ulcers measuring from several morning.
millimeters to a few centimeters in size. They
are more commonly found in the pretibial Background Information
region. Associated symptoms may include Achilles tendinitis is associated with overuse
palpable coolness and trophic changes such from repetitive loading of the involved ten-
as hair loss. don. This injury is most common in 15- to
45-year-old active individuals, most com-
Background Information
monly runners. Diagnosis is often made
This condition commonly is associated
clinically. If the diagnosis is difficult, then
with hypertension or peripheral vascular
confirmation may be made with magnetic
disease with ulcerations at the ends of the
resonance imaging. Usual treatment is non-
arterial branches.32 Wound management in
surgical, consisting of anti-inflammatory
combination with endovascular or bypass
medication, rest, and gentle exercise.
surgical interventions may be necessary as
treatments.
■ Extensor Digitorum Longus
■ Systemic Vasculitis With Tendinitis
Ulceration Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can be characterized by
This presentation may include subcutaneous pain over the dorsum of the foot and tender-
erythematous nodules on the lower leg that are ness to palpation along the extensor digitorum
usually painful and may develop into puru- longus tendons. Resisted extension of the lesser
lent ulcerations. toes and passive flexion of the lesser toes
often increase the pain. Pain may also be
Background Information elicited with resisted dorsiflexion of the ankle.
This condition results from inflammation Walking and running are often painful.
of the blood vessels that may result from
systemic lupus erythematosus, polyarteritis Background Information
nodosa, rheumatoid arthritis, Wegener’s The pathology of this tendinitis is usually the
granulomatosis, or sickle cell anemia. Diag- result of overuse. Diagnosis is usually made
nosis may be focused toward determining clinically, but may be confirmed with mag-
the tissue involved via histological analysis or netic resonance or ultrasound imaging.
angiography. Typical treatment involves Nonsurgical treatments may be indicated,
wound management in combination with such as anti-inflammatory medication, rest,
treatment for the underlying pathology. and gentle exercise.
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Chapter 22 Achilles Tendinosis 413

■ Extensor Hallucis Longus the eccentric loading of the tendon and mus-

LOWER LEG PAIN


Tendinitis cle. Pain is often increased with passive
Chief Clinical Characteristics stretch into plantarflexion or resisted dorsi-
This presentation may include pain and pal- flexion of the ankle. The pathology of tibialis
pable tenderness along the extensor hallucis anterior tendinitis is usually overuse.33
tendon. Resisted extension of the hallux and Diagnosis is usually made clinically, but may
passive flexion of the hallux often increase be confirmed with magnetic resonance or
the pain. Pain may also be elicited with resis- ultrasound imaging. Usual treatment is non-
ted dorsiflexion of the ankle. Walking and surgical, consisting of anti-inflammatory
running are often painful. medication, rest, and gentle exercise.

Background Information ■ Tibialis Posterior Tendinitis


The pathology of this tendinitis is usually the Chief Clinical Characteristics
result of overuse.33 Diagnosis is usually This presentation may involve pain and
made clinically, but may be confirmed with swelling with palpable tenderness along the
magnetic resonance or ultrasound imaging. medial aspect of the midfoot and sometimes
Treatment usually involves nonsurgical in- posterior to the medial malleolus. Pain is
terventions, such as anti-inflammatory med- often worsened with weight bearing, walk-
ication, rest, and gentle exercise. ing, performing a heel rise, or stretching
■ Peroneal Tendinitis the tendon. Attenuation of this tendon is
sometimes seen and can lead to a flat foot
Chief Clinical Characteristics deformity. Palpation may be assisted with
This presentation can involve pain anywhere muscle contraction of the tibialis posterior
along the lateral lower leg to the posterior lat- muscle.
eral malleolus and along the lateral foot at the
location of the cuboid or at the plantar aspect Background Information
of the foot where the peroneus longus tendon Injury to this tendon is often the result of
inserts at the base of the first metatarsal. The overuse that is caused by a hypermobile
pain may also be present at the base of the fifth midfoot and excessive foot pronation. The
metatarsal where the peroneus brevis tendon tibialis posterior is the most significant
inserts. The pain is often aggravated with a active stabilizer of the arch.34 Diagnosis is
stretch into foot and ankle dorsiflexion and usually made clinically, but may be con-
inversion or a muscle contraction into foot and firmed with magnetic resonance or ultra-
ankle plantarflexion and/or eversion. sound imaging. Nonsurgical treatments may
be indicated, such as anti-inflammatory
Background Information medication, rest, and gentle exercise.
Common causes of peroneal tendon injury in-
clude overuse and inversion injury mecha- TENDINOSES
nisms. Diagnosis is usually made clinically, but
may be confirmed with magnetic resonance or ■ Achilles Tendinosis
ultrasound imaging. Nonsurgical treatments Chief Clinical Characteristics
may be indicated, such as anti-inflammatory This presentation typically includes sharp,
medication, rest, and gentle exercise. aching, or burning pain with point tender-
ness usually along the medial aspect of the
■ Tibialis Anterior Tendinitis middle one-third of the Achilles tendon. Some
Chief Clinical Characteristics of these patients are pain free. Symptoms, if
This presentation typically includes pain present, may be reproduced with palpation
and tenderness to palpation of the tibialis of the Achilles tendon, passive dorsiflexion,
anterior tendon. or active resisted plantarflexion. Palpable
increased thickening of the tendon may be
Background Information present in comparison to the uninvolved ten-
Pain is often present at initial contact and don. Calf atrophy, limitations in ankle dor-
during the loading response of gait, due to siflexion, and reports of increased stiffness
1528_Ch22_392-416 07/05/12 1:53 PM Page 414

414 Chapter 22 Extensor Digitorum Longus Tendinosis

in the morning may be observed. Pain is ■ Extensor Hallucis Longus


LOWER LEG PAIN

often worse with weight bearing and walk- Tendinosis


ing, and these patients often have difficulty Chief Clinical Characteristics
performing a heel rise. This presentation typically includes pain and
Background Information palpable tenderness along the extensor hallu-
Tendinosis is a state of increased tenocyte cis tendon. Walking and running are often
and cellular activity that leads to disorgan- painful. Resisted extension of the hallux and
ized collagen and vascular hyperplasia.35,36 passive flexion of the hallux often increase
Tendinosis is usually a chronic degenerative the pain. Pain may also be elicited with resis-
process that is initiated by an injury to the ted dorsiflexion of the ankle.
tendon. Diagnosis is usually based on the Background Information
clinical presentation. If pathology is severe Extensor hallucis longus tendinosis is a state
or the clinical diagnosis is unclear, then of increased tenocyte and cellular activity
magnetic resonance or ultrasound imaging that leads to disorganized collagen and vas-
may confirm the diagnosis and allow for cular hyperplasia.35,36 Tendinosis is usually a
prognosis and staging of the tendinosis. A chronic degenerative process that is initiated by
combination of patient/client education, an injury to the tendon. Clinical examination
unloading the affected musculotendinous confirms the diagnosis. If pathology is severe
unit, controlled mechanical reloading, and or the clinical diagnosis is unclear, then mag-
preventive measures appears to be effective netic resonance or ultrasound imaging may
for managing this condition.37 confirm the diagnosis and allow for progno-
sis and staging of the tendinosis. A combina-
■ Extensor Digitorum Longus tion of patient/client education, unloading
Tendinosis the affected musculotendinous unit, con-
Chief Clinical Characteristics trolled mechanical reloading, and preventive
This presentation typically includes pain measures appears to be effective for manag-
over the dorsum of the foot and tenderness ing this condition.37
to palpation along the extensor digitorum
longus tendons. Walking and running often ■ Peroneal Tendinosis
are painful. Resisted extension of the lesser Chief Clinical Characteristics
toes and passive flexion of the lesser toes This presentation typically includes pain
often increase the pain. Pain also may anywhere along the lateral lower leg to the
be elicited with resisted dorsiflexion of the posterior lateral malleolus and along the
ankle. lateral foot at the location of the cuboid or
at the plantar aspect of the foot where the
Background Information
peroneus longus tendon inserts at the base
Tendinosis is a state of increased tenocyte
of the first metatarsal. The pain may also be
and cellular activity that leads to disorgan-
present at the base of the fifth metatarsal
ized collagen and vascular hyperplasia.35,36
where the peroneus brevis tendon inserts. The
Tendinosis is usually a chronic degenerative
pain is often aggravated with a stretch into
process that is initiated by an injury to the
dorsiflexion and inversion or a muscle
tendon. Clinical examination confirms the
contraction into plantarflexion and/or ever-
diagnosis. If pathology is severe or the clini-
sion. Weakness may be noted into plantarflex-
cal diagnosis is unclear, then magnetic reso-
ion and/or eversion due to degenerative thick-
nance or ultrasound imaging may confirm
ening, lengthening, and tearing of the
the diagnosis and allow for prognosis and
tendon.
staging of the tendinosis. A combination of
patient/client education, unloading the af- Background Information
fected musculotendinous unit, controlled Common causes of peroneal tendon injury
mechanical reloading, and preventive meas- include overuse and inversion injury mecha-
ures appears to be effective for managing this nisms that may lead to the chronic degener-
condition.37 ative process of tendinosis. Tendinosis is a
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Chapter 22 Tibialis Posterior Tendinosis 415

state of increased tenocyte and cellular activ- Background Information

LOWER LEG PAIN


ity that leads to disorganized collagen and Injury to this tendon is often the result of
vascular hyperplasia.35,36 Final diagnosis is overuse with a hypermobile midfoot. The
confirmed with magnetic resonance or tibialis posterior is the most significant ac-
ultrasound imaging. A combination of tive stabilizer of the arch.34 Tendinosis is a
patient/client education, unloading the state of increased tenocyte and cellular activ-
affected musculotendinous unit, controlled ity that leads to disorganized collagen and
mechanical reloading, and preventive meas- vascular hyperplasia. Tendinosis is usually a
ures appears to be effective for managing this chronic degenerative process that is initiated
condition.37 by injury to the tendon. Diagnosis is usually
based on the clinical presentation; however,
■ Tibialis Anterior Tendinosis magnetic resonance imaging may confirm
Chief Clinical Characteristics the diagnosis and allow for prognosis and
This presentation typically includes pain and staging of the tendinosis. A combination of
tenderness to palpation of the tibialis anterior patient/client education, unloading the af-
tendon. fected musculotendinous unit, controlled
mechanical reloading, and preventive meas-
Background Information ures appears to be effective for managing this
Pain is often present at initial contact and condition.37
during the loading response of gait, due to
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LOWER LEG PAIN

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Finding cancer in primary care outpatients with low netic resonance imaging in stress fractures and shin
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CHAPTER23
Ankle Pain
■ Jason R. Cozby, PT, DPT, OCS ■ Lisa Meyer, PT, DPT, OCS
■ Stephen F. Reischl, PT, DPT, OCS

Description of the Symptom Special Concerns


■ Ankle symptoms associated with fever
This chapter describes pathology that may lead ■ Decreased sensation
to ankle pain, including generalized, lateral, ■ Diminished popliteal, dorsalis pedis, or
medial, anterior, and posterior ankle pain. tibial arterial pulses
Local causes of ankle pain are defined as ■ Inability to bear weight
pathology occurring between the distal one- ■ Pain that increases at night and/or with rest
third of the tibia and the ankle joint, inclusive ■ Skin breakdown
of musculoskeletal and neurovascular struc- ■ Warmth and swelling
tures that cross the ankle joint. Remote causes
are defined as occurring outside this area.

Medial
Posterior
Anterior

Lateral

Anterior view Posterior view

417
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418 Chapter 23 Ankle Pain

CHAPTER PREVIEW: Conditions That May Lead to Ankle Pain


ANKLE PAIN

T Trauma
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Lumbar radiculopathies: Fractures: Anterolateral shin splints 427
• L4 radiculopathy 425 • Lateral malleolus 429 Fractures:
• L5 radiculopathy 425 • Medial malleolus 430 • Lateral malleolus 429
• S1 radiculopathy 426 Ligament sprains: Ligament sprains:
• Anterior talofibular • Anterior talofibular ligament 432
ligament 432 • Subtalar joint 434
• Calcaneofibular ligament Peroneal tendon tear or rupture 436
(isolated) 433 Traumatic dislocation of the
• Deltoid ligament 434 peroneal tendons 442
UNCOMMON
Not applicable Ankle dislocation 427 Fractures:
Fractures: • Lateral process of the talus 429
• Anterior or posterior lip of the • Lateral tubercle of the posterior
tibial articular surface 429 process of the talus 430
• Talus, neck or body 431 • Osteochondral fracture of the
talar dome 430
• Stress fracture of the fibula 431
Ligament sprains:
• Anterior tibiofibular ligament 432
Peroneal tendon tear or rupture 436
Traumatic dislocation of the
peroneal tendons 442
RARE
Not applicable Ankle dislocation 427 Not applicable

I Inflammation
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Aseptic Aseptic
Reiter’s syndrome 437 Tendinitis:
Rheumatoid arthritis 437 • Peroneal tendinitis 440
Rubella and rubella
vaccine–associated Septic
arthritis 437 Not applicable

Septic
Not applicable
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Chapter 23 Ankle Pain 419

ANKLE PAIN
LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Fractures: Anterior ankle impingement 427 Not applicable


• Medial malleolus 430 Tibiofibular synostosis 441
• Stress fracture of the tibia 431
Ligament sprains:
• Deltoid ligament 434
Posteromedial shin splints 436
Tarsal tunnel syndrome 438

Fractures: Fractures: Rupture of the Achilles


• Medial tuberosity of the • Anterior lip of the distal tibial tendon 438
posterior process of the talus 430 articular surface 429 Talar compression
• Osteochondral fracture of the Ligament sprains: syndrome/impingement 438
talar dome 430 • Anterior tibiofibular
• Sustentaculum tali 431 ligament 432
• Cruciate crural ligament 434

Not applicable Not applicable Fractures:


• Posterosuperior calcaneal
tuberosity 430

LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Aseptic Aseptic Aseptic


Tendinitis: Paratenonitis of extensor Tendinitis:
• Tibialis posterior tendinitis 440 tendons of the foot and • Achilles tendinitis 439
toes 436
Septic Septic
Infectious periostitis 432 Septic Not applicable
Not applicable

(continued)
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420 Chapter 23 Ankle Pain

Inflammation (continued)
ANKLE PAIN

REMOTE LOCAL GENERALIZED LOCAL LATERAL


UNCOMMON
Not applicable Aseptic Not applicable
Acute rheumatic fever 427
Arthritis associated with
inflammatory bowel
disease 427
Complex regional pain
syndrome 428
Hepatitis B–associated
arthritis 432
Reiter’s syndrome 437
Septic
Lyme arthritis 434
Septic arthritis 438
RARE
Not applicable Aseptic Not applicable
Rubella and rubella vaccine–
associated arthritis 437
Septic
Osteomyelitis 436

M Metabolic
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Distal symmetric Not applicable
polyneuropathy, including
from acquired
immunodeficiency
syndrome, alcohol/drug
abuse, carcinoma, diabetes
mellitus, and kidney
failure 428
Gout 432
UNCOMMON
Not applicable Pseudogout 437 Distal symmetric polyneuropathy,
including from acquired
immunodeficiency syndrome,
alcohol/drug abuse, carcinoma,
diabetes mellitus, and kidney
failure 428

RARE
Not applicable Transient migratory Not applicable
osteoporosis 442
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Chapter 23 Ankle Pain 421

ANKLE PAIN
LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Aseptic Aseptic Aseptic


Tendinitis: Tendinitis: Achilles paratenonitis 426
• Flexor digitorum longus • Extensor digitorum longus Bursitis:
tendinitis 439 tendinitis 439 • Retrocalcaneal bursitis 428
• Flexor hallucis longus • Extensor hallucis longus • Subcutaneous Achilles
tendinitis 440 tendinitis 439 bursitis 428
• Tibialis anterior tendinitis 440
Septic Septic
Septic Not applicable Not applicable
Not applicable

Not applicable Not applicable Not applicable

LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Distal symmetric polyneuropathy, Distal symmetric Distal symmetric


including from acquired polyneuropathy, including polyneuropathy,
immunodeficiency syndrome, from acquired including from acquired
alcohol/drug abuse, immunodeficiency syndrome, immunodeficiency
carcinoma, diabetes mellitus, alcohol/drug abuse, syndrome, alcohol/drug
and kidney failure 428 carcinoma, diabetes mellitus, abuse, carcinoma, diabetes
and kidney failure 428 mellitus, and kidney
failure 428

Not applicable Not applicable Not applicable

(continued)
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422 Chapter 23 Ankle Pain


ANKLE PAIN

Va Vascular
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Hemophilic arthropathy 432 Not applicable
Osteochondritis dissecans 435
RARE
Not applicable Acute hemarthrosis 426 Not applicable

De Degenerative
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Osteoarthrosis/osteoarthritis of Tendinosis:
the ankle joint 435 • Peroneal tendinosis 441
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Tu Tumor
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Malignant Primary: Not applicable
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Dysplasia epiphysealis
hemimelica (Trevor’s disease) 429
• Osteoid osteoma 435
RARE
Not applicable Malignant Primary, such as: Not applicable
• Osteosarcoma 436
Malignant Metastatic, such as:
• Metastases to the ankle,
including from primary breast,
kidney, lung, prostate, and
thyroid disease 434
• Multiple myeloma 435
Benign:
Not applicable
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Chapter 23 Ankle Pain 423

ANKLE PAIN
LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Tendinosis: Tendinitis: Tendinosis:


• Tibialis posterior tendinosis 441 • Tibialis anterior tendinitis 440 • Achilles tendinosis 440

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

(continued)
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424 Chapter 23 Fear and Avoidance


ANKLE PAIN

Co Congenital
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL GENERALIZED LOCAL LATERAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Fear and avoidance 424 Not applicable Ganglion cyst 431
RARE
Psychological effect of Not applicable Not applicable
complex regional pain
syndrome 426

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Ankle Pain Description of Conditions That


May Lead to Ankle Pain
Pain that is experienced in the ankle region is
often due to injury or mechanical disorders Remote
affecting the underlying structures, especially ■ Fear and Avoidance
because the ankle experiences very high forces
from the ground reaction forces, forces orig- Chief Clinical Characteristics
inating from functional activities, and forces This presentation may include avoiding the use
that arise during attempts to control body of an extremity as a result of apprehension of
weight and momentum. However, pain also onset of pain.
may be associated with vascular compromise, Background Information
a proximal nerve compression, or systemic Injury to the foot and ankle may contribute
disease. The lower leg, ankle, and foot are end to the disuse of the lower extremity even with
target sites for multiple disease processes, so minimal injury. Fear of reinjury, pain, or
all potential diagnoses should be considered other factors will need to be explored. In
even if mechanical trauma is associated with all cases the basis of pathology must be
the presentation. thoroughly explored before this presentation
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Chapter 23 L5 Radiculopathy 425

ANKLE PAIN
LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL MEDIAL LOCAL ANTERIOR LOCAL POSTERIOR

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

is to be considered. If this condition is Background Information


present, a team approach ensures optimal A lumbar disk herniation is the most com-
treatment. mon cause for this condition. The diagnosis is
confirmed with magnetic resonance imaging.
LUMBAR RADICULOPATHIES Surgical intervention may be indicated in
severe cases of lower extremity pain accompa-
■ L4 Radiculopathy
nied by neurological signs.
Chief Clinical Characteristics
This presentation can be characterized by ■ L5 Radiculopathy
pain in the lumbar spine and paresthesias Chief Clinical Characteristics
radiating from the anterior aspect of the hip, This presentation includes pain in the lum-
thigh, and knee, sometimes extending antero- bar spine and paresthesias radiating from the
medially from the knee to the foot. Depend- lateral aspect of the hip and buttock to the
ing on the severity, the presentation may also lateral aspect of the knee, extending antero-
include a decreased or absent patellar ten- laterally down to the foot. Depending on the
don reflex and motor loss in the muscles severity, the presentation may also include
innervated by the L4 nerve. Prone knee bend motor loss in the muscles innervated by the
may reproduce symptoms. L5 nerve root.
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426 Chapter 23 S1 Radiculopathy

Background Information spinal electrical nerve stimulation, and surgical


ANKLE PAIN

A lumbar disk herniation is the most com- or pharmacologic sympathectomy. However, a


mon cause for this condition. The diagnosis team approach ensures optimal treatment for
is confirmed with magnetic resonance imag- individuals with this condition.
ing. Surgical intervention may be indicated
in severe cases of lower extremity pain ac- Local
companied by neurological signs.
■ Achilles Paratenonitis
■ S1 Radiculopathy Chief Clinical Characteristics
Chief Clinical Characteristics This presentation involves diffuse pain, tender-
This presentation typically includes pain in ness, and thickening and swelling along the
the lumbar spine and paresthesias radiat- Achilles tendon, especially 2 to 6 cm from the
ing from the buttock to the posterior aspect calcaneal insertion. However, the painful palpa-
of the knee and extending posterolaterally ble thickening does not move with dorsiflexion
from the knee to the foot. Depending on the or plantarflexion because the tendon moves with
severity, the presentation may also include this action but the paratenon does not. Pain is
a decreased or absent Achilles tendon reflex greatest in the mornings or at the start of a phys-
and motor loss in the muscles innervated by ical activity, but the pain will often decrease with
the S1 nerve. continued walking or activity as the tendon be-
gins to move more freely inside the paratenon.
Background Information
A lumbar disk herniation is a common cause Background Information
for this condition. The diagnosis is con- The paratenon is usually the site of inflam-
firmed with magnetic resonance imaging. matory injury related to overuse, but it may
Surgical intervention may be indicated in also be damaged from direct traumatic pres-
severe cases of lower extremity pain accom- sure. The diagnosis is determined clinically,
panied by neurological signs. but magnetic resonance or ultrasound imag-
ing may be necessary for confirmation if the
■ Psychological Effect of Complex clinical diagnosis is unclear. Treatment in-
Regional Pain Syndrome cludes rest, inflammation control, improving
Chief Clinical Characteristics flexibility of the calf muscle group, and im-
This presentation typically includes a traumatic proving strength of the antipronator muscle
onset of severe chronic ankle and foot pain ac- groups.
companied by allodynia, hyperalgesia, and
■ Acute Hemarthrosis
trophic, vasomotor, and sudomotor changes in
later stages. This condition is characterized by Chief Clinical Characteristics
disproportionate responses to painful stimuli. This presentation typically includes pain,
swelling, tenderness to palpation, decreased
Background Information range of motion with a possible soft end feel, dif-
This regional neuropathic pain disorder pres- ficulty bearing weight, and pain with squat-
ents either without direct nerve trauma (Type I) ting. Individuals with hemophilia are more at
or with direct nerve trauma (Type II) in any risk to develop a hemarthrosis.3
region of the body.1 This condition may pre-
cipitate due to an event distant to the affected Background Information
area. Thermography may confirm associated This condition is rare in the ankle; however,
sympathetic dysfunction. This condition has injury to the joint capsule can lead to a
been shown to have a psychological compo- hemarthrosis. The presence of blood in aspi-
nent and may be influenced by cortical mecha- rated synovial fluid aspirated from affected
nisms.2 Treatment may include physical ther- joints confirms the diagnosis. Complications
apy interventions to improve patient/client include risks associated with acute bleeding, as
functioning, biofeedback, analgesic or anti- well as the arthropathy that may result from
inflammatory medication, transcutaneous or chronic bleeding.4 The patient should seek
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Chapter 23 Arthritis Associated with Inflammatory Bowel Disease 427

medical attention to treat the acute bleeding may be tender to palpation if osteophytes are

ANKLE PAIN
and prevent arthropathy.4 present on the talus or tibia.
■ Acute Rheumatic Fever Background Information
Chief Clinical Characteristics The impingement is caused by compression of
This presentation may involve warm, red, osseous or soft tissue structures, possibly in-
swollen, and tender joints with systemic signs cluding a meniscoid lesion of organized scar tis-
of infection including fever, abdominal pain, sue that may be present due to thickening of the
anorexia, lethargy, malaise, and fatigue. Rheu- anterior capsule after multiple sprains and de-
matic fever usually has five major manifesta- creased talar gliding. Usual treatment is nonsur-
tions that include migratory polyarthritis, cardi- gical with possible arthroscopic debridement in
tis, chorea, subcutaneous nodules, and erythema cases of recalcitrant symptoms and disability.
marginatum (flat to slightly indurated lesions
■ Anterolateral Shin Splints
on the skin of the extensor surfaces of the extrem-
ities or trunk). Chief Clinical Characteristics
This presentation may involve pain and palpa-
Background Information ble tenderness along the involved anterior tib-
This condition is an acute inflammation as a ial muscles immediately adjacent to the tibia.
result of group A streptococcal infection.5 The The pain usually lessens following warm-up
pain is usually the result of an aseptic inflam- exercises of the involved muscles, but is often pres-
mation of the joints. Diagnosis is made with ent during activity with soreness following the
confirmation of a group A streptococcal infec- activity. Pain usually is absent at night.
tion in association with two of the five major
manifestations. Monoarthritis and low-grade Background Information
fever are important considerations to avoid Shin splints are the result of eccentric overuse,
underdiagnosis.5 Usual treatment consists of leading to an inflammatory musculotendinous
antibiotic, anti-inflammatory, and antipyretic injury along the lateral aspect of the tibia, with
medications. potential muscular avulsion from the tibia.6
Diagnosis is usually based on the clinical
■ Ankle Dislocation presentation. Imaging is usually unhelpful,
Chief Clinical Characteristics although fat-suppressed magnetic resonance
This presentation can involve severe pain with imaging may allow differentiation between
gross deformity, usually with an inability to stress fractures and shin splints.7 Usual treat-
weight bear through the involved extremity. ment is nonsurgical.
Background Information ■ Arthritis Associated with
Trauma is usually severe and directed to the Inflammatory Bowel Disease
tibia on a fixed foot. Most cases result in either
Chief Clinical Characteristics
complete rupture of the ankle ligaments,
This presentation typically includes migratory
fracture of the ankle, or various combinations
polyarthritic pain with abdominal pain, diar-
of the two. Standard radiographs of the ankle
rhea, abdominal cramping, and other gastrointesti-
usually visualize these fractures and confirm
nal symptoms. Commonly affected joints include
the diagnosis of the dislocation. Treatment
the metatarsophalangeal joints, ankle, knee, elbow,
consists of surgical reduction and stabiliza-
wrist, and hand. Other extraintestinal manifes-
tion. Acute dislocations are a medical emer-
tations include episcleritis, ankylosing spondyli-
gency secondary to potential compromise of
tis, sacroiliitis, anterior uveitis, aphthous stomati-
neurovascular structures.
tis, erythema nodosum, pyoderma gangrenosum,
■ Anterior Ankle Impingement or growth and development retardation.
Chief Clinical Characteristics Background Information
This presentation includes pain at the anterior One-third of patients with inflammatory
ankle during dorsiflexion activities. The ankle bowel disease have at least one musculoskeletal
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428 Chapter 23 Retrocalcaneal Bursitis

manifestation, and in children the extraintesti- Treatment is nonsurgical, and typically in-
ANKLE PAIN

nal manifestations may dominate.8 Colonoscopy volves footwear modification.


with mucosal biopsy confirms the diagnosis.
Treatment typically involves aminosalicylates, ■ Complex Regional Pain Syndrome
corticosteroids, anti–tumor necrosis factor Chief Clinical Characteristics
medications, diet changes, and surgical resec- This presentation may include a traumatic
tion of the involved portion of bowel. onset of severe chronic ankle and foot pain
accompanied by allodynia, hyperalgesia, and
BURSITIS
trophic, vasomotor, and sudomotor changes in
■ Retrocalcaneal Bursitis later stages. This condition is characterized
Chief Clinical Characteristics by disproportionate responses to painful
This presentation can involve pain, palpa- stimuli.
ble warmth, tenderness to palpation, and Background Information
some swelling at the medial and lateral This regional neuropathic pain disorder pres-
aspects of the Achilles tendon. Pain is ents either without direct nerve trauma (Type I)
reproduced with firm palpation of the tis- or with nerve trauma (Type II) in any region of
sue anterior to the tendon, as well as with the body.1 This condition may precipitate due
dorsiflexion of the ankle. to an event distant to the affected area. Ther-
Background Information mography may confirm associated sympathetic
This condition is the result of inflammation dysfunction. Treatment may include physical
within the bursa that lies between the therapy interventions to improve patient and
Achilles tendon and the calcaneus. Predis- client functioning, biofeedback, analgesic or
posing factors include trauma, systemic anti-inflammatory medication, transcutaneous
disease, and biomechanical or structural or spinal electrical nerve stimulation, and surgi-
factors such as prominent posterosuperior cal or pharmacologic sympathectomy.
calcaneal tuberosity (Haglund’s deformity)
and rearfoot varus. Clinical examination ■ Distal Symmetric
confirms the diagnosis. Treatment is non- Polyneuropathy, Including From
surgical, and typically involves footwear Acquired Immunodeficiency
modification. Syndrome, Alcohol/Drug Abuse,
Carcinoma, Diabetes Mellitus,
■ Subcutaneous Achilles Bursitis and Kidney Failure
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes local pain, tender- This presentation may be characterized by
ness to palpation, and swelling just posterior decreased distal deep tendon reflexes, as well
to the Achilles tendon insertion at the cal- as pain, dysesthesia, or paresthesia in the feet
caneal tuberosity. Palpable warmth and and toes.
observable redness may be present in the same
Background Information
area.
Peripheral neuropathy is one of the more
Background Information common complications in patients presenting
Subcutaneous Achilles bursitis is a result of in- with acquired immunodeficiency syndrome,
flammation and swelling of the bursa between diabetes, renal failure, hypothyroidism, toxins,
the Achilles tendon and the skin. This may malignancy, and malnutrition. It is character-
occur from mechanical trauma to the tendon ized by retrograde axonal degeneration, so this
or it may be associated with insertional condition commonly progresses from distal to
Achilles tendinopathy (tendinitis/tendinosis) proximal. Electromyographic studies and
or retrocalcaneal bursitis. Continued irritation biopsy confirm the diagnosis. Vibration and
of the inflamed and swollen bursa may lead to light touch sensation (10-g Semmes Weinstein
thickening of the bursal walls and fibrosis. monofilament) tests are useful to assess protec-
Clinical examination confirms the diagnosis. tive sensation. Curative treatment of this
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Chapter 23 Lateral Process of the Talus 429

condition remains controversial, so the focus of ■ Anterior or Posterior Lip of the

ANKLE PAIN
intervention typically includes patient educa- Tibial Articular Surface
tion to maintain blood glucose control and Chief Clinical Characteristics
care for the insensate regions to prevent pro- This presentation may involve limited weight
gression and complications secondary to this bearing on the affected leg as well as pain,
condition. tenderness, and swelling around the location
■ Dysplasia Epiphysealis of the fracture.
Hemimelica (Trevor’s Disease) Background Information
Chief Clinical Characteristics These fractures usually do not traverse into
This presentation can include a limp that may the articular cartilage of the talocrural joint,
be accompanied by joint swelling, restricted but may be associated with ligamentous
joint movement, or muscle wasting. Males are injuries. The fracture to the anterior lip of
more likely to be affected than females. the tibia more often occurs with an injury
with the foot pronated and the ankle in dor-
Background Information siflexion. The fracture to the posterior lip of
This condition is a congenital bone develop- the tibia may occur with the foot and ankle
ment disorder that is the result of an intra- in various positions; however, it is more
articular osteochondromatosis (localized over- commonly associated with talar eversion
growth of the cartilage) that is present in the and posterior displacement. Standard radi-
epiphyses of the joint. This affects both sides of ograph views of the ankle confirm the diag-
the epiphyses and joint deformity is often the nosis. If the fracture is unstable, surgical
result. This condition is most common at fixation may be necessary.
the medial ankle, but it also may be seen in
the femur, the wrist, or the foot. Radiographs ■ Lateral Malleolus
often reveal the structural changes to the Chief Clinical Characteristics
joint. Treatment usually consists of surgical This presentation can include significant pain
excision of the mass, as well as correction at the lateral malleolus, swelling, and possi-
of any angular deformity that may have ble ecchymosis with pain increasing with
resulted. Nonsurgical intervention includes inversion, eversion, plantarflexion, and
immobilization. dorsiflexion motions.
FRACTURES Background Information
■ Anterior Lip of the Distal Tibial The fracture usually results during a forceful
Articular Surface inversion sprain of the lateral ligaments,
but may also occur during eversion sprains,
Chief Clinical Characteristics direct trauma, or torsional injuries. Associ-
This presentation typically includes limited ated injuries include medial malleolar
weight bearing on the affected leg as well as lesions, ligamentous injury, talar dome and
pain, tenderness, and swelling around the body lesions, or peroneal tendon injury.
location of the fracture. Diagnosis may be made clinically, assisted by
Background Information the Ottawa ankle rules, and radiographs are
These fractures usually do not traverse into necessary for confirmation of a fracture.9
the articular cartilage of the talocrural Surgery is indicated for displaced fractures;
joint, but may be associated with ligamen- nondisplaced fractures typically are treated
tous injuries. The fracture to the anterior with immobilization.
lip of the tibia more often occurs with
an injury with the foot pronated and the ■ Lateral Process of the Talus
ankle in dorsiflexion. Standard radiograph Chief Clinical Characteristics
views of the ankle confirm the diagnosis. This presentation may be characterized by
Surgery may be required if the fracture is pain, swelling, and tenderness along the
unstable. anterior and inferior lateral ankle located
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430 Chapter 23 Lateral Tubercle of the Posterior Process of the Talus

directly over the lateral process of the talus. Pain with the clinical examination, and the frac-
ANKLE PAIN

increases with weight bearing, talocrural ture can be confirmed with plain radi-
plantarflexion and dorsiflexion, and subtalar ographs. Surgical treatment is necessary for
joint movement. The mechanism of injury is nonunion or displaced fractures.
usually a dorsiflexion and inversion motion
common in motor vehicle accidents, direct ■ Medial Tuberosity of the
trauma, or snowboarding accidents. Posterior Process of the Talus
Chief Clinical Characteristics
Background Information
This presentation typically includes ankle pain
Chronic pain may be indicative of avascular
that is posterior to the medial malleolus and
necrosis. Bone scans, computed tomography,
anterior to the Achilles tendon. The injury of-
and magnetic resonance imaging often assist
ten results from dorsiflexion-pronation ankle
in the diagnosis, staging, and prognosis of
injuries, ankle dislocation, or possibly an avul-
the injury. Surgical treatment is indicated
sion fracture from a posterior tibiotalar injury.
for large or displaced fractures; otherwise,
nonsurgical treatment is indicated.10 Background Information
Bone scan, computed tomography scan, or
■ Lateral Tubercle of the Posterior magnetic resonance imaging may assist in
Process of the Talus the diagnosis, staging, and prognosis. In the
Chief Clinical Characteristics event of unstable fractures or nonunion, sur-
This presentation can involve posterolateral gical fixation may be necessary.
ankle pain, swelling, and tenderness over the
lateral tubercle. Pain increases while walk- ■ Osteochondral Fracture of the
ing downhill or downstairs, as well as with Talar Dome
ankle and subtalar movement, active first toe Chief Clinical Characteristics
flexion, or forced first toe dorsiflexion. This presentation includes variable symp-
Background Information toms based on the site of the osteochondral le-
This condition results from talocalcaneal sion. Lateral dome lesions commonly present
ligament avulsion, posterior talofibular liga- with localized pain at the central and lateral
ment avulsion, or a compressive force. It is ankle anterior to the lateral malleolus. Me-
associated with lateral ankle sprains, lateral dial dome lesions present with tenderness
malleolus avulsion fractures, and osteochon- posterior to the medial malleolus and at the
dral defects on the talar dome. Clinical ex- posterior talus.
amination findings and radiographs confirm Background Information
the diagnosis.10 Surgical treatment is neces- Osteochondral defects may loosen and cause
sary for nonunion or displaced fractures.10 joint pain, locking, and swelling. Lateral dome
■ Medial Malleolus injuries are frequently associated with trauma,
usually the result of an inversion-dorsiflexion
Chief Clinical Characteristics mechanism. Medial dome injuries may either
This presentation typically involves local pain, be the result of inversion-plantarflexion
swelling, and point tenderness present over the trauma or present insidiously. Radiographs,
fracture site. Pain is most severe within the first magnetic resonance imaging, and arthroscopy
24 hours of the injury. The fracture usually is confirm the diagnosis. Patients should be
the result of an abduction or external rotation monitored because joint degeneration is com-
force on a pronated foot, but also can result mon following these injuries.11
from a high-force inversion sprain that may
lead to a compression fracture. ■ Posterosuperior Calcaneal
Background Information Tuberosity
Associated findings may include a deltoid Chief Clinical Characteristics
ligament rupture, syndesmosis tear, lateral This presentation can include pain, swelling,
ankle sprain, lateral malleolus fracture, or and possible ecchymosis located at the cal-
subtalar dislocation. Diagnosis is confirmed caneal insertion of the Achilles tendon, the
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Chapter 23 Ganglion Cyst 431

posterior heel, and the posterior ankle. The Background Information

ANKLE PAIN
patient will often have a functional loss of This condition occurs in runners and is asso-
plantarflexion, such as with a single-leg heel ciated with an increase in training. A clinical
raise, due to the avulsion fracture. This con- examination and bone scan confirm the
dition is most common in older individuals diagnosis. This condition typically is treated
with advanced age, diabetes mellitus, or with relative rest, including general condi-
osteoporosis. tioning in an environment that is character-
ized by low-impact training.
Background Information
The mechanism of injury is a rapid pull of ■ Sustentaculum Tali
the Achilles tendon. A positive Thompson Chief Clinical Characteristics
test and a palpable sulcus may be present if This presentation may involve pain, swelling,
the tendon is avulsed. Associated findings and tenderness over the medial heel and
may include an Achilles tendon tear or ankle, inferior to the medial malleolus. Pain
other extra-articular calcaneal fractures. is increased with passive rearfoot inversion and
Plain radiographs confirm the diagnosis. passive hyperextension of the first phalanx.
Surgical fixation under traction may be
necessary for displaced fractures. Background Information
This fracture may result from a fall onto the
■ Stress Fracture of the Fibula heel with an inverted foot, but it is uncom-
Chief Clinical Characteristics mon to have this condition as an isolated
This presentation may involve focused, local- injury. Plain radiographs confirm the diag-
ized, superficial aching or sharp pain directly nosis. Complications may include nonunion,
at the site of the fracture (usually within nerve irritation, and toe flexor injury. Sur-
15 cm [6 in.] of the distal fibula). Pain is worse gery is indicated if the fracture is unstable.
with prolonged standing and weight-bearing
■ Talus, Neck or Body
activities that require dorsiflexion and rota-
tion. Pain is rarely present at rest unless the Chief Clinical Characteristics
fracture is severe. This presentation includes pain, swelling, and
tenderness at the sinus tarsi or just distal to
Background Information the lateral malleolus, following extreme com-
This condition occurs as a result of abnor- pression or shear loading of the foot. Pain
mal loading from a sudden increase in or usually is present with active or passive
change in training, improper footwear, or subtalar motion. This injury is commonly
ankle and foot dysfunction that may lead associated with spasm of the peroneal mus-
to excessive loading of the fibula. Clinical cles, resulting in palpable tenderness. A val-
presentation and bone scan confirm the di- gus deformity of the hindfoot may be present.
agnosis. This condition typically is treated
with relative rest, including general condi- Background Information
tioning in an environment that is character- The diagnosis is confirmed with imaging
ized by low-impact training. that may include plain radiographs, mag-
netic resonance imaging, and bone scan.
■ Stress Fracture of the Tibia This condition includes fractures of the talar
Chief Clinical Characteristics neck and body, as well as osteochondral
This presentation can be characterized by lesions of the trochlear surface that often
pain and palpable tenderness over the postero- require surgical treatment.12
medial border of the tibia, more commonly at
■ Ganglion Cyst
the middle to proximal one-third of the tibia.
Pain also may be present at the distal tibia, Chief Clinical Characteristics
leading to ankle pain. Pain usually is aggra- This presentation often involves the presence of
vated with weight bearing and running and a mildly painful or painless cystic lesion over the
alleviated with rest in a non–weight-bearing posterior, inferolateral aspect of the ankle usu-
position. ally detected following an inversion ankle
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432 Chapter 23 Gout

sprain. Rest will sometimes decrease the pres- elbows, and ankles. The diagnosis is confirmed
ANKLE PAIN

sure and alleviate some of the pain. by the presence of blood in aspirated synovial
fluid from affected joints and joint deteriora-
Background Information
tion apparent on plain radiographs. Immedi-
Cysts are often tender to palpation; they may
ate medical treatment of acute hemarthrosis is
arise from a joint or tendon sheath, increasing
necessary to minimize the extent of arthropa-
in size and pressure during ambulation as the
thy. This may include immobilization of
associated motion increases the pressure. Clin-
the affected joint and systemic infusion of the
ical examination confirms the diagnosis. If the
deficient blood factor.
pain causes dysfunction or is recalcitrant, then
aspiration and steroid treatment may help. If ■ Hepatitis B–Associated Arthritis
nonsurgical treatment is unsuccessful, surgical
excision of the ganglion is required. Recur- Chief Clinical Characteristics
rence is possible if excision is incomplete. This presentation typically includes a symmet-
rical polyarthralgia that can affect the toes and,
■ Gout more commonly, the fingers. The common
symptoms include anorexia, malaise, nausea,
Chief Clinical Characteristics vomiting, and fever. Jaundice usually develops
This presentation includes severe pain, edema, in approximately 1 to 2 weeks.
warmth, erythema, and very localized tender-
ness. Systemic findings may include fever, chills, Background Information
malaise, and sweating. The areas typically in- Hepatitis is an inflammation of the liver as a
volved are the first metatarsophalangeal joint, result of viral infection with necrosis of the
the talocrural joint, the calcaneal region, and liver. The arthralgia is usually the result of an
the tarsals of the instep. Pain is usually aseptic inflammation of the joints. Blood tests
provoked by hard movements, or may follow confirm the diagnosis.
alcohol abuse, dehydration, trauma, surgery,
septic arthritis, protein fasting, excessive purine ■ Infectious Periostitis
ingestion, and allopurinol or uricosuric agents. Chief Clinical Characteristics
Background Information This presentation may be characterized by
Sustained hyperuricemia levels lead to deposi- aching to severe pain with tenderness and
tion of monosodium urate crystals in and edema of the infected bone. Suppuration may
around the tendons and joints. Men are eight be present and an individual with this condi-
times more likely to be affected than women.13 tion will often experience systemic signs of
Needle aspiration is required to confirm the di- infection, such as fever, chills, and fatigue.
agnosis. Treatment commonly involves a combi- Background Information
nation of anti-inflammatory medication and This condition involves a chronic infection of
colchicine in individuals with normal liver func- the periosteum from a hematogenous source
tion. Preventive treatment should be undertaken or trauma to the affected region. Not all cases
to control uric acid levels by way of diet and of periostitis are the result of an infection;
medication in individuals with chronic gout. therefore, the diagnosis is confirmed by radi-
ographic imaging revealing necrosis of the
■ Hemophilic Arthropathy affected bone and bone scan revealing in-
Chief Clinical Characteristics creased uptake. This condition is usually
This presentation can involve a painful, swollen, managed with antibiotic medication appropri-
and tender joint culminating in range-of-motion ately directed to the infective agent.
limitation and difficulty bearing weight through
the affected limb. LIGAMENT SPRAINS
Background Information ■ Anterior Talofibular Ligament
Recurrent hemarthroses lead to inflammation Chief Clinical Characteristics
of the joint and result in joint degeneration.3 This presentation can include pain, swelling,
This condition commonly affects the knees, tenderness to palpation, and possible
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Chapter 23 Calcaneofibular Ligament (Isolated) 433

ecchymosis just anterior and inferior to the mechanism of injury, and a positive anterior

ANKLE PAIN
lateral malleolus, directly over the ligament translation test of the talus. If pain or insta-
(Fig. 23-1). Weight bearing and combined bility persists, then radiographs are helpful to
inversion/plantarflexion motions of the foot rule out fractures, osteochondral defects, or
will be painful and may be limited by pain. structural instability with stress. Nonsurgical
If the ligament is completely torn, then intervention is commonly indicated; however,
pain may or may not be present with surgical stabilization may be indicated in the
excessive inversion. event of a mechanically unstable joint.
Background Information ■ Anterior Tibiofibular Ligament
Injury to the ligament is usually caused by an Chief Clinical Characteristics
inversion and plantarflexion mechanism to This presentation may involve tenderness
the ankle. Associated injuries may include cal- and pain at the superomedial aspect of the
caneofibular ligament sprain, syndesmotic lig- lateral malleolus and the anterior aspect
ament sprain, sinus tarsi syndrome with a of the syndesmosis. Pain is present with
subtalar sprain, superficial peroneal nerve weight bearing, forced dorsiflexion of the
injury, fifth metatarsal fracture, medial ankle ankle, and extreme eversion or external
impingement, a medial or lateral malleolar rotation of the ankle, and is often repro-
fracture, talar or osteochondral fractures, duced with the squeeze test or external
or peroneal tendon injury. Diagnosis is rotation of the foot while the knee is flexed
usually based on the clinical presentation, to 90 degrees.14,15
Background Information
This injury (commonly referred to as a high
ankle sprain) usually occurs with abduction
or abduction-external rotation injuries di-
rectly to the foot and ankle, or with forced
internal rotation of the body over a planted
foot. Associated injuries include lateral ankle
ligament sprains, interosseous membrane
injury, deltoid ligament rupture, fibular frac-
ture, bone bruising or osteochondral defects,
and avascular necrosis. Diagnosis is based on
patient presentation, mechanism of injury,
Anterior and external rotation stress radiographs.
talofibular Nonsurgical intervention is commonly indi-
ligament
cated; however, surgical stabilization may
be indicated in the event of a mechanically
unstable joint.
■ Calcaneofibular Ligament
(Isolated)
Chief Clinical Characteristics
This presentation can be characterized by
swelling and tenderness along the lateral
Calcaneofibular ankle, inferior to the lateral malleolus, along
ligament the ligament (see Fig. 23-1). Pain increases with
foot inversion.
FIGURE 23-1 Lateral ankle sprain, including tear
of the anterior talofibular ligament, calcaneofibular Background Information
ligament, and posterior talofibular ligament. The an- Injury to the ligament occurs during an
terior drawer test (inset) is one method used to grade inversion mechanism of the ankle, with the an-
lateral ankle sprains. kle in neutral or even sometimes dorsiflexion.
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434 Chapter 23 Cruciate Crural Ligament

This injury rarely occurs independently, and commonly indicated; however, surgical sta-
ANKLE PAIN

is commonly associated with anterior bilization may be indicated in the event of a


talofibular ligament sprains, lateral malleo- mechanically unstable joint.
lus fractures, and syndesmotic injuries.
Diagnosis is based on the clinical presenta- ■ Subtalar Joint
tion, and the use of radiographs may be Chief Clinical Characteristics
useful to rule out any additional injuries or This presentation often includes pain at the
complications. Nonsurgical intervention is lateral ankle with tenderness in the sinus tarsi
commonly indicated; however, surgical sta- and pain aggravated with excessive inversion
bilization may be indicated in the event of a or subtalar motion.
mechanically unstable joint.
Background Information
■ Cruciate Crural Ligament This condition may be associated with an in-
version ankle injury followed by persistent
Chief Clinical Characteristics
difficulty with walking on uneven surfaces.
This presentation may include pain and
The injury also may be associated with grade II
tenderness to palpation along the ligament on
calcaneofibular ligament injuries occurring
the dorsum of the ankle and foot. Pain is
in a neutral or plantarflexed position, and
intensified with plantarflexion of the toes,
synovitis of the subtalar joint if excessive mo-
but is present even at rest. Paresthesias or
tion occurs. If the clinical examination is not
weakness may be present if the injury causes
definitive, then magnetic resonance imaging
increased intracompartmental pressure.
may be necessary. Nonsurgical intervention is
Background Information commonly indicated; however, surgical fusion
Injury to this ligament may be due to either may be indicated in the uncommon event of
direct or indirect trauma. Complications a mechanically unstable joint.
may include injury to the blood vessels or
nerves on the surface of the foot. Palpation ■ Lyme Arthritis
for pedal pulses, neurological examination, Chief Clinical Characteristics
and possibly a Doppler should be used to de- This presentation typically includes arthralgia
termine the extent of the injury to the nerves and swelling of the ankle joint or other joints,
or vasculature. Treatment will often consist myalgias, or signs of meningitis. Symptoms also
of rest to allow for healing, provided that may be associated with fever, altered mental
nerve and vascular injuries are absent. status, and an erythematous and pruritic skin
lesion that demonstrates central clearing and
■ Deltoid Ligament satellite rings.
Chief Clinical Characteristics
Background Information
This presentation involves medial ankle pain,
This condition results when the Borrelia burgdor-
swelling, ecchymosis, tenderness along the lig-
feri infection is transferred through a tick bite.
aments, pain with weight bearing, and pain
Serologic testing confirms the diagnosis. In most
with eversion of the ankle.
cases, this condition can be treated with prompt
Background Information administration of oral antibiotics or parenteral
Injury results from an abduction and/or agent. However, musculoskeletal and neurologi-
external rotation force on the ankle with se- cal consequences appear in a minority of indi-
vere eversion of the calcaneus, or when the viduals despite the medication used.
body moves over a fixed foot. The injury can
occur independently or may be associated ■ Metastases to the Ankle, Including
with lateral malleolus fractures, fracture of From Primary Breast, Kidney,
the anterior distal tip of the tibial articular Lung, Prostate, and Thyroid
surface, or distal tibiofibular syndesmosis Disease
injuries. Diagnosis is made based on the Chief Clinical Characteristics
clinical presentation and magnetic reso- This presentation typically includes unremit-
nance imaging. Nonsurgical intervention is ting pain in individuals with these risk
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Chapter 23 Osteoid Osteoma 435

factors: previous history of cancer, age 50 years Background Information

ANKLE PAIN
or older, failure to improve with conservative The etiology of osteoarthritis is either idio-
therapy, and unexplained weight change of pathic or secondary to conditions that affect the
more than 10 pounds in 6 months.16 intra-articular environment (such as infection,
inflammation, genetic defects, instability, or
Background Information
trauma). The clinical presentation will lead to
The skeletal system is the third most common
a preliminary diagnosis with radiographs
site of metastatic disease.17 Symptoms also
confirming the diagnosis and revealing the
may be related to pathological fracture in
extent of joint damage. Treatment for this
affected sites. Common primary sites causing
condition ranges from nonsurgical options,
metastases to bone include breast, prostate,
such as physical therapy intervention and intra-
lung, and kidney. Bone scan confirms the
articular corticosteroid injections, to total joint
diagnosis. Common treatments for metastases
arthroplasty.
include surgical resection, chemotherapy, radi-
ation treatment, and palliation, depending on ■ Osteochondritis Dissecans
the tumor type and extent of metastasis. Chief Clinical Characteristics
■ Multiple Myeloma This presentation involves pain, swelling, and
tenderness at the sinus tarsi, just distal to the lat-
Chief Clinical Characteristics eral malleolus or around the peroneal tendons
This presentation involves unexplained skele- as a result of muscle spasm. Symptoms increase
tal pain in the feet, lower legs, arms, hands, with subtalar motion and a valgus deformity
back, and thorax. This may be associated with of the hindfoot may be present.
renal failure, recurrent bacterial infections,
pathological fractures, and anemia. Background Information
This condition, also called osteochondral
Background Information lesion of the talus, occurs on both the medial
This condition is an insidious plasma cell cancer and lateral trochlea and has been linked to in-
that originates in the bone marrow and can juries related to movement of the talus inside
affect multiple areas with multiple lytic and the mortise of the ankle following ligamen-
osteosclerotic effects. Pain results from either tous disruption. The medial lesions are seen
neuropathy or bone pain. Radiographically, most often in bilateral lower extremities, in-
the bones will have lesions of a punched-out dicating some genetic or predisposition to
appearance with generalized osteoporosis. Diag- these injuries. This condition can occur sec-
nosis may be confirmed with blood tests, urinal- ondary to abnormal blood flow, resulting in a
ysis, serum protein electrophoresis, bone scan, poor prognosis.18 Bone scan, computed to-
magnetic resonance imaging, and bone marrow mography, and magnetic resonance imaging
aspiration and biopsy. Depending on the aggres- assist in the diagnosis, staging, and prognosis
siveness of this condition, treatment may range of this pathology. This condition is often
from “watchful waiting” to chemotherapy and staged based on magnetic resonance imaging
bone marrow transplantation. findings. Four stages are used to describe the
extent of articular damage as well as the sta-
■ Osteoarthrosis/Osteoarthritis
bility of the joint. Intervention may include
of the Ankle Joint
immobilization during early-stage disease
Chief Clinical Characteristics or arthroscopic debridement and drilling for
This presentation includes pain with localized advanced lesions.
tenderness at the involved joint, range-
of-motion limitations, a hard end feel, and ■ Osteoid Osteoma
crepitus, depending on the extent of the joint Chief Clinical Characteristics
damage. Onset of pain is usually gradual, symp- This presentation typically includes focal bone
toms are worsened by exercise and weight- pain at the site of the tumor that is associated
bearing activities, and patients usually report with tenderness and warmth to palpation, with
increased pain and stiffness in the morning and a significant increase in pain with activity and
following prolonged static postures. at night, and with substantial and immediate
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436 Chapter 23 Osteomyelitis

relief of pain with use of anti-inflammatory ■ Paratenonitis of Extensor


ANKLE PAIN

medication. This condition is more common in Tendons of the Foot and Toes
males than females, and it rarely presents in Chief Clinical Characteristics
people younger than 5 or older than 40 years This presentation often involves pain, possible
of age. swelling, erythema, and tenderness to palpation
Background Information over the anterior ankle and may include crepi-
The pathology of this condition includes tus with active or passive movements if the
abnormal production of osteoid and primitive tendon sheaths are inflamed.
bone. The proximal femur is the most com- Background Information
mon site for this tumor. Pain associated with This condition usually occurs with a sudden
this condition is self-limiting and may resolve onset following increases in walking intensity
spontaneously over the course of 2 to 4 years.19 or distance, change of shoe, tightness in the
Relief also may be obtained by surgical shoelaces over the tendons, midtarsal joint
removal of the tumor. hyperostosis, or tightness of the posterior
■ Osteomyelitis calf muscles. Differentiation among extensor
tendons can be achieved by passive stretching
Chief Clinical Characteristics and active contraction of the specific muscles.
This presentation typically includes localized Clinical examination confirms the diagnosis.
tenderness, warmth, erythema, swelling, and Typical intervention is nonsurgical, involving
systemic signs of infection such as weight loss and gentle exercise, physical modalities, and foot-
fatigue. The toes may present with a “sausage wear modification.
toe” deformity.
Background Information ■ Peroneal Tendon Tear or Rupture
This condition is an inflammation and necro- Chief Clinical Characteristics
sis of bone as a result of an infection. The cal- This presentation can include lateral ankle pain,
caneus is the most common site of infection in edema, weakness, and tenderness between the tip
the foot, followed by the metatarsals, tarsals, of the fibula and the fifth metatarsal. Painful weak-
and then the phalangeal bones. Biopsy, mag- ness is present with resistance into plantarflexion
netic resonance imaging, and histology con- and eversion, and ankle instability is common.
firm the diagnosis.20 Treatment may involve an
aggressive regimen of antibiotic medications, Background Information
with surgical resection of the affected areas The presentation of this condition differs from
potentially necessary. peroneal tendinopathy (tendinitis/tendinosis)
because symptoms often will persist even with
■ Osteosarcoma treatment. If there is a complete rupture,
which is more common with the peroneus
Chief Clinical Characteristics
longus tendon, an audible pop may have been
This presentation may be characterized by an
observed. This injury often is associated with
insidious onset of pain that persists for weeks
ankle fractures, severe lateral ankle sprains,
or months. Pain due to this condition may be
peroneal tenosynovitis, or a previous history
aggravated with activity, causing limping.
of peroneal tendon injuries. Magnetic reso-
Background Information nance imaging confirms the diagnosis. Treat-
This condition is frequently found in adoles- ment ranges from nonsurgical options, such
cents because of the active bone growth in this as physical therapy intervention, to surgical
age group. African American individuals are options, such as debridement of partial tears
affected slightly more often than Caucasian in- and repair of complete tears.
dividuals. This condition can be especially
fatal if metastasized to the lungs. Plain films ■ Posteromedial Shin Splints
confirm the diagnosis. Treatment includes Chief Clinical Characteristics
chemotherapy and surgery to remove the This presentation often is characterized by pain
cancerous cells or tumors. and palpable tenderness along the muscles
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Chapter 23 Rubella and Rubella Vaccine–Associated Arthritis 437

attached to the posteromedial aspect of the tibia Other symptoms can include tendon synovitis,

ANKLE PAIN
within the deep posterior compartment. Pain fasciitis, back pain, mucocutaneous lesions, skin
is usually less often located directly over the lesions, or cardiac involvement. Joint pain
tibia. Pain is aggravated with stretching into foot accompanies urethritis and conjunctivitis.
dorsiflexion and eversion, and with active con-
traction of the involved muscles. Pain usually Background Information
lessens following warm-up of the muscles. Pain This disease typically follows either an episode
is usually present during activity with soreness of dysentery or infectious arthritis, and persons
following the activity, but pain does not usually with the HLA-B27 genetic makeup are at greater
occur during the night. risk.24 Diagnosis is usually based on the patient’s
history, radiographs, and blood tests to rule out
Background Information other forms of arthritis or potentially associated
Shin splints are due to an eccentric overuse, infections. This is usually a self-limiting condi-
leading to musculotendinous injury along the tion and resolves in 3 to 4 months. When
posteromedial attachment of the tibia, result- provided, treatment involves a combination of
ing in a periostitis.21,22 Diagnosis is based on antibiotic medications directed toward the infec-
the clinical presentation. Imaging is usually tive agent that is responsible for the autoimmune
unhelpful, although fat-suppressed magnetic response that characterizes this condition, in
resonance imaging may help differentiate combination with anti-inflammatory medication.
between stress fractures and shin splints.7
Usual intervention is nonsurgical. ■ Rheumatoid Arthritis
■ Pseudogout Chief Clinical Characteristics
This presentation can involve symmetric foot
Chief Clinical Characteristics
pain with localized tenderness, swelling, and
This presentation may include pain, edema,
early morning stiffness. The feet may be the
warmth, erythema, and tenderness of the in-
first region affected by the arthritis; however,
volved joint or joints. The arthritis may mimic
multiple joints are often affected. After the ini-
other types of arthritis, but attacks usually are
tial stages, subcutaneous rheumatoid nodules
less severe than those experienced with gout. The
and joint deformities may present, most com-
first metatarsophalangeal joint, ankle, dorsum
monly flexion deformities. The metatarsopha-
of the foot, knees, wrists, or shoulders may be
langeal joints are the joints most commonly
affected. This condition is prevalent in indi-
affected by rheumatoid arthritis.25
viduals over 60 years of age.
Background Information Background Information
Causes for the contributory calcium pyrophos- This is a progressive systemic condition with
phate crystal deposition in synovial joints are unknown etiology. Diagnosis, according to the
unknown. Diagnosis is based on the clinical American Rheumatism Association, includes
presentation, the pattern of joints involved, at least a 6-week presentation with any four of
radiographic intra-articular and periarticular the seven following manifestations: morning
involvement, and aspiration of calcium py- stiffness for more than 1 hour, arthritis of
rophosphate crystals in the joint fluid.23 Anti- three or more joints, arthritis of the hand
inflammatory medication comprises the usual joints, symmetric arthritis, rheumatoid nod-
treatment for this condition. Unlike with gout, ules, serum rheumatoid factor, or radiographic
uric acid–lowering medications are not used. changes. Treatment typically includes a variety
of steroidal, nonsteroidal, and biological
■ Reiter’s Syndrome anti-inflammatory medications.
Chief Clinical Characteristics
This presentation typically includes asymmet- ■ Rubella and Rubella
ric and varying severity of joint pain and Vaccine–Associated Arthritis
stiffness of the subtalar joint, the metatarsopha- Chief Clinical Characteristics
langeal joints, interphalangeal joints, tarsal This presentation includes symmetric pol-
joints, calcaneal abnormalities, and knees. yarthralgia that is often correlated with joint
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438 Chapter 23 Rupture of the Achilles Tendon

stiffness and preceded by a prodromal stage ■ Talar Compression


ANKLE PAIN

of malaise, low-grade fever, and lymphadenopa- Syndrome/Impingement


thy and by skin rash. The rash is similar in Chief Clinical Characteristics
appearance to the measles. Arthralgias are more This presentation can involve pain, swelling,
common in children, whereas the joint stiffness and tenderness to palpation over the posterior
is more common in adults. aspect of the lateral ankle, between the Achilles
Background Information tendon and peroneals, and that pain is increased
This condition is caused by a ribonucleic acid with forced plantarflexion.
viral infection and diagnosis is usually con- Background Information
firmed with laboratory tests (serology) and The mechanism of injury is a compression of
clinical examination. It is usually self-limiting the posterior structures, between the posterior
and the symptoms usually alleviate in several tibia and the talar process, or an os trigonum
weeks. (accessory center of ossification), if present.
■ Rupture of the Achilles Tendon This injury is common in ballet dancers, gym-
nasts, and ice skaters. Associated injuries
Chief Clinical Characteristics include flexor hallucis longus stenosing
This presentation may be characterized by tenosynovitis, hypertrophic posterior capsuli-
a sudden onset of pain, palpable tenderness, tis, and calcific debris. Diagnosis is based
significant weakness, and sometimes a palpa- on the clinical examination, and imaging for
ble sulcus, usually 2 to 6 cm proximal to the confirmation if a clinical diagnosis is unclear.
distal insertion of the Achilles tendon. Relative rest with progressive reloading com-
Background Information prises the typical treatment for this condition,
This condition often occurs spontaneously although arthroscopic debridement may be
during an activity that places loading on the necessary in cases of recalcitrant disability.
plantarflexors. It is associated with age over
40 years and a history of Achilles tendinitis/ ■ Tarsal Tunnel Syndrome
tendinosis. The Thompson test and magnetic Chief Clinical Characteristics
resonance or ultrasound imaging confirm This presentation may include hypesthesias,
the diagnosis. Surgical intervention is indi- pain, and paresthesias due to compression of the
cated for repair of the Achilles tendon in most tibial nerve at the ankle, located around the
individuals. medial aspect of the ankle and the plantar sur-
face of the foot. Symptoms often are worse
■ Septic Arthritis with walking, with compression, and with a
Chief Clinical Characteristics dorsiflexion-eversion test. They present with a
This presentation includes achy to throbbing positive Tinel’s sign. Rest alleviates symptoms.
pain, erythema, edema, and palpable warmth. Weakness of the flexor hallucis and flexor
Systemic signs of infection may include fever or digitorum brevis muscles is rarely present.
malaise. This condition more often is monar-
Background Information
ticular and can have a sudden onset.
Common etiologies are space-occupying
Background Information lesions, fractures, edema, tendinitis or tenosyn-
Septic arthritis can result from a fungal, bacte- ovitis, accessory bones, or prolonged direct
rial, or viral pathogen infecting the periarticu- and indirect pressures from inflammation and
lar or synovial tissues; Staphylococcus aureus is fluid retention as a result of systemic diseases
the most common cause in all ages. Computed (rheumatoid arthritis, ankylosing spondylitis)
tomography and magnetic resonance imaging or significant hindfoot pronation that causes
combined with culture and staining of aspi- tibial nerve stretch. Diagnosis is confirmed
rated joint fluid commonly confirm the diag- with the clinical examination and nerve con-
nosis. Treatment usually involves a combina- duction studies. Nonsurgical treatment—such
tion of an aggressive antibiotic medication and as physical therapy intervention, orthoses,
joint drainage, perhaps by way of open and corticosteroid injections—may be used to
drainage and/or surgical lavage. address this condition. Surgical intervention
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Chapter 23 Flexor Digitorum Longus Tendinitis 439

may be considered for individuals with persist- clinically, but may be confirmed with mag-

ANKLE PAIN
ent disability. netic resonance or ultrasound imaging.
Nonsurgical treatments may be indicated,
TENDINITIS such as anti-inflammatory medication, rest,
and gentle exercise.
■ Achilles Tendinitis
Chief Clinical Characteristics ■ Extensor Hallucis Longus
This presentation can involve a sharp, Tendinitis
aching, or burning pain with point tender- Chief Clinical Characteristics
ness 2 to 6 cm proximal to the calcaneal This presentation may include pain and pal-
insertion. Insertional tendinopathy may be pable tenderness along the extensor hallucis
located at the distal insertion at the calca- tendon. Walking and running are often
neus. Symptoms are reproduced with palpa- painful. Resisted extension of the hallux and
tion of the tendon, passive dorsiflexion, and passive flexion of the hallux often increase
active resisted plantarflexion. Palpable the pain. Pain may also be elicited with resis-
crepitus may be present with movement, ted dorsiflexion of the ankle.
and an increased thickening of the tendon
that moves with tendon excursion may be Background Information
palpable in comparison to the asympto- This pathology is usually the result of over-
matic side. Calf atrophy may be observed, use of the affected musculotendinous unit.26
the patient may have limitations in ankle Diagnosis is usually made clinically, but may
dorsiflexion, and the patient may report be confirmed with magnetic resonance or
increased stiffness in the morning. ultrasound imaging. Treatment usually in-
volves nonsurgical interventions, such as
Background Information anti-inflammatory medication, rest, and
Achilles tendinitis is associated with over- gentle exercise.
use from repetitive loading of the involved
tendon. This injury is most common in ■ Flexor Digitorum Longus
15- to 45-year-old active individuals, most Tendinitis
commonly runners. Diagnosis is often Chief Clinical Characteristics
made clinically. If the diagnosis is difficult, This presentation can be characterized by
then confirmation may be made with pain, tenderness, and swelling along the plan-
magnetic resonance imaging. Usual treat- tar hindfoot and midfoot with tenderness
ment is nonsurgical, consisting of anti- along the tendon of the flexor digitorum
inflammatory medication, rest, and gentle longus. Pain is aggravated with walking and
exercise. weight bearing.
■ Extensor Digitorum Longus Background Information
Tendinitis A common site of irritation is deep to the
Chief Clinical Characteristics flexor retinaculum at the posteromedial
This presentation can be characterized ankle. Palpation can be aided with the use of
by pain over the dorsum of the foot and ten- muscle contraction or active movement of the
derness to palpation along the extensor dig- toes. If the tendons are inflamed, then pain is
itorum longus tendons. Resisted extension of often reproduced with stretching of the toes
the lesser toes and passive flexion of the into extension or contraction into flexion.
lesser toes often increases the pain. Pain may The pathology is often related to trauma or
also be elicited with resisted dorsiflexion overuse of the affected musculotendinous
of the ankle. Walking and running are often unit. Diagnosis is often determined clinically
painful. and may be confirmed with magnetic reso-
nance or ultrasound imaging. Treatment
Background Information usually involves nonsurgical interventions,
The pathology of this tendinitis is usually the such as anti-inflammatory medication, rest,
result of overuse. Diagnosis is usually made and gentle exercise.
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440 Chapter 23 Flexor Hallucis Longus Tendinitis

■ Flexor Hallucis Longus ■ Tibialis Anterior Tendinitis


ANKLE PAIN

Tendinitis Chief Clinical Characteristics


Chief Clinical Characteristics This presentation typically includes pain and
This presentation typically includes pain tenderness to palpation of the tibialis anterior
and tenderness to the flexor hallucis longus tendon.
tendon. Pain is aggravated with walking,
Background Information
weight bearing, or stretching of the tendon.
Pain is often present at initial contact and
Swelling is often noted in the region of the
during the loading response of gait, due to the
pathology.
eccentric loading of the tendon and muscle.
Background Information Pain is often increased with passive stretch
The location of the pathology is often at into plantarflexion or resisted dorsiflexion of
the medial aspect of the midfoot and can the ankle. Tibialis anterior tendinitis is usually
usually be isolated along the tendon with caused by overuse.26 Diagnosis is usually
palpation. A common site of irritation is made clinically, but may be confirmed with
under the flexor retinaculum at the pos- magnetic resonance or ultrasound imaging.
teromedial ankle. Palpation can be aided Usual treatment is nonsurgical, consisting
with the use of muscle contraction or of anti-inflammatory medication, rest, and
movement of the hallux. The pathology is gentle exercise.
often related to overuse of the affected
musculotendinous unit. Diagnosis is usu- ■ Tibialis Posterior Tendinitis
ally made clinically, but may be confirmed Chief Clinical Characteristics
with magnetic resonance or ultrasound This presentation may involve pain and
imaging. Management typically involves a swelling with palpable tenderness along the
combination of anti-inflammatory med- medial aspect of the midfoot and sometimes
ication, rest, and gentle exercise. posterior to the medial malleolus. Pain is
often worsened with weight bearing, walk-
■ Peroneal Tendinitis ing, performing a heel rise, or stretching the
Chief Clinical Characteristics tendon. Attenuation of this tendon is some-
This presentation can involve pain anywhere times seen and can lead to a flat-foot defor-
along the lateral lower leg to the posterior lat- mity. Palpation may be assisted with muscle
eral malleolus and along the lateral foot at contraction of the tibialis posterior muscle.
the location of the cuboid or at the plantar Background Information
aspect of the foot where the peroneus longus Injury to this tendon is often the result of
tendon inserts at the base of the first overuse that is caused by a hypermobile
metatarsal. The pain may also be present at midfoot and excessive foot pronation. The
the base of the fifth metatarsal where the tibialis posterior is the most significant
peroneus brevis tendon inserts. The pain is active stabilizer of the arch.27 Diagnosis is
often aggravated with a stretch into foot and usually made clinically, but may be con-
ankle dorsiflexion and inversion or a mus- firmed with magnetic resonance or ultra-
cle contraction into foot and ankle plan- sound imaging. Nonsurgical treatments may
tarflexion and/or eversion. be indicated, such as anti-inflammatory
Background Information medication, rest, and gentle exercise.
Common causes of peroneal tendon injury
TENDINOSIS
include overuse and inversion injury mech-
anisms. Diagnosis is usually made clini- ■ Achilles Tendinosis
cally, but may be confirmed with magnetic Chief Clinical Characteristics
resonance or ultrasound imaging. Nonsur- This presentation typically includes sharp,
gical treatments may be indicated, such aching, or burning pain with point tenderness
as anti-inflammatory medication, rest, and usually along the medial aspect of the middle
gentle exercise. one-third of the Achilles tendon. Some
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Chapter 23 Tibiofibular Synostosis 441

patients with Achilles tendinosis are pain free. state of increased tenocyte and cellular

ANKLE PAIN
Symptoms, if present, may be reproduced with activity that leads to disorganized collagen
palpation of the Achilles tendon, passive dor- and vascular hyperplasia.28,29 Final diagnosis
siflexion, or active resisted plantarflexion. is confirmed with magnetic resonance or
Palpable increased thickening of the tendon ultrasound imaging. A combination of
may be present in comparison to the unin- patient/client education, unloading of the
volved tendon. Calf atrophy, limitations in affected musculotendinous unit, controlled
ankle dorsiflexion, and reports of increased mechanical reloading, and preventive meas-
stiffness in the morning may be observed. Pain ures appears to be effective to manage this
is often worse with weight bearing and walk- condition.30
ing, and these patients often have difficulty
performing a heel rise. ■ Tibialis Posterior Tendinosis
Background Information Chief Clinical Characteristics
Tendinosis is a state of increased tenocyte and This presentation typically includes pain and
cellular activity that leads to disorganized col- swelling with palpable tenderness along the
lagen and vascular hyperplasia.28,29 Tendinosis medial aspect of the midfoot and posterior
is usually a chronic degenerative process that is to the medial malleolus. Pain is often worse
initiated by an injury to the tendon. Diagnosis with weight bearing and walking, and these
is usually based on the clinical presentation. If patients often have difficulty performing a heel
pathology is severe or the clinical diagnosis is rise. Attenuation of this tendon is sometimes
unclear, then magnetic resonance or ultra- seen as a result of tendinosis and the develop-
sound imaging may confirm the diagnosis ment of posterior tibialis tendon dysfunction
and allow for prognosis and staging of the may lead to a flat-foot deformity and a caudal
tendinosis. A combination of patient/client drop of the navicular. These patients have a very
education, unloading of the affected musculo- difficult time contracting the tibialis posterior
tendinous unit, controlled mechanical reload- muscle, even during a manual muscle test.
ing, and preventive measures appears to be Background Information
effective to manage this condition.30 Injury to this tendon is often the result of
■ Peroneal Tendinosis overuse with a hypermobile midfoot. The tib-
ialis posterior is the most significant
Chief Clinical Characteristics active stabilizer of the arch.27 Tendinosis is a
This presentation typically includes pain any- state of increased tenocyte and cellular activity
where along the lateral lower leg to the pos- that leads to disorganized collagen and vascu-
terior lateral malleolus and along the lateral lar hyperplasia. Tendinosis is usually a chronic
foot at the location of the cuboid or at the degenerative process that is initiated by injury
plantar aspect of the foot where the peroneus to the tendon. Diagnosis is usually based on
longus tendon inserts at the base of the first the clinical presentation; however, magnetic
metatarsal. The pain may also be present at resonance imaging may confirm the diagnosis
the base of the fifth metatarsal where the per- and allow for prognosis and staging of the
oneus brevis tendon inserts. The pain is often tendinosis. A combination of patient/client
aggravated with a stretch into dorsiflexion education, unloading of the affected musculo-
and inversion or a muscle contraction into tendinous unit, controlled mechanical reload-
plantarflexion and/or eversion. Weakness ing, and preventive measures appears to be
may be noted into plantarflexion and/or effective to manage this condition.30
eversion due to degenerative thickening,
lengthening, and tearing of the tendon. ■ Tibiofibular Synostosis
Background Information Chief Clinical Characteristics
Common causes of peroneal tendon injury This presentation typically includes pain at the
include overuse and inversion injury mecha- anterior ankle and lower leg and instability
nisms that may lead to the chronic degener- with activities such as cutting, pivoting, running,
ative process of tendinosis. Tendinosis is a or performing agility activities.
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442 Chapter 23 Transient Migratory Osteoporosis

Background Information of the peroneals, and commonly occurs during


ANKLE PAIN

This condition is associated with a history of skiing, ice skating, running, basketball, and
recurrent inversion ankle sprains that remain football. Associated injuries include lateral an-
symptomatic due to widening of the inferior kle retinaculum tear, avulsion fracture of the
tibiofibular joint and bone formation in the inferior tip of the lateral malleolus, and poste-
interosseous membrane. The mechanism of rior talofibular ligament injury. Diagnosis is
injury for the fracture is the same as the origi- based on the clinical examination. Treatment
nal injury. With a synostosis present in the ab- ranges from nonsurgical reduction and immo-
sence of a fracture, pain may be present with bilization to surgical fixation of the dislocated
dorsiflexion and pushing off. Injuries to asso- tendons.
ciated structures could include the anterior
and posterior inferior tibiofibular ligaments, References
the interosseous membrane, the lateral ankle 1. Merskey H, Bogduk N. Classification of Chronic Pain:
Descriptions of Chronic Pain Syndromes and Definitions
ligaments, and possibly the talar dome lesions. of Pain Terms. Seattle, WA: IASP Press; 1994.
A clinical impingement test and radiographs 2. Moseley GL. Imagined movements cause pain and
confirm the diagnosis. Typical intervention is swelling in a patient with complex regional pain syn-
nonsurgical. drome. Neurology. May 11, 2004;62(9):1644.
3. Abshire T. An approach to target joint bleeding in
■ Transient Migratory Osteoporosis hemophilia: prophylaxis for all or individualized treat-
ment? J Pediatr. Nov 2004;145(5):581–583.
Chief Clinical Characteristics 4. Rodriguez-Merchan EC. Management of musculoskele-
This presentation typically includes pain and tal complications of hemophilia. Semin Thromb Hemost.
Feb 2003;29(1):87–96.
swelling at the ankle joint, progressing over time 5. Carapetis JR, Currie BJ. Rheumatic fever in a high
to adjacent joints with symptoms usually lasting incidence population: the importance of monoarthritis
6 months to a year. Pitting edema, palpable and low grade fever. Arch Dis Child. Sep 2001;85(3):
warmth, erythema, or trophic changes are unlikely. 223–227.
6. Slocum DB. The shin splint syndrome. Medical aspects
Background Information and differential diagnosis. Am J Surg. Dec 1967;114(6):
The etiology of this condition is unknown. 875–881.
7. Aoki Y, Yasuda K, Tohyama H, Ito H, Minami A. Mag-
Transient migratory osteoporosis differs from netic resonance imaging in stress fractures and shin
senile osteoporosis in the presentation of pain splints. Clin Orthop. Apr 2004(421):260–267.
and edema, in addition to multiple joints 8. Salvarani C, Vlachonikolis IG, van der Heijde DM, et al.
affected in the same individual. Radiographic Musculoskeletal manifestations in a population-based
cohort of inflammatory bowel disease patients. Scand
evidence of osteoporosis in the presence of J Gastroenterol. Dec 2001;36(12):1307–1313.
normal laboratory tests confirms the diagno- 9. Springer BL, Clarkson PM. Two cases of exertional
sis.31 It is sometimes difficult to differentiate rhabdomyolysis precipitated by personal trainers. Med
from avascular necrosis. This condition is usu- Sci Sports Exerc. Sep 2003;35(9):1499–1502.
10. Judd DB, Kim DH. Foot fractures frequently misdiag-
ally self-limiting and resolves within 1 year. nosed as ankle sprains. Am Fam Physician. Sep 1, 2002;
66(5):785–794.
■ Traumatic Dislocation of the 11. Canale ST, Belding RH. Osteochondral lesions of the
Peroneal Tendons talus. J Bone Joint Surg Am. Jan 1980;62(1):97–102.
12. Loomer R, Fisher C, Lloyd-Smith R, Sisler J, Cooney T.
Chief Clinical Characteristics Osteochondral lesions of the talus. Am J Sports Med.
This presentation typically includes sudden on- Jan–Feb 1993;21(1):13–19.
set of posterolateral ankle pain and swelling, 13. Kramer HM, Curhan G. The association between gout
with possible snapping posterior to and over and nephrolithiasis: the National Health and Nutrition
Examination Survey III, 1988–1994. Am J Kidney Dis.
the lateral malleolus and tenderness over the Jul 2002;40(1):37–42.
peroneal tendons. Resisted dorsiflexion on 14. Xenos JS, Hopkinson WJ, Mulligan ME, Olson EJ,
a plantarflexed and everted foot will often Popovic NA. The tibiofibular syndesmosis. Evaluation
reproduce pain and may reproduce the tendon of the ligamentous structures, methods of fixation,
and radiographic assessment. J Bone Joint Surg Am.
dislocation. Jun 1995;77(6):847–856.
15. Sangeorzan BJ. Painful disorders of the ankle and heel.
Background Information In Loeser JD, eds. Bonica’s Management of Pain. 3rd ed.
This injury results from dorsiflexion force on Philadelphia, PA: Lippincott Williams & Wilkins;
an inverted ankle during muscle contraction 2001:1637–1646.
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Chapter 23 References 443

16. Joines JD, McNutt RA, Carey TS, Deyo RA, Rouhani R. 24. Eapen BR. A new insight into the pathogenesis of

ANKLE PAIN
Finding cancer in primary care outpatients with low Reiter’s syndrome using bioinformatics tools. Int
back pain: a comparison of diagnostic strategies. J Gen J Dermatol. Mar 2003;42(3):242–243.
Intern Med. 2001;16(1):14–23. 25. Smyth CJ, Janson RW. Rheumatologic view of the
17. Holman PJ, Suki D, McCutcheon I, Wolinsky JP, Rhines rheumatoid foot. Clin Orthop. Jul 1997(340):7–17.
LD, Gokaslan ZL. Surgical management of metastatic 26. Karlsson J, Lundin O, Lossing IW, Peterson L. Partial
disease of the lumbar spine: experience with 139 rupture of the patellar ligament. Results after operative
patients. J Neurosurg Spine. 2005;2(5):550–563. treatment. Am J Sports Med. Jul–Aug 1991;19(4):
18. Kelberine F, Frank A. Arthroscopic treatment of osteo- 403–408.
chondral lesions of the talar dome: a retrospective study 27. Thordarson DB, Schmotzer H, Chon J, Peters J.
of 48 cases. Arthroscopy. Jan–Feb 1999;15(1):77–84. Dynamic support of the human longitudinal arch.
19. Crist BD, Lenke LG, Lewis S. Osteoid osteoma of the A biomechanical evaluation. Clin Orthop. Jul 1995(316):
lumbar spine. A case report highlighting a novel 165–172.
reconstruction technique. J Bone Joint Surg Am. 28. Mafi N, Lorentzon R, Alfredson H. Superior short-term
Feb 2005;87(2):414–418. results with eccentric calf muscle training compared to
20. Lipman BT, Collier BD, Carrera GF, et al. Detection of concentric training in a randomized prospective multi-
osteomyelitis in the neuropathic foot: nuclear medicine, center study on patients with chronic Achilles tendi-
MRI and conventional radiography. Clin Nucl Med. nosis. Knee Surg Sports Traumatol Arthrosc. 2001;9(1):
Feb 1998;23(2):77–82. 42–47.
21. Michael RH, Holder LE. The soleus syndrome. A cause 29. Kraushaar BS, Nirschl RP. Tendinosis of the elbow (ten-
of medial tibial stress (shin splints). Am J Sports Med. nis elbow). Clinical features and findings of histological,
Mar–Apr 1985;13(2):87–94. immunohistochemical, and electron microscopy stud-
22. Beck BR, Osternig LR. Medial tibial stress syndrome. ies. J Bone Joint Surg Am. Feb 1999;81(2):259–278.
The location of muscles in the leg in relation to symp- 30. Davenport TE, Kulig K, Matharu Y, Blanco CE. The
toms. J Bone Joint Surg Am. Jul 1994;76(7):1057–1061. EdUReP model for nonsurgical management of
23. Cassetta M, Gorevic PD. Crystal arthritis. Gout and tendinopathy. Phys Ther. Oct 2005;85(10):1093–1103.
pseudogout in the geriatric patient. Geriatrics. Sep 2004; 31. McCarthy EF. The pathology of transient regional
59(9):25–30; quiz 31. osteoporosis. Iowa Orthop J. 1998;18:35–42.
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CHAPTER 24
Foot Pain
■ Jason R. Cozby, PT, DPT, OCS ■ Lisa Meyer, PT, DPT, OCS
■ Stephen F. Reischl, PT, DPT, OCS

Description of the Symptom Special Concerns


■ Decreased sensation
This chapter describes pathology that may lead ■ Diminished dorsalis pedis or tibial pulses
to foot pain, including generalized, dorsal foot, ■ Pain that increases at night or with rest
forefoot and toe, hindfoot, and midfoot. Local ■ Skin breakdown
causes of foot pain are defined as pathology ■ Warmth and edema
that occurs within the foot, either deep to or
distal to the medial and lateral malleoli of the
tibia. Remote causes are defined as occurring
outside this region.

CHAPTER PREVIEW: Conditions That May Lead to Foot Pain

T Trauma
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Lumbar radiculopathies: Nerve entrapments: Bite injuries 459
• L5 radiculopathy 455 • Tarsal tunnel syndrome 475 Fractures:
• S1 radiculopathy 456 • Metatarsal 466

444
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FOOT PAIN
Dorsal foot pain Hindfoot pain

Midfoot pain Midfoot pain

Forefoot pain Forefoot pain

LOCAL LOCAL LOCAL


FOREFOOT AND TOE HINDFOOT MIDFOOT

Calluses 460 Fractures: Longitudinal arch


Corns 461 • Calcaneus 464 strain 472
Crush injury 461 Ligament sprains: Plantar fasciitis 477
Fractures: • Anterior talofibular Positional faults:
• Hallucal sesamoid disorders 465 ligament 471 • Calcaneocuboid joint 477
• Metatarsal 466 • Calcaneofibular ligament • Talonavicular joint 478
• Toe 468 (isolated) 472
Hammer toe deformity 469 Nerve entrapments:
Ligament sprains: • Tarsal tunnel syndrome 475
• Forefoot 472 Plantar fasciitis 477
Metatarsalgia 473 Strain of the peroneal
Shoe vamp ulcer and bursitis 480 muscles 481
(continued)

445
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446 Chapter 24 Foot Pain

Trauma (continued)
FOOT PAIN

LOCAL LOCAL
REMOTE GENERALIZED DORSAL
UNCOMMON
Lumbar radiculopathies: Bite injuries 459 Fractures:
• L4 radiculopathy 455 Cold injuries 460 • Osteochondral fracture of the
Crush injury 461 subtalar joint 466
Nerve entrapments: Ligament sprains:
• Deep peroneal nerve 475 • Cruciate crural ligament 472
• Superficial peroneal nerve 475 Nerve entrapments:
• Sural nerve 475 • Deep peroneal nerve 475
• Superficial peroneal
nerve 475

RARE
Not applicable Not applicable Not applicable

I Inflammation
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Aseptic Aseptic
Dermatitis 462 Tendinitis:
Reiter’s syndrome 479 • Extensor digitorum longus
Rheumatoid arthritis 479 tendinitis 481
• Extensor hallucis longus
tendinitis 482
• Tibialis anterior tendinitis 482
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Chapter 24 Foot Pain 447

FOOT PAIN
LOCAL LOCAL LOCAL
FOREFOOT AND TOE HINDFOOT MIDFOOT

Dislocations: Calcaneal apophysitis (Sever’s Fractures:


• Interphalangeal joint 462 disease) 459 • Fifth metatarsal 464
Ligament sprains: Fat pad syndrome 464 • Navicular 466
• Metatarsophalangeal joint 472 Fractures: Interosseous myositis 470
Mallet toe deformity 473 • Lateral process of the talus 465
Morton’s neuroma 474 • Lateral tubercle of the
posterior process of the
talus 465
• Medial process of the
sustentaculum tali of the
calcaneus 465
• Medial tuberosity of the
posterior process of the
talus 466
• Osteochondral fracture of the
talar dome 466
• Posterosuperior calcaneal
tuberosity 467
• Stress fracture of the
calcaneus 467
• Stress fracture of the lateral
malleolus 467
Nerve entrapments:
• First branch of the lateral
plantar nerve 475
• Sural nerve 475
Ruptures:
• Achilles tendon 480
• Plantar fascia 480

Dislocations: Nerve entrapments: Fractures:


• Metatarsophalangeal joint 462 • Calcaneal branch • Osteochondral fracture of
Joplin’s neuroma 471 neurodynia 474 the subtalar joint 466
• Tarsometatarsal joint
(Lisfranc’s) fracture or
dislocation 467

LOCAL LOCAL LOCAL


FOREFOOT AND TOE HINDFOOT MIDFOOT

Aseptic Aseptic Not applicable


Not applicable Achilles paratenonitis 456
Bursitis:
Septic • Retrocalcaneal bursitis 459
Paronychia 477 • Subcutaneous Achilles
Tinea pedis (athlete’s foot) 485 bursitis 459
Warts 486
(continued)
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448 Chapter 24 Foot Pain

Inflammation (continued)
FOOT PAIN

LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Septic Septic
Bite injuries 459 Cellulitis 460
Cellulitis 460
Tinea pedis (athlete’s foot) 485

UNCOMMON
Not applicable Aseptic Aseptic
Acute rheumatic fever 457 Interosseous myositis 470
Ankylosing spondylitis 457
Arthritis associated with Septic
inflammatory bowel Not applicable
disease 458
Complex regional pain
syndrome 461
Hepatitis B–associated
arthritis 469
Herpes zoster 469
Hypersensitivity vasculitis 470
Polyarteritis nodosa 477
Rheumatoid arthritis–like
diseases of the foot:
• Psoriatic arthritis 479
• Systemic lupus erythematosus 479
Rubella and rubella vaccine–
associated arthritis 480
Septic
Abscess 456
Hand-foot-and-mouth disease
469
Osteomyelitis 476
RARE
Not applicable Aseptic Aseptic
Allergic angiitis and Herpes zoster 469
granulomatosis (Churg-Strauss
disease) 457 Septic
Erosive lichen planus 463 Abscess 456
Fixed drug eruption 464 Osteomyelitis 476
Septic arthritis 480
Septic
Necrotizing fasciitis 474
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Chapter 24 Foot Pain 449

FOOT PAIN
LOCAL LOCAL LOCAL
FOREFOOT AND TOE HINDFOOT MIDFOOTUNCOMMON

Tendinitis:
• Achilles tendinitis 481
• Peroneal tendinitis 482
• Tibialis posterior
tendinitis 483
Septic
Not applicable

Aseptic Aseptic Aseptic


Idiopathic metatarsophalangeal Haglund’s syndrome 468 Tendinitis:
synovitis 470 Tendinitis: • Flexor hallucis longus
• Flexor digitorum longus tendinitis 482
Septic tendinitis 482 • Peroneal tendinitis 482
Candidiasis 460 • Flexor hallucis longus • Tibialis posterior
Infected blister 470 tendinitis 482 tendinitis 483
Septic Septic
Not applicable Abscess 456
Osteomyelitis 476

Aseptic Aseptic Not applicable


Not applicable Not applicable
Septic Septic
Osler’s nodules 476 Osteomyelitis 476
Pulp-space infection 478
Septic arthritis 480
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450 Chapter 24 Foot Pain


FOOT PAIN

M Metabolic
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Benign muscle cramps 459 Envenomation 463
Distal symmetric Gout 468
polyneuropathy, including
from acquired
immunodeficiency syndrome,
alcohol/drug abuse,
carcinoma, diabetes mellitus,
and kidney failure 462
Envenomation 463
Gout 468
UNCOMMON
Not applicable Pseudogout 478 Pseudogout 478

RARE
Not applicable Fabry’s disease 463 Not applicable
Intermittent acute porphyria 470
Primary amyloidosis 478
Vitamin B deficiency 485

Va Vascular
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Arterial insufficiency 457 Not applicable
Sickle cell crisis 480

UNCOMMON
Not applicable Arterial occlusion 457 Not applicable
Cholesterol embolism 460

RARE
Not applicable Arteriosclerosis obliterans 458 Avascular necrosis of the
Compartment syndrome 461 navicular 458
Cryoglobulinemia 461
Erythromelalgia 463
Thromboangiitis (Buerger’s
disease) 485
Waldenstrom’s
macroglobulinemia 486
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Chapter 24 Foot Pain 451

FOOT PAIN
LOCAL LOCAL LOCAL
FOREFOOT AND TOE HINDFOOT MIDFOOT

Gout 468 Not applicable Not applicable

Distal symmetric polyneuropathy, Not applicable Not applicable


including from acquired
immunodeficiency syndrome,
alcohol/drug abuse, carcinoma,
diabetes mellitus, and kidney
failure 462
Pseudogout 478

Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


FOREFOOT AND TOE HINDFOOT MIDFOOT

Ischemic ulceration of the Not applicable Not applicable


toes 471
Subungual hematoma 481

Arterial insufficiency 457 Not applicable Not applicable


Freiberg’s disease 468
Raynaud’s disease 478

Not applicable Not applicable Avascular necrosis of the


navicular 458
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452 Chapter 24 Foot Pain


FOOT PAIN

De Degenerative
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Not applicable Osteoarthrosis/osteoarthritis:
• Midfoot (tarsometatarsal
joints) 476
Tendinosis:
• Extensor digitorum longus
tendinosis 483
• Extensor hallucis longus
tendinosis 483
• Tibialis anterior tendinosis 484
UNCOMMON
Not applicable Not applicable Not applicable

RARE
Not applicable Not applicable Not applicable

Tu Tumor
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable

RARE
Not applicable Malignant Primary, such as: Not applicable
• Leukemia 471
• Lymphoma 473
• Multiple myeloma 474
Malignant Metastatic, such as:
• Metastases to the foot,
including from primary breast,
kidney, lung, prostate, and
thyroid disease 473
Benign:
Not applicable
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Chapter 24 Foot Pain 453

FOOT PAIN
LOCAL LOCAL LOCAL
FOREFOOT AND TOE HINDFOOT MIDFOOT

Hallux rigidus 469 Tendinosis: Osteoarthrosis/osteoarthritis:


Hallux valgus 469 • Achilles tendinosis 483 • Midfoot (tarsometatarsal
Osteoarthrosis/osteoarthritis: • Peroneal tendinosis 484 joints) 476
• Interphalangeal joint 476 • Tibialis posterior
• Small toes 476 tendinosis 485

Not applicable Atrophic fat pad disorder 458 Tendinosis:


Tendinosis: • Flexor hallucis longus
• Flexor digitorum longus tendinosis 484
tendinosis 484 • Peroneal tendinosis 484
• Flexor hallucis longus • Tibialis posterior
tendinosis 484 tendinosis 485

Calcific tendinitis of the flexor Not applicable Not applicable


hallucis longus or brevis 460

LOCAL LOCAL LOCAL


FOREFOOT AND TOE HINDFOOT MIDFOOT

Not applicable Not applicable Not applicable

Malignant Primary: Malignant Primary: Not applicable


Not applicable Not applicable
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign, such as: Benign, such as:
• Submetatarsal cyst 481 • Fat pad separation secondary
to a cyst 463
• Piezogenic papules 477

Not applicable Malignant Primary: Not applicable


Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Glomus tumor 468
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454 Chapter 24 Fear and Avoidance


FOOT PAIN

Co Congenital
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Dysplasia epiphysealis Not applicable
hemimelica (Trevor’s
disease) 462
RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
LOCAL LOCAL
REMOTE GENERALIZED DORSAL
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Fear and avoidance 454 Not applicable Not applicable
RARE
Psychological effect of Not applicable Not applicable
complex regional pain
syndrome 456

Note: These are estimates of relative incidence because few data are available for the less common conditions.

an end target site for multiple disease


Overview of Foot Pain
processes, so all potential diagnoses should
Pain that is experienced in the foot is com- be considered even if mechanical trauma is
monly the result of traumatic injury or me- associated with the presentation.
chanical disorders that affect the underlying
structures. The foot is the structure of the Description of Conditions That
lower extremity that comes in direct contact May Lead to Foot Pain
with the ground and therefore experiences
very high forces due to ground reaction forces, Remote
forces originating from functional activities, ■ Fear and Avoidance
and forces that arise during attempts to con-
Chief Clinical Characteristics
trol body weight and momentum. Injury to
This presentation may include avoiding the use
the foot may lead to alignment issues as well as
of an extremity as a result of apprehension of
damage to multiple joints or tissues. Further-
onset of pain.
more, damage to multiple structures may lead
to a more generalized pain. Nevertheless, the Background Information
foot also may experience pain as a result of Injury to the foot and ankle may contribute to
vascular compromise, proximal nerve com- the disuse of the lower extremity even with
pression, or systemic disease. The foot often is minimal injury. Fear of reinjury, pain, or other
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Chapter 24 L5 Radiculopathy 455

FOOT PAIN
LOCAL LOCAL LOCAL
FOREFOOT AND TOE HINDFOOT MIDFOOT

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LOCAL LOCAL


FOREFOOT AND TOE HINDFOOT MIDFOOT

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

factors will need to be explored. In all cases the the L4 nerve. Prone knee bend may reproduce
basis of pathology must be thoroughly explored symptoms.
before this presentation is to be considered.
If this condition is present, a team approach Background Information
ensures optimal treatment. A lumbar disk herniation is the most com-
mon cause for this condition. The diagnosis
LUMBAR RADICULOPATHIES is confirmed with magnetic resonance imag-
■ L4 Radiculopathy ing. Surgical intervention may be indicated
Chief Clinical Characteristics in severe cases of lower extremity pain
This presentation can be characterized by accompanied by neurological signs.
pain in the lumbar spine and paresthesias
radiating from the anterolateral thigh, the ■ L5 Radiculopathy
anteromedial lower leg, the medial malleo- Chief Clinical Characteristics
lus, and distally into the medial aspect of the This presentation includes pain in the lumbar
foot. The patient may have weakness of the tib- spine and paresthesias radiating from the
ialis anterior muscle. Depending on the sever- lateral aspect of the hip and buttock to
ity, the presentation may also include a the lateral aspect of the knee, extending from
decreased or absent patellar tendon reflex the anterolateral lower leg down into the dor-
and motor loss in the muscles innervated by sum of the foot. Depending on the severity,
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456 Chapter 24 S1 Radiculopathy

the presentation may also include motor loss and may be influenced by cortical mecha-
FOOT PAIN

in the muscles innervated by the L5 nerve root, nisms.2 Treatment may include physical
including extensor hallucis longus and exten- therapy interventions to improve patient/
sor digitorum longus. client functioning, biofeedback, analgesic or
anti-inflammatory medication, transcuta-
Background Information
neous or spinal electrical nerve stimulation,
A lumbar disk herniation is the most com-
and surgical or pharmacologic sympathectomy.
mon cause for this condition. The diagnosis
However, a team approach ensures optimal
is confirmed with magnetic resonance imag-
treatment for individuals with this condition.
ing. Surgical intervention may be indicated
in severe cases of lower extremity pain Local
accompanied by neurological signs.
■ Abscess
■ S1 Radiculopathy
Chief Clinical Characteristics
Chief Clinical Characteristics This presentation often includes a severe, deep,
This presentation typically includes pain in and throbbing pain, with pus surrounded by a
the lumbar spine and paresthesias radiating region of erythema, edema, and warmth, as well
from the buttock to the posterior aspect of as systemic signs of infection that may include
the knee and extending posterolaterally along fever, chills, malaise, anorexia, and delirium.
the lower leg, the lateral heel, and the lateral
plantar foot. Depending on the severity, the Background Information
presentation may also include a decreased This condition most commonly affects the
or absent Achilles tendon reflex and motor central plantar compartment. It often follows
loss in the muscles innervated by the S1 nerve, minor skin trauma. Localized cellulitis may
including the peroneal muscles, gastrocne- accompany the abscess. Magnetic resonance
mius, and soleus. imaging with contrast confirms the diagnosis.3
Treatment may involve options ranging from
Background Information
rest and antibiotic medications to surgical
A lumbar disk herniation is a common cause
drainage.
for this condition. The diagnosis is confirmed
with magnetic resonance imaging. Surgical ■ Achilles Paratenonitis
intervention may be indicated in severe cases
of lower extremity pain accompanied by Chief Clinical Characteristics
neurological signs. This presentation involves diffuse pain, tender-
ness, and thickening and swelling along the
■ Psychological Effect of Complex Achilles tendon, especially 2 to 6 cm from the
Regional Pain Syndrome calcaneal insertion. However, the painful pal-
Chief Clinical Characteristics pable thickening does not move with dorsiflex-
This presentation typically includes a traumatic ion or plantarflexion because the tendon moves
onset of severe chronic ankle and foot pain with this action but the paratenon does not.
accompanied by allodynia, hyperalgesia, and Pain is greatest in the mornings or at the start
trophic, vasomotor, and sudomotor changes in of a physical activity, but the pain will often
later stages. This condition is characterized by decrease with continued walking or activity as
disproportionate responses to painful stimuli. the tendon begins to move more freely inside the
paratenon.
Background Information
This regional neuropathic pain disorder pres- Background Information
ents either without direct nerve trauma (Type I) The paratenon is usually the site of inflamma-
or with direct nerve trauma (Type II) in any tory injury related to overuse, but it may also
region of the body.1 This condition may pre- be damaged from direct traumatic pressure.
cipitate due to an event distant to the affected The diagnosis is determined clinically, but
area. Thermography may confirm associated magnetic resonance or ultrasound imaging
sympathetic dysfunction. This condition has may be necessary for confirmation if the clini-
been shown to have a psychological component cal diagnosis is unclear. Treatment includes
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Chapter 24 Arterial Occlusion 457

rest, inflammation control, improving flexibil- ■ Ankylosing Spondylitis

FOOT PAIN
ity of the calf muscle group, and improving Chief Clinical Characteristics
strength of the antipronator muscle groups. This presentation may include an insidious on-
set of low back and symmetric posterior hip pain
■ Acute Rheumatic Fever
associated with a slowly progressive and signif-
Chief Clinical Characteristics icant loss of general spinal mobility. Some indi-
This presentation may involve warm, red, swollen, viduals with late-stage disease also will develop
and tender joints with systemic signs of infection characteristic syndesmophytes in the foot region.
including fever, abdominal pain, anorexia,
lethargy, malaise, and fatigue. Rheumatic fever usu- Background Information
ally has five major manifestations that include Symptoms may be worse in the morning and
migratory polyarthritis, carditis, chorea, subcuta- improve with light exercise. This condition is
neous nodules, and erythema marginatum (flat more common in males, as well as people of
to slightly indurated lesions on the skin of the American indigenous descent, less than 40 years
extensor surfaces of the extremities or trunk). of age, or who carry the human leukocyte
antigen B27. It also may be associated with
Background Information fever, malaise, and inflammatory bowel disease.
This condition is an acute inflammation as a The diagnosis is confirmed with plain radi-
result of group A streptococcal infection.4 The ographs of the sacroiliac joints and lumbar
pain is usually the result of an aseptic inflam- spine, which reveal characteristic findings of
mation of the joints. Diagnosis is made with sacroiliitis and “bamboo spine.” Blood panels
confirmation of a group A streptococcal infec- including erythrocyte sedimentation rate are
tion in association with two of the five major useful to track disease activity. Treatment typi-
manifestations. Monoarthritis and low-grade cally includes a combination of steroidal, nons-
fever are important considerations to avoid teroidal, and biological anti-inflammatory
underdiagnosis.4 Usual treatment consists of medications combined with physical therapy to
antibiotic, anti-inflammatory, and antipyretic address postural and movement considerations
medications. associated with changing spinal position.
■ Allergic Angiitis and ■ Arterial Insufficiency
Granulomatosis (Churg-Strauss Chief Clinical Characteristics
Disease) This presentation may involve pain in the foot
Chief Clinical Characteristics and lower leg that is worst at night, possibly
This presentation can be characterized by causing awakening from sleep. Symptoms often
fever, abdominal pain, peripheral neuropathy, decrease when the leg is placed in a dependent
mononeuritis multiplex, weakness, weight loss, position or after walking.
hypertension, edema, oliguria, and uremia in
Background Information
association with pulmonary symptoms. This
More severe cases of arterial insufficiency may
disorder is always associated with bronchial
be associated with skin ulceration. This condi-
asthma.
tion is a result of decreased mean arterial pres-
Background Information sure while sleeping; ischemia causes foot pain.
Other signs and symptoms include skin Doppler ultrasonography confirms the diag-
nodules, eosinophilia, mononeuropathy or nosis. The goal of treatment is to prevent the
polyneuropathy, and small blood vessel occlu- progression of this condition to acute arterial
sion that may result in toe and foot gangrene. occlusion.5 Treatment may involve exercise,
Drug allergies or infection are theoretical risk factor modification, stenting, balloon
causes. Clinical examination confirms the angioplasty, bypass grafting, or amputation.
diagnosis in the presence of rhinitis, sinusitis,
or asthma. Usual treatment includes corticos- ■ Arterial Occlusion
teroids. Because of the multisystem nature of Chief Clinical Characteristics
this condition, other supportive interventions This presentation includes an abrupt onset of
also may be necessary. deep achy and/or burning pain, often on the
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458 Chapter 24 Arteriosclerosis Obliterans

plantar surface of the foot. Diminished sensa- extraintestinal manifestations include episcle-
FOOT PAIN

tion, weakness, pallor, coldness, and cyanotic ritis, ankylosing spondylitis, sacroiliitis, ante-
mottling also may be present. The pulses in the rior uveitis, aphthous stomatitis, erythema
feet, and often the entire lower extremity, may nodosum, pyoderma gangrenosum, or growth
be absent. and development delay.
Background Information Background Information
Common causes of this condition include One-third of patients with inflammatory bowel
trauma, embolus, or a thrombus.6 It is located disease have at least one musculoskeletal mani-
at the bifurcation just distal to the last festation, and in children the extraintestinal
detectable pulse. If the occlusion is present for manifestations may dominate.9 Colonoscopy
longer than 6 hours, the patient is likely to with mucosal biopsy confirms the diagnosis.
contract gangrene and/or have residual symp- Treatment typically involves aminosalicylates,
toms of weakness or sensory impairment. corticosteroids, anti–tumor necrosis factor
Diagnosis is most often confirmed with medications, diet changes, and surgical resec-
Doppler ultrasonography; however, angiogra- tion of the involved portion of bowel.
phy, assessment of systolic blood pressure, and
magnetic resonance angiography may be uti- ■ Atrophic Fat Pad Disorder
lized. This condition is a medical emergency in Chief Clinical Characteristics
its acute form due to the risk for compromise This presentation can involve pain on the
of the distal tissues. Treatment usually involves plantar surface of the heel with tenderness to
pharmacologic or surgical thrombolysis. palpation. Pain increases with barefoot walk-
ing, especially in the morning.
■ Arteriosclerosis Obliterans
Background Information
Chief Clinical Characteristics This condition results from thinning or
This presentation typically includes painful toes spreading of the fat pad that occurs more often
or feet in association with symptoms of palpa- in the elderly, long-distance runners, individu-
ble coolness, and trophic changes such as hair als who train on hard surfaces, and individuals
loss or nail changes. Affected toes may be blue who wear open-heeled or unsupportive shoes.
or black in color. Pulses are diminished or Clinical examination confirms the diagnosis.
absent in the lower leg and foot. Treatment usually involves nonsurgical inter-
Background Information ventions, such as orthoses and footwear mod-
This condition is a vascular disease in which ification, relative rest, and strengthening and
the arteries become thickened and lose elastic- flexibility exercises for the lower quarter.
ity, resulting in vessel occlusion.7,8 It can lead ■ Avascular Necrosis of the
to ulceration or gangrene of the foot or toes. Navicular
The kidneys, brain, and heart also may be
affected. The diagnosis is confirmed with Chief Clinical Characteristics
Doppler ultrasonography or angiography. This presentation includes severe medial mid-
Treatment may involve exercise, risk factor mod- foot pain accompanied by edema and erythema.
ification, stenting, balloon angioplasty, bypass This condition is more common in individuals
grafting, or amputation. with a severe pes planus foot posture.
Background Information
■ Arthritis Associated with This condition results from an alteration in
Inflammatory Bowel Disease blood supply that does not allow for proper
Chief Clinical Characteristics healing after initial trauma to the bone, most
This presentation may be characterized by mi- commonly as a dorsal avulsion fracture or
gratory polyarthritic pain with abdominal pain, direct trauma (such as a blow to the bone).
diarrhea, abdominal cramping, and other gas- The diagnosis is confirmed with plain radi-
trointestinal symptoms. Commonly affected ographs. Intervention may include immobi-
joints include the metatarsophalangeal joints, lization in early-stage disease or arthroscopic
ankle, knee, elbow, wrist, and hand. Other debridement and drilling for advanced lesions.
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Chapter 24 Calcaneal Apophysitis (Sever’s Disease) 459

■ Benign Muscle Cramps Achilles tendon and the calcaneus. Biome-

FOOT PAIN
Chief Clinical Characteristics chanical or structural factors, such as
This presentation involves painful muscle Haglund’s deformity or rearfoot varus, may
cramps, palpable tightness, and induration that lead to increased stress on this region. Sys-
is often correlated with a sustained contraction temic inflammatory diseases or direct
of the affected muscle.10 This condition may trauma also may lead to this disorder. Clin-
last several minutes to approximately one-half ical examination confirms the diagnosis.
hour, with tenderness for several hours follow- Usual intervention includes activity and
ing an acute episode. footwear modification.

Background Information ■ Subcutaneous Achilles Bursitis


This condition is associated with an insidious Chief Clinical Characteristics
onset following muscle overuse. Other poten- This presentation can include local pain, ten-
tial causes include dehydration, hyponatremia, derness to palpation, and edema just poste-
hypomagnesemia, geriatric nocturnal leg rior to the Achilles tendon insertion at the
cramps, or association with pregnancy.10 Treat- calcaneal tuberosity. Warmth and redness
ment typically involves addressing the underly- may be present.
ing cause of muscle cramps. Quinine sulfate is
an established pharmacologic treatment that Background Information
also has potential adverse cardiac side effects. This condition results from inflammation
and edema of the bursa located between the
■ Bite Injuries Achilles tendon insertion and the skin.
Chief Clinical Characteristics Continued irritation of the inflamed and
This presentation may involve pain with swollen bursa may lead to thickening of the
ecchymosis, edema, abrasion, and lesions or bursal walls and fibrosis. Clinical examina-
puncture wounds at the location of the bite. tion confirms the diagnosis. Treatment is
typically nonsurgical, including physical
Background Information
therapy intervention, orthoses, and footwear
Common bites include those by humans,
and activity modification.
dogs, and cats. Potential complications in-
clude nerve damage, blood vessel damage, or ■ Calcaneal Apophysitis (Sever’s
crush injury resulting in tissue damage or a Disease)
fracture. Any bite injury may develop sep-
ticemia and its associated signs of systemic in- Chief Clinical Characteristics
fection. The history will reveal the cause and This presentation may include pain along the
circumstances of the bite. This condition is medial and lateral heel or distal Achilles inser-
treated prophylactically with antibiotics due tion. Edema and warmth may be present. Pain
to the risk for septicemia.11,12 increases with higher level weight-bearing
activities and decreases with rest. This condi-
BURSITIS tion is common in young athletes.
■ Retrocalcaneal Bursitis Background Information
Calcaneal apophysitis occurs when the open
Chief Clinical Characteristics
calcaneal physis of adolescents is disrupted
This presentation typically includes pain,
by direct trauma, excessive shearing forces,
palpable warmth, tenderness to palpation,
or traction injury from tight calf muscula-
and some edema at the medial and lateral
ture. This condition resolves with maturity
aspects of the Achilles tendon. Pain is
and the closing of the epiphysis. The diagno-
reproduced with compressing the tissue
sis is confirmed with the clinical examina-
anterior to the tendon, as well as with dor-
tion. This condition is typically treated
siflexion of the ankle.
nonsurgically with rehabilitative interven-
Background Information tions, including physical modalities and
This condition is the result of inflamma- gentle flexibility and strengthening exercises
tion within the bursa that lies between the for the lower quarter.
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460 Chapter 24 Calcific Tendinitis of the Flexor Hallucis Longus or Brevis

■ Calcific Tendinitis of the Flexor the region of the initial infection. Vesicles, bul-
FOOT PAIN

Hallucis Longus or Brevis lae, necrosis, petechiae, and ecchymosis may


Chief Clinical Characteristics be present. Fever, chills, headaches, malaise,
This presentation may involve pain, edema, hypotension, tachycardia, and delirium are the
redness, and palpable tenderness on the plan- common signs of systemic involvement.
tar aspect of the first ray along the tendon. Pain Background Information
is aggravated with bearing weight on the affected This condition often results from a wound
limb, walking, and passive stretching or action complication. Edema is a significant risk fac-
of the hallux flexors. tor. There are multiple causative pathogens, of
Background Information which Staphylococcus and Streptococcus species
The etiology of this condition is unclear. It is are the most common. Laboratory tests con-
self-limiting. The diagnosis is confirmed with firm the causative pathogen. Most cases are
plain radiographs and biopsy. This condition self-limiting. However, severe cases involve
is usually treated nonsurgically; however, sur- necrosis and may require surgical debridement
gical debridement of the affected tendon may and antibiotic therapy.
be necessary in cases of recalcitrant disability.
■ Cholesterol Embolism
■ Calluses Chief Clinical Characteristics
This presentation can be characterized by uni-
Chief Clinical Characteristics
lateral or bilateral foot pain associated with toes
This presentation includes tenderness located at
of a blue-black color, foot and toe gangrene, foot
a broad and diffuse hyperkeratotic lesion.
ulceration, and ischemia. Diminished or absent
Background Information pulse is common in the affected artery.
This condition usually is the result of excessive Background Information
friction or pressure, and usually is located under The embolism may develop as an atheroscle-
the first metatarsal head. Clinical examination rotic plaque is dislodged, resulting in complete
confirms the diagnosis. Treatment usually in- or partial arterial occlusion. It is a rare but
cludes mitigation of the callus with an abrasive potentially fatal complication of cardiac
block. In the case of recurring disease, analysis of catheterization, particularly using the descend-
plantar foot pressures should be undertaken in ing thoracic aorta as a route.13 Complications
order to determine the optimal preventive include renal embolism, penile gangrene,
footwear modification of orthoses. bowel infarction, or spinal cord infarction. The
■ Candidiasis diagnosis is confirmed with Doppler ultra-
sonography, abnormal laboratory blood tests,
Chief Clinical Characteristics histological conformation of involved tissues,
This presentation can include burning pain, or angiography. Treatment is symptomatic.
maceration, weeping lesions, and itching be-
tween the toes. Individuals with poor general ■ Cold Injuries
health or immunosuppression are at particular
risk for this condition. Chief Clinical Characteristics
This presentation often involves pain, pares-
Background Information thesias, and numbness followed by hyperemia
This condition results from a fungal infection and hypesthesias for at least several weeks
from the Candida species. Microscopic labora- following the injury. Frostnip is a reversible
tory culture confirms the diagnosis. Topical epithelial injury presenting as firm, cold white
antifungal medication is the usual treatment areas on the affected skin, changing to burning
of choice for this condition. sensation with redness during rewarming,
followed by peeling and blistering 24 to 72 hours
■ Cellulitis after the presentation. Frostbite results in more
Chief Clinical Characteristics serious tissue damage as a result of cells
This presentation may involve pain, erythema, freezing. It presents as cold, white, hard, and
edema, warmth, and tenderness spreading from anesthetic skin. Frostbitten skin may develop
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Chapter 24 Cryoglobulinemia 461

blistering with dry or wet gangrene of the deeper ■ Corns

FOOT PAIN
tissue. Chief Clinical Characteristics
Background Information This presentation includes painful and ten-
Clinical examination confirms the diagnosis. der hyperkeratotic lesions that are small and
Treatment typically includes progressive re- conical. Pain often is localized to the site of
warming of affected tissue. Tissues injured by the lesion with increased pain during weight
cold tend toward additional damage during bearing.
rewarming as a result of inflammation.14 Background Information
■ Compartment Syndrome This condition develops at the site of excessive
or repetitive mechanical trauma or friction.
Chief Clinical Characteristics The most common type is a hard corn. Soft
This presentation includes paresthesia and corns result from the absorption of a consider-
hypesthesia, followed by significant pain, mas- able amount of perspiration, so they are usu-
sive edema, ecchymosis, soft tissue tenderness, ally more painful than hard corns. Clinical
erythema, palpable warmth, and weakness of examination confirms the diagnosis. Treat-
the plantarflexors or toe flexors. Pulses and ment usually includes mitigation of the corn.
capillary refill often remain normal until the In the case of recurring disease, analysis of
pressure increases to the level of severe injury. plantar foot pressures should be undertaken to
Background Information determine the optimal preventive footwear
Compartment syndrome often is the result of modification or orthoses.
trauma; however, it can occur spontaneously
in patients with diabetes mellitus.15 Altered ■ Crush Injury
vessel perfusion and edema result in increased Chief Clinical Characteristics
intercompartmental pressure that disrupts This presentation can be characterized by pain
the oxygen diffusion and results in ischemia. with possible inability to bear weight on the
Clinical examination and intercompartmental affected limb following a crush injury.
pressure measurements confirm the diagnosis.
Background Information
This condition is a medical emergency and re-
Clinical examination should determine if the
quires urgent surgical decompression.
source of pain is related to epithelial injury,
■ Complex Regional Pain Syndrome soft tissue injury, or fracture. The nature and
Chief Clinical Characteristics extent of the injury depends on the weight
This presentation may include a traumatic and composition of the object as well as the
onset of severe chronic ankle and foot pain duration of time that it is applied to the foot.
accompanied by allodynia, hyperalgesia, and Even if the injury seems insignificant, the diag-
trophic, vasomotor, and sudomotor changes in nosis may require clinical imaging to confirm
later stages. This condition is characterized by the specific structures that are affected. Treat-
disproportionate responses to painful stimuli. ment depends on the extent and type of tissue
involvement.
Background Information
This regional neuropathic pain disorder pres- ■ Cryoglobulinemia
ents either without direct nerve trauma (Type I) Chief Clinical Characteristics
or with direct nerve trauma (Type II) in any This presentation typically includes pain, weak-
region of the body.1 This condition may precip- ness, paresthesia, sensory loss, Raynaud’s
itate due to an event distant to the affected area. phenomenon, toe gangrene, ulceration of the feet
Thermography may confirm associated sympa- or toes, purpura, signs of glomerulonephritis, or
thetic dysfunction. Treatment may include phys- gastrointestinal bleeding. Tissues and skin of
ical therapy interventions to improve patient the extremities are commonly affected.
and client functioning, biofeedback, analgesic or
anti-inflammatory medication, transcutaneous Background Information
or spinal electrical nerve stimulation, and surgi- This condition is the result of macroglobulins
cal or pharmacologic sympathectomy. that precipitate when plasma cools, leading to
1528_Ch24_444-488 07/05/12 1:54 PM Page 462

462 Chapter 24 Dermatitis

small-vessel damage in the tissues. Blood tests ■ Metatarsophalangeal Joint


FOOT PAIN

confirm the diagnosis. Usual treatments in- Chief Clinical Characteristics


volve a combination of anti-inflammatory, This presentation may be characterized by
interferon, and monoclonal antibody medica- pain, edema, tenderness, and gross deformity
tions with plasmapheresis. of the metatarsophalangeal joint. Hallux
■ Dermatitis dislocation is the most common of all metatar-
sophalangeal dislocations.
Chief Clinical Characteristics
This presentation may involve superficial Background Information
skin irritation characterized by epidermal This condition may be complicated with in-
edema, vesicles, erythema, oozing, crusting, jury to the sesamoid complex of the hallux.
scaling, usually pruritus, and lichenification. This dislocation usually is related to high-
Sweating may be normal, diminished, or impact trauma and often involves injury to
excessive. the joints of the midfoot. Plain radiographs
confirm the diagnosis. Reduction of the dislo-
Background Information cated joint is a usual treatment, and immobi-
The three major types of dermatitis in the feet lization or surgical fixation may be necessary.
include contact dermatitis, atopic dermatitis,
and dyshidrosis (pompholyx). Most cases of ■ Distal Symmetric
contact or atopic dermatitis are related to Polyneuropathy, Including From
exposure of the skin to an irritating agent. Acquired Immunodeficiency
Atopic dermatitis has a genetic susceptibility Syndrome, Alcohol/Drug Abuse,
and exacerbations may be related to environ- Carcinoma, Diabetes Mellitus,
mental irritants, emotional stress, temperature and Kidney Failure
changes, bacterial skin infections, certain Chief Clinical Characteristics
fabric softeners, nutritional deficiencies, and This presentation may be characterized by de-
wool. Most cases of dyshidrosis are chronic creased distal deep tendon reflexes, as well as pain,
forms of dermatitis and are idiopathic. Clini- dysesthesia, or paresthesia in the feet and toes.
cal examination confirms the diagnosis. Treat-
ment typically involves a combination of risk Background Information
factor modification with topical medication Peripheral neuropathy is one of the more com-
appropriately directed at the source of der- mon complications in patients presenting with
matitis if it can be identified. acquired immunodeficiency syndrome, dia-
betes, renal failure, hypothyroidism, toxins,
DISLOCATIONS malignancy, and malnutrition. It is character-
■ Interphalangeal Joint ized by retrograde axonal degeneration, so this
condition commonly progresses from distal to
Chief Clinical Characteristics proximal. Electromyographic studies and biopsy
This presentation can include pain, edema, dif- confirm the diagnosis. Vibration and light touch
ficulty weight bearing, tenderness to palpation, sensation (10-g Semmes Weinstein monofila-
and gross deformity of the hallux interpha- ment) tests are useful to assess protective sensa-
langeal joint. tion. Curative treatment of this condition re-
Background Information mains controversial, so the focus of intervention
The interphalangeal joint of the hallux is the typically includes patient education to maintain
most common to dislocate, usually caused by blood glucose control and care for the insensate
axial loading of the hallux. The dislocation regions to prevent progression and complica-
occurs in a dorsal direction. The sesamoid tions secondary to this condition.
bone may become lodged between the distal
phalanx and proximal phalanx as a possible ■ Dysplasia Epiphysealis
complication. Plain radiographs confirm the Hemimelica (Trevor’s Disease)
diagnosis. Reduction of the dislocated joint is Chief Clinical Characteristics
a usual treatment, and immobilization or This presentation can include a limp that may
surgical fixation may be necessary. be accompanied by joint swelling, restricted
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Chapter 24 Fat Pad Separation Secondary to a Cyst 463

joint movement, or muscle wasting. Males are suspected causes. Clinical examination confirms

FOOT PAIN
more likely to be affected than females. the diagnosis. These lesions are self-limiting
and may take months or years to resolve.16
Background Information
This condition is a congenital bone develop- ■ Erythromelalgia
ment disorder that is the result of an intra- Chief Clinical Characteristics
articular osteochondromatosis (localized This presentation involves symmetric burn-
overgrowth of the cartilage) that is present in ing pain in the hands and feet of mild to
the epiphyses of the joint. This affects both disabling intensity with observable redness.
sides of the epiphyses and joint deformity is Pain usually presents as attacks that are often
often the result. This condition is most com- aggravated by vasodilation, such as exposure
mon at the medial ankle, but it also may be to warmth or exercise. Alleviating factors
seen in the femur, the wrist, or the foot. Radi- include rest, elevation, and cryotherapy. The
ographs often reveal the structural changes to lower extremities are more often involved than
the joint. Treatment usually consists of surgi- the upper extremities.
cal excision of the mass, as well as correction
of any angular deformity that may have re- Background Information
sulted. Nonsurgical intervention includes This disease is a result of paroxysmal vasodila-
immobilization. tion of unknown etiology. Doppler ultrasonog-
raphy, transcutaneous oximetry, and nerve con-
■ Envenomation duction studies confirm the diagnosis. Treatment
Chief Clinical Characteristics may include anti-inflammatory medications.
This presentation may include immediate pain
and redness at the location of the sting or ven- ■ Fabry’s Disease
omous bite. Depending on the nature of the Chief Clinical Characteristics
sting, the patient may have ecchymosis, edema, This presentation often includes males with
bullae, necrosis, ulceration, or gangrene. symmetrical skin lesions on the lower trunk
(angiokeratomas), with common symptoms of
Background Information
burning pain in the lower extremities and feet.
The extent of the injury will be determined by
Pain in the hands or more proximal extremities,
the nature of the bite or sting and the type of
corneal opacities, cardiac involvement, and
venom. Most stings are from sea urchins, fish,
renal disease are other associated symptoms.
insects, or arthropods. Most common ven-
omous bites are from snakes and spiders. Background Information
Snake venom may be neurotoxic, hemotoxic, This condition is a rare X-linked genetic disorder
or cardiotoxic. Spider venom may be neuro- that leads to abnormal lipid metabolism. Het-
toxic or necrotizing. Clinical examination erozygous females are usually asymptomatic, but
confirms the diagnosis. This condition can be may display an attenuated version of this disease
a medical emergency. that is characterized by corneal opacities. Blood
test or prenatal assay confirms the diagnosis.
■ Erosive Lichen Planus Treatment includes supportive intervention for
Chief Clinical Characteristics the multisystem effects of this condition, as well
This presentation typically includes cutaneous as enzyme replacement therapy.
lesions that involve itchy and violaceous polyg-
onal papules. Characteristic lesions are marked ■ Fat Pad Separation Secondary
by Wickham’s striae (itchy, violaceous, flat to a Cyst
papules that are highlighted by white dots or Chief Clinical Characteristics
lines). Papules often unite to form rough, scaly This presentation may involve pain at the plan-
patches. tar heel, edema, and tenderness to palpation and
Background Information pressure.
Skin degeneration may occur with ulceration on Background Information
the plantar, medial, or lateral surfaces of the This condition is caused by a fluid-filled bursa
foot. Several drug interactions and infections are or cyst. The cyst or bursa may be palpable and
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464 Chapter 24 Fat Pad Syndrome

aid in the diagnosis. Treatment requires ecchymosis, tenderness, and occasional widen-
FOOT PAIN

drainage of the cyst and rest to allow the fat ing of the heel or development of a valgus de-
pad to adhere to the calcaneus. formity. Pain may be described as diffuse or
sharp. Pain and edema presenting inferior to
■ Fat Pad Syndrome the medial malleolus and increased with
Chief Clinical Characteristics inversion of the calcaneus or hyperextension
This presentation includes pain and palpable of the great toe may indicate sustentaculum
tenderness on the plantar surface of the heel. Pain tali involvement. Pain inferior to the medial
often increases with bearing weight on the malleolus and reproduction of symptoms with
affected limb, especially with barefoot walking forced dorsiflexion of the foot or toes may
and in the morning. indicate possible medial calcaneal process
Background Information involvement.
Atrophy or inflammation of the heel fat pad Background Information
may cause this condition, often as the result This condition is caused by a fall or jump
of repetitive traumatic compression of the from height. Associated conditions may in-
fat pad secondary to repetitive overuse, over- clude diabetes mellitus, spinal and ankle
training, weak or fatigued lower extremity fractures, severe ankle sprains, bilateral
muscles, or training on hard surfaces. Clinical calcaneal fractures, peroneal injuries, flexor
examination confirms the diagnosis. Usual hallucis longus injury, and compartment
treatment involves footwear modification, syndrome. Complications may include loss
orthoses, activity modification, and strength- of sensation and paresthesias of the distal
ening weak muscles that may contribute to this foot, excruciating pain with toe movements,
condition. compartment syndrome, or subtalar arthri-
tis. Plain radiographs confirm the diagnosis.
■ Fixed Drug Eruption
Treatment ranges from immobilization and
Chief Clinical Characteristics protected weight-bearing status in the event
This presentation can often involve either of a stable fracture, to surgical fixation for
immediate or delayed eruptions of the skin or unstable or nonunion fractures.
mucous membranes as a result of administra-
tion of a drug. Redness, edema, pain, and ■ Fifth Metatarsal
tenderness of the affected skin often are Chief Clinical Characteristics
observed. Responses can vary from a mild rash This presentation involves pain and tender-
to epidermal necrolysis. ness at the lateral aspect of the foot around the
Background Information base of the fifth metatarsal.
In the feet, this condition often is secondary to Background Information
oral drug administration (such as Antabuse, Typical fractures are either avulsion frac-
isoniazid, and other drugs that may cause tures or diaphyseal fractures. Avulsion frac-
sensory or sensorimotor neuropathy). Clinical tures result from an inversion motion that
examination confirms the diagnosis. Treat- is correlated with reflexive contraction of
ment typically includes ceasing the medication the peroneus brevis. Diaphyseal fractures
responsible for this condition. Individuals are caused by loading through a foot that is
suspected of having this condition should be in a plantarflexed and inverted position.
referred to a physician for further investigation Pain at the base of the fifth metatarsal or
and to supervise changes in an individual’s inability to bear weight on the affected limb
medication regimen. may lead the clinician to utilize radi-
FRACTURES ographs, which largely confirm the diagno-
sis.17 Usual treatment ranges from immobi-
■ Calcaneus lization and protected weight-bearing
Chief Clinical Characteristics status in the event of a stable fracture, to
This presentation includes severe heel pain surgical fixation for unstable or nonunion
associated with significant plantar edema, fractures.
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Chapter 24 Medial Process of the Sustentaculum Tali of the Calcaneus 465

■ Hallucal Sesamoid Disorders and magnetic resonance imaging often

FOOT PAIN
Chief Clinical Characteristics assist in the diagnosis, staging, and
This presentation often involves pain and ten- prognosis of the injury. Surgical treatment
derness with palpation of the involved is indicated for large or displaced frac-
sesamoid, as well as dorsiflexion of the first tures; otherwise, nonsurgical treatment is
metatarsophalangeal joint (Fig. 24-1). indicated.18

Background Information ■ Lateral Tubercle of the Posterior


Fracture of either the medial (tibial) or Process of the Talus
lateral (fibular) sesamoid can occur with Chief Clinical Characteristics
direct trauma, an avulsion injury, or from This presentation can involve posterolat-
repetitive trauma. Bone scan, magnetic eral ankle pain, swelling, and tenderness
resonance imaging, or computed tomogra- over the lateral tubercle. Pain increases
phy confirms the diagnosis. Typically, while walking downhill or downstairs, as
treatment involves immobilization and pro- well as with ankle and subtalar movement,
tected weight-bearing status. In the event of active first toe flexion, or forced first toe
recalcitrant pain and disability, however, dorsiflexion.
surgical excision of the affected sesamoid(s)
may be considered. Background Information
This condition results from talocalcaneal
■ Lateral Process of the Talus ligament avulsion, posterior talofibular
Chief Clinical Characteristics ligament avulsion, or a compressive force.
This presentation may be characterized by It is associated with lateral ankle sprains,
pain, swelling, and tenderness along the an- lateral malleolus avulsion fractures, and
terior and inferior lateral ankle located di- osteochondral defects on the talar dome.
rectly over the lateral process of the talus. Pain Clinical examination findings and radi-
increases with weight bearing, talocrural ographs confirm the diagnosis.18 Surgical
plantarflexion and dorsiflexion, and sub- treatment is necessary for nonunion or dis-
talar joint movement. The mechanism of placed fractures.18
injury is usually a dorsiflexion and inversion ■ Medial Process of the
motion common in motor vehicle accidents, Sustentaculum Tali of the
direct trauma, or snowboarding accidents. Calcaneus
Background Information Chief Clinical Characteristics
Chronic pain may be indicative of avascular This presentation typically includes pain,
necrosis. Bone scans, computed tomography, edema, and tenderness over the medial ankle,
just inferior to the medial malleolus and along
the heel. Pain increases with passive rearfoot
inversion. Passive hyperextension of the first
phalanx may increase pain due to the position
of the flexor hallucis longus, just inferior to this
structure.
Background Information
This condition may result from a fall onto
the heel with an inverted foot; it is uncom-
mon in isolation. Complications may
include nonunion, nerve irritation, and
toe flexor injury. Bone scan, computed to-
mography, or magnetic resonance imaging
Lateral Medial confirms the diagnosis. Surgical fixation may
FIGURE 24-1 Position of medial and lateral hallucal be necessary in the event of an unstable or
sesamoids. nonunion fracture.
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466 Chapter 24 Medial Tuberosity of the Posterior Process of the Talus

■ Medial Tuberosity of the trauma. They may present without significant


FOOT PAIN

Posterior Process of the Talus edema, while traumatic or avulsion fractures


Chief Clinical Characteristics typically present with edema. Traumatic
This presentation typically includes ankle pain fractures are due to avulsion of the distal in-
that is posterior to the medial malleolus and sertion of the posterior tibialis tendon; less
anterior to the Achilles tendon. The injury commonly, trauma to the navicular or the
often results from dorsiflexion-pronation calcaneus may lead to a direct fracture or dis-
ankle injuries, ankle dislocation, or possibly an location. Diagnosis is confirmed with plain
avulsion fracture from a posterior tibiotalar radiographs, magnetic resonance imaging, or
injury. computed tomography. Usually, treatment
depends on severity and ranges from nonsur-
Background Information gical to surgical interventions.
Bone scan, computed tomography scan, or
magnetic resonance imaging may assist in ■ Osteochondral Fracture of the
the diagnosis, staging, and prognosis. In the Subtalar Joint
event of unstable fractures or nonunion, Chief Clinical Characteristics
surgical fixation may be necessary. This presentation typically includes pain,
edema, and tenderness at the sinus tarsi or
■ Metatarsal just distal to the lateral malleolus. Pain is
Chief Clinical Characteristics worsened with subtalar passive motion.
This presentation may be characterized by Peroneal muscle spasm and tenderness may
pain, tenderness, and sometimes edema be present.
around the location of the injury. Pain is Background Information
aggravated when bearing weight on the Often, osteochondral lesions of the subtalar
affected limb and alleviated with rest in a joint are idiopathic. The diagnosis is con-
non–weight-bearing position. firmed with magnetic resonance imaging.
Background Information Treatment is usually nonsurgical, with
Most commonly, this condition involves arthrodesis considered for individuals with
a fracture along one or more of the recalcitrant pain and disability.20
metatarsal shafts. Repetitive overuse com-
monly causes this condition, although it ■ Osteochondral Fracture of the
also could result from acute trauma. Com- Talar Dome
mon fractures may include an avulsion, Chief Clinical Characteristics
diaphyseal fracture, or stress fracture, which This presentation includes variable symp-
may share a common clinical presentation. toms based on the site of the osteochondral
Clinical examination confirms the diagno- lesion. Lateral dome lesions commonly pres-
sis. Additional certainty may be obtained ent with localized pain at the central and
with plain radiographs.19 Treatment de- lateral ankle anterior to the lateral malleolus.
pends on severity and ranges from nonsur- Medial dome lesions present with tenderness
gical to surgical interventions. posterior to the medial malleolus and at the
posterior talus.
■ Navicular
Background Information
Chief Clinical Characteristics
Osteochondral defects may loosen and cause
This presentation includes pain and tender-
joint pain, locking, and swelling. Lateral
ness that is located at the medial and dorsal
dome injuries are frequently associated
aspect of the midfoot. Pain is provoked with
with trauma, usually the result of an inver-
weight-bearing activities and running.
sion-dorsiflexion mechanism. Medial dome
Background Information injuries may either be the result of inversion-
Stress fractures are more common in the ath- plantarflexion trauma or present insidiously.
letic population due to repetitive high-impact Radiographs, magnetic resonance imaging,
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Chapter 24 Tarsometatarsal Joint (Lisfranc’s) Fracture or Dislocation 467

and arthroscopy confirm the diagnosis. modification, orthoses, and strengthening

FOOT PAIN
Patients should be monitored because joint and flexibility exercises for weak or tight
degeneration is common following these lower quarter muscles that may be responsi-
injuries.21 ble for contributory pathomechanics.

■ Posterosuperior Calcaneal ■ Stress Fracture of the Lateral


Tuberosity Malleolus
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation may be characterized by This presentation may involve focused, local-
pain, swelling, and possible ecchymosis lo- ized, superficial aching or sharp pain directly
cated at the calcaneal insertion of the Achilles on the lateral malleolus at the site of the frac-
tendon, the posterior heel, and the posterior ture (usually within 15 cm [6 in.] of the
ankle. The patient will often have a func- distal fibula). Mild edema may be present but
tional loss of plantarflexion, such as with a is not required for the diagnosis. Tenderness
single-leg heel raise, due to the avulsion frac- usually is present with direct palpation, and
ture. This condition is most common in older pain may be provoked with vibration testing.
individuals with advanced age, diabetes Pain rarely is present at rest unless the
mellitus, or osteoporosis. fracture is severe.

Background Information Background Information


The mechanism of injury is a rapid pull of This condition occurs as a result of abnormal
the Achilles tendon. A positive Thompson loading from a sudden increase in training,
test and a palpable sulcus may be present if repetitive forces, training on hard surfaces,
the tendon is avulsed. Associated findings improper footwear, or ankle and foot dys-
may include an Achilles tendon tear or other function (such as a valgus deformity or
extra-articular calcaneal fractures. Plain severe flat-foot deformity). Bone scan con-
radiographs confirm the diagnosis. Treat- firms the diagnosis. Treatment typically
ment may range from nonsurgical interven- involves protected weight bearing in combi-
tions, such as protected weight bearing nation with activity modification, footwear
and immobilization, to surgical fixation of modification, orthoses, and strengthening
unstable or nonunion fractures. and flexibility exercises for weak or tight
lower quarter muscles that may be responsi-
ble for contributory pathomechanics.
■ Stress Fracture of the Calcaneus
Chief Clinical Characteristics ■ Tarsometatarsal Joint
This presentation can include diffuse pain, (Lisfranc’s) Fracture or
edema, and possible erythema in the heel. Dislocation
Pain increases with bearing weight on the Chief Clinical Characteristics
affected limb and decreases with rest. This presentation can include pain, tenderness,
and edema at the location of the involved
Background Information
joint or joints. Pain may be reproduced with
This condition develops through repetitive
active supination, pronation, or weight bear-
overuse, overtraining, weak or fatigued
ing, and deformity may be present.
lower extremity muscles, or training on hard
surfaces. This presentation may be associ- Background Information
ated with low to flat arch height and weak This condition is caused by axial loading
intrinsic foot musculature. Clinical exami- through a plantarflexed foot. Even seem-
nation, including the squeeze test, confirms ingly mild injuries may lead to degeneration
the diagnosis. Additional certainty may be at the tarsometatarsal joints, which lead
obtained with bone scan. Usual treatment to future complications and pain.22 This
involves protected weight bearing in combi- injury is commonly associated with tar-
nation with activity modification, footwear sometatarsal ligament sprain. The diagnosis
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468 Chapter 24 Toe

is confirmed with plain radiographs. Treat- magnetic resonance imaging. Surgical excision
FOOT PAIN

ment commonly involves surgical reduction is a common treatment.


and fixation.
■ Gout
■ Toe Chief Clinical Characteristics
Chief Clinical Characteristics This presentation includes severe pain, edema,
This presentation involves pain, edema, ten- warmth, erythema, and very localized tender-
derness to palpation, ecchymosis, and often a ness. Systemic findings may include fever, chills,
subungual hematoma. This condition is asso- malaise, and sweating. The areas typically
ciated with difficulty bearing weight on the involved are the first metatarsophalangeal joint,
affected lower extremity, with terminal stance the talocrural joint, the calcaneal region, and
gait abnormalities to reduce loading on the the tarsals of the instep. Pain is usually pro-
forefoot and toes. voked by hard movements, or may follow
alcohol abuse, dehydration, trauma, surgery,
Background Information septic arthritis, protein fasting, excessive purine
This condition usually occurs as a result of ingestion, and allopurinol or uricosuric agents.
direct trauma to a toe. Ligament damage is a
potential complication. Plain radiographs Background Information
confirm the diagnosis. Usual treatment in- Sustained hyperuricemia levels lead to deposi-
cludes immobilization and protected weight tion of monosodium urate crystals in and
bearing; however, reduction and fixation around the tendons and joints. Men are eight
may be considered for unstable or nonunion times more likely to be affected than women.23
fractures. Needle aspiration is required to confirm the di-
agnosis. Treatment commonly involves a combi-
■ Freiberg’s Disease nation of anti-inflammatory medication and
Chief Clinical Characteristics colchicine in individuals with normal liver func-
This presentation often involves pain, tender- tion. Preventive treatment should be undertaken
ness, and edema over the second and third to control uric acid levels by way of diet and
metatarsal heads. The second metatarsal head medication in individuals with chronic gout.
is involved 75% of the time and females are ■ Haglund’s Syndrome
more commonly affected.
Chief Clinical Characteristics
Background Information This presentation can be characterized by pain
This condition is a form of osteochondrosis and edema along the posterior heel and Achilles
with avascular necrosis that follows repetitive tendon, near the distal insertion at the calcaneus.
trauma. Radiography, radionuclide bone scan, Symptoms are reproduced with direct palpation,
and magnetic resonance imaging confirm the passive or forceful dorsiflexion, and resisted
diagnosis. Immobilization is a primary treat- plantarflexion. Walking and running are very
ment, with surgical debridement reserved for painful during late stance phase, and high shoe
cases of advanced disease. heel counters also will aggravate symptoms.
■ Glomus Tumor Background Information
This condition is a prominent superior cal-
Chief Clinical Characteristics
caneal tuberosity. Haglund’s syndrome is the
This presentation typically includes localized
combination of retrocalcaneal bursitis, super-
pain and tenderness on the plantar heel. A pal-
ficial Achilles bursitis, Achilles tendinopathy,
pable nodule may be present. Pain will not in-
and Haglund’s deformity—a prominent pos-
crease with dorsiflexion of the toes, although cold
terior superior tuberosity of the calcaneus.24
often aggravates the symptoms.
The diagnosis is confirmed with the combina-
Background Information tion of plain radiographs and clinical exami-
This condition results from an organized arte- nation. Treatment is typically nonsurgical,
riovenous anastomosis. Diagnosis may be con- with surgical reshaping of the superior cal-
firmed with imaging, including ultrasound or caneal tuberosity combined with debridement
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Chapter 24 Herpes Zoster 469

of the Achilles tendon insertion reserved for Background Information

FOOT PAIN
cases of recalcitrant symptoms and disability. A hammer toe deformity is a position of prox-
imal interphalangeal joint flexion with distal
■ Hallux Rigidus interphalangeal joint extension. The injuries to
Chief Clinical Characteristics the toes are a result of this deformity, whether
This presentation can include pain, decreased flexible or rigid, that leads to altered mechani-
range of motion, edema, possible bony prominence cal pressure and friction. Typical treatments
with redness, and tenderness at the first metatar- for clinically significant forms of this condi-
sophalangeal joint. Common gait abnormali- tion that cause intolerable pain and disability
ties include a decreased terminal stance phase and involve surgical procedures to straighten the
rolling the foot into supination. Pain may be toes.
present with metatarsophalangeal extension.
■ Hand-Foot-and-Mouth Disease
Background Information
Chief Clinical Characteristics
Hallux rigidus or limitus results from degener-
This presentation can include vesicular eruption
ative changes to the first metatarsophalangeal
around the buccal mucosa or the tongue, as well
joint. Clinical examination confirms the diag-
as similar lesions on the palms of the hands and
nosis. Usual nonsurgical treatments involve
the soles of the feet. Skin lesions are painful to
physical therapy intervention, footwear modi-
palpation and pressure.
fication, and orthoses. Surgical treatments
include wedge osteotomy. Background Information
The disease is a result of a viral infection, most
■ Hallux Valgus often by Coxsackie virus A 16.25 This condition
Chief Clinical Characteristics is more common in young children than
This presentation may involve medial pain, adults. Molecular assay confirms the diagno-
edema, redness, metatarsalgia pain, and tender- sis.26 Typically, treatment is supportive pending
ness as a result of the prominence of the great resolution of the underlying viral infection.
toe. This condition may progress to the point that
deviation develops in other toes. An adventitious ■ Hepatitis B–Associated Arthritis
bursa may develop, and patients also may have Chief Clinical Characteristics
difficulty wearing desired footwear. This presentation involves a symmetric pol-
yarthralgia that can affect the toes, and more
Background Information
commonly, the fingers. The common symptoms
This condition is truly an abductus defor-
include anorexia, malaise, nausea, vomiting,
mity, better termed hallux abductovalgus. It is
and fever. Jaundice usually develops in
associated with cartilage damage within the
approximately 1 to 2 weeks.
first metatarsophalangeal joint; the degree of
cartilage damage and angle of deformity are Background Information
directly related. Clinical examination con- Hepatitis is an inflammation of the liver as
firms the diagnosis. Usual nonsurgical treat- a result of viral infection with necrosis of
ments involve physical therapy intervention, the liver. Arthralgia usually is the result of an
footwear modification, and orthoses. Surgical aseptic inflammation of the joints. Blood tests
treatments include a variety of techniques to confirm the diagnosis.
correct the deformity.
■ Herpes Zoster
■ Hammer Toe Deformity Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically includes an exquis-
This presentation typically includes pain, red- itely painful rash or blisters along a specific der-
ness, tenderness, blister formation, or the pres- matomal pattern accompanied by flu-like
ence of a corn or callus formation. Some of symptoms. Individuals with this condition also
these patients will present with a history of demonstrate a previous history of varicella
subungual hematomas as a result of the repet- exposure or infection. Pain associated with this
itive trauma. condition may be disproportionate to the extent
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470 Chapter 24 Hypersensitivity Vasculitis

of skin irritation. The initial presentation of unremarkable. Nonsurgical interventions are


FOOT PAIN

this condition may be confused with radicu- often preferred, including footwear modifica-
lopathy due to the distribution of symptoms. The tion, orthoses, taping, and rehabilitative exer-
presence of the rash, extreme pain, general cises. Synovectomy may be required in individ-
malaise, and unclear association with spinal uals with persistent symptoms and disability.
movement aids in differential diagnosis.
■ Infected Blister
Background Information Chief Clinical Characteristics
The virus remains dormant in the spinal gan- This presentation can be characterized by burn-
glia until its reactivation during a period of ing and pain at the site of a blister. Purulence
stress, infection, or physical exhaustion. Treat- of the blister fluid with redness and edema
ment includes the administration of antiviral around the blister are associated signs. Signs of
agents as soon as the zoster eruption is noted, systemic involvement may include fever, chills,
ideally within 48 to 72 hours. If timing is malaise, or myalgia.
greater than 3 days, treatment is aimed at con-
trolling pain and pruritus and minimizing the Background Information
risk of secondary infection.27 Blisters of the feet may result from friction be-
tween the skin and a sock or shoe.29 Clinical
■ Hypersensitivity Vasculitis examination confirms the diagnosis. Treat-
ment for this condition usually includes risk
Chief Clinical Characteristics
factor mitigation, wound dressing, and oral
This presentation often includes pruritic skin
antibiotic medication.
lesions, necrosis, and painful ulcerations of
the feet and toes. Associated conditions may ■ Intermittent Acute Porphyria
include joint pain, hepatosplenomegaly, lym- Chief Clinical Characteristics
phadenopathy, or glomerulonephritis. This presentation includes neurovisceral symp-
Background Information toms with abdominal pain attacks and associ-
This condition is an inflammation of the ated symptoms of nausea, vomiting, constipa-
postcapillary venule that is caused by a drug tion, or diarrhea. Exacerbating factors include
interaction or immune complex interaction, drugs, hormones, or dietary restrictions (eg,
resulting in leukocytoclastic angiitis.28 Biopsy low-calorie or low-carbohydrate diets).
and subsequent histological investigation Background Information
confirm the diagnosis. This condition is often Neuropathy can lead to burning pain in the
self-limiting and resolves without specific feet and/or hands, paresthesias, numbness,
treatment. Topical or oral anti-inflammatory dysesthesias, myalgias, and symmetrical or
medication may be necessary for individuals asymmetrical weakness. Associated symptoms
with persistent symptoms. also may include bladder and urinary symp-
■ Idiopathic Metatarsophalangeal toms, behavioral changes, hypertension, tachy-
Synovitis cardia, and cardiac or respiratory failure. Re-
flexes may be diminished or absent. This
Chief Clinical Characteristics condition is an autosomal dominant disorder
This presentation may involve tenderness, that results from porphobilinogen deaminase
pain, and edema of the involved joints. Pain deficiency.30 Urine, blood, and deoxyribonu-
is aggravated with forced flexion of the metatar- cleic acid tests confirm the diagnosis. Treat-
sophalangeal joint. Monoarticular metatar- ment involves medications to reduce heme
sophalangeal joint synovitis is more common synthesis, as well as supportive interventions
than multiple joint involvement. Commonly, for the multisystem effects of this condition.
the second ray is more affected.
Background Information ■ Interosseous Myositis
A history of trauma or injury is not frequently Chief Clinical Characteristics
reported. Clinical examination confirms the This presentation involves pain and palpable ten-
diagnosis, and plain radiographs often will be derness in between the metatarsal bones with
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Chapter 24 Anterior Talofibular Ligament 471

pain aggravated by weight-bearing and frontal ■ Leukemia

FOOT PAIN
plane movements. Pain usually is worst in the Chief Clinical Characteristics
morning. This presentation typically includes a variety of
Background Information symptoms including bony foot pain, malaise,
Midfoot and forefoot support often alleviate fatigue, excessive bruising or bleeding, night
the pain associated with this condition, such as sweats, and weight loss. Acute disorders have a
when wearing shoes. Overuse injury of the in- more rapid onset with illness.31
terosseous muscles may cause this condition. Background Information
Magnetic resonance imaging confirms the di- Leukemic cells will accumulate in the bone
agnosis. The usual treatment for this condition marrow and replace the normal hematopoietic
is nonsurgical, including footwear and activity cells, which may then infiltrate any other organ.
modification, orthoses, and oral nonsteroidal Acute myelogenous leukemias constitute 85%
anti-inflammatory medication. of adult acute leukemias. Chronic lymphocytic
leukemia is responsible for 30% of all leukemias
■ Ischemic Ulceration of the Toes
in the Western countries and the median age of
Chief Clinical Characteristics onset is the seventh decade; whereas acute lym-
This presentation can involve pain, tenderness, phoblastic leukemia is responsible for 80% of
ulceration, and blackening of the great toe. childhood cases.32 Foot pain may result from
The foot or toe may be cold, pulseless, and either an arthritis or symmetrical peripheral
pale. polyneuropathy. The diagnosis is confirmed
Background Information with the presence of disabling bone pain, night
This condition results from tissue ischemia, pain, hematologic findings in the blood tests,
and the nature of vascular compromise leucopenia, or positive findings in a bone mar-
determines the affected distribution of foot row biopsy.33–35 Typical treatments for this
and toes. This condition is caused by throm- condition involve chemotherapy, radiation,
bus, embolism, vasculitis, or small artery monoclonal antibody medication, and stem cell
occlusion. Doppler ultrasonography con- transplantation.
firms the diagnosis. Treatment includes LIGAMENT SPRAINS
wound management strategies combined
with interventions to improve tissue perfu- ■ Anterior Talofibular Ligament
sion. Interventions to improve tissue perfu- Chief Clinical Characteristics
sion are appropriately directed to the cause of This presentation can include pain, swelling, ten-
ischemia. derness to palpation, and possible ecchymosis
just anterior and inferior to the lateral malle-
■ Joplin’s Neuroma olus, directly over the ligament. Weight bearing
Chief Clinical Characteristics and combined inversion/plantarflexion mo-
This presentation can include pain and pares- tions of the foot will be painful and may be
thesia at the medial plantar surface of the foot, limited by pain. If the ligament is completely torn,
just distal to the first metatarsophalangeal joint then pain may or may not be present with
and into the first digit. Pain may be worsened excessive inversion.
during weight bearing on the affected limb or Background Information
when wearing certain shoes. Injury to the ligament is usually caused by an
Background Information inversion and plantarflexion mechanism to
Entrapment of the medial plantar nerve near the ankle. Associated injuries may include
the hallux causes this condition. Clinical exam- calcaneofibular ligament sprain, syndesmotic
ination, including Tinel’s or compression sign ligament sprain, sinus tarsi syndrome with a
along the involved nerve, confirms the diagno- subtalar sprain, superficial peroneal nerve
sis. Usual treatment is nonsurgical, with surgi- injury, fifth metatarsal fracture, medial ankle
cal intervention reserved for cases in which the impingement, a medial or lateral malleolar
site of entrapment is well characterized. fracture, talar or osteochondral fractures, or
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472 Chapter 24 Calcaneofibular Ligament (Isolated)

peroneal tendon injury. Diagnosis is usually determine the extent of the injury to the
FOOT PAIN

based on the clinical presentation, mecha- nerves or vasculature. Treatment will often
nism of injury, and a positive anterior trans- consist of rest to allow for healing, provided
lation test of the talus. If pain or instability that nerve and vascular injuries are absent.
persists, then radiographs are helpful to rule
out fractures, osteochondral defects, or ■ Forefoot
structural instability with stress. Nonsurgical Chief Clinical Characteristics
intervention is commonly indicated; how- This presentation may be characterized by
ever, surgical stabilization may be indicated pain, edema, and tenderness to palpation.
in the event of a mechanically unstable joint. Symptoms are aggravated with range of mo-
tion or weight bearing through a dorsiflexed
■ Calcaneofibular Ligament or extended joint. Deformity may result if
(Isolated) there is significant damage to the ligamentous
Chief Clinical Characteristics and other passive stabilizing structures.
This presentation can be characterized by Background Information
swelling and tenderness along the lateral This condition results from movement be-
ankle, inferior to the lateral malleolus, yond the normal range of the joint in any of
along the ligament. Pain increases with foot the planes of movement, usually through a
inversion. hyperextension injury of the metatarsopha-
Background Information langeal joints. Clinical examination confirms
Injury to the ligament occurs during an in- the diagnosis, and plain radiographs may be
version mechanism of the ankle, with the useful to rule out fracture. Treatment usually
ankle in neutral or even sometimes dorsi- consists of activity and footwear modification
flexion. This injury rarely occurs independ- along with anti-inflammatory medications,
ently, and is commonly associated with ante- with arthrodesis necessary in the presence
rior talofibular ligament sprains, lateral of symptoms and disability related to gross
malleolus fractures, and syndesmotic in- mechanical instability.
juries. Diagnosis is based on the clinical ■ Metatarsophalangeal Joint
presentation and the use of radiographs may
be useful to rule out any additional injuries Chief Clinical Characteristics
or complications. Nonsurgical intervention This presentation typically includes pain,
is commonly indicated; however surgical edema, and tenderness to palpation. Pain is
stabilization may be indicated in the event of aggravated with range of motion or bearing
a mechanically unstable joint. weight on the affected limb through the affected
joint while it is dorsiflexed.
■ Cruciate Crural Ligament
Background Information
Chief Clinical Characteristics This condition is commonly caused by a
This presentation may include pain and hyperextension injury of the metatarsopha-
tenderness to palpation along the ligament on langeal joint. Clinical examination confirms
the dorsum of the ankle and foot. Pain is the diagnosis. Plain radiographs may be
intensified with plantarflexion of the toes, helpful to exclude avulsion fractures and dis-
but is present even at rest. Paresthesias or ruptions of the sesamoid and flexor hallucis
weakness may be present if the injury causes longus complex. Typical treatment involves
increased intracompartmental pressure. rest and footwear modification along with
Background Information anti-inflammatory medications.
Injury to this ligament may be due to either
direct or indirect trauma. Complications ■ Longitudinal Arch Strain
may include injury to the blood vessels or Chief Clinical Characteristics
nerves on the surface of the foot. Palpation This presentation involves pain on the plantar
for pedal pulses, neurological examination, aspect of the foot, primarily along the medial
and possibly a Doppler should be used to aspect of the foot. Pain is aggravated by weight
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Chapter 24 Metatarsalgia 473

bearing or dorsiflexion of the foot, and allevi- deformity, whether flexible or rigid, that leads

FOOT PAIN
ated by rest or plantarflexion of the foot. to altered mechanical pressure and friction.
Typical treatments for clinically significant
Background Information
forms of this condition that cause intolerable
Prolonged weight bearing that results in liga-
pain and disability involve surgical procedures
ment sprain or muscle fatigue causes this
to straighten the toes.
condition. Individuals with excessive foot
pronation, pes cavus, pes planus, or excessive ■ Metastases to the Foot, Including
ligament laxity are usually more prone to this From Primary Breast, Kidney,
condition. Clinical examination confirms the Lung, Prostate, and Thyroid
diagnosis. Typical treatment involves rest and Disease
footwear modification along with anti-
inflammatory medications, as well as stretch- Chief Clinical Characteristics
ing and strengthening of muscle length and This presentation can be characterized by
strength deficits that may contribute to this unremitting pain in individuals with these risk
condition. factors: previous history of cancer, age 50 years
or older, failure to improve with conservative
■ Lymphoma therapy, and unexplained weight change of
Chief Clinical Characteristics more than 10 pounds in 6 months.37
This presentation may include foot pain, tingling, Background Information
paresthesias, or weakness that results from a The skeletal system is the third most com-
sometimes present polyneuropathy.36 Some of mon site of metastatic disease.38 Symptoms
the most common symptoms include fever, night also may be related to pathological fracture
sweats, or weight loss. in affected sites. Common primary sites
Background Information causing metastases to bone include breast,
Congestion and edema of the face and neck as prostate, lung, and kidney. Bone scan con-
well as ureteral compression are common in in- firms the diagnosis. Common treatments for
dividuals with non-Hodgkin’s lymphoma. metastases include surgical resection,
Hodgkin’s lymphoma is caused by malignant chemotherapy, radiation treatment, and
proliferation of tumor cells in the lymphoretic- palliation, depending on the tumor type and
ular system, while non-Hodgkin’s lymphoma is extent of metastasis.
proliferation of lymphoid cells within the im-
mune system. Diagnosis may be confirmed ■ Metatarsalgia
with histological study of a biopsy or excised Chief Clinical Characteristics
tissue. Treatment will vary based on the type This presentation can include pain and palpa-
of lymphoma. Common treatments include ble tenderness on the plantar aspect of the
chemotherapy, radiation, and bone marrow metatarsal heads. Pain is aggravated with
transplantation. weight bearing, especially with a heel rise or dur-
ing the terminal stance in gait.
■ Mallet Toe Deformity
Background Information
Chief Clinical Characteristics This condition is the result of imbalanced
This presentation can involve pain, redness, weight distribution across the metatarsal
tenderness, blister formation, or the presence heads, leading to overuse trauma to the
of a corn or callus formation. Some of these bones, joints, and surrounding tissues. Clini-
patients will present with a history of subun- cal examination confirms the diagnosis.
gual hematomas as a result of the repetitive Additional certainty may be obtained by
trauma. plantar pressure measurement during walk-
Background Information ing. Typical treatment involves rest and
A mallet toe deformity is a position of distal footwear modification, anti-inflammatory
and proximal interphalangeal joint flexion. This medications, and gentle exercise for lower
condition is often termed “claw toe” deformity. quarter muscle length and strength deficits
The injuries to the toes are a result of this that may lead to this condition.
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474 Chapter 24 Morton’s Neuroma

■ Morton’s Neuroma neuropathy or bone pain. Radiographically, the


FOOT PAIN

Chief Clinical Characteristics bones will have lesions of a punched-out


This presentation involves sharp or burning appearance with generalized osteoporosis. Diag-
forefoot pain or numbness that may be gener- nosis may be confirmed with blood tests, urinal-
alized or in the distribution of a specific digi- ysis, serum protein electrophoresis, bone scan,
tal nerve (Fig. 24-2). Pain may be particularly magnetic resonance imaging, and bone marrow
present while wearing shoes, with additional in- aspiration and biopsy. Depending on the aggres-
creases while bearing weight on the metatarsal siveness of this condition, treatment may range
heads, and during walking, standing, or active from “watchful waiting” to chemotherapy and
ankle dorsiflexion. bone marrow transplantation.

Background Information ■ Necrotizing Fasciitis


This condition is caused by thickening and Chief Clinical Characteristics
fibrosis of the interdigital nerve; it is most com- This presentation often is characterized by pain
mon in the third intermetatarsal space. Clinical with erythema, warmth, swelling, possible skin
examination, including palpation and Mulder’s discoloration, blisters, gangrene, and joint crepi-
test, and response to local corticosteroid injec- tus. Systemic signs of an infection may include
tions confirm the diagnosis.39 Corticosteroid fever, tachycardia, hypotension, diaphoresis,
injection is a diagnostic test as well as a form of chills, malaise and altered mental status. The
treatment. Other usual treatments include wound may be odorous and sometimes subcu-
footwear and activity modification, orthoses, taneous gas is observed.
anti-inflammatory medications, and gentle ex-
ercise for lower quarter muscle length and Background Information
strength deficits that may lead to this condition. The underlying infection often is caused by a
bacterium following trauma or infection of an
■ Multiple Myeloma open wound or ulcer. This condition often is
Chief Clinical Characteristics mistaken for cellulitis in its early stages. Diag-
This presentation may involve unexplained nosis may be confirmed with radiographs or
skeletal pain in the feet, lower legs, arms, hands, abnormal blood serum tests, and a Gram stain
back, and thorax. This may be associated with of the infected tissue will often provide the
renal failure, recurrent bacterial infections, best diagnostic information. This condition is
pathological fractures, and anemia. a medical emergency. Treatment includes
debridement of necrotic tissue and an aggres-
Background Information sive regimen of antibiotic medication.
This condition is an insidious plasma cell cancer
that originates in the bone marrow and can NERVE ENTRAPMENTS
affect multiple areas with multiple lytic and ■ Calcaneal Branch Neurodynia
osteosclerotic effects. Pain results from either
Chief Clinical Characteristics
This presentation includes local medial heel
pain, located inferior and posterior to the
tarsal tunnel. Pain often is increased with
weight bearing, direct palpation, or neural
tension testing with the ankle in dorsiflexion
and foot eversion. A valgus hindfoot posture
is common.
Background Information
This condition results from compression or
tension on the calcaneal branch of the tibial
nerve. It may be caused by space-occupying
lesions, fractures, calluses, arthritis and
FIGURE 24-2 Distribution of symptoms in Morton’s osteophytes, edema, bursitis, tendinitis or
neuroma. tenosynovitis, accessory bones, or prolonged
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Chapter 24 Tarsal Tunnel Syndrome 475

direct and indirect pressures. The clinical anterolateral lower leg and dorsum of the

FOOT PAIN
examination, including neurodynamic test- foot. Plantarflexion and foot inversion are
ing, confirms the diagnosis. Usual treat- often aggravating factors, and Tinel’s sign
ment is nonsurgical, with surgical release of over the superficial peroneal nerve also may
entrapment sites reserved for cases in which reproduce pain. Associated motor deficits may
the site of entrapment is well characterized. include weakness or foot eversion.

■ Deep Peroneal Nerve Background Information


Etiology includes compression due to
Chief Clinical Characteristics trauma or idiopathic entrapment.40 This
This presentation involves pain, numbness, condition is a diagnosis of exclusion after
dysesthesias, and/or paresthesias at the first lumbar radiculopathy has been ruled out.
dorsal web space of the foot. Ankle plantarflex- The clinical examination, including the
ion or foot inversion may aggravate symp- straight leg raise test and a standard neuro-
toms. This condition may present as antero- logical examination, and electrodiagnostic
lateral ankle pain or weakness of ankle tests confirm the diagnosis. Treatment is
dorsiflexion. typically nonsurgical, with surgical release of
Background Information entrapment sites reserved for cases in which
The extensor retinaculum is the most com- the site of entrapment is well characterized.
mon site of entrapment. However, other
sources of compression may include tight ■ Sural Nerve
shoelaces, anterior compartment syn- Chief Clinical Characteristics
drome, ankle fracture, ganglion, and other This presentation may often include hypesthe-
forms of trauma. Electromyographic tests sia or paresthesia and pain along the posterior
confirm the diagnosis. Treatment is typi- calf, posterolateral ankle, or dorsolateral foot.
cally nonsurgical, with surgical exploration Tinel’s sign also may reproduce pain. This
and release of entrapment sites reserved for condition does not include motor involvement
cases in which the site of entrapment is well because the sural nerve is a sensory afferent.
characterized.
Background Information
■ First Branch of the Lateral Common etiologies include ankle sprains,
Plantar Nerve stretch injury, Achilles injury, trauma, nerve
compression due to ganglion cyst, or a cal-
Chief Clinical Characteristics
caneal fracture. This condition is a diagnosis
This presentation may involve pain and ten-
of exclusion after lumbar radiculopathy is
derness present over the plantar aspect of the
ruled out. The clinical examination, includ-
heel, just slightly anterior and medial to the
ing the straight leg raise test and a standard
plantar fascia insertion on the calcaneal
neurological examination, and electrodiag-
tuberosity. Pain also may be palpable at the dis-
nostic tests confirm the diagnosis. Typically,
tal tibial nerve, along the medial plantar foot.
treatment is nonsurgical, with surgical
Background Information exploration and release of entrapment sites
The most common entrapment site is distal reserved for cases in which the site of entrap-
to the tarsal tunnel. Clinical examination, in- ment is well characterized.
cluding neurodynamic testing, confirms the
diagnosis. Treatment is typically nonsurgical, ■ Tarsal Tunnel Syndrome
with surgical exploration and release of Chief Clinical Characteristics
entrapment sites reserved for cases in which This presentation involves hypesthesia, pain,
the site of entrapment is well characterized. and paresthesia due to compression of the tib-
ial nerve at the ankle, located around the
■ Superficial Peroneal Nerve medial aspect of the ankle and the plantar
Chief Clinical Characteristics surface of the foot. Symptoms often are worse
This presentation can include pain, pares- with walking, with compression, and with a
thesia, dysesthesia, or numbness on the dorsiflexion-eversion test. They present with
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476 Chapter 24 Osler’s Nodules

a positive Tinel’s sign. Rest alleviates symptoms. ■ Midfoot (Tarsometatarsal


FOOT PAIN

Weakness of the flexor hallucis and flexor Joints)


digitorum brevis muscles is rarely present. Chief Clinical Characteristics
Background Information This presentation typically includes pain,
Common etiologies are space-occupying localized tenderness, crepitus, and joint noise
lesions, fractures, edema, tendinitis or located at the involved joint. Patients usually
tenosynovitis, accessory bones, or prolonged report increased pain and stiffness in the
direct and indirect pressures from inflamma- morning and after prolonged static postures.
tion and fluid retention as a result of systemic Onset of pain is usually gradual and symptoms
diseases (rheumatoid arthritis, ankylosing are often worsened by exercise.
spondylitis) or significant hindfoot prona- Background Information
tion that causes tibial nerve stretch. Diagno- In the midfoot, some of the more common
sis is confirmed with the clinical examination sites of osteoarthrosis include the first
and nerve conduction studies. Usual treat- metatarsocuneiform joint and the cuboid-
ment is nonsurgical, with surgical release of metatarsal joint. Osteoarthrosis is a progres-
entrapment sites reserved for cases in which sive disorder that usually starts in the second
the site of entrapment is well characterized. or third decade of life. The cause of os-
■ Osler’s Nodules teoarthrosis is often thought of as either
idiopathic or secondary to conditions that
Chief Clinical Characteristics affect the environment of the joint (such as
This presentation may be characterized by infection, inflammation, genetic defects, or
painfully tender, small, red lesions with a cen- trauma). Radiographs will often confirm the
tral elevation on the tips of the toes. Systemic signs diagnosis and reveal damage to the involved
of infection may include fever, myalgia, pallor, joint, such as narrowing of the joint space,
anorexia, a heart murmur, and encephalopathy. formation of osteophytes, and formation of
Background Information pseudocysts in the subchondral bone. Usual
This condition involves small microabscesses treatment is nonsurgical, with arthrodesis
that are associated with bacterial endocarditis. considered for cases of recalcitrant symp-
Blood cultures and echocardiography confirm toms and disability.
the diagnosis. Treatment involves antibiotic
intervention directed toward the underlying ■ Small Toes
organism, which is Staphylococcus aureus. Chief Clinical Characteristics
This presentation typically includes pain, ten-
OSTEOARTHROSIS/ derness, and edema of the involved joint or
OSTEOARTHRITIS joints. Active and passive range of motion
■ Interphalangeal Joint may reproduce symptoms.
Chief Clinical Characteristics Background Information
This presentation typically includes pain, ten- This condition usually is initiated by trauma
derness, and edema. Pain is worsened with or increased stress that results from changes in
active and passive range of motion to the joint. lower extremity alignment. Plain radiographs
Background Information that demonstrate joint space narrowing and
The pain and arthritis usually are initiated by bone spurs confirm the diagnosis. Typically,
either acute trauma or cumulative stress that treatment is nonsurgical, except in cases of
results from changes in anatomical and intractable symptoms and disability in which
functional alignment. Plain radiographs that arthrodesis may be considered.
indicate spurring or joint space narrowing
confirm the diagnosis. Treatment is typically ■ Osteomyelitis
nonsurgical, except in cases of intractable Chief Clinical Characteristics
symptoms and disability in which arthrode- This presentation typically includes localized
sis may be considered. tenderness, warmth, erythema, edema, and
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Chapter 24 Calcaneocuboid Joint 477

systemic signs of infection such as weight loss and plantar fascia, as well the plantar aspect of the

FOOT PAIN
fatigue. The toes may present with a “sausage midfoot. Pain is more intense with the first few
toe” deformity. steps in the morning and activities that involve
bearing weight on the affected limb, such as
Background Information
walking, running, and ankle dorsiflexion with
This condition is an inflammation and necrosis
extension of the toes.
of bone as a result of an infection. The calca-
neus is the most common site of infection in the Background Information
foot, followed by the metatarsals, tarsals, and Overuse and overloading of the plantar fascia
then the phalangeal bones. Biopsy, magnetic cause this condition. Plain radiographs may
resonance imaging, and histology confirm the reveal a calcaneal heel spur. However, these
diagnosis and guide medical intervention.41 have been shown as the source of pain in only
Treatment may involve an aggressive regimen a minority of cases. Clinical examination
of antibiotic medications, with surgical resec- confirms the diagnosis. Treatment commonly
tion of affected areas potentially necessary. consists of nonsurgical interventions, such as
footwear modification, orthoses, and gentle
■ Paronychia exercise to improve ankle dorsiflexion and
Chief Clinical Characteristics plantar fascial mobility. In individuals with
This presentation typically includes pain and ten- recalcitrant symptoms and disability, surgical
derness with possible redness to the parony- debridement also may be considered.
chium around the nail. Purulent drainage is
common in the region of the infection. ■ Polyarteritis Nodosa
Chief Clinical Characteristics
Background Information This presentation typically includes fever,
Chronic cases tend to be nonsuppurative. This abdominal pain, peripheral neuropathy,
condition involves infection to the epidermis mononeuritis multiplex, weakness, weight loss,
surrounding the nail, which usually is caused hypertension, edema, oliguria, and uremia (in
by some puncture or laceration to the tissue. order of commonness). This condition is most
The diagnosis is confirmed by clinical exami- common in males between 40 and 50 years
nation. Treatment typically involves drainage of age.
and oral antibiotic medications.
Background Information
■ Piezogenic Papules Neuropathy can result in sensory loss, hypes-
Chief Clinical Characteristics thesia, or weakness. Pain associated with this
This presentation typically includes pain and condition results from peripheral neuropa-
tenderness at the plantar heel. Symptoms are thy, myalgia, or arthralgia. This condition
worsened when bearing weight on the affected involves inflammation and necrosis of the
limb, especially in a rigid shoe or when barefoot medium-sized arteries that result in ischemia,
on a hard floor. and it may occur as an effect of hepatitis B.42
The diagnosis is confirmed with angiography,
Background Information tissue biopsy, or magnetic resonance imag-
Oversized fat nodules may cause compressive ing.43,44 Corticosteroid medication combined
pain on the fat pad. These nodules are often with cyclophosphamide comprises a com-
observable clinically. Pain may result from the mon treatment.
subcutaneous fat herniations. Diagnosis is
made clinically. Footwear modifications, or- POSITIONAL FAULTS
thoses, and activity modification may be
considered as common treatments. ■ Calcaneocuboid Joint
Chief Clinical Characteristics
■ Plantar Fasciitis This presentation typically includes pain at the
Chief Clinical Characteristics plantar aspect of the calcaneocuboid joint or
This presentation typically includes pain and lateral to the joint line. Pain usually is increased
tenderness at the calcaneal insertion of the with impact activities, when performing a
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478 Chapter 24 Talonavicular Joint

heel raise, or with a manual muscle test of suspected, then a tissue biopsy may confirm
FOOT PAIN

the peroneus longus. Localized edema and this diagnosis. Prognosis may range from mild
peroneus longus weakness may be present. changes to death. Typical goals of treatment
include addressing contributory conditions
Background Information
and organ failure.
Theoretical causes include lateral ankle
sprains, calcaneocuboid joint abnormalities, ■ Pseudogout
or torque from the peroneus longus tendon.
Diagnosis is usually made by clinical presen- Chief Clinical Characteristics
tation, weakness of the peroneus longus, and This presentation may include pain, edema,
localized palpable pain. The diagnosis is con- warmth, erythema, and tenderness of the in-
firmed with the clinical examination, and volved joint or joints. The arthritis may mimic
radiographs usually are unhelpful. Common other types of arthritis but attacks usually are
treatment is nonsurgical, involving mobi- less severe than those experienced with gout. The
lization or manipulation of the cuboid. first metatarsophalangeal joint, ankle, dorsum
of the foot, knees, wrists, or shoulders may be af-
■ Talonavicular Joint fected. This condition is prevalent in individu-
Chief Clinical Characteristics als over 60 years of age.
This presentation typically includes pain at the Background Information
plantar and medial aspect of the foot, around Causes for the contributory calcium py-
the navicular. Pain is aggravated with weight rophosphate crystal deposition in synovial
bearing on the affected limb, impact activi- joints are unknown. Diagnosis is based on
ties, and manual muscle testing of the the clinical presentation, the pattern of joints
posterior tibialis muscle. Weakness of the involved, radiographic intra-articular and
posterior tibialis muscle, mild edema, and periarticular involvement, and aspiration of
hypomobility of the talonavicular joint usu- calcium pyrophosphate crystals in the joint
ally are present. fluid.45 Anti-inflammatory medication com-
Background Information prises the usual treatment for this condition.
Torsion of the medial forefoot is thought to Unlike with gout, uric acid–lowering medica-
medially displace the navicular, causing this tions are not used.
condition. Clinical examination confirms
the diagnosis, and radiographs usually are ■ Pulp-Space Infection
unhelpful. Common treatment is nonsurgi- Chief Clinical Characteristics
cal, involving mobilization or manipulation This presentation typically includes pain,
of the navicular. redness, edema, tenderness, and sometimes pal-
pable warmth at the plantar aspect of the first
■ Primary Amyloidosis toe. Signs of systemic involvement include fever,
Chief Clinical Characteristics malaise, weight loss, or fatigue.
This presentation typically includes pain, pares-
thesia, hypesthesia, and loss of light touch and Background Information
temperature sensation in the feet and toes. The cause of this rare condition is a puncture
wound. Clinical examination confirms the
Background Information diagnosis. Treatment typically involves oral
This condition occurs when amyloid, a protein- antibiotic medications.
like substance, is deposited in between cells
in many tissues of the body. Renal failure ■ Raynaud’s Disease
with proteinemia and uremia are the most Chief Clinical Characteristics
common complications; however, the patient This presentation typically includes a period of
also may have cardiac and gastrointestinal in- pallor and cyanosis followed by skin rubor and
volvement. Diagnosis of primary amyloidosis pain. Symptoms are often aggravated with cold
neuropathy must correlate the neuropathy or emotional stress. It is most common in the
with other comorbidities that may result fingers, although toes also may be affected. Pares-
from amyloidosis. If organ involvement is thesia tends to occur during the later stages.
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Chapter 24 Systemic Lupus Erythematosus 479

Background Information the seven following manifestations: morning

FOOT PAIN
This phenomenon may be idiopathic, or stiffness for more than 1 hour, arthritis of
related to vascular disease or a drug reaction. three or more joints, arthritis of the hand
Clinical examination confirms the diagnosis. joints, symmetric arthritis, rheumatoid
Typical treatment of this condition involves nodules, serum rheumatoid factor, or radi-
prevention of future episodes. ographic changes. Treatment typically includes
a variety of steroidal, nonsteroidal, and biolog-
■ Reiter’s Syndrome ical anti-inflammatory medications.
Chief Clinical Characteristics
RHEUMATOID ARTHRITIS–LIKE
This presentation typically includes asymmet-
DISEASES OF THE FOOT
ric and varying severity of joint pain and stiff-
ness of the subtalar joint, metatarsophalangeal ■ Psoriatic Arthritis
joints, interphalangeal joints, tarsal joints, cal- Chief Clinical Characteristics
caneal abnormalities, and knees. Other symp- This presentation can be characterized by an
toms can include tendon synovitis, fasciitis, insidious onset of lumbopelvic and hip pain
back pain, mucocutaneous lesions, skin lesions, associated with psoriasis. The severity of
and cardiac involvement. Joint pain accompa- arthritis is uncorrelated with the extent of
nies urethritis and conjunctivitis. skin involvement.48 Pitting nail lesions occur
Background Information in 80% of individuals with this condition.
This disease typically follows either an episode Dactylitis, tenosynovitis, and peripheral
of dysentery or infectious arthritis, and per- arthritis also are common.
sons with the HLA-B27 genetic makeup are at Background Information
greater risk.46 Diagnosis is usually based on the Radiographs of the distal phalanges may
patient’s history, radiographs, and blood tests reveal a characteristic “pencil in cup” defor-
to rule out other forms of arthritis or poten- mity. Blood panels including erythrocyte
tially associated infections. This usually is a sedimentation rate are useful to track disease
self-limiting condition and resolves in 3 to activity. Management usually includes use of
4 months. When provided, treatment involves salicylates, corticosteroids, antibiotics, and im-
a combination of antibiotic medications di- munosuppressants depending on the severity
rected toward the infective agent that is re- of the pathology.49
sponsible for the autoimmune response that
characterizes this condition, in combination ■ Systemic Lupus Erythematosus
with anti-inflammatory medication. Chief Clinical Characteristics
This presentation can involve fatigue and
■ Rheumatoid Arthritis joint pain/swelling affecting the hands, feet,
Chief Clinical Characteristics knees, and shoulders. This condition affects
This presentation can involve symmetric foot mostly women of childbearing age, but men
pain with localized tenderness, swelling, and also may be affected.
early morning stiffness. The feet may be the
Background Information
first region affected by the arthritis; however,
This condition is a chronic autoimmune
multiple joints are often affected. After the
disorder that can affect virtually any organ
initial stages, subcutaneous rheumatoid nodules
system of the body, including skin, joints,
and joint deformities may present, most
kidneys, brain, heart, lungs, and blood.
commonly flexion deformities. The metatar-
There may be some ankle tendon involve-
sophalangeal joints are the joints most com-
ment, more often in the Achilles tendon. The
monly affected by rheumatoid arthritis.47
diagnosis is confirmed by the presence of
Background Information skin lesions; heart, lung, or kidney involve-
This is a progressive systemic condition with ment; and laboratory abnormalities includ-
unknown etiology. Diagnosis, according to the ing low red or white cell counts, low platelet
American Rheumatism Association, includes counts, or positive ANA and anti-DNA anti-
at least a 6-week presentation with any four of body tests.42
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480 Chapter 24 Rubella and Rubella Vaccine–Associated Arthritis

■ Rubella and Rubella Background Information


FOOT PAIN

Vaccine–Associated Arthritis A history of plantar fasciitis with one or


Chief Clinical Characteristics more corticosteroid injections may predis-
This presentation typically includes symmetric pose individuals to this condition. It may
polyarthralgia that often is correlated with joint be caused by degeneration of the plantar
stiffness and preceded by a prodromal stage of fascia, chronic microtrauma, or a single
malaise, low-grade fever, and lymphadenopa- acute trauma. Clinical examination con-
thy and by skin rash. The rash is similar in firms the diagnosis. Surgical repair may be
appearance to the measles. Arthralgias are more indicated.
common in children, whereas the joint stiffness ■ Septic Arthritis
is more common in adults.
Chief Clinical Characteristics
Background Information This presentation includes achy to throbbing
This condition is caused by a ribonucleic acid pain, erythema, edema, and palpable warmth.
viral infection and diagnosis usually is con- Systemic signs of infection may include fever or
firmed with laboratory tests (serology) and malaise. This condition more often is monoar-
clinical examination. It usually is self-limiting ticular and can have a sudden onset.
and the symptoms usually alleviate in several
Background Information
weeks.
Septic arthritis can result from a fungal, bacter-
RUPTURES ial, or viral pathogen infecting the periarticular
or synovial tissues; Staphylococcus aureus is the
■ Achilles Tendon
most common cause in all ages. Computed
Chief Clinical Characteristics tomography and magnetic resonance imaging
This presentation may involve a sudden combined with culture and staining of aspi-
onset of pain, palpable tenderness, signifi- rated joint fluid commonly confirms the diag-
cant weakness, and sometimes a palpable sul- nosis. Treatment usually involves a combination
cus, usually 2 to 6 cm proximal to the distal of an aggressive antibiotic medication and
insertion of the Achilles tendon. joint drainage, perhaps by way of open drainage
Background Information potentially combined with surgical lavage.
This condition often occurs spontaneously ■ Shoe Vamp Ulcer and Bursitis
during an activity that places loading on the
plantarflexors. It is associated with age over Chief Clinical Characteristics
40 years and a history of Achilles tendinopa- This presentation may involve erythema,
thy. The Thompson test and magnetic reso- tenderness, pain, and superficial ulceration to
nance or ultrasound imaging confirm the the dorsal aspect of the hallux.
diagnosis. This condition is treated with sur- Background Information
gical repair of the affected tendon. Ulceration of skin, bursitis, and blistering
occur from friction with the shoe vamp. Ulcers
■ Plantar Fascia in patients with vascular or neuropathic
Chief Clinical Characteristics deficits are of concern because healing may
This presentation can include sudden onset of be compromised. Clinical examination con-
severe pain during walking, running, jump- firms the diagnosis. Treatment usually in-
ing, or climbing stairs. Pain is described as a cludes footwear modification to reduce fric-
“tearing” or “ripping” pain in the heel and tion between the shoe vamp and foot.
midfoot that prevents activities that involve
bearing weight on the affected limb. Ecchymo- ■ Sickle Cell Crisis
sis along the plantar fascia may appear 24 to Chief Clinical Characteristics
48 hours following the injury. Pain may be This presentation includes bone pain about the
reproduced with weight bearing, palpation, hip joint that is worsened by cold weather,
or with dorsiflexion of the ankle and exten- overexertion, dehydration, and being overly
sion of the toes. fatigued.
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Chapter 24 Extensor Digitorum Longus Tendinitis 481

Background Information Background Information

FOOT PAIN
This condition involves abnormal red blood Trauma or repetitive use, a medication reac-
cell morphology that causes the cells to become tion, systemic pathology, and aging may initi-
rigid and sticky, disrupting blood flow to ate the characteristic hematoma under the
bones, and resulting in painful bone infarcts. nail. Clinical examination confirms the diag-
This condition may cause complications in nosis with the appearance of a blackened
multiple organ systems, including stroke, skin toenail. Usual treatment involves drainage.
ulcers, and blindness. Diagnosis may be con-
firmed with a blood test, the most common TENDINITIS
being hemoglobin electrophoresis. This condi- ■ Achilles Tendinitis
tion is a medical emergency. Chief Clinical Characteristics
■ Strain of the Peroneal Muscles This presentation can involve a sharp,
aching, or burning pain with point tender-
Chief Clinical Characteristics ness 2 to 6 cm proximal to the calcaneal
This presentation involves pain, tenderness, insertion. Insertional tendinopathy may be
and possible edema or ecchymosis at the lateral located at the distal insertion at the calcaneus.
lower leg and ankle, in the region of the peroneal Symptoms are reproduced with palpation of
muscles. Pain may be aggravated with passive the tendon, passive dorsiflexion, and active
dorsiflexion and inversion of the foot, or with resisted plantarflexion. Palpable crepitus
contraction of the muscle with active plan- may be present with movement, and an
tarflexion and eversion of the foot. Bearing increased thickening of the tendon that
weight on the affected limb may be difficult, moves with tendon excursion may be palpa-
such as with walking and running. Focal ble in comparison to the asymptomatic side.
tenderness may be present. Calf atrophy may be observed, the patient
Background Information may have limitations in ankle dorsiflexion,
This condition occurs as a result of rapid and the patient may report increased stiff-
eccentric muscle contractions involved with ness in the morning.
activities like acceleration and deceleration.
Background Information
Clinical examination confirms the diagnosis.
Achilles tendinitis is associated with overuse
Additional certainty may be obtained with
from repetitive loading of the involved
magnetic resonance imaging or computed
tendon. This injury is most common in
tomography. Usual treatment is nonsurgical,
15- to 45-year-old active individuals, most
including physical therapy intervention, or-
commonly runners. Diagnosis is often made
thoses, and footwear and activity modification.
clinically. If the diagnosis is difficult then
■ Submetatarsal Cyst confirmation may be made with magnetic
resonance imaging. Usual treatment is non-
Chief Clinical Characteristics
surgical, consisting of anti-inflammatory
This presentation can involve a tender cyst just
medication, rest, and gentle exercise.
plantar to the metatarsal heads.
Background Information ■ Extensor Digitorum Longus
This condition is a dermal plantar mass that is Tendinitis
benign and usually is residing in the dermal Chief Clinical Characteristics
layer. As opposed to a submetatarsal bursa, the This presentation can be characterized by
cyst is palpable and adhered to the dermis. pain over the dorsum of the foot and tender-
Sebaceous material may discharge through a ness to palpation along the extensor digito-
small pore. Treatment may include drainage. rum longus tendons. Resisted extension of
the lesser toes and passive flexion of the
■ Subungual Hematoma lesser toes often increases the pain. Pain may
Chief Clinical Characteristics also be elicited with resisted dorsiflexion of
This presentation involves a painful toenail the ankle. Walking and running are often
that is very tender to pressure. painful.
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482 Chapter 24 Extensor Hallucis Longus Tendinitis

Background Information ■ Flexor Hallucis Longus


FOOT PAIN

The pathology of this tendinitis is usually the Tendinitis


result of overuse. Diagnosis is usually made Chief Clinical Characteristics
clinically, but may be confirmed with mag- This presentation typically includes pain and
netic resonance or ultrasound imaging. tenderness to the flexor hallucis longus tendon.
Nonsurgical treatments may be indicated, Pain is aggravated with walking, weight bear-
such as anti-inflammatory medication, rest, ing, or stretching of the tendon. Swelling is
and gentle exercise. often noted in the region of the pathology.
■ Extensor Hallucis Longus Background Information
Tendinitis The location of the pathology is often at the
Chief Clinical Characteristics medial aspect of the midfoot and can usually
This presentation may include pain and be isolated along the tendon with palpation. A
palpable tenderness along the extensor hallu- common site of irritation is under the flexor
cis tendon. Walking and running are often retinaculum at the posteromedial ankle. Pal-
painful. Resisted extension of the hallux and pation can be aided with the use of muscle
passive flexion of the hallux often increase contraction or movement of the hallux. The
the pain. Pain may also be elicited with resis- pathology is often related to overuse of the
ted dorsiflexion of the ankle. affected musculotendinous unit. Diagnosis is
usually made clinically, but may be confirmed
Background Information with magnetic resonance or ultrasound imag-
The pathology of this tendinitis is usually the ing. Management typically involves a combi-
result of overuse.50 Diagnosis is usually nation of anti-inflammatory medication, rest,
made clinically, but may be confirmed with and gentle exercise.
magnetic resonance or ultrasound imaging.
Treatment usually involves nonsurgical in- ■ Peroneal Tendinitis
terventions, such as anti-inflammatory Chief Clinical Characteristics
medication, rest, and gentle exercise. This presentation can involve pain anywhere
■ Flexor Digitorum Longus along the lateral lower leg to the posterior lat-
Tendinitis eral malleolus and along the lateral foot at the
location of the cuboid or at the plantar aspect
Chief Clinical Characteristics of the foot where the peroneus longus tendon
This presentation typically includes pain, ten- inserts at the base of the first metatarsal. The
derness, and swelling along the plantar hind- pain may also be present at the base of the fifth
foot and midfoot with tenderness along the metatarsal where the peroneus brevis tendon
tendon of the flexor digitorum longus. Pain is inserts. The pain is often aggravated with a
aggravated with walking and weight bearing. stretch into foot and ankle dorsiflexion and in-
Background Information version or a muscle contraction into foot and
A common site of irritation is deep to the ankle plantarflexion and/or eversion.
flexor retinaculum at the posteromedial Background Information
ankle. Palpation can be aided with the use of Common causes of peroneal tendon injury
muscle contraction or active movement of the include overuse and inversion injury mecha-
toes. If the tendons are inflamed, then pain is nisms. Diagnosis is usually made clinically, but
often reproduced with stretching of the toes may be confirmed with magnetic resonance or
into extension or contraction into flexion. ultrasound imaging. Nonsurgical treatments
The pathology is often related to trauma or may be indicated, such as anti-inflammatory
overuse of the affected musculotendinous medication, rest, and gentle exercise.
unit. Diagnosis is often determined clinically
and may be confirmed with magnetic reso- ■ Tibialis Anterior Tendinitis
nance or ultrasound imaging. Treatment usu- Chief Clinical Characteristics
ally involves nonsurgical interventions, such This presentation typically includes pain and
as anti-inflammatory medication, rest, and tenderness to palpation of the tibialis anterior
gentle exercise. tendon.
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Chapter 24 Extensor Hallucis Longus Tendinosis 483

Background Information ankle dorsiflexion, and reports of increased

FOOT PAIN
Pain is often present at initial contact and stiffness in the morning may be observed. Pain
during the loading response of gait, due to the is often worse with weight bearing and walk-
eccentric loading of the tendon and muscle. ing, and these patients often have difficulty per-
Pain is often increased with passive stretch forming a heel rise.
into plantarflexion or resisted dorsiflexion of
Background Information
the ankle. Tibialis anterior tendinitis is usually
Tendinosis is a state of increased tenocyte
caused by overuse.50 Diagnosis is usually
and cellular activity that leads to disorgan-
made clinically, but may be confirmed with
ized collagen and vascular hyperplasia.52,53
magnetic resonance or ultrasound imaging.
Tendinosis is usually a chronic degenerative
Usual treatment is nonsurgical, consisting
process that is initiated by an injury to the
of anti-inflammatory medication, rest, and
tendon. Diagnosis is usually based on the
gentle exercise.
clinical presentation. If pathology is severe
■ Tibialis Posterior Tendinitis or the clinical diagnosis is unclear, then mag-
netic resonance or ultrasound imaging may
Chief Clinical Characteristics
confirm the diagnosis and allow for progno-
This presentation may involve pain and
sis and staging of the tendinosis. A combina-
swelling with palpable tenderness along the
tion of patient/client education, unloading
medial aspect of the midfoot and sometimes
of the affected musculotendinous unit, con-
posterior to the medial malleolus. Pain is of-
trolled mechanical reloading, and preventive
ten worsened with weight bearing, walking,
measures appears to be effective to manage
performing a heel rise, or stretching the ten-
this condition.54
don. Attenuation of this tendon is sometimes
seen and can lead to a flat-foot deformity. ■ Extensor Digitorum Longus
Palpation may be assisted with muscle con- Tendinosis
traction of the tibialis posterior muscle.
Chief Clinical Characteristics
Background Information This presentation is characterized by pain
Injury to this tendon is often the result of over the dorsum of the foot and tenderness to
overuse that is caused by a hypermobile palpation along the extensor digitorum longus
midfoot and excessive foot pronation. The tendons. Resisted extension of the lesser toes
tibialis posterior is the most significant or passive flexion of the lesser toes often in-
active stabilizer of the arch.51 Diagnosis is creases the pain. Pain may also be elicited
usually made clinically, but may be con- with resisted dorsiflexion of the ankle. Walk-
firmed with magnetic resonance or ultra- ing and running are often painful.
sound imaging. Nonsurgical treatments may Background Information
be indicated, such as anti-inflammatory The pathology of this tendinosis is usually
medication, rest, and gentle exercise. the result of sustained overuse. Diagnosis
TENDINOSIS is usually made clinically, but may be con-
firmed with magnetic resonance or ultra-
■ Achilles Tendinosis sound imaging. Nonsurgical treatments may
Chief Clinical Characteristics be indicated, such as anti-inflammatory
This presentation typically includes sharp, medication, rest, and gentle exercise.
aching, or burning pain with point tenderness
usually along the medial aspect of the middle ■ Extensor Hallucis Longus
one-third of the Achilles tendon. Some pa- Tendinosis
tients with Achilles tendinosis are pain free. Chief Clinical Characteristics
Symptoms, if present, may be reproduced with This presentation may include pain and pal-
palpation of the Achilles tendon, passive dor- pable tenderness along the extensor hallucis
siflexion, or active resisted plantarflexion. tendon. Walking and running are often
Palpable increased thickening of the tendon painful. Resisted extension of the hallux or pas-
may be present in comparison to the unin- sive flexion of the hallux often increases the
volved tendon. Calf atrophy, limitations in pain. Pain may also be elicited with resisted
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484 Chapter 24 Flexor Digitorum Longus Tendinosis

dorsiflexion of the ankle. Walking and running flexor retinaculum at the posteromedial
FOOT PAIN

are often painful. ankle. Palpation can be aided with the use
of muscle contraction or movement of the
Background Information
hallux. The pathology is often related to
The pathology of this tendinosis is usually the
chronic overuse of the affected musculo-
result of sustained overuse.50 Diagnosis is
tendinous unit. Diagnosis is usually made
usually made clinically, but may be confirmed
clinically, but may be confirmed with mag-
with magnetic resonance or ultrasound imag-
netic resonance or ultrasound imaging.
ing. Treatment usually involves nonsurgical
Management typically involves a combina-
interventions, such as anti-inflammatory
tion of anti-inflammatory medication, rest,
medication, rest, and gentle exercise.
and gentle exercise.
■ Flexor Digitorum Longus ■ Peroneal Tendinosis
Tendinosis
Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically includes pain
This presentation typically includes pain, anywhere along the lateral lower leg to
tenderness, and swelling along the plantar the posterior lateral malleolus and along
hindfoot and midfoot with tenderness along the lateral foot at the location of the cuboid
the tendon of the flexor digitorum longus. or at the plantar aspect of the foot where
Pain is aggravated with walking and weight the peroneus longus tendon inserts at the
bearing. base of the first metatarsal. The pain may
Background Information also be present at the base of the fifth
A common site of irritation is deep to the metatarsal where the peroneus brevis ten-
flexor retinaculum at the posteromedial an- don inserts. The pain is often aggravated
kle. Palpation can be aided with the use of with a stretch into dorsiflexion and inver-
muscle contraction or active movement of the sion or a muscle contraction into plan-
toes. If the tendons are chronically inflamed, tarflexion and/or eversion. Weakness may be
then pain is often reproduced with stretching noted into plantarflexion and/or eversion due
of the toes into extension or contraction into to degenerative thickening, lengthening, and
flexion. The pathology is often related to tearing of the tendon.
sustained trauma or overuse of the affected
Background Information
musculotendinous unit. Diagnosis is often
Common causes of peroneal tendon injury
determined clinically and may be confirmed
include overuse and inversion injury mecha-
with magnetic resonance or ultrasound imag-
nisms that may lead to the chronic degener-
ing. Treatment usually involves nonsurgical
ative process of tendinosis. Tendinosis is a
interventions, such as anti-inflammatory
state of increased tenocyte and cellular activ-
medication, rest, and gentle exercise.
ity that leads to disorganized collagen and
■ Flexor Hallucis Longus vascular hyperplasia.52,53 Final diagnosis
Tendinosis is confirmed with magnetic resonance or
ultrasound imaging. A combination of
Chief Clinical Characteristics
patient/client education, unloading of the
This presentation typically includes pain and
affected musculotendinous unit, controlled
tenderness to the flexor hallucis longus tendon.
mechanical reloading, and preventive meas-
Pain is aggravated with walking, weight
ures appears to be effective to manage this
bearing, or stretching of the tendon. Swelling
condition.54
is often noted in the region of the pathology.
Background Information ■ Tibialis Anterior Tendinosis
The location of the pathology is often at the Chief Clinical Characteristics
medial aspect of the midfoot and can usually This presentation typically includes pain and
be isolated along the tendon with palpation. tenderness to palpation of the tibialis anterior
A common site of irritation is under the tendon.
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Chapter 24 Vitamin B Deficiency 485

Background Information ■ Thromboangiitis (Buerger’s

FOOT PAIN
Pain is often present at initial contact and Disease)
during the loading response of gait, due to Chief Clinical Characteristics
the eccentric loading of the tendon and mus- This presentation typically includes pain that is
cle. Pain is often increased with passive worse in early stages, as well as coldness, numb-
stretch into plantarflexion or resisted dorsi- ness, tingling, paresthesia, ulcerations, and gan-
flexion of the ankle. The pathology of tibialis grene. Signs of intermittent claudication are
anterior tendinosis is usually caused by sus- common in the calf or the arch of the foot. Dis-
tained overuse.50 Diagnosis is usually made tal pulses of the lower and upper extremities
clinically, but may be confirmed with mag- are often decreased or absent with a proximal
netic resonance or ultrasound imaging. progression. This condition is prevalent in males
Usual treatment is nonsurgical, consisting of ages 20 to 40 years with a history of smoking.
anti-inflammatory medication, rest, and
gentle exercise. Background Information
This condition is characterized by an oblitera-
■ Tibialis Posterior Tendinosis tion of the small and medium arteries and
Chief Clinical Characteristics veins through autoimmunity triggered by
This presentation typically includes pain and smoking nicotine.55,56 Angiography confirms
swelling with palpable tenderness along the the diagnosis. The only known effective inter-
medial aspect of the midfoot and posterior to vention is nicotine smoking cessation.
the medial malleolus. Pain is often worse
with weight bearing and walking, and these ■ Tinea Pedis (Athlete’s Foot)
patients often have difficulty performing a heel Chief Clinical Characteristics
rise. Attenuation of this tendon is sometimes This presentation typically includes itching,
seen as a result of tendinosis and the devel- pain, inflammation, vesiculation, and macer-
opment of posterior tibialis tendon dysfunc- ation of affected web spaces, as well as
tion may lead to a flat-foot deformity and a thickening and distortion of infected toenails.
caudal drop of the navicular. These patients Symptoms range from mild to severe.
have a very difficult time contracting the tib-
ialis posterior muscle, even during a manual Background Information
muscle test. This condition usually starts in the third web
space, and may spread to the plantar surface of
Background Information the feet. The common infecting organism is
Injury to this tendon is often the result of the Trichophyton rubrum fungus, which is
overuse with a hypermobile midfoot. The transmitted from person to person or animal
tibialis posterior is the most significant to person. Fungal culture confirms the diagno-
active stabilizer of the arch.51 Tendinosis sis. Topical antifungal agents form the most
is a state of increased tenocyte and cellular common treatment for this condition.
activity that leads to disorganized collagen
and vascular hyperplasia. Tendinosis is ■ Vitamin B Deficiency
usually a chronic degenerative process Chief Clinical Characteristics
that is initiated by injury to the tendon. This presentation typically includes burning
Diagnosis is usually based on the clinical pain on the soles of the feet with erythematous
presentation; however, magnetic resonance mottling and systemic symptoms include
imaging may confirm the diagnosis and anorexia, weakness, and weight loss.
allow for prognosis and staging of the
tendinosis. A combination of patient/client Background Information
education, unloading of the affected This condition can result in damage to periph-
musculotendinous unit, controlled me- eral nerves, the heart, and the central nervous
chanical reloading, and preventive meas- system. In developed countries, this deficiency
ures appears to be effective to manage this is more likely in individuals with a history
condition.54 of chronic alcohol abuse, debilitating illness,
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486 Chapter 24 Waldenstrom’s Macroglobulinemia

long-term unsupplemented parenteral nutri- 2. Moseley GL. Imagined movements cause pain and
FOOT PAIN

tion, and pernicious vomiting. Blood tests con- swelling in a patient with complex regional pain
syndrome. Neurology. May 11, 2004;62(9):1644.
firm the diagnosis. Vitamin B supplementation 3. Ledermann HP, Schweitzer ME, Morrison WB. Nonen-
is a common treatment for this condition, as hancing tissue on MR imaging of pedal infection: char-
well as addressing underlying diseases that may acterization of necrotic tissue and associated limitations
contribute to this condition. for diagnosis of osteomyelitis and abscess. Am
J Roentgenol. Jan 2002;178(1):215–222.
4. Carapetis JR, Currie BJ. Rheumatic fever in a high inci-
■ Waldenstrom’s dence population: the importance of monoarthritis and
Macroglobulinemia low grade fever. Arch Dis Child. Sep 2001;85(3):
223–227.
Chief Clinical Characteristics 5. Weitz JI, Byrne J, Clagett GP, et al. Diagnosis and treat-
This presentation typically includes bleeding and ment of chronic arterial insufficiency of the lower
peripheral neuropathy. Neuropathy associated extremities: a critical review. Circulation. Dec 1,
with this condition may present as lower extrem- 1996;94(11):3026–3049.
6. Mosley JG. Arterial problems in athletes. Br J Surg.
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7. Williams KJ, Tabas I. The response-to-retention hypoth-
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Common associated findings include fatigue, May 1995;15(5):551–561.
weakness, visual disturbances, headaches, and 8. Schwartz SM, deBlois D, O’Brien ER. The intima. Soil
skin and mucosal bleeding. Cardiovascular for atherosclerosis and restenosis. Circ Res. Sep 1995;77
(3):445–465.
impairment, recurrent infections, sensitivity 9. Salvarani C, Vlachonikolis IG, van der Heijde DM, et al.
to cold, lymphadenopathy, hepatosplenomegaly, Musculoskeletal manifestations in a population-based
or engorgement of retinal veins also may be cohort of inflammatory bowel disease patients. Scand
present. This disease is a result of bone mar- J Gastroenterol. Dec 2001;36(12):1307–1313.
10. Parisi L, Pierelli F, Amabile G, et al. Muscular cramps:
row infiltration that leads to increased proposals for a new classification. Acta Neurol Scand.
amounts of macroglobulin circulating in the Mar 2003;107(3):176–186.
plasma. The survival rate usually is in the 11. Morgan MS. Prophylaxis should be considered even
range of 5 to 10 years.57 Laboratory tests that for trivial animal bites. BMJ. May 10, 1997;314(7091):
1413.
reveal altered immunoglobulin M mono- 12. Toolan BC, Sangeorzan BJ, Hansen ST, Jr. Complex re-
clonal proteins and neoplastic infiltration construction for the treatment of dorsolateral peritalar
of tissues confirm the diagnosis. Treatments subluxation of the foot. Early results after distraction
for this condition may include nucleoside arthrodesis of the calcaneocuboid joint in conjunction
with stabilization of, and transfer of the flexor digito-
analogs, alkylators, combination chemother- rum longus tendon to, the midfoot to treat acquired pes
apy, and stem cell transplantation.58 planovalgus in adults. J Bone Joint Surg Am. Nov 1999;81
(11):1545–1560.
■ Warts 13. Fukumoto Y, Tsutsui H, Tsuchihashi M, Masumoto A,
Takeshita A. The incidence and risk factors of choles-
Chief Clinical Characteristics terol embolization syndrome, a complication of cardiac
This presentation typically includes pain and catheterization: a prospective study. J Am Coll Cardiol.
tenderness to palpation or weight bearing on Jul 16, 2003;42(2):211–216.
14. Murphy JV, Banwell PE, Roberts AH, McGrouther DA.
the lesion. Frostbite: pathogenesis and treatment. J Trauma.
Jan 2000;48(1):171–178.
Background Information 15. Jose RM, Viswanathan N, Aldlyami E, Wilson Y,
Warts result from human papillomavirus in- Moiemen N, Thomas R. A spontaneous compartment
fection; plantar warts are commonly present syndrome in a patient with diabetes. J Bone Joint Surg
beneath points that bear high pressures. This Br. Sep 2004;86(7):1068–1070.
16. Handa S, Sahoo B. Childhood lichen planus: a study of
condition involves skin lesions that have a 87 cases. Int J Dermatol. Jul 2002;41(7):423–427.
rough keratotic surface with an outer rim of 17. Pao DG, Keats TE, Dussault RG. Avulsion fracture of
thickened skin and punctate black dots, which the base of the fifth metatarsal not seen on conventional
are thrombosed capillaries. Clinical examina- radiography of the foot: the need for an additional
projection. Am J Roentgenol. Aug 2000;175(2):549–552.
tion confirms the diagnosis. 18. Judd DB, Kim DH. Foot fractures frequently misdiag-
nosed as ankle sprains. Am Fam Physician. Sep 1,
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SECTION III
Adult Non-Pain

CHAPTER25
Foundations of Neurological Differential
Diagnosis
■ Bernadette M. Currier, PT, DPT, MS, NCS ■ Beth E. Fisher, PT, PhD

IN THIS CHAPTER: physical therapy services. As specialists in the


■ Rationale for considering nervous system diagnosis and treatment of movement dys-
pathology in diagnosis function, physical therapists must have the
■ Summary of relevant clinical neuroanatomy knowledge base and clinical skills to confirm
■ Relationship between neuroanatomy and that impairments are consistent with a given
clinical syndromes medical diagnosis. As Gordon and Watts
discuss in Chapter 1, physical therapists must
OUTLINE engage in the diagnostic process as part of their
overall evaluation. The process must lead to a
Introduction 489 decision regarding the probable pathological
Nervous System Pathology is or pathophysiological cause of an individual’s
Important to Consider in Diagnostic problem, followed by a determination of
Reasoning 489 whether physical therapy intervention is the
Summary of Neurological Differential most appropriate to address the pathology
Diagnosis 490 underlying the condition. We advocate that the
Chart Review 491 use of this process should extend to physical
History/Subjective Examination 492 therapists working with individuals who
Objective Examination 492 display symptoms and signs consistent with
Clinical Neurological Tests 492 nervous system pathology.
Diagnostic Impression 493
Linking Examination Findings to
Neuroanatomy 493
Nervous System Pathology is
Anatomy and Functions of the Nervous
Important to Consider in
System 493
Diagnostic Reasoning
Neurological Impairments and Associated We believe it is imperative for physical thera-
Anatomical Regions 502 pists to be as skilled in the process of diagnosis
Conclusion 503 for individuals reporting concerns of activity
restrictions for reasons other than pain, as it
is when diagnosing individuals reporting
Introduction concerns of pain. As Landel describes in
Chapter 2, it is the responsibility of the physi-
Numbness, tingling, weakness, and dizziness cal therapist to encourage individuals to de-
are just a few symptoms commonly encoun- scribe what activities are limited as a result of
tered in physical therapist practice that may their chief concern. Unlike individuals with
indicate pathology of the neurological system. pain, however, individuals with neurological
Resultant difficulty in mobility and function is diagnoses rarely report problems at the level
what frequently drives an individual to seek of body structures and functions as a chief

489
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490 Chapter 25 Foundations of Neurological Differential Diagnosis

concern. Individuals are not likely to state that therapist applying a backward reasoning
FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

they are experiencing ataxia, spasticity, or thought process to establish differential diag-
problems with force production. Rather, these noses would further investigate the possible rea-
problems manifest as limitations specific to sons for difficulty tying shoes along with multi-
activity or participation in a social context, and ple episodes of “visual changes.”
these limitations are frequently the chief con- Given that the patient history in this case in-
cern. Regardless of whether activity restric- cludes the possible presence of special concerns
tions are the result of pain or impairments related to the nervous system, the physical ther-
resulting from nervous system pathology, the apist should immediately consider causative
physical therapist is encouraged to conduct the pathologies beyond an isolated musculoskeletal
examination to establish causative pathology system disorder such as shoulder impingement.
and the appropriate plan of care. Determining Watts discusses common special concerns in
appropriateness for physical therapy and iden- Chapter 4. In addition, Box 25-1 provides a list
tifying the primary problems influencing the of signs and symptoms caused by neurological
movement dysfunction are the primary goals pathology that should alert the physical thera-
of the physical therapy evaluation. pist as to the appropriate course of action—
To illustrate the importance of considering emergent or nonemergent referral to a medical
nervous system pathology during the process provider. Optic neuritis, tumor, and multiple
of differential diagnosis, throughout the chap- sclerosis are additional examples of pathologies
ter we will use the example of a 34-year-old that should be included in the list of hypotheses.
Caucasian female schoolteacher whose chief Neurological testing should include men-
concern involves not being able to reach high tal status, cranial nerve function, force produc-
enough to effectively write on the chalkboard. tion, deep tendon and pathological reflexes,
Patient interview reveals the individual has ex- coordination, gait, and sensory integrity.1 For
perienced this difficulty reaching for the past the example in this chapter, an abnormal
3 weeks. Past medical history is unremarkable Babinski reflex and upper limb ataxia are
with the exception of nearsightedness for found. As described by Landel in Chapter 2,
which the individual wears corrective lenses. when determining if managing the causative
The individual states that she has experienced pathology is within the scope of physical ther-
intermittent problems with her vision for the apy practice, the decision-making process
past several months, and attributes this to involves three possible outcomes:
missing her eye exam this year with the need
● Physical therapy is not indicated;
for a new eyeglass prescription.
As Davenport introduces in Chapter 3 and ● Physical therapy is indicated but a consulta-
Watts elaborates on in Chapter 4, a process of tion to another health care provider is
backward reasoning should be used to create required; or
and test clinical hypotheses related to the cause ● Physical therapy can proceed independent
of this individual’s symptoms and signs. A of additional consultation.
physical therapist who incorporates a backward The constellation of findings from the his-
reasoning pattern will start with a list of all the tory and physical examination in the case of the
possible pathologies that may cause the pa- 34-year-old schoolteacher should indicate that
tient’s disablement in order to guide additional physical therapy is not indicated. The physical
history and physical examination. Further therapist should refer this individual to a neu-
questioning of this individual reveals she has rologist promptly for further medical workup.
been experiencing difficulty tying her students’
shoes during the past few weeks, and attributes Summary of Neurological
this to work-related stress. She also admits to an Differential Diagnosis
episode of visual changes a few years prior to
this visit. Because those changes resolved spon- How did the physical therapist in the above
taneously within just a few days, the individual scenario determine that the individual was
did not feel the need to seek medical consulta- not only inappropriate for physical therapy
tion. Upon hearing these concerns, the physical services, but also in need of referral to a
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Chapter 25 Foundations of Neurological Differential Diagnosis 491

FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


BOX 25-1 Special Concerns Specific to Nervous System Pathology2

Emergency Situations (Immediate Medical Assistance Required)


● Loss of consciousness or difficult to arouse ● Evidence of motor neuron disease (fascicula-

● Extreme confusion not consistent with premor-


tion, atrophy, weakness in limbs or trunk) not
bid status previously diagnosed
● New onset of involuntary movements or tremor
● Uncontrolled seizure activity (status epilepticus)
● Change in autonomic status (RR, HR, HP, body
● Acute infection with other associated neurolog-
ical signs such as nuchal rigidity or intense, temperature, autonomic dysreflexia, orthostatic
localized back pain hypotension)
● Bulbar and other cranial nerve signs/symptoms,
● Rapid onset of focal neurological deficits
(suggesting stroke in progress): progressive change in swallowing, dysarthria,
● Weakness, numbness or tingling of face, arm,
voice hoarseness, visual changes such as gaze
or leg, especially on one side of body palsies or report of diplopia, nystagmus, ptosis,
● Blurred or decreased vision in one or both
impaired papillary responses, facial weakness or
eyes or double vision sensory changes, hearing loss, dizziness, vertigo
● Loss of speech or trouble understanding speech
(Note: Acute onset or changes in any of these
● Headache that is sudden, severe, and unusual
signs or symptoms would require immediate
● Dizziness, loss of balance or coordination with
medical assistance.)
any of other signs. ● Constant headache that worsens over time

● Evidence of spinal column instability (eg, decre- ● Patient report of transient ischemic attack
ment in motor and sensory status with position symptoms (stroke-in-progress symptoms from
change from supine to sit or sit to stand, bilateral above that resolve within 24 hours)
upper extremity paresthesias with neck flexion) ● Vertebral artery insufficiency (neck motions
● Nonresponsive autonomic dysreflexia discontinued immediately)
Nonemergency Situations (Outside ● Neurological signs inconsistent with diagnosis
Referral Required) ● Signs or symptoms of systemic illness (fever,
● Acute onset of neurological signs such as incon- diaphoretic, poor exercise tolerance)
tinence, saddle paresthesia, abnormal reflexes ● Significant changes in personality or cognitive
● Progressive neurological signs in a known neuro- status (memory loss, impaired language ability,
logical diagnosis that is not degenerative (ie, visuospatial deficits)
decrement in motor, sensory, or cognitive status)

neurologist for further medical evaluation? model, information from the chart review,
How is the symptom-based diagnostic process subjective examination, and objective testing
described in the previous chapters applied to are combined to create a hypothesized
individuals with neurological diagnoses? Con- anatomical lesion location. The hypothesized
sistent with pathology of other body systems, lesion location can assist physical therapists
we encourage the use of a hypothesis-driven with determining which among the list of hy-
reasoning strategy to formulate a list of possi- pothesized forms of pathology can be ruled
ble diagnoses and establish an appropriate less likely. In turn, forms of pathology that can
course of action when physical therapists sus- be excluded will assist in the decision to treat
pect that pathology related to the nervous sys- the individual, refer him or her to another
tem is the probable cause for an individual’s health care provider for additional evaluation,
chief concern. or both.
Sullivan and colleagues2 adapted an exist-
Chart Review
ing process of neurological differential
diagnosis3 to provide physical therapists with a When available in physical therapy settings,
framework for establishing a hypothesized the medical record can be a primary source of
lesion location within the neuraxis. In this information for obtaining the individual’s
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492 Chapter 25 Foundations of Neurological Differential Diagnosis

temporal features such as age, history of pres- Objective Examination


FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

ent illness, past medical history, past surgical


history, lab and radiology reports, family his- Following the subjective interview, an objec-
tory, and medications. It is the responsibility of tive examination should be performed (1) to
the physical therapist to confirm that the in- determine if the individual’s clinical presen-
formation gathered from the medical record is tation is consistent with pathology that
consistent with the individual’s chief concern is potentially amenable to physical therapy
and clinical presentation. To determine this, and then (2) to establish causes for move-
the physical therapist must extract pertinent ment dysfunction including the identifica-
information from the subjective examination tion of direct impairments and compensa-
and combine it with information obtained tions. Given that the individual’s chief
from the objective examination. In some set- concern is often a limitation at the func-
tings, however, medical records are inaccessi- tional level, observation and analysis of task
ble and the physical therapist must use solely performance is the next step in the objective
his or her interaction with the individual to examination. Comparison of what the phys-
gain this information. ical therapist considers to be “ideal” with
the actual demonstration of the task gives
History/Subjective Examination the clinician valuable insight into what
impairments and compensations might be
The patient interview should include a skilled influencing the production of movement.4
set of questions posed by the physical therapist The physical therapist can then test those
with the goal of distinguishing among hypo- impairments to confirm or negate working
thetical causes of the patient’s disablement. hypotheses as to the cause(s) of the present
If the individual is unable to fully participate movement dysfunction.
in the subjective interview due to cognitive
or language barriers, a caregiver or inter-
preter, respectively, may be a necessary par- Clinical Neurological Tests
ticipant in the interview. Key pieces of infor- In addition to testing hypothesized impair-
mation should include the individual’s goals ments, clinical neurological tests should be
for physical therapy, the family’s goals for performed for any individual whose given
physical therapy if the individual is unable medical diagnosis, subjective history, or
to express these, current and prior levels of clinical presentation suggests underlying
function, perceived limitations to the individ- pathology of the nervous system. These tests
ual’s participation in societal roles and rele- include examination of mental status, cranial
vant activities, social history, and temporal nerve function, force production, deep ten-
features. don and pathological reflexes, coordination,
The physical therapist should then deter- gait, and sensory integrity.1 The following
mine, based on the information gathered examples of presentations should cue the
from the subjective interview, whether or not clinician to perform additional neurological
it is appropriate to continue with the physi- testing:
cal therapy examination of the individual. If
the information from the subjective history ● Mental status: Confusion, inability to follow
reveals information consistent with the re- simple directions, no recall of events from
ferred medical diagnosis, the physical thera- the recent past, or emotional lability.
pist is encouraged to proceed with the exam. ● Cranial nerve function: Eyes that are offset
In the event that the individual’s history from one another, facial asymmetry or
either does not appear consistent with the weakness, slurred speech, drooling, poor
given diagnosis or suggests the presence of hearing, or balance problems.
special concerns, the physical therapist is ● Force production: Slowness in movement or
encouraged to continue by performing a neu- signs of weakness. Often these signs are no-
rological screen and utilizing those results to ticed during observation of functional tasks
determine the appropriate course of action such as moving from a sitting position to
(see Box 25-1). standing, walking, and reaching.
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Chapter 25 Foundations of Neurological Differential Diagnosis 493

● Deep tendon and pathological reflexes: Neck Linking Examination Findings to

FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


or low back pain with radiating pain or Neuroanatomy
sensory disturbances down an extremity.
Pathological reflex testing should be per- We suggest that the decision to refer the
formed to help determine the presence of an patient to a medical care provider in the case
upper motor neuron lesion, which is of suspected nervous system pathology is facil-
described in a later section of this chapter. itated by the physical therapist’s ability to in-
● Coordination: Uncoordinated trunk or ex- terpret examination findings and concisely
tremity movement, indicated by tremors or communicate the most important findings. To
inability to accurately reach targets. Gait be able to accurately associate impairments
may appear unsteady and individuals may with neuroanatomical locations, a physical
lose their balance while walking. therapist must have a sound background in
● Gait: Stagger, limp, maintain a wide base of neuroanatomy with the ability to efficiently
support, or require an assistive device in utilize neuroanatomy resources to supplement
order to be able to walk. his or her knowledge as needed. In this section,
● Sensory integrity: Inconsistent foot place- the anatomy and function of the nervous sys-
ment or the need to look down at the floor tem, and pathological processes associated
while walking, or report of sensory distur- with specific brain regions, spinal cord re-
bances such as numbness or tingling. gions, and peripheral nerves, are reviewed in
order to assist the reader in determining possi-
ble lesion locations as a part of the differential
Diagnostic Impression diagnosis process.
The results of clinical neurological testing
Anatomy and Functions of the
in combination with other information
Nervous System
gleaned through movement analysis and
specific impairment testing should enable The nervous system is divided into two main
the physical therapist to hypothesize lesion parts, the central nervous system (CNS) and
location(s). Based on the lesion location, this the peripheral nervous system (PNS). The
hypothesis will aid in the decision to proceed CNS is comprised of the brain and spinal cord.
with physical therapy and/or refer the It is made up of white matter (myelinated
individual to a neurologist. Box 25-1 lists axons) and gray matter (cell bodies; Fig. 25-1).
signs and symptoms that may warrant refer- The PNS is comprised of the cranial, spinal,
ral to a medical provider. In addition to the sympathetic and parasympathetic nerves, and
signs and symptoms listed in Box 25-1, if ganglia. Neuroanatomical structures work
involvement of specific neuroanatomical individually and together to perform specific
structures is suspected in an individual with functions. We discuss the anatomy and com-
an otherwise unknown pathology, referral mon functions of the neuroanatomical
to a neurologist should take place. structures in this section.
To provide an example of how going The ultrastructure of the brain is charac-
through this process will generate a diagnostic terized by two cerebral hemispheres, each of
impression, let’s consider the 34-year-old female which has frontal, parietal, temporal, and
who reports difficulty reaching, tying shoes, occipital lobes (Fig. 25-2). The interhemi-
and seeing clearly. Because impairment testing spheric fissure separates the two hemispheres,
revealed an abnormal Babinski sign and upper while the corpus callosum connects the paired
limb ataxia, involvement of neuroanatomical lobes of each hemisphere. The central sulcus
structures should be suspected. Specifically, the divides the brain into anterior (precentral)
abnormal Babinski reflex suggests the presence and posterior (postcentral) regions. Cortices
of a disorder that affects the upper motor made up of gray matter cover the surfaces of
neurons, and the presence of the ataxia suggests the cerebral hemispheres and serve important
a disorder that involves the cerebellum. The functions. The primary motor cortex is located
process of linking examination findings to in the precentral gyrus of the frontal lobe. The
neuroanatomy is elaborated on next. primary somatosensory cortex is within the
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494 Chapter 25 Foundations of Neurological Differential Diagnosis

Cerebral hemispheres
FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

(Frontal section)
White matter
Gray matter:
Thalamus
Basal ganglia
Cortex

Brainstem
(Transverse section)
Gray matter
White matter

Spinal cord
(Transverse section)
Gray matter
White matter

FIGURE 25-1 Relative position of gray matter and white matter in the neuraxis. In the brain, the gray
matter is primarily located in the periphery, the thalamus, and the basal ganglia. By contrast, the gray matter
is located centrally within the spinal cord.

Sylvian Central Corpus


fissure sulcus callosum
Frontal Parietal Frontal Parietal
lobe lobe lobe lobe

Temporal Occipital Temporal Occipital


lobe lobe lobe lobe
A Lateral view B Midsagittal view
FIGURE 25-2 Ultrastructure of the brain, including (A) lateral view and (B) midsagittal view of the cerebrum
and its component lobes.
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Chapter 25 Foundations of Neurological Differential Diagnosis 495

postcentral gyrus of the parietal lobe. The of the main regions within the cerebral hemi-

FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


primary visual cortex is in the occipital spheres. The functions of other structures in-
lobe. The primary auditory cortex is in the volved in motor pathways are as follows:
temporal lobe. ● Basal ganglia: Regulates the descending
The cerebral cortex is topographically organ- motor pathway, eye movements, emotion,
ized. The somatotopic map is characterized by and cognition.
the motor and sensory homunculi, by which ● Brainstem: Cardiorespiratory function; pro-
motor and sensory regions of the body are repre- vides sensory and motor innervation to face
sented by regions of the brain. The motor ho- and neck, passageway for ascending sensory
munculus represents the areas within the motor tracts and descending motor pathways, regu-
cortex that control movement of respective parts lation of consciousness and sleep/wake cycle.
of the body, and the sensory homunculus within
● Cerebellum: Integrates information to pro-
the sensory cortex receives sensory input from
duce smooth movement, motor planning,
respective parts of the body.
and learning.
Several brain structures are involved in the
production of movement. These include the ● Lateral motor pathways (lateral corticospinal
cerebral cortex (Table 25-1), brainstem, thala- and rubrospinal tracts): Control movement
mus, basal ganglia, and cerebellum. Movement of the extremities.
information is transferred between these struc- ● Lower motor neurons: Provide the connec-
tures via motor pathways. The lateral corti- tion between upper motor neurons and the
cospinal tract is the primary motor pathway. periphery.
The cell bodies, or upper motor neurons, are lo- ● Medial motor pathways (anterior corti-
cated within the primary motor cortex. cospinal, vestibulospinal, reticulospinal, and
Axons then travel down as white matter tectospinal tracts): Control movements of
through the brainstem to cross over at the level the trunk and its relationship to postural
of the medulla to reach the spinal cord, specifi- stability and balance, head and neck orien-
cally to project to lower motor neurons within tation, and automaticity of gait.
the anterior horn. The cerebellum, basal gan- ● Thalamus: Conveys sensory, motor, and
glia, and thalamus are subcortical structures lo- limbic information to the cerebral cortex.
cated within both the right and left sides of the ● Upper motor neurons: Involved in move-
brain. Table 25-1 reviews the primary functions ment and tone of the trunk and extremities.

TABLE 25-1 ■ Functions of Selected Brain Structures


BRAIN STRUCTURE FUNCTION(S)
Frontal lobe Initiation, judgment, memory, impulse control,
Broca’s area (left hemisphere) sequencing, social behavior
Expressive language
Parietal lobe Sensory integration, awareness of body image
and environment
Temporal lobe Long-term memory, auditory processing
Wernicke’s area (left hemisphere) Processing of speech
Occipital lobe Visual field, color discrimination
Cerebral cortex
Motor cortex (motor homunculus) Contralateral control of movement
Somatosensory cortex (sensory homunculus) Contralateral perception of sensation
Visual cortex Contralateral perception of vision
Auditory cortex Bilateral reception of auditory input
Corpus callosum Facilitates communication between the right and
left hemispheres of the brain
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496 Chapter 25 Foundations of Neurological Differential Diagnosis

Each motor pathway described above orig- ipsilaterally up the spinal cord, where it crosses
FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

inates at the primary motor cortex. The axons over at the level of the medulla to then ascend
come together to form the corona radiata and through the rest of the brainstem and thala-
internal capsule prior to traversing through the mus to eventually be received and integrated
brainstem to reach the spinal cord and finally within the somatosensory cortex.
synapse with alpha motor neurons in the ven- The spinothalamic tracts carry the sensory
tral horns. The interrelationships among struc- modalities of pain and temperature. As is the
tures that form specific motor pathways are case with the pathways of proprioception, vibra-
depicted in Figure 25-3. tion, and light touch, peripheral sensory recep-
A common way of describing pathology to tors initially receive the stimuli, and information
the CNS or PNS is by using the terms upper is then carried from ascending peripheral nerves
motor neuron (UMN) disorder or lower motor through spinal nerves to reach the spinal cord.
neuron (LMN) disorder. The term UMN disor- Pain and temperature modalities are transmit-
der signifies that there is a lesion within the ted in the anterolateral rather than dorsal col-
CNS, specifically involving the descending umn of the spinal cord. Rather than ascending
motor pathways, whereas the term LMN disor- ipsilaterally as the dorsal column does, pathways
der signifies that there is a lesion within the carrying pain and temperature cross over
PNS. Certain classic signs, summarized in through Lissauer’s tract to the opposite side of
Table 25-2, suggest the presence of a lesion. the spinal cord within a few levels of entry to
Somatic sensation travels through the as- then ascend the anterolateral tract contralater-
cending pathways of the somatosensory sys- ally. The spinothalamic tracts ascend through
tem, the dorsal column/medial lemniscus the brainstem and thalamus to also be received
pathways, and the anterolateral pathways. The and integrated within the somatosensory cortex.
axons traveling through the dorsal column Figure 25-4 depicts the structures responsible
cross at the level of the medulla prior to as- for somatosensory processing, which are gener-
cending to the contralateral thalamus in order ally organized into the dorsal column–medial
to reach the somatosensory cortex. Axons of lemniscal pathway and the anterolateral system.
the anterolateral pathway cross within the gray The brainstem houses the reticular forma-
matter of the spinal cord and ascend through tion and cranial nerves. It is comprised of the
the thalamus to terminate at the somatosen- midbrain, pons, and medulla. The brainstem
sory cortex. has many important functions, especially
Somatosensory pathways convey informa- those responsible for maintaining vital life
tion from the periphery to the brain: functions:
● Anterolateral pathways: Convey pain and ● Midbrain: Controls consciousness, mainte-
temperature sensations. nance of open eyelids, pupil dilation; acts as
● Medulla: Area where the dorsal column/ the pathway for ascending and descending
medial lemniscal pathways cross over. long tracts.
● Dorsal column: Conveys proprioception, ● Pons: Controls facial sensation, muscles of
vibration, and light touch. mastication, eye movements, facial expres-
● Somatosensory cortex: Receives and inte- sion, vestibular and hearing sense; acts as
grates sensory information. the pathway for ascending and descending
● Thalamus: Conveys sensory, motor, and long tracts.
limbic information to the cerebral cortex. ● Medulla: Relays signals between the brain
and spinal cord; controls autonomic func-
To summarize, the dorsal column carries tions including respiration, blood pressure,
the sensory modalities of proprioception, vi- vomiting, and reflexes.
bration, and light touch. Peripheral sensory
receptors initially receive the stimuli, and in- The paired internal carotid arteries (ICAs)
formation is carried from ascending periph- and vertebral arteries provide the blood supply
eral nerves through spinal nerves to reach the to the brain. Anterior circulation is provided
dorsal column of the spinal cord. The medial by the ICAs, which give rise to the anterior and
lemniscal pathways carry this information middle cerebral arteries. The anterior cerebral
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FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


Cortex

Midbrain

Pons

Rostral
medulla

Cervico-
medullary Caudal
junction medulla
(decussation)

Spinal cord

A B C

Cortex

Midbrain

Pons

Rostral
medulla

Cervical
spinal cord

D E
FIGURE 25-3 Motor pathways. The central nervous system has two motor pathways that originate in primary
motor cortex: (A) the lateral corticospinal tract and (B) the anterior corticospinal tract. Additionally, motor path-
ways originate in the midbrain: (C) rubrospinal tract and pons and rostral medulla; (D) vestibulospinal tracts and
midbrain and pons; (E) tectospinal tract and reticulospinal tract.
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498 Chapter 25 Foundations of Neurological Differential Diagnosis


FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

TABLE 25-2 ■ Comparison of Clinical Findings for Upper Motor Neuron (UMN) and Lower
Motor Neuron (LMN) Disease
UMN LMN
Deep tendon reflexes Hyperreflexica Hyporeflexic
Pathological reflexes Present Absenta
Fasciculationsb Absent Present
Atrophyb Present Present
Tone Increaseda Decreased
aExceptin the case of newborns and individuals in the acute stage of stroke or spinal injury.
bFasciculationsand atrophy may be present in both UMN and LMN disorders, but are more prominent in LMN disorders
due to profound weakness.

Frontal
section
of brain
(Cerebrum) Cortex

Midbrain
Pons
Rostral
medulla
Caudal Midbrain
medulla
Cervical
spinal
cord Pons

Rostral
medulla

Caudal
medulla

Cervical
spinal cord
A B
FIGURE 25-4 Somatosensory pathways. The central nervous system contains two somatosensory pathways
that originate in primary sensory cortex: (A) the anterolateral system and (B) the dorsal column–medial
lemniscal pathway.

artery supplies the anterior medial surface of form the basilar artery. The posterior cerebral
the cortex, and the middle cerebral artery sup- artery arises from the basilar artery. The
plies the lateral frontal, temporal, and parietal basilar artery supplies the brainstem and cere-
lobes. Posterior circulation is provided by the bellum, while the posterior cerebral artery
vertebral arteries, which come together to supplies the brainstem, cerebellum, inferior
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Chapter 25 Foundations of Neurological Differential Diagnosis 499

and medial temporal lobes, and medial occip- C1

FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


C1
ital cortex. C2
C2
The ICAs and basilar artery meet to form C3
C3 Cervical
the circle of Willis, which is an anastomotic C4
C4 roots
C5
ring that gives rise to additional arteries that Cervical
C5
C6
supply the cerebral hemispheres. This struc- enlarge-
C6 C7
ture allows brain vasculature to be somewhat C7 C8
ment T1
redundant, so that if one vessel is damaged and T1
T2
cannot provide blood supply to a certain re- T2
T3
gion of the brain, another will provide some T3
degree of circulation. T4
T4
The spinal cord is housed within the bony T5
vertebral canal (Fig. 25-5). The vertebral canal T5
is made up of 7 cervical, 12 thoracic, and T6 T6
5 lumbar vertebrae, along with a fused sacrum. T7 T7 Thoracic
The spinal cord ends at the level of the first or roots
T8
second lumbar vertebrae. Both gray and white T8
matter are located within the cord, with the T9

gray matter housing cell bodies and myelinated T10 T9


axons that travel within the posterior column
T11 T10
and anterolateral ascending pathways. The
single anterior and two posterior spinal arteries Conus
T12
T11
provide the blood supply to the spinal cord. medullaris
The spinal cord is a central component of T12
L1
the pathways that transmit information to and Lumbo- L1
from the brain and periphery. Structures sacral L2
within the spinal cord have many functions enlarge- L2
ment Lumbar
that serve this purpose. The posterior column L3 L3
receives ipsilateral axons to convey informa- roots
Cauda
tion of proprioception, vibration, and light L4
L4
equina
touch sensation to the brain. Within the poste-
L5
rior column, the gracile fasciculus transmits L5
information from the lower trunk and legs,
and the cuneate fasciculus transmits informa- S1
S
tion from the upper trunk, arms, and neck. S2 Sacral
The anterolateral pathways receive contralat- S3 and
eral axons two to three spinal segments coccygeal
superior to convey information of pain and S4 roots
temperature sensation to the brain S5
The PNS is comprised of lower motor neu- CO1
rons (LMNs), peripheral nerves (including
cranial nerves), spinal nerve roots, and the au- Anterolateral view
tonomic nervous system. LMNs have cell bod- FIGURE 25-5 The spinal cord, composed of the
ies located within the brainstem and anterior cervical, thoracic, lumbar, and sacral cord. The spinal
horns of the spinal cord. cord terminates at the conus medullaris (T12–L1
There are 12 pairs of cranial nerves (CNs) level). Traversing lumbosacral roots continue within
(Table 25-3). CN I, the olfactory tract, trav- the spinal canal in the cauda equina.
erses the surface of the frontal lobes (Fig. 25-6).
CN II, the optic nerve, comes together at the CN V exits the pons, CN VI the ponto-
optic chiasm, forming the optic tracts that medullary junction, and CNs VII tkhrough X
enter the thalamus. The remainder of the cra- the pontomedullary junction and medulla, in-
nial nerves are located within and exit from the feriorly in that respective order. The cerebello-
brainstem. CNs III and IV exit the midbrain, pontine angle is the exit point for CNs VII, VIII,
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500 Chapter 25 Foundations of Neurological Differential Diagnosis


FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

TABLE 25-3 ■ Cranial Nerves and Functions


NUMBER NAME FUNCTION
I Olfactory nerve Sense of smell
II Optic nerve Visual acuity, color vision, visual fields, papillary
responses
III Oculomotor nerve Smooth pursuit, convergence
IV Trochlear nerve Smooth pursuit, convergence
V Trigeminal nerve Facial sensation, muscles of mastication
VI Abducens nerve Smooth pursuit, convergence
VII Facial nerve Facial expression, taste
VIII Vestibulocochlear nerve Vestibular and hearing sensation, vestibulo-ocular
reflex
IX Glossopharyngeal nerve Palate elevation and gag reflex
X Vagus nerve Palate elevation and gag reflex, visceral innervation
XI Spinal accessory nerve Cervical rotation, side-bending, flexion, and shoulder
elevation
XII Hypoglossal nerve Tongue movements

and IX. CN XI is located within the cervical The brachial plexus receives sensory informa-
spinal cord, and CN XII exits the medulla. tion from and provides a conduit for motor in-
The spinal cord gives rise to nerve roots at nervation to the upper extremities. The brachial
each level bilaterally (see Fig. 25-6). There are plexus forms from the roots of C5–T1 spinal
8 cervical (C1–C8), 12 thoracic (T1–T12), 5 levels to then merge to become the superior,
lumbar (L1–L5), 5 sacral (S1–S5) and 1 coc- middle, and inferior trunks. Each trunk then di-
cygeal (Co1) pairs of nerve roots. Nerve roots vides in two to form anterior and posterior divi-
exit each segment of the spinal cord through sions. These six divisions then merge to become
the intervertebral foramina to then merge into the posterior, lateral, and medial cords. Branches
peripheral nerves that traverse the upper ex- of these cords (in addition to a few exceptions of
tremities, trunk, and lower extremities. Two branches from previous structures within the
major plexuses are formed by the nerve roots plexus) make up the peripheral nerves, which are
and peripheral nerves: the brachial plexus, the dorsal scapular, long thoracic, subclavius,
which supplies the upper extremities, and the suprascapular, lateral pectoral, musculocuta-
lumbosacral plexus, which supplies the lower neous, median, upper subscapular, thoracodor-
extremities. sal, lower subscapular, axillary, radial, medial
The spinal cord terminates as the conus pectoral, medial cutaneous of the arm, medial
medullaris at the level of L1 or L2. At or around cutaneous of the forearm, and ulnar nerves. An
this level, nerve roots converge to become the analogy to this structure is found in the lower ex-
cauda equina. Both the conus medullaris and tremities in the lumbosacral plexus, which re-
cauda equina are considered parts of the PNS. ceives sensory information from and provides
The conus medullaris is the caudal end of the motor innervation to the legs. The lumbosacral
spinal cord where lumbar, sacral, and coccygeal plexus forms from the 12 thoracic, 5 lumbar,
nerve roots begin to form. The cauda equina is 5 sacral, and 1 coccygeal spinal nerves. The
a collection of L1–S2 nerve roots that receive branches of these spinal nerves form the periph-
sensory information and provide motor inner- eral nerves, which include the iliohypogastric,
vation to the legs; they also control bowel and ilioinguinal, genitofemoral, lateral femoral
bladder function. cutaneous, obturator, femoral, superior gluteal,
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Chapter 25 Foundations of Neurological Differential Diagnosis 501

FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


Superior

Right Left

Inferior

Olfactory
CNI Olfactory bulb
nerve
CNII Optic
nerve
Rostral
CNIII
Oculomotor
nerve
CNIV Thalamus
Trochlear Optic chiasm
nerve
CNV
Trigeminal Mandibular
nerve Midbrain
nerve

CNVI Abducens
nerve Maxillary
nerve Pons
CNVII
Facial
nerve

CNVIII
Vestibulo- Medulla
cochlear
nerve
CNIX
Glosso- Spinal
pharyngeal cord
nerve
CNX
Vagus Caudal
CNXI
nerve Spinal CNXII
accessory Hypoglossal
nerve nerve
FIGURE 25-6 Twelve cranial nerves exit from the midbrain, pons, and brainstem.

inferior gluteal, posterior cutaneous femoral, by the same spinal nerve. Dysfunction in
sciatic, common fibular, tibial, pudendal, and dermatomal distributions, myotomal distribu-
coccygeal nerves. tions, or both indicates pathology that causes a
Dermatomes are an area of skin that is spinal nerve root lesion.
primarily supplied by a single spinal nerve. The autonomic nervous system (ANS)
Myotomes are a group of muscles innervated is comprised of the sympathetic and
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502 Chapter 25 Foundations of Neurological Differential Diagnosis

parasympathetic divisions. The sympathetic Sensory Abnormalities1


FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS

division arises from T1–L2, and the parasym- ● Joint position sense, vibration, and fine,
pathetic from the cranial nerves and S2–S4. discriminative touch:
The ANS primarily controls consciousness and ● Peripheral nervous system: sensory recep-
visceral functions. Its sympathetic division ele- tors, ascending peripheral nerves, spinal
vates blood pressure and heart rate, causes nerves, trigeminal nerve
bronchodilation, and increases pupil size, ● Central nervous system: dorsal column,
whereas its parasympathetic division decreases medial lemniscus of spinal cord, brain-
heart rate and pupil size and increases gastric stem, thalamus, primary somatosensory
secretions. cortex
● Pain, temperature, and crude touch:
Neurological Impairments and
● Peripheral nervous system: sensory recep-
Associated Anatomical Regions
tors, ascending peripheral nerves, spinal
It is rare that an individual with a neurologi- nerves, trigeminal nerve (CN V)
cal diagnosis would report a problem that is ● Central nervous system: anterolateral
at the impairment level. Individuals typically pathway of spinal cord, brainstem, thala-
report the problems they are having with mus, primary somatosensory cortex
activities, such as standing up from a seated ● Visual acuity and fields:
position, walking, or reaching for an object. ● Peripheral nervous system: visual recep-
Movement analysis of these identified func- tors, optic nerves (CN II)
tional activities points to the appropriate tests ● Central nervous system: optic chiasm,
for impairments. Once specific impairments thalamus, visual cortex, occipital lobe
have been identified, the possible source list ● Smell:
of neuroanatomical regions becomes key in ● Peripheral nervous system: olfactory
the diagnostic process. This approach is par- receptors, olfactory nerves (CN I)
ticularly important for individuals who do ● Central nervous system: olfactory tracts,
not have a known neurological diagnosis, olfactory cortex, temporal lobe
such as those individuals described at the ● Taste:
beginning of this chapter. ● Peripheral nervous system: facial (CN VII)
The following section provides examples and glossopharyngeal (CN IX) nerves
of how neurological health conditions may be ● Central nervous system: medulla, thala-
considered in the differential diagnostic mus, taste cortex, insula
process, organized by neurological impair-
ments commonly seen in physical therapy Cognition1
settings. These include imbalance, sensory ● Memory: medial temporal lobes, hip-
abnormalities, cognition, abnormal move- pocampus, amygdala
ment, stiffness, and weakness. A thorough list ● Language: dominant hemisphere (usually
of neuroanatomical regions is provided for the left)
each of the impairments, and the clinician ● Expressive aphasia: Broca’s area (frontal
must follow up by narrowing down the hypo- lobe)
thetical source list to the regions most likely ● Receptive aphasia: Wernicke’s area (tem-
affected. poral lobe)
Imbalance5 ● Attention: frontal lobe
● Peripheral nervous system: inner ear ● Perseveration: frontal lobe
(membranous and bony labyrinths, hair ● Impulsivity: frontal lobe
cells), sensory receptors, ascending periph-
eral nerves, spinal nerves, vestibulocochlear Abnormal Movement
nerve (CN VIII) ● Tremor: cerebellum, basal ganglia
● Central nervous system: spinal cord, brain- ● Incoordination: spinocerebellar pathways,
stem, cerebellum, thalamus, basal ganglia cerebellum, basal ganglia
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Chapter 25 Foundations of Neurological Differential Diagnosis 503

Stiffness1 Conclusion

FOUNDATIONS OF NEUROLOGICAL DIFFERENTIAL DIAGNOSIS


● Spasticity: descending motor pathways at
the cortical, brainstem, or spinal cord levels The purpose of this chapter was to outline the
● Rigidity: basal ganglia
foundations of neurological differential diagno-
sis. To review, the process of neurological differ-
● Hypertonicity: descending motor pathways,
ential diagnosis entails formation of a “data-
especially the reticulospinal and corticospinal
base” of hypothetical causes for disablement;
● Hypotonicity: cerebellum, descending chart review; focused subjective examination to
motor pathways, and spinal cord in the determine the chief concern and help to discern
acute stage of injury the type of pathology that may be responsible
Weakness for causing the chief concern; objective exami-
● Upper extremity: nation, including focused testing that can rule
● Peripheral nervous system: spinal nerves, more or less likely involvement of pathology
brachial plexus, peripheral nerves affecting various neuroanatomical structures;
● Central nervous system: upper extremity
identification of neurological impairments and
region of motor homunculus, primary associated neuroanatomical regions; and syn-
motor cortex, descending motor path- thesis of information to determine the appro-
ways, brainstem, cervical spinal cord priate disposition.
● Lower extremity:
References
● Peripheral nervous system: spinal nerves,
1. Blumenfeld H. Neuroanatomy Through Clinical Cases.
lumbosacral plexus, cauda equina, conus Sunderland, MA: Sinauer Associates; 2002.
medullaris, peripheral nerves 2. Sullivan KJ, Hershberg J, Howard R, Fisher BE. Neuro-
● Central nervous system: lower extremity logic differential diagnosis for physical therapy. J Neurol
Phys Ther. 2004;28:162–168.
region of motor homunculus, primary 3. Victor M, Ropper A. Adams and Victor’s Principles of
motor cortex, descending motor path- Neurology. 7th ed. New York, NY: McGraw-Hill; 2001.
ways, brainstem 4. Fisher B, Yakura J. Movement analysis: A different
● Trunk: perspective. Orthop Phys Ther Clin North Am.
1993;2:1–24.
● Peripheral nervous system: spinal nerves,
5. Herdman SJ. Vestibular Rehabilitation. 3rd ed. Philadelphia,
peripheral nerves PA: F. A. Davis; 2007.
● Central nervous system: trunk region
of motor homunculus, primary motor
cortex, descending motor pathways,
brainstem, thoracic spinal cord
● Face:
● Peripheral nervous system: facial nerve
(CN VII)
● Central nervous system: face region of
motor homunculus, primary motor cor-
tex, corticobulbar pathways.
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CHAPTER26
Cardiovascular and Pulmonary Clues
from Examination
■ Jesus F. Dominguez, PT, PhD

IN THIS CHAPTER: Tonal Quality of Sound Transmitted Through the


■ Rationale for diagnosis of cardiac and pul- Thorax Provides Information Regarding the
monary pathologies by physical therapists Composition of the Lungs 511
■ Diagnostic relevance of the clinical Vital Signs Provide Direct Information
examination for cardiac and pulmonary Regarding Various Aspects of
pathologies Cardiovascular and Pulmonary
■ Interpretation of common examination Functioning 512
findings with respect to pathology affecting Heart Rate Indicates the Cardiovascular
the cardiovascular and pulmonary systems System’s Response to Different Levels of
Activity 513
OUTLINE Pulse Rhythm Suggests the Status of Electrical
Introduction 504 Conduction and Cardiac Output 513
General Observation is an Important Pulse Quality Provides Information Regarding
Initial Basis for Diagnosing the Status of Contractile Force and Cardiac
Cardiovascular and Pulmonary Output 514
Pathologies 505 Blood Pressure Responds to Changes in
Assessment of Skin Provides Clues Position and Activity in a Predictable Fashion
Regarding the Status of Cardiac and Provides Important Clues to Organ
and Pulmonary Function 506 Perfusion 515
Color May Indicate the Presence of Impaired Respiratory Rate Suggests the Status of Ventilatory
Blood Oxygenation 506 Mechanics and Blood Oxygenation 518
Surface Temperature Reflects the Rate of Heart and Lung Sounds Reveal Clues
Underlying Tissue Metabolism 508 About Cardiac and Pulmonary
Mobility and Turgor Suggest the Status of Nutrition, Pathology 519
Hydration, and Vascular Competence 508 Cardiac Auscultation 519
Lesions and Marks can Provide Clues to Pulmonary Auscultation 522
the Presence of Important Medical Conclusion 524
Comorbidities 508
Examination of the Thorax Provides
Diagnostic Information Regarding Introduction
the Cardiovascular and Pulmonary
Physical therapy is becoming a more common
Systems 509
route of first entry into the health care delivery
Configuration of the Chest Wall May Indicate
system. This development necessitates that
the Presence of Chronic Pulmonary
physical therapists quickly and accurately rec-
Pathology 509
ognize the pathology underlying patients’ clin-
Chest Wall Excursion Reflects the Status
ical presentations, especially those pathologies
of Ventilatory Mechanics 509
that require referral to other health care
Palpation of the Thorax Provides Information
providers. Of particular importance is the ap-
Regarding the Physical Composition
preciation of cardiovascular and pulmonary
and Orientation of Thoracic Organs 510
disorders that may present in patients who are

504
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 505

being seen for unrelated musculoskeletal or chronic obstructive pulmonary disease may
neuromuscular dysfunction. also avoid tasks that require the arms to be held
Most patients who experience acute cardio- at or above shoulder level because of the altered
vascular or pulmonary symptoms do not ini- ventilatory mechanics and increased energy
tially seek the services of a physical therapist. cost associated with overhead activities.1
Patients may mention these symptoms to a The effects of gravity on the distribution of
physical therapist or manifest signs of compro- pulmonary edema secondary to chronic con-
mised cardiovascular or pulmonary function gestive heart failure may lead some patients to
during the course of treatment for another dis- assume a semi-Fowler’s position (the head of
order. In such situations, the physical therapist the bed is elevated and the knees may be
should establish the patient’s cardiovascular and slightly elevated) during bedtime and they will
pulmonary status quickly by means of a thor- state that they require one or several pillows
ough line of questioning, evaluation of current under their head and shoulders to prevent the
signs and symptoms, and application of appro- shortness of breath associated with lying flat
priate definitive assessment tools. on their back. Once in this position, the pul-
During this process, the physical therapist monary edema is preferentially distributed to
must decide on the appropriate course of action the lung bases and the patient is afforded some
to ensure the health and safety of the patient in a relief from dyspnea. This condition is called
timely manner. Depending on the patient’s med- orthopnea. The therapist may rate the degree
ical stability, this can include expeditious referral of cardiopulmonary compromise by the num-
to the patient’s primary care physician, direct ber of pillows used by the patient. For exam-
contact with the primary care provider while ple, “two-pillow orthopnea” occurs when the
the patient is still in the presence of the physical patient uses two pillows to achieve relief.
therapist, administration of basic cardiopul- The patient’s general appearance can often
monary life support techniques, or activation of suggest the presence of latent or undiagnosed
the emergency medical services system. cardiopulmonary disorders.2 The following
This chapter is not intended to present a physical characteristics represent clues to pri-
comprehensive cardiovascular and pulmonary mary disorders that have secondary cardiopul-
examination sequence; rather, it provides an monary implications. Anasarca, which is
overview of how observation and fundamental edema or general accumulation of serous fluid
evaluation tools contribute to the diagnostic in various tissues of the body, may result from
process with respect to cardiovascular and congestive heart failure, cirrhosis of the liver,
pulmonary pathology. renal failure, or hypoalbuminemia. A tall and
thin frame with long extremities, pes cavus,
General Observation is an genu valgum, pectus excavatum or carinatum,
Important Initial Basis for and flushed cheeks suggest a patient with ho-
Diagnosing Cardiovascular mocystinuria and a corresponding predisposi-
and Pulmonary Pathologies tion toward increased thrombus formation
and embolic incidents. Patients with large neck
Patients with various cardiovascular and circumferences who are morbidly obese and
pulmonary diseases commonly adopt recog- appear somnolent may have obstructive sleep
nizable body positions that should prompt the apnea and associated sequelae that include sys-
therapist to examine further the pathological temic hypertension and cor pulmonale.
reasons behind these preferences. Individuals Individuals with Duchenne muscular
with chronic obstructive pulmonary disease dystrophy present with accentuated lumbar
often assume the “professorial position,” which lordosis, a waddling gait, and pseudohyper-
consists of leaning forward with hands or trophy of the calves. Duchenne muscular
elbows resting on knees or another stationary dystrophy is associated with hypertrophic
object. This strategy helps to stabilize the cardiomyopathy and respiratory failure as
shoulder girdle so that the accessory muscles of the disease progresses. Young children who
ventilation can function more optimally in ele- present with cyanosis, fatigue easily, and
vating the clavicle and upper ribs. Patients with exhibit a preferential squatting posture are
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506 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

likely to have tetralogy of Fallot. This posture


serves as a compensatory strategy by increas-
ing peripheral and aortic pressure so that the
right-to-left shunt through the ventricular 3-5 cm normal
septal defect is lessened and more blood enters
the pulmonary circulation to be oxygenated.
Adiposity in the abdomen and upper torso,
peripheral muscle atrophy and weakness, alope-
cia in women, the presence of a round, plump
face, and a fat deposit in the back of the neck and Sternal
upper shoulders are highly suggestive of Cush- angle of
ing’s syndrome. Associated findings include hy- Louis
pertension and elevated blood sugar levels in the
diabetic range. Acromegaly leads to enlargement
of the hands, feet, and nasal bone with protru-
sion of the lower jaw and brow. Patients with this FIGURE 26-1 Assessment of jugular venous distension.
condition typically develop systemic hyperten-
sion and cardiomyopathy, and often succumb to pneumothorax). A corneal arcus (thin, grayish
lethal dysrhythmias. Women with Turner’s syn- white or yellowish arc around the outer edges of
drome present with a webbed neck, short stature, the cornea) in young people suggests, but does
low nuchal hairline, and edema of the lower ex- not confirm, hyperlipoproteinemia, and indi-
tremities. This condition may be associated with viduals with dental caries and advanced gingivi-
hypertension or coarctation of the aorta. tis are at risk for bacterial endocarditis and
Careful inspection and palpation of the head cardiac valvular lesions. Jugular venous disten-
and neck areas during the course of the initial tion (JVD) is a manifestation of heart failure,
interview and subsequent physical exam also cardiac tamponade, or constrictive pericarditis.
can reveal important clues regarding underlying JVD is measured while the patient is semire-
cardiac or pulmonary pathology. For example, cumbent with the upper torso at a 45-degree
patients with myxedema (cutaneous and dermal angle (Fig. 26-1). A ruler is used to measure the
edema secondary to deposition of connective vertical distance between the sternal angle
tissues often associated with severe hypothy- (angle of Louis) and the highest point of jugular
roidism) present with a dull and puffy facies, venous pulsation. A value greater than 3 to 5 cm
significant periorbital edema, course hair, and above the sternal angle is considered the criteria
dry skin. In contrast, patients with hyperthy- for elevated jugular venous pressure.
roidism exhibit exophthalmos, palpitations, ex-
cessive sweating, and enlargement of the thyroid Assessment of Skin Provides Clues
gland (goiter), which is noted by both visual Regarding the Status of Cardiac
inspection and palpation. Hypertrophy of the and Pulmonary Function
accessory muscles of ventilation (ie, the scalenes
and sternocleidomastoids), excessive upper rib Observation of the patient’s skin also provides
cage and clavicular movement, and nasal flaring important clues regarding potential underly-
suggest the presence of chronic obstructive pul- ing cardiovascular or pulmonary pathology.
monary disease as the patient attempts to ex- When assessing the patient’s skin for signs of
pand an already hyperinflated thoracic cage. cardiovascular and/or pulmonary involve-
These same patients may exhibit pursed-lip ment, the physical therapist should evaluate
breathing and prolonged expiratory time in color, temperature, mobility, and turgor and
attempts to maintain airway patency and maxi- also identify the presence of lesions or marks.
mize expired air volume.
Color May Indicate the Presence
Deviation of the trachea from the midline
of Impaired Blood Oxygenation
may indicate the presence of a tumor, mediasti-
nal shift, or pneumothorax (deviation toward a Changes in skin color can be evaluated by
simple pneumothorax and away from a tension examining the lips, the mucous membranes
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 507

including the oral mucosa, and the periph- of a parrot). A cardinal feature of digital
eral extremities including the nailbeds. clubbing is a positive Schamroth sign and it
Cyanosis is a bluish or purplish discol- is often used to confirm the observation. The
oration of the skin and mucous membranes sign is observed by having the patient place
and may be better appreciated in the perioral the dorsal surfaces of two opposing fingers
area and nailbeds. Cyanosis can result from (usually the thumbs) together. Normally, a
desaturating disorders that include conges- diamond-shaped window is formed between
tive heart failure with pulmonary edema and the distal interphalangeal (DIP) joints and
chronic obstructive pulmonary disease. the tips of the nails. A positive Schamroth
Cyanosis is also caused by impaired blood sign is associated with obliteration of the
flow associated with peripheral arterial dis- window as the distal fingertips approximate
ease. These conditions lead to oxygen desatu- one another (Fig. 26-2). Digital clubbing is
ration, which causes the bluish appearance usually associated with such cardiovascular
characteristic of cyanotic tissue. In the case of and pulmonary diseases as congenital cyanotic
impaired flow, an excess amount of oxygen is heart disease, endocarditis, atrial myxoma,
removed from the circulation and the venous idiopathic pulmonary fibrosis, chronic lung
blood is further desaturated. Hypoxemia that infections, malignancy, and interstitial pul-
is associated with cyanosis may suggest the monary diseases.
presence of various congenital heart defects Once cyanosis is identified and hypoxemia
producing right-to-left shunts, such as tetral- is suspected, further evaluation is warranted by
ogy of Fallot, ventricular septal defect, and means of pulse oximetry to assess hemoglobin
patent ductus arteriosus, or abnormalities in oxygen saturation. Cyanosis with associated
hemoglobin structure, including methemo- rapid heart and respiratory rates and altered
globinemia and sulfhemoglobinemia.3
Cyanosis can be classified as central or
peripheral, depending on the etiology. Periph-
eral cyanosis is localized only to the extremi-
ties and nailbeds, suggesting compromised
blood flow through the arterial tree. Anxiety
or cold weather–induced arterial vasoconstric-
tion can also present as transient peripheral
cyanosis. Central cyanosis is best appreciated
in the lips, oral mucosa, and tongue. It sug-
gests a primary cardiovascular or pulmonary
etiology. It is generally accepted that central A
cyanosis associated with hypoxemia becomes
evident only when there are 5 g/dL of unsat-
urated hemoglobin in the capillaries, and
that patients with normal hemoglobin levels
manifest cyanosis at higher partial pressures
of oxygen and higher hemoglobin saturation
levels than patients with lower hemoglobin
values.4 It is likely that patients with low
hemoglobin levels will demonstrate signs of
acute hypoxemia well before cyanosis be-
comes evident.
B
In addition to cyanosis, patients who are
chronically hypoxemic often present with FIGURE 26-2 Schamroth sign: (A) normal appear-
digital clubbing (a condition in which the ance of diamond-shaped window between DIP joints
distal segments of the fingers and toes be- and tips of nails (negative Schamroth sign); (B) pos-
come enlarged) and the nailbeds take on a itive Schamroth sign (obliteration of diamond-shaped
more convex appearance (similar to the beak window).
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508 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

mental status are signs that emergency medical of the pitting within 30 to 40 seconds is typi-
intervention is required. cally associated with low plasma albumin levels,
and the persistence of pitting beyond 1 minute
Surface Temperature Reflects favors increased vascular hydrostatic pressure
the Rate of Underlying Tissue as the contributing source. The latter is termed
Metabolism congestive pitting edema and may be due
Physical therapists can make a general assess- to such conditions as congestive heart failure,
ment of skin temperature using the dorsum hypertension, medication-induced vasodila-
of the hand and fingers. Warmth noted over tion, or venous obstruction. In cases where
an area of localized tenderness, erythema, edema is accompanied by rapid and signifi-
and swelling may indicate the presence of ve- cant weight gain, such as an increase of 2 to
nous obstruction such as deep venous throm- 3 pounds overnight, the patient should be
bosis or cellulitis, whereas extremities that referred to his or her medical practitioner for
appear cold to the touch are often associated further workup, because this finding is sugges-
with arterial insufficiency. This latter finding tive of acute fluid retention and heart failure.
can be associated with the presence of dimin- The severity of edema has traditionally
ished pulses. On auscultation, a harsh, vibra- been assessed on a 0 to 4+ scale, although con-
tory noise secondary to interruption of nor- siderable variation in opinion exists between
mal laminar flow through a vessel may be the precise depth of tissue depression for each
appreciated. This noise is called a bruit. If the value.5 Alternative methods include taking cir-
arterial obstruction or occlusion is severe, cumferential or figure-8 girth measurements
a palpable vibration or thrill that may also at several sites along the limb or recording vol-
be felt with the palmar aspect of the hand. umetric water displacement by immersing the
Generalized warmth is often associated with involved limb in a container filled with water.6
fever or hyperthyroidism, whereas patients When utilizing these methods, the therapist
with hypothyroidism exhibit generalized skin should remember to record the precise land-
coolness. marks used for measurements so that subse-
quent assessments will more accurately track
Mobility and Turgor Suggest true changes in limb girths and volumes.
the Status of Nutrition, Hydration,
and Vascular Competence Lesions and Marks can Provide
Clues to the Presence of Important
Evaluation of skin mobility and turgor can be
Medical Comorbidities
accomplished by gently lifting up a fold
of skin in the distal extremities and observing The presence of certain skin lesions or marks
how quickly it returns to its place. Persistence may at times alert the physical therapist to
of elevated skinfolds after they are released in- the possibility of underlying cardiovascular
dicates decreased turgor. This finding usually and pulmonary disorders in their patients.
occurs in patients with dehydration or malnu- For example, xanthomas, which are raised,
trition. Edema is characterized by reduced yellowish, waxy-appearing skin lesions due
tissue mobility secondary to extravascular to deposits of fatty material under the sur-
congestion. It can be classified as nonpitting face of the skin, are often associated with an
or pitting, with the anterior tibia being a use- increase in blood lipid levels. These lesions
ful site for distinguishing between the two appear most frequently on the elbows,
forms. The therapist presses on the skin over a knees, hands, feet, or buttocks. Underlying
bony area for 10 seconds using three or four metabolic disorders often linked with the
fingers and looks or feels for hills and valleys presence of xanthomas include diabetes, bil-
(depressions and ridges) after the pressure is iary cirrhosis, and familial hypercholes-
released. If the depressions made by the fin- terolemia. Xanthomas can also appear on
gers resolve immediately, the edema is nonpit- the eyelids and are termed xanthelasma
ting. If however, the depressions persist, the palpebra. A butterfly-shaped rash that covers
edema is considered to be pitting. Resolution both cheeks and often crosses the bridge of
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 509

the nose, or malar rash, is often observed in pulmonary hyperinflation secondary to


patients with systemic lupus erythematosus. chronic obstructive pulmonary disease.
Patients with this condition can develop A patient with traumatic flail chest sec-
pericarditis or pleurisy some time during the ondary to rib fractures will present with
progression of the disease. Vitiligo, which depression of the involved area during inhala-
refers to white patches of skin appearing tion and protrusion during expiration, which
on different parts of the body secondary to is an example of a paradoxical breathing pat-
destruction of melanocytes, may present in tern. Severe deformities of the chest wall and
patients with hyperthyroidism. thoracic spine may impair ventilatory me-
chanics. For example, patients with moderate
Examination of the Thorax to severe scoliosis or kyphoscoliosis may man-
Provides Diagnostic Information ifest symptoms of hypoinflation, restrictive
Regarding the Cardiovascular and lung disease, or cor pulmonale secondary to
Pulmonary Systems excessive pulmonary vascular resistance if
chest wall mechanics are significantly com-
Examination of the chest yields useful infor- promised. In patients who are morbidly obese
mation about the function and integrity of the (especially those with central adiposity), the
cardiopulmonary system and provides clues to increased abdominal mass may impair the de-
the presence of pathology. When evaluating scent of the diaphragm and lead to restrictive
the chest wall, the therapist should routinely lung dysfunction. Visible pulsations of the tis-
observe the general contours of the thoracic sue overlying the thorax or abdomen in symp-
cage from an anteroposterior (AP) and lateral tomatic patients are often suggestive of large
perspective and assess for obvious asymme- vessel pathology (ie, aneurysm or dissection)
tries between the left and right sides. Examina- and constitute a medical emergency. In most
tion of chest wall excursion, together with cases, there is a palpable mass beneath the area
breathing rate, rhythm, and depth, should be of pulsation and a bruit is commonly heard
noted in order to assess ventilatory pump upon auscultation with a stethoscope.
function and identify potential impairments
in oxygenation capacity. Palpation, percussion, Chest Wall Excursion Reflects the
and auscultation may often be required to ap- Status of Ventilatory Mechanics
preciate the origin of identified impairments
During the initiation of inspiration, the di-
more fully. Examination of the thorax should
aphragm descends and the rib cage expands
include assessment of the chest wall configura-
by way of contraction of the parasternals and
tion and excursion, chest palpation, ausculta-
scalenes to generate the negative intrapleural
tion, and performance of mediate percussion.
pressure necessary for lung expansion. The
descent of the diaphragm also compresses
Configuration of the Chest Wall
the abdominal contents, causing the abdom-
May Indicate the Presence
inal wall to protrude slightly. Normally, the
of Chronic Pulmonary Pathology
chest and abdominal walls rise to a similar
The normal circumferential pattern of the extent during inspiration, although thoracic
chest wall is elliptical with an AP-to-lateral excursion is less obvious when the patient is
diameter ratio of 1:2. Additionally, the ribs in the supine position. The therapist may
typically form an angle of 45 degrees below choose to evaluate chest wall expansion in
horizontal where they articulate with the ver- any patient who presents with symptoms of
tebral bodies. AP-to-lateral diameter ratios potential cardiac or pulmonary pathology,
greater than 1:2 and rib angles that approach but overt abnormalities in chest wall config-
the horizontal result in the thoracic cage uration warrant special attention to thoracic
taking on a more rounded contour that mobility.
adversely affects chest wall dynamics. The Several techniques are available for evalu-
term barrel chest is used to describe this ating the extent and quality of chest wall ex-
observation and is usually associated with pansion. One method is to place the thumbs
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510 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

on the posterior aspect of the patient’s thorax The magnitude of chest expansion and
at the midline and the fingers in parallel ori- symmetry of movement can provide the thera-
entation to the rib cage approximately at the pist with clues as to the presence of cardiopul-
level of the 10th rib. The thumbs are then monary dysfunction. For example, a patient
brought close together so that a fold of skin is with lobar pneumonia, hemi-diaphragm paral-
formed at the midline of the patient’s back. As ysis secondary to phrenic nerve injury during
the patient is instructed to breathe in deeply, cardiac bypass surgery, or a thoracic surgical
the hands are allowed to travel with the move- incision may present with diminished excur-
ment of the rib cage (Fig. 26-3). The same pro- sion on the involved side, whereas a patient
cedure can be utilized to evaluate motion in with chronic obstructive pulmonary disease
the midthorax, with thumbs placed at the level may demonstrate a symmetric decrease in
of the xiphoid process and fingers parallel with chest wall motion.
the rib cage, and upper thorax, with thumbs Globally diminished chest wall excursion
placed at the level of the suprasternal notch may be observed in patients with ankylosing
and fingers perpendicular to the clavicles. spondylitis as the disease progresses. Unilateral
An alternate method is to place a measur- lag usually indicates pathology of the underly-
ing tape around the upper, middle, and lower ing lung or pleura. Paradoxical breathing pat-
thorax and evaluate excursion as the patient terns may be noted in individuals with spinal
breathes deeply. The tape should be held cord injury and severe chronic obstructive
loosely so that chest movement is not re- pulmonary disease. In individuals with spinal
stricted. In general, the typical excursion in cord injury, the limited abdominal and inter-
most areas of the thoracic cage ranges from costal muscle control causes exaggerated ab-
3 to 10 cm.7 The therapist is reminded to dominal protrusion and intercostal retraction
measure chest wall excursion both during during inspiration. This is characterized as an
quiet breathing and with deep breathing to upper chest paradox with pronounced abdom-
evaluate possible restrictions in range of inal rise. Individuals with chronic obstructive
motion. Although excursion deficits should pulmonary disease typically present with ab-
be noted in all cases, the measurements dominal and lower rib cage retraction during
obtained are best suited for purposes of track- inspiration, while expansion of the upper
ing changes within a particular patient. chest is accentuated because of accessory mus-
cle overuse. This pattern is characterized as an
abdominal paradox with pronounced upper
chest rise. Retraction of the intercostal spaces
during inspiratory effort suggests the presence
of severe airway obstruction, which is typically
accompanied by a loud, clearly audible, high-
pitched wheezing sound called stridor. These
signs indicate that the patient is rapidly
decompensating and in need of immediate
medical attention.

Palpation of the Thorax Provides


Information Regarding the Physical
Composition and Orientation
of Thoracic Organs
Tactile fremitus is a normal phenomenon
resulting from transmission of vocal sounds
via the bronchopulmonary tree to the surface
of the thorax. The vibration of tactile fremi-
FIGURE 26-3 Evaluation of chest wall excursion. tus is best appreciated with the palmar
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 511

surface of the metacarpal heads or the with ventricular hypertrophy or dilation,


hypothenar eminence. The therapist in- aortic dilation or aneurysm, pulmonary
structs the patient to repeat the words artery enlargement, pneumothorax, or a
“one–two–three” or “ninety-nine” as both space-occupying tumor. An extreme right-
sides of the posterior thorax are palpated ward deviation of the apical impulse is asso-
simultaneously to compare intensity and ciated with the rare condition of dextrocar-
symmetry of the vibration. Because of the dia in which the heart is rotated 180 degrees
location of the scapulae and heart, as well as and is anatomically located in the right
the natural decay in transmission volume, margin of the mediastinum. Additional
tactile fremitus is most prominent in the investigation of these palpatory findings
interscapular regions, while the left side and should include cardiac auscultation.
posterior lung bases are associated with Masses observed on the abdominal or
decreased vibration. thoracic walls should be palpated to identify
With experience, the novice therapist the presence of thrills as the result of vascular
will be able to discern normal from abnormal obstruction or dilation. Some of the most
patterns. Because of the physical properties of common conditions associated with palpable
different media, sound volume is transmitted thrills are aortic aneurysms, aortic dissections,
more effectively through fluid as compared atherosclerosis, arteriovenous malformations,
to air. Consolidation of the underlying lung or arteriovenous fistulas. The therapist will of-
tissue secondary to fluid accumulation or ten appreciate a bruit when auscultating over
pneumonia enhances the intensity of tactile the area where the thrill is identified. This
fremitus. Conversely, other conditions that finding further supports the presence of vas-
cause impedance of sound transmission are cular pathology and warrants timely consulta-
associated with diminished tactile fremitus, tion with the patient’s physician.
such as bronchial obstruction, hyperinflation
associated with chronic obstructive pul-
Tonal Quality of Sound Transmitted
monary disease, pneumothorax, and obesity.
Through the Thorax Provides
The therapist should auscultate with a stetho-
Information Regarding the
scope over areas of altered vibration to assess
Composition of the Lungs
further for the presence of underlying pul-
monary involvement. The ability to appreciate the subtle, yet
Palpation over the precordium can yield distinct, sounds produced by percussion of
valuable information about the patient’s car- the chest wall and discriminate sound quality
diac status as well. The apical impulse refers that suggests the presence of air, fluid, or con-
to the point at which the apex of the heart solidation within the underlying tissues is
strikes the thoracic wall during ventricular vital to the emerging trend of autonomous
systole, whereas the point of maximal im- practice in physical therapy. The technique of
pulse is a term used to identify the specific mediate percussion begins with hyperexten-
location where the heart strikes the thoracic sion of the middle finger (referred to as the
wall with the greatest force. The apical im- pleximeter) of the nondominant hand and
pulse and the point of maximal impulse can placement of its distal interphalangeal joint
be grossly identified by palpation using the on the surface to be percussed. Care should
palmar aspect of the phalanges; finer local- be taken to keep the other four fingers raised
ization can then be accomplished using one away from the patient’s skin so as not to
finger. Normally, both occur at the fifth in- dampen the transmission of sound. The tip
tercostal space along the midclavicular line. of the middle finger of the dominant hand
At times, the apical impulse and the point of (referred to as the plexor) is used to strike the
maximal impulse are not coincident and the distal interphalangeal joint of the pleximeter
point of maximal impulse may be displaced sharply by allowing the wrist to flex freely
medially or laterally from the fifth inter- (Fig. 26-4). After the strike, the plexor is
costal space. This may occur in individuals quickly withdrawn.
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512 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

nature of pulmonary pathology is related to the


Point of tonal quality that results from mediate percus-
motion sion (Table 26-1).8

Vital Signs Provide Direct


Information Regarding Various
Aspects of Cardiovascular
and Pulmonary Functioning
The arterial pulse is produced when the left
ventricle contracts and ejects blood through
the aorta and out to the peripheral arterial
tree. Thus, the pulse is coincident with the
period of ventricular systole. The pulse is
measured at the wrist by palpating the radial
artery and counting the number of beats felt
in 10, 15, or 30 seconds and multiplying by 6,
4, or 2, respectively, to arrive at an average
heart rate (HR) in beats per minute (bpm). If
the pulse is noted to be irregular, as discussed
FIGURE 26-4 The technique of mediate percussion. later in this section, then the length of assess-
ment should be increased to 60 seconds in
The resulting sound is categorized according order to minimize error in estimation.
to its quality and should prompt the therapist to The therapist should routinely assess the
evaluate the area in question more thoroughly by pulse quality bilaterally to rule out asymmetries
employing more directed auscultatory tech- between limbs, which is suggestive of peripheral
niques (discussed later in this chapter). From a vascular compromise. It is also wise practice to
physical therapy perspective, the most likely can- assess all peripheral arteries in order to compare
didates for the application of mediate percussion pulse characteristics of the neck (carotid), up-
techniques are those patients who present with per extremities (ie, brachial and radial arteries),
pulmonary symptoms and in whom ausculta- and lower extremities (femoral, popliteal, pos-
tory findings warrant further evaluation. The terior tibial, and dorsalis pedis arteries). For

TABLE 26-1 ■ Characteristics of Percussion Notes and Associated Pathologies


RELATIVE
INTENSITY RELATIVE EXAMPLE OF EXAMPLE OF
PERCUSSION OR PITCH OR RELATIVE LOCATION FOR ASSOCIATED
NOTE LOUDNESS FREQUENCY DURATION COMPARISON CONDITION
Flatness Soft High Short Thigh Large pleural
effusion
Dullness Medium Medium Medium Liver Lobar pneumonia
Resonance Loud Low Long Normal lung Normal, chronic
bronchitis
Hyperresonance Very loud Lower Longer None normally Emphysema,
pneumothorax
Tympany Loud Higha a Gastric air bubble Large
or puffed-out pneumothorax
cheek
aDistinguished mainly by its musical timbre.
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 513

example, diminished strength or absence of


BOX 26-1 How is the Appropriateness
pulses in the left vs. right upper extremity or the
of Heart Rate Determined
lower vs. upper extremity is associated with for a Given Functional
coarctation of the aorta or advanced peripheral Activity?
vascular disease. Evaluation of the rate, rhythm,
and quality of the patient’s pulse can provide The chronotropic index (ratio of heart rate re-
the therapist with information regarding the serve to metabolic reserve) is often used in the
clinic to assess the appropriateness of the heart
status of the cardiac pump.
rate response to a particular workload. For a
given workload, the percentage of heart rate
Heart Rate Indicates (HR) reserve used is represented by the follow-
the Cardiovascular System’s ing equation:
Response to Different Levels
% HR reserve = [(HRstage – HRrest) /
of Activity (220 – age in years – HRrest)] ⫻ 100.
The normal resting heart rate lies within the Similarly, the percentage of metabolic reserve
range of 60 to 100 bpm. This is referred to as a used is represented by the following equation:
normal sinus rhythm. Patients may present
with a resting pulse that is less than 60 bpm % Metabolic reserve = [(METstage – METrest) /
(METpeak – METrest)] ⫻ 100,
(bradycardia) or greater than 100 bpm (tachy-
cardia) which may manifest symptoms of where MET refers to the metabolic equivalent
underperfusion, including dizziness, light- (ie, 1 MET = 3.5 mL O2/kg/min consumed at
headedness, diaphoresis, visual disturbance, rest) and METpeak refers to the peak oxygen
cognitive impairment, shortness of breath/ consumption during a maximal symptom-limited
chest discomfort, or syncope. However, resting exercise test.
heart rates that deviate from the normal range The chronotropic index is expressed as the ratio
do not always indicate the presence of pathol- between the % HR reserve and the % metabolic
ogy. Because of the potential for deficits in car- reserve. A ratio of <0.8 is considered to be a low
diac output, bradycardia or tachycardia must chronotropic index and suggestive of chronotropic
be evaluated within the context of the patient’s incompetence. An alternative determination is that
heart rate should increase by approximately 10 to
entire clinical picture.
15 bpm from resting values during normal walking
The therapist can further evaluate the car- speeds (ie, 2.0 to 3.0 mph). Chronotropic incom-
diac rate response to various perturbations, in- petence is highly suggestive of sympathetic auto-
cluding ventilatory maneuvers and positional nomic nervous system dysfunction and warrants
changes (Box 26-1). For example, some indi- referral to the appropriate health care practitioner.
viduals exhibit a variation in heart rate that is
influenced by the ventilatory cycle and is me-
diated by the respiratory centers in the
medulla. The heart rate is felt to increase as the to maintain left ventricular output. It has been
patient inhales and to decrease as the patient suggested that no change or a decrease in heart
exhales. This phenomenon is called respiratory rate when assuming a more vertical position is
sinus dysrhythmia and is considered to be a indicative of a compromised cardiovascular
normal variant in children and young adults, as system.9
well as highly conditioned athletes. Addition-
ally, asking an individual to move from the Pulse Rhythm Suggests the Status
supine to sitting or standing position renders of Electrical Conduction and Cardiac
the cardiovascular system more susceptible to Output
the influence of gravity. This maneuver induces
Heartbeat rhythm is described by one of the
temporary venous pooling in the lower extrem-
following three terms:
ities and would lead to a reduction in venous
return and cardiac output if it were not for 1. Regular,
the normal reflexive response of increasing the 2. Regularly irregular, or
heart rate in addition to vascular adjustments 3. Irregularly irregular.
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514 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

A regular rhythm implies that the cadence characteristics discussed next may also be
of the palpated pulse does not vary appreciably identified. They are typically appreciated best
from beat to beat and usually, although not at the carotid artery but can, on occasion, be
always, identifies normal sinus rhythm. A felt in other peripheral arteries.
rhythm that is regularly irregular refers to an
COLLAPSING PULSE
irregularity in the palpated pulse that occurs in
The pulse is noted to be strong initially and
a regular, repeating pattern. Examples of this
then disappears quickly. This finding is also
include normal sinus rhythm with premature
common in patients with severe aortic regur-
atrial contractions, premature junctional con-
gitation and is caused by a larger than normal
tractions, premature ventricular contractions,
stroke volume yielding a forceful contraction
and supraventricular tachycardias with a fixed
followed by rapid collapse of the pulse
atrial/ventricular block.
strength as blood regurgitates back into the left
Atrial fibrillation is perhaps the best exam-
ventricle. For this reason, the difference be-
ple of an irregularly irregular rhythm, and it is
tween systolic and diastolic blood pressures
characterized by erratic variations in the time
will be wide.
between successive beats. The pulse rhythm
varies from beat to beat and can alternate be- PULSUS BISFERIENS
tween fast and slow rates. An irregular rhythm In pulsus bisferiens (bis = two and feriere = to
should prompt the therapist to auscultate beat), the pulse is noted to have two distinct
the heart with a stethoscope while palpating the systolic peaks separated by a slight drop during
pulse to correlate cardiac contraction with the midsystole. The dip is primarily due to regur-
peripheral pulse. In some instances, premature gitation of blood back into the left ventricle
beats do not generate a peripheral pulse because during midsystole. This finding is common in
cardiac diastolic filling time is compromised. patients with significant aortic regurgitation
This is referred to as a pulse deficit. Irregular and hypertrophic cardiomyopathy. In the lat-
rhythms may affect cardiac output and should ter, the mechanism involves rapid ejection of
therefore be a consideration for referral to the blood in early systole, followed by a trough,
appropriate health care practitioner. culminating in a second ventricular ejection
wave (the second peak). Pulsus bisferiens is
Pulse Quality Provides Information also commonly associated with a wide pulse
Regarding the Status of Contractile pressure.
Force and Cardiac Output
PULSUS ALTERNANS
In general terms, the quality of the pulse In an otherwise healthy individual, the pulse
refers to its strength when palpated and to the strength may decrease slightly during inspi-
presence of associated findings such as palpa- ration as the result of pulmonary vascular
ble thrills. Physical therapists should consider engorgement associated with decreased in-
examining the brachial and carotid pulses in trapleural pressures. This, in turn, dimin-
the event of abnormal findings on radial ar- ishes return to the left heart and the pulse is
tery examination. The pulse can be character- noted to be softer because of the slightly
ized based on its strength (Table 26-2). The lower stroke volume. Although not commonly

TABLE 26-2 ■ Classification of Peripheral Pulses


GRADE PHYSICAL FINDING EXAMPLE OF ASSOCIATED CONDITION
0 Absent Arterial obstruction or cardiovascular collapse
1+ Weak and thready Hypovolemic or hypotensive
2+ Normal force Normal
3+ Full Elevated temperature (fever), anxiety, or exercise
4+ Bounding Hypervolemic or hypertensive
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 515

observed, this is considered to be a normal combine to cause the diminished pulse strength
phenomenon. However, significant variations and systolic blood pressure during this maneu-
in pulse strength timed with the breathing cy- ver and the magnitude of the drop is exagger-
cle typically indicate the presence of a patho- ated by disease states. Pulsus paradoxus is asso-
logical condition. The appreciation of a strong ciated with cardiopulmonary pathologies that
pulse alternating with a weak pulse in a cyclic include cardiac tamponade, constrictive peri-
pattern is referred to as pulsus alternans. carditis, acute myocardial infarction, pulmonary
If pulsus alternans is suspected, the patient is embolus, and severe asthma.
asked to hold his or her breath during mid-inspi-
BIGEMINAL PULSE
ration to avoid ventilatory cycle influence. The
The term used to describe a pulse in which the
physical therapist should then assess the pulse
beats seem to occur in pairs with a slight pause
strength for alternating weak and strong beats.
between coupled beats is referred to as pulsus
The systolic pressure is noted to alternate be-
bigeminus. The most likely cause of this finding
tween higher and lower values as well. It is
is premature contraction of the ventricles,
thought that the variation in pulse strength is a
which may be of ventricular or supraventricu-
manifestation of decreased myocardial contrac-
lar origin. Typically, the second beat is noted to
tility secondary to failure of certain myocytes
be weaker than the first, suggesting that the
to contract on alternate cardiac cycles. The de-
beat occurred prematurely and limited ventric-
creased myocardial contractility leads to de-
ular diastolic filling. Although the distinction is
creased stroke volume (weaker pulse). This re-
typically made on the basis of ECG criteria,
sults in increased end-diastolic volume prior to
longer pauses between paired beats suggests an
the next systole. Via the Frank-Starling mecha-
ectopic focus of ventricular origin.
nism, the next systole produces a more forceful
contraction (stronger pulse). The cycle then re- PALPABLE VIBRATION (THRILL)
peats itself. Pulsus alternans is almost always as- Normally, blood flowing through vessels does
sociated with severe left ventricular systolic dys- so in a streamlined fashion, a phenomenon re-
function. In some individuals, electrical ferred to as laminar flow. In peripheral arteries,
alternans (visible as fluctuations in QRS ampli- when there is an obstruction to flow or when
tude or electrical axis on ECG) is also present. flow velocities are enhanced, the resulting tur-
This is often observed in patients with severe bulence is appreciated as a palpable thrill, often
pericardial effusion and cardiac temponade. It is described as a vibration best felt with the pal-
thought to result from the heart “wobbling” back mar aspect of the metacarpals or hypothenar
and forth within the fluid-filled pericardial sac.10 eminence. The thrill is almost always associated
with a bruit when the vessel is auscultated with
PULSUS PARADOXUS
a stethoscope. Bruits in the peripheral vessels
The term pulsus paradoxus was coined by
are thus analogous to cardiac murmurs. As
Adolph Kussmaul in 1873 to describe an absence
mentioned previously, palpable thrills are ob-
of the palpated pulse and auscultated contrac-
served in patients with various conditions, in-
tion correlated to the ventilatory cycle.11 The
cluding peripheral vascular disease, cardiac
pulse strength diminishes slightly during inspi-
valvular stenosis or regurgitation, arteriove-
ration and returns again during expiration.
nous malformations, or arteriovenous fistulas.
More appropriately, the term is used to describe
an exaggerated fall (≥10 mm Hg) in systolic
Blood Pressure Responds
blood pressure during inspiration. During in-
to Changes in Position
spiration, venous return to the right heart is en-
and Activity in a Predictable
hanced and this causes a bowing of the interven-
Fashion and Provides Important
tricular septum toward the left ventricle,
Clues to Organ Perfusion
reducing the left ventricle’s dimensions during
diastole and limiting end-diastolic volume. Measurement of arterial pressure is a skill that
In addition, the reduction in intrapleural pres- every physical therapist can master with expe-
sure during inspiration favors pooling in the rience and diligence. This often requires little
pulmonary vasculature and further reduces left more effort than routinely measuring blood
ventricular end-diastolic volume. Both factors pressure (BP) in all patients coming into the
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516 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

clinic or being seen at the bedside. With the pa- is a better estimate of diastolic pressure in in-
tient seated quietly and his or her arm sup- fants and children. Table 26-3 represents the
ported by the therapist, a cuff is placed around most recent AHA recommendations for resting
the patient’s arm and inflated until the pressure BP values in adults and their classification.12
in the cuff exceeds the systolic blood pressure. Blood pressure measurements should be
The degree to which the cuff should be inflated routinely taken in both arms when seeing
is easily estimated by palpating the patient’s ra- the patient for the first time. It is common for
dial or brachial pulse as the pressure in the cuff BP to differ between right and left arms on the
increases. The therapist should note the point order of ≤10 mm Hg (although greater differ-
at which the pulse becomes absent; this is a ences have been recorded) with the higher
general approximation of the systolic blood reading frequently measured in the right arm.
pressure. The cuff is deflated and the patient is Clinical evidence suggests that the mortality
allowed to rest for 1 minute. The therapist then hazard (risk of all-cause death) increases sig-
inflates the cuff 20 to 30 mm Hg higher than nificantly for every 10 mm Hg difference in
the point where the pulse became absent and systolic BP between arms.13 As a matter of
begins listening to the Korotkoff sounds with a convention, the arm with the higher reading is
stethoscope as the bladder is deflated.12 used to monitor blood pressure and determine
To ensure inter- and intra-tester reliability the presence of hypertension. A difference of
and accurately monitor patient status, the thera- >10 mm Hg usually necessitates further inves-
pist should be familiar with the American Heart tigation, including assessment of lower ex-
Association (AHA) standardization guidelines tremity pressures. Depending on location,
for the measurement of blood pressure.12 coarctation of the aorta and aortic dissection
According to these guidelines, the systolic are associated with higher pressures in the
blood pressure is recorded when the therapist right vs. left arm, or higher pressures in the up-
first hears discernable tapping sounds as the per vs. lower extremities.
pressure in the cuff is released (coincident with The therapist can gather important infor-
the first Korotkoff sound). The cuff is allowed mation about a patient’s cardiovascular
to continue deflating and the diastolic pressure health by measuring blood pressure while the
is recorded when the sounds disappear alto- patient assumes different body positions.
gether (or at the point when they are last Blood pressure is measured in the supine po-
heard). Diastolic blood pressure corresponds to sition after the patient has been lying quietly
this fifth Korotkoff sound. In some cases (eg, in for 5 to 10 minutes and then immediately
infants and small children), the tapping sounds after the patient stands up. In most cases,
may be heard all the way down to the 0 mm Hg diastolic pressure is lower in the supine com-
mark on the dial. When this occurs, the dias- pared to the standing position. The increase
tolic pressure should be recorded when the in diastolic BP with standing reflects the
therapist hears the tapping sound muffle dis- effects of gravity on venous pooling that are
tinctly (corresponding to the fourth Korotkoff offset by an increase in peripheral vascular
sound). Most experts feel that the fifth Ko- tone. In contrast, systolic BP changes very
rotkoff sound (disappearance) more closely little during the transition from supine to
approximates true adult diastolic pressure, standing in otherwise healthy individuals (a
whereas the fourth Korotkoff sound (muffling) finding that is consistent with maintenance of

TABLE 26-3 ■ American Heart Association Classification for Adult Blood Pressure Values
CLASSIFICATION SYSTOLIC BP (mm HG) DIASTOLIC BP (mm HG)
Normal <120 <80
Prehypertension 120–139 80–89
Stage 1 hypertension 140–159 90–99
Stage 2 hypertension ≥160 ≥100
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 517

venous return supported by increased dias- efficiently when the patient stands because of
tolic pressure). A diastolic blood pressure that the reduced venous return.9
drops when the patient stands likely indicates As mentioned during the discussion of pulse
vascular incompetence or a dysfunctional au- assessment, blood pressure also demonstrates
tonomic nervous system (especially if there is variations timed with the ventilatory cycle.
no compensatory increase in heart rate). By Specifically, systolic blood pressure is lower dur-
definition, orthostatic hypotension refers to a ing inspiration because of the associated pul-
drop in systolic and/or diastolic BP when monary vascular engorgement and decreased
going from the supine position to sitting or left ventricular end-diastolic volume. The typi-
standing. The accepted criteria is a drop of cal difference in systolic pressure between inspi-
≥20 mm Hg in systolic pressure and/or a drop ration and expiration is on the order of ≤10 mm
of ≥10 mm Hg in diastolic pressure within Hg. Certain pathologies exaggerate the inspira-
3 minutes of standing up.14 Associated symp- tory drop in systolic blood pressure, including
toms can include dizziness, light-headedness, cardiac tamponade and constrictive pericarditis.
shortness of breath, chest discomfort, urinary When these conditions are suspected, the thera-
incontinence, and syncope. pist should check for the presence of pulsus
In some patients, an exaggerated reflex paradoxus. First, the therapist takes the patient’s
tachycardia (usually an increase in heart rate of blood pressure as usual. The therapist then asks
30 bpm) is noted as the sympathetic nervous the patient to inspire and hold their breath.
system attempts to compensate for the drop in Blood pressure is now taken while the patient
arterial pressure.15 The condition is termed continues to hold his or her breath and systolic
positional orthostatic tachycardia syndrome. pressure is noted. A drop of >10 mm Hg in sys-
Absence of a blood pressure increase in the tolic BP with inspiration confirms pulsus para-
presence of hypotension may imply a neuro- doxus.16 An alternate approach is to have the pa-
logical component to the disorder. tient breathe normally as the therapist takes the
Other factors to consider in the diagnosis blood pressure by deflating the cuff at a slightly
of orthostatic hypotension include the pa- slower rate. This will allow the therapist to note
tient’s neurological status, use of vasoactive when the systolic blood pressure is heard only
medications, prolonged bed rest, and hemor- during expiration and then when it is heard dur-
rhagic or hypovolemic states. Furthermore, ing both inspiration and expiration.
maintenance of (or an increase in) systolic Systolic BP demonstrates typical responses
blood pressure in the face of a lower diastolic to increasing physical workload, which can be
blood pressure in the standing position may altered in various disease states (Table 26-4).17,18
be an early sign of cardiac pump failure. In Because systolic BP is a function of cardiac
this example, the left ventricle operates more output and total peripheral resistance, the

TABLE 26-4 ■ Criteria for Normal and Abnormal Blood Pressure (BP) Responses to Graded
Exercise
DESCRIPTION CRITERIA EXAMPLE OF ASSOCIATED CONDITION
Normal Systolic BP: 7–12 mm Hg increase Normal BP response
per MET
Diastolic BP: ⫾10 mm Hg from
resting values throughout test
Hypertensive Systolic BP: ≥15–20 mm Hg Systemic hypertension, coronary artery disease,
increase per MET arteriosclerosis
Diastolic BP: ≥90 mm Hg
Blunted or flat Systolic BP: <5–7 mm Hg increase Overmedication, ventricular failure, coronary
per MET artery disease
Hypoadaptive Systolic BP: ≥20 mm Hg drop after Severe ventricular failure, multivessel coronary
an initial rise artery disease, significant overmedication
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518 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

relationship can be expressed in terms of the respiratory rate subconsciously if they know
following equation: systolic BP ≈ CO ⫻ TPR. they are being watched, the therapist should
Recalling also that cardiac output is the product adopt a strategy that minimizes this con-
of heart rate and left ventricular stroke volume, founding factor. One strategy is to assess the
increasing workload leads to an increase in patient’s pulse for 30 seconds and continue to
cardiac output (because of its direct effects on palpate the pulse for another 30 seconds while
heart rate and stroke volume). To support this inconspicuously counting breaths. The respi-
increase in cardiac output, systemic vascular ratory rate is then calculated by multiplying
tone is adjusted such that total peripheral resist- the breaths counted in 30 seconds by 2.
ance actually decreases as the intensity of exer- Breathing rhythm is important to observe,
cise progresses. because various forms of pathology may affect it
In response to graded exercise, systolic BP (Table 26-5). Normally, the inspiratory phase is
increases in a fairly linear fashion, such that half as long as the expiratory phase. Patients with
for every 1 metabolic equivalent (MET) increase small airway obstruction often present with pro-
in workload, the systolic BP increases 7 to longed expiratory times (and may exhibit
12 mm Hg from resting values.17 The conclusion pursed-lip breathing), whereas patients with re-
drawn from this observation is that cardiac strictive lung disease may shorten both the inspi-
output increases to a greater degree than total ratory and expiratory phases leading to a re-
peripheral resistance decreases, and the net result duced tidal volume. In the latter case, respiratory
is an increase in systolic BP. In contrast, diastolic rate is usually elevated and represents a pattern
blood pressure is more directly influenced by termed tachypnea. Patients with restrictive lung
total peripheral resistance and changes very little disease often adopt this pattern of breathing be-
from resting values in response to exertion (on cause of the relatively high metabolic cost associ-
the order of ⫾10 mm Hg). ated with maintaining normal tidal volume.
Other diagnoses associated with tachypnea
Respiratory Rate Suggests the Status
include pleuritic or incisional chest pain, ele-
of Ventilatory Mechanics and Blood
vated or hemi-diaphragm, and partial upper
Oxygenation
airway obstruction. Although hyperventilation
Measurement of respiratory rate provides im- has been used historically in reference to either
portant basic diagnostic information regard- rapid or deep breathing, the term hyperpnea is
ing pulmonary function. At rest, the typical probably more appropriate and indicates that
respiratory rate in adults is 10 to 20 breaths the breathing pattern is rapid and deep. Hyper-
per minute; in infants it may be 20 to 40 pnea may be caused by relatively benign factors
breaths per minute. In newborns, the respira- that include exercise and anxiety, but can
tory rate may be as high as 40 to 50 breaths per also be associated with more serious conditions
minute. Because most individuals alter their such as metabolic acidosis. Patients with

TABLE 26-5 ■ Characteristics of Abnormal Breathing Patterns


BREATHING PATTERN CHARACTERISTICS EXAMPLE OF ASSOCIATED CONDITION
Cheyne-Stokes breathing Periods of hyperpnea Uncompensated congestive heart failure,
followed by periods of drug-induced respiratory depression, brain
apnea (no breathing) injury, uremia secondary to kidney failure
Biot’s (ataxic) breathing Periods of hyperpnea, Meningitis, brain injury
tachypnea, or apnea
with no predictable
regularity
Apneustic breathing Deep inspiration Brain injury (pontine lesion)
followed by a pause,
ending in rapid
exhalation
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 519

diabetes mellitus and severe ketoacidosis may Sounds produced by the aortic valve are
demonstrate a characteristic deep, labored, and usually heard best in the aortic area (second in-
gasping breathing pattern called Kussmaul tercostal space along the right sternal border),
breathing. Likewise, bradypnea, which is whereas the pulmonic valve sounds are appreci-
slow breathing in which the respiratory rate is ated best in the pulmonic area (second inter-
<10 breaths per minute, may be a manifestation costal space along the left sternal border). The
of an exercise-trained pulmonary system or tricuspid area (fourth or fifth intercostal space
concentration on a specific task, but can also along the left sternal border) and mitral area
result from drug-induced respiratory depres- (fifth intercostal space at the midclavicular line)
sion, diabetic coma, or traumatic brain injury represent the remaining landmarks for ausculta-
with elevation of intracranial pressure. tion (Fig. 26-5). These sites are typically auscul-
With respect to graded exercise, the first tated with the patient supine, but having the pa-
adjustment made by the ventilatory system is tient assume the left lateral decubitus position or
usually an increase in tidal volume. As exercise sitting up and leaning forward while holding the
intensity progresses, the respiratory rate in- breath can be useful to accentuate heart sounds
creases in order to support the metabolic de- that are otherwise faint or inaudible.
mands of the exercising muscles. Anxiety can Normal heart sounds are thought to origi-
often lead to a rapid increase in respiratory nate from the closure of specific heart valves
rate at the start of an exercise bout and should (or the reverberating sound made by blood
not be interpreted as an abnormal finding. colliding with a valve as it is closing). The first
normal heart sound (the lub in lub-dub) is
Heart and Lung Sounds Reveal called S1 and is associated with closure of
Clues About Cardiac and the mitral (M1 component of S1) and tricuspid
Pulmonary Pathology (T1 component of S1) valves. The second nor-
mal heart sound (the dub in lub-dub) is called
Although a comprehensive tutorial on cardiac S2 and is associated with closure of the aortic
and pulmonary auscultation is beyond the (A2 component of S2) and pulmonic (P2 com-
scope of this chapter, all physical therapists ponent of S2) valves. Ventricular systole, then,
should be able to identify more obvious ab- is represented by the period between S1 and S2,
normal heart sounds that can be readily distin- whereas diastole occurs between S2 and the
guished from normal heart sounds. We advo- next S1. Although S1 and S2 are high frequency
cate that any stethoscope that will be used for in nature and usually appreciated as single
auscultating heart and lung sounds must have
a diaphragm (the flat head that is pressed
firmly against the skin and used for listening to Aortic Pulmonic
high-frequency sounds) and a bell (the cup- area area
shaped head that is placed softly against the
skin and is useful for hearing low-frequency
sounds). Using a stethoscope that only has a
diaphragm will result in failure to appreciate 2
3
some abnormal cardiac sounds.
4
Cardiac Auscultation 5

To perform a competent auscultatory exami-


nation of the heart, the therapist should adopt
a systematic approach which includes:
1. Proper positioning of the patient,
2. Listening to the major auscultatory areas on Tricuspid Mitral
the chest wall with the diaphragm, and area area
3. Listening to those same areas again with the FIGURE 26-5 Primary sites for cardiac auscultation
bell of the stethoscope. of the anterior chest wall.
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520 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

sounds when using the diaphragm, individual is thought to result from the reverberating
components of each sound are sometimes sound produced by blood as it enters the
distinguishable. In this case, the sound is said ventricle during the early part of diastole
to be split. This occurs because cardiac me- and collides against a stiff myocardium that
chanical events on the left slightly precede has lost normal compliance.19 Another
those on the right. That is to say, the mitral name for an S3 is a ventricular gallop. In
valve closes slightly before the tricuspid valve addition to congestive heart failure, an S3 can
and the aortic valve closes slightly before the be heard in patients with thyrotoxicosis, sig-
pulmonic valve. nificant anemia, and conditions character-
A useful maneuver to aid the therapist in ized by large left-to-right shunts such as ven-
distinguishing between the S1 and S2 sounds tricular septal defect and patent ductus
(and especially difficult task with faster pulse arteriosus. The sound can be of right or left
rates) is to palpate the carotid artery while aus- ventricular origin and is heard best at the car-
cultating the heart. The carotid pulse is coinci- diac apex. When an S3 is heard in children,
dent with the beginning of ventricular systole, pregnant females, or young, well-conditioned
and so it is timed with S1. Because S1 is associ- individuals, it is considered to be a normal
ated with closure of the mitral and tricuspid variant (possibly secondary to hyperdynamic
valves, S1 is louder than S2 in the mitral and tri- ventricular function) and is called a physio-
cuspid areas. Conversely, S2 is louder than S1 in logical S3. All other patients exhibiting an S3
the aortic and pulmonic areas. The tricuspid should be referred to the appropriate health
area is the best place to listen for a normal split- care provider for further evaluation.
ting of S1, whereas the pulmonic area is best for ● An S4 (the fourth heart sound) is also heard
appreciating a splitting of S2 (the pulmonic with the bell of the stethoscope in patients
valve closes slightly later than the aortic valve). with cardiovascular syndromes that include
While a split S1 is not affected by the ventilatory chronic systemic hypertension, hypertensive
cycle, a split S2 can be further evaluated on the heart disease, myocardial infarction, and
basis of changes related to inspiration or expi- acute mitral regurgitation secondary to
ration. A physiological split S2 (a normal phe- chordae tendineae rupture. The mechanism
nomenon) exhibits a widening of the A2 and P2 of sound production is similar to that of an
sounds upon inspiration and a fusion of the S3, with the exception that the sound occurs
two sounds upon expiration. The drop in in- immediately after atrial contraction forces
trathoracic pressure during inspiration leads to blood into the ventricle during late ventric-
increased venous return and prolongs right ular diastole (the sound occurs immediately
ventricular ejection, causing the pulmonic before S1). Because of its atrial component,
valve to close later than usual. an S4 is also referred to as an atrial gallop.
Several abnormal heart sounds can be
auscultated: When the novice first hears any of these
abnormal heart sounds, a question may arise
● A split S2 that either: as to how they can be distinguished from a
1. is not affected by the ventilatory cycle split S2. To make the distinction consistently,
(fixed split S2) the therapist can rely on a physical property of
2. widens with inspiration and approxi- the stethoscope itself. As discussed, high-fre-
mates, but does not fuse, with expiration quency sounds are best heard by pressing the
(persistent split S2); or diaphragm firmly against the patient’s skin so
3. widens with expiration and fuses with as to leave a slight impression when it is re-
inspiration (paradoxical split S2); these are moved. The bell, on the other hand, should be
associated with various cardiac anatomical held gently with little more than its own
and conduction abnormalities. weight resting on the patient’s skin so that it
● A third heart sound (S3) can be heard with can transmit low-frequency sounds through
the bell of the stethoscope in patients with its cone. If firm pressure is applied to the bell,
congestive heart failure and occurs as a low- the patient’s own skin acts as a diaphragm
frequency sound immediately after S2. An S3 (like the skin of a drum) and the bell is less
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 521

able to transmit lower frequencies. The thera- and decays in late systole. It is heard best at the
pist need only adjust the pressure applied to aortic area with the diaphragm of the stetho-
the bell in order to differentiate between the scope. When severe, the sound may radiate to
normal variant and the abnormal sounds. For the carotid arteries. Symptoms associated
example, a sound that is heard with the with aortic stenosis include dyspnea, chest
stethoscope’s bell held gently on the skin and pain, and near or frank syncope. The patient
not when the bell is pressed firmly favors an S3 may occasionally present with palpitations,
or S4 (the latter two are then distinguished on fatigue, and a narrowed pulse pressure.
the basis of proximity to S1 or S2). ● Hypertrophic obstructive cardiomyopathy
Cardiac murmurs may be appreciated is a result of abnormal blood flow through the
during auscultation of the heart. Cardiac left ventricular outflow tract. The obstruction
murmurs are usually the result of: is below the level of the aortic valve and in-
1. High-velocity flow through normal or abnor- volves thickening of the interventricular sep-
mal heart valves, tum and left ventricular free wall. When the
2. Forward flow through a restricted (stenotic) ventricle contracts, the thickened septum and
valve, past an area of obstruction, or into a free wall approximate below the aortic valve
dilated vessel/chamber, and the anterior mitral leaflet is sometimes
3. Backward flow through an incompetent pulled into the outflow path.21 The obstruc-
(regurgitant) valve, or tion is less severe if the end-diastolic volume
4. High-velocity flow through nonvalvular (and thus, the left ventricular dimensions) is
communications between cardiac chambers increased. This murmur is a harsh or rough
or large vessels. murmur whose intensity is usually main-
tained throughout all of systole (holosystolic).
An entry-level practitioner can acquire a It is heard loudest along the left sternal border
level of aptitude in auscultating cardiac mur- with the diaphragm of the stethoscope and
murs by remembering a few key concepts.20 often radiates to the apex rather than the aor-
First, the timing of the murmur will indicate if it tic area. Patients with significant hypertrophic
is systolic (heard between S1 and S2) or diastolic obstructive cardiomyopathy present with
(heard between S2 and S1). Once the timing is symptoms similar to aortic stenosis.
noted, the auscultatory area where the murmur ● Aortic regurgitation is characterized by a
is heard loudest provides further clues to its ori- high-pitched, blowing murmur heard dur-
gin. Finally, remembering the state of the valve ing diastole that has a decrescendo pattern
during the particular phase of the cardiac cycle that decreases in intensity with time. It is
will usually implicate the specific valvular dys- usually loudest along the left sternal border
function. For example, a murmur that is heard and heard best with the diaphragm of the
between S1 and S2 and is loudest at the second stethoscope. It may radiate to the cardiac
intercostal space along the left sternal border apex. Aortic regurgitation can be the result
would suggest pulmonic stenosis, since the pul- of such pathologies as rheumatic fever,
monary valve should be open at that time. chronic systemic hypertension (leading to
Murmurs are also graded in terms of their dilation of the aortic root and aortic annu-
relative loudness and associated physical find- lus), and congenital bicuspid valve. Patients
ings. Some murmurs merit special considera- with Marfan’s syndrome and ankylosing
tion in the clinical setting: spondylitis are at higher risk for developing
● Aortic stenosis is a pathological narrowing of this condition. The typical symptoms in-
the aortic valve that can impede left ventricu- clude signs and symptoms of left heart fail-
lar outflow during systole. Calcification of the ure, worsening fatigue, hypotension, pallor,
stenotic leaflets can further impair left ven- tachycardia, palpitations, and chest pain.
tricular outflow. The murmur of aortic steno- Associated findings often include a widened
sis is usually a harsh, medium- to high- pulse pressure and pulsus bisferiens.
pitched sound with a crescendo–decrescendo ● Mitral stenosis exhibits a low-frequency,
quality, which rises to a peak in midsystole rumbling diastolic murmur that is heard best
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522 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

with the bell of the diaphragm at the cardiac Pulmonary Auscultation


apex. The etiology is almost always rheumatic
valvular disease. The typical presentation in- As with cardiac auscultation, pulmonary aus-
cludes exertional dyspnea, fatigue, pulmonary cultation begins with proper positioning of the
edema, and hemoptysis secondary to elevated patient. The lung fields are best auscultated
pulmonary vascular pressure. with the patient sitting at the edge of the bed or
plinth so that the therapist can assess both the
● Mitral regurgitation can be due to any factor anterior and posterior chest wall. Because the
that interferes with the normal function of majority of lung sounds are of medium to high
the mitral complex, including papillary mus- frequency, the diaphragm of the stethoscope
cle dysfunction, rupture of the chordae should be used for auscultation. Posteriorly, the
tendineae, and rheumatic valvular disease. therapist should begin by listening to the apices
The holosystolic murmur is high pitched and and then move down along either side of the
has a blowing quality. It is heard best with the vertebral column (asking the patient to protract
diaphragm of the stethoscope at the cardiac the shoulders prevents scapular interference),
apex and often radiates to the left axilla. Pa- finally terminating in the basal-lateral aspects of
tients with mitral regurgitation can present the rib cage. The anterior chest should be aus-
with pulmonary edema, fatigue, dyspnea, cultated in a similar fashion while avoiding di-
edema, and tachycardia. Associated findings rect auscultation over the sternum (Fig. 26-6).
can include mitral valve prolapse in which one
of the leaflets bulges back into the left atrium
during systole (possibly due to elongation of
the chordae tendineae). Mitral valve prolapse
occurs more often in females than males and
exhibits a high-pitched click occurring in
mid- to late systole that is heard best with the
diaphragm of the stethoscope. Quite often,
the disorder is benign and patients remain
asymptomatic.22 Patients with clinically sig-
nificant mitral valve prolapse may present
with symptoms of dizziness, weakness, chest
pain, awareness of the heartbeat, fatigue, anx-
iety, and palpitations.
● Pericardial friction rubs are often described
as squeaking or scratching sounds heard best Anterior
with the diaphragm of the stethoscope and
are likened to the rubbing of sandpaper or
dry leather. Friction rubs indicate the pres-
ence of pericarditis that is more severe in na-
ture, often involving both the visceral and
parietal layers. Pericarditis may result from
such conditions as myocardial infarction or
infection or may occur post-pericardiotomy
or as a result of trauma. Patients often report
chest pain and may present with tachycardia,
tachypnea, hypotension, and fever. A simple
maneuver to help the therapist distinguish
between a pericardial friction rub and a rub
of pulmonary origin is to ask the patient to
hold his or her breath during auscultation. If Posterior
the sound persists, then it is pericardial in FIGURE 26-6 Primary sites for pulmonary ausculta-
nature. tion of the chest wall.
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Chapter 26 Cardiovascular and Pulmonary Clues from Examination 523

Most therapists find it efficient to alternate may have such a degree of pulmonary hyperin-
from right to left at each level so as to better flation that breath sounds are poorly transmit-
compare discrepancies between sides. Ausculta- ted to the surface of the chest. In this case, sig-
tion through clothes dampens sound transmis- nificantly diminished breath sounds may be
sion and may simulate pathological sounds, so appreciated during auscultation. Finally, a
the therapist should auscultate over bare skin patient with a pneumothorax will present with
while maintaining proper draping techniques. absent breath sounds over the involved area.
The patient should be asked to breathe deeply When sound transmission is altered, the
through the mouth to maximize airflow and therapist can apply several techniques to fur-
enhance sound fidelity. ther characterize the degree or severity of pul-
Normal breath sounds can be classified monary involvement. Egophony (also called
into three main groups: the E to A change) is observed when the patient
is asked to say “E” and the auscultated sound is
1. Bronchial, “A.” The second abnormal sound characteristic
2. Bronchovesicular, and is called bronchophony and is demonstrated
3. Vesicular. by asking the patient to repeat “ninety-nine” or
“one–two–three.” The therapist will hear the
Bronchial breath sounds are loud, high-
words with greater intensity and higher pitch
pitched sounds that have an almost equal inspi-
during auscultation (normally, the words are
ratory and expiratory phase separated by a dis-
muffled and hard to decipher). Egophony and
cernable pause. They are heard primarily over
bronchophony usually occur together in pa-
the trachea and mainstem bronchi. Bron-
tients with diffuse pulmonary consolidation,
chovesicular sounds share similar characteristics
pleural effusion, or compression atelectasis.
with bronchial sounds, but they are much softer
The last abnormal characteristic is referred
and lack a discernable pause between phases
to as whispered pectoriloquy, and is elicited by
(the inspiratory component fuses with the expi-
asking the patient to whisper the words “ninety-
ratory component). They are heard over the
nine” or “one–two–three” and the words are
junctions of the mainstem bronchi and the var-
clearly heard through the stethoscope (nor-
ious segmental bronchi (in the interscapular
mally, the words are unintelligible). Whispered
region). Vesicular sounds are very soft, low-
pectoriloquy may be appreciated even in the
pitched sounds having an expiratory phase that
absence of egophony and bronchophony, and
is approximately one-third of the inspiratory
allows for the identification of more subtle con-
phase (at times, the expiratory phase may actu-
solidation or hyperinflation. As a general rule,
ally be inaudible). They also lack a discernable
louder and more pronounced words are heard
pause between phases and are heard over most
in the presence of consolidation, whereas hyper-
of the peripheral lung fields.
inflation states are associated with softer and less
Generally speaking, the absence of a normal
intelligible sound transmission.
breath sound when one would be expected or
Adventitious breath sounds refer to
appreciation of a normal breath sound over an
abnormal extrinsic breath sounds that can be
area in which it is not usually heard should
heard throughout the bronchopulmonary
prompt further examination. For example, a
tree. According to the American Thoracic
patient with pneumonia may have an area of
Society, they are classified as being either con-
consolidation in the peripheral lung fields sec-
tinuous or discontinuous:
ondary to accumulation of secretions in the
alveoli. In this case, fluid has displaced the air in ● Wheezes are continuous, high- or low-
the peripheral lung tissue, increasing its relative pitched sounds of varying duration auscul-
density. The increased density causes the sounds tated during expiration (although they may
from the larger airways to be transmitted more also be heard during inspiration). High-
readily to the surface of the chest. As a result, pitched expiratory wheezes are associated
bronchial breath sounds may be heard in an area with intrathoracic airway obstruction as
where vesicular sounds are normally heard. can occur with bronchospasm secondary
Conversely, a patient with severe emphysema to asthma or obstruction by secretions
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524 Chapter 26 Cardiovascular and Pulmonary Clues from Examination

secondary to chronic bronchitis. Although friction rubs are distinguished from pericar-
some clinicians still label low-pitched, con- dial friction rubs on the basis of the effect
tinuous sounds as rhonchi, they are more breath-holding has on the sound, where dis-
correctly referred to as low-pitched wheezes. appearance of the sound indicates a pleural
Inspiratory wheezes usually indicate a more friction rub while persistence of the sound
severe airway obstruction and may coexist favors a pericardial friction rub.
with expiratory wheezes. A laryngeal or tra-
cheal obstruction produces a loud, high- Conclusion
pitched sound that is readily audible without
a stethoscope. Examples of large airway ob- This chapter provided an overview of the phys-
struction include foreign body aspiration, ical clues suggestive of cardiovascular and/or
epiglottitis, croup, and tumors. This sound is pulmonary dysfunction and reviewed specific
referred to as stridor and is usually heard dur- clinical evaluation tools that are available to the
ing inspiration, but may be heard during physical therapist to further assess its presence
both phases of the ventilatory cycle. The and severity. Understanding the clues given by
presence of stridor necessitates expeditious cardiovascular and pulmonary pathologies will
evaluation and treatment (especially when provide physical therapists with the tools nec-
associated with cyanosis) because it repre- essary to suspect and determine the presence of
sents a potentially life-threatening condition. cardiovascular and/or pulmonary involvement
● Crackles are discontinuous, low-pitched in their patients who may be receiving physical
sounds similar in quality to the carbonation therapy intervention for seemingly unrelated
of a soft drink or Velcro ripping and can be disorders. Awareness of the possibility of car-
associated with either restrictive or obstruc- diovascular and pulmonary comorbidities in
tive lung diseases. The therapist can simulate patients will also allow the physical therapist to
the sound by rubbing hair between the design appropriate treatment plans, accurately
fingertips next to the ear. Crackles are heard interpret the patient’s physiological responses
primarily during inspiration (but can also to exertion, modify assessment and treatment
be heard during expiration) and are further approaches as needed, and initiate a referral to
described as fine or coarse. The mechanism an appropriate health care provider when the
of sound production usually involves the patient’s needs fall outside the scope of physical
sudden opening of previously closed airways therapist practice.
(including alveoli) or the movement of air
through secretions that have accumulated References
in the air spaces. Early inspiratory crackles 1. Panka GFL, Oliveira MM, Franca DC, et al. Ventilatory
and muscular assessment in healthy subjects during an
are consistent with chronic obstructive activity of daily living with unsupported arm elevation.
pulmonary disease, whereas late inspiratory Rev Bras Fisioter. Jul/Aug 2010;14(4);337–344.
crackles suggest the presence of restrictive 2. Mangione S. The skin, the neck, the cardiovascular sys-
lung diseases, such as congestive heart tem, and chest inspection, palpation, and percussion. In:
Mangione S, ed. Physical Diagnosis Secrets. Philadelphia,
failure, pneumonia, atelectasis, and intersti- PA; Elsevier Health Sciences; 2008.
tial pulmonary fibrosis. 3. Stack AM. Etiology and evaluation of cyanosis in chil-
● Inflammation of the pleural membranes dren. In: UpToDate, Basow DS (Ed), UpToDate, Waltham,
MA, 2009.
typically produces another adventitious 4. Martin L, Khalil H. How much reduced hemoglobin is
lung sound referred to as a pleural necessary to generate central cyanosis? Chest. Jan
friction rub. The sound made by a pleural 1990;97(1):182–185.
rub is likened to that of two pieces of 5. Welsh JR, Arzouman JM, Holm K. Nurses’ assessment
and documentation of peripheral edema. Clin Nurse
dry leather or sandpaper being rubbed Spec. Jan 1996;10(1):7–10.
together. It is almost always heard during in- 6. Brodovicz KG, McNaughton K, Uemura N, et al. Reliabil-
spiration and expiration. Patients often will ity and feasibility of methods to quantitatively assess
report chest pain with a pleuritic compo- peripheral edema. C M & R. 2009;7(1/2):21–31
7. Plathow C, Ley S, Fink C, et al. Evaluation of chest motion
nent in that the pain worsens with deep in- and volumetry during the breathing cycle by dynamic
spiration and is aggravated by coughing or MRI in healthy subjects: comparison with pulmonary
sneezing. As mentioned previously, pleural function tests. Invest Radiol. Apr 2004;39(4):202–209.
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8. Bickley LS, Szilagyi PG. Bates’ Guide to Physical Exami- 15. Stewart JM. Chronic orthostatic intolerance and
nation and History Taking. Philadelphia, PA: Lippincott the postural tachycardia syndrome (POTS). J Pediatr.
Williams & Wilkins; 2003. Dec 2004;145(6):725–730.
9. Cahalin LP. Cardiovascular evaluation. In: DeTurk WE, 16. Khasnis A, Lokhandwala Y. Clinical signs in medicine:
Cahalin LP, eds. Cardiovascular and Pulmonary Physical pulsus paradoxus. J Postgrad Med. Jan–Mar 2002;
Therapy: An Evidence–Based Approach. New York, NY: 48(1):46–49.
McGraw Hill Medical; 2011. 17. Le V-V, Mitiku T, Sungar G, et al. The blood pressure re-
10. Kodama M, Kato K, Hirono S. Linkage between sponse to dynamic exercise testing: a systematic review.
mechanical and electrical alternans in patients with Prog Cardiovasc Dis. Sept/Oct 2008;51(2);135–160.
chronic heart failure. J Cardiovasc Electrophysiol. 18. Watchie J. Cardiopulmonary implications of specific
2004;15(3):295–299. diseases. In: Hillegass E, ed. Essentials of Cardiopul-
11. Wagner HR. Paradoxical pulse: 100 years later. Am J monary Physical Therapy. St. Louis, MO: Elsevier Saun-
Cardiol. Jul 1973;32(1):91–92. ders; 2011.
12. Pickering TG, Hall JE, Appel LJ, et al. Recommenda- 19. Richardson TR, Moody JM, Jr. Bedside cardiac exami-
tions for blood pressure measurement in humans and nation: constancy in a sea of change. Curr Probl Cardiol.
experimental animals: part 1: blood pressure meas- Nov 2000;25(11):783–825.
urement in humans: a statement for professionals 20. Chizner MA. Cardiac auscultation: rediscovering the
from the Subcommittee of Professional and Public lost art. Curr Prob Cardiol. July 2008;33:326–408.
Education of the American Heart Association Council 21. Lee CT, Dec GW, Lilly LS. The cardiomyopathies. In:
on High Blood Pressure Research. Circulation. Feb 8, Lilly LS, ed. Pathophysiology of Heart Disease: A Collab-
2005;111(5):697–716. orative Project of Medical Students and Faculty. 5th ed.
13. Agarwal R, Bunaye Z, Bekele DM. Prognostic significance Baltimore, MD: Lippincott Williams & Wilkins: 2011.
of between-arm blood pressure differences. Hypertension. 22. Freed LA, Benjamin EJ, Levy D, et al. Mitral valve pro-
2008;51:657–662. lapse in the general population: the benign nature of
14. Bradley JG, Davis KA. Orthostatic hypotension. Am echocardiographic features in the Framingham Heart
Fam Physician. Dec 15, 2003;68(12):2393–2398. Study. J Am Coll Cardiol. Oct 2, 2002;40(7):1298–1304.
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CHAPTER 27
Dizziness
■ Rob Landel, PT, DPT, OCS, CSCS, FAPTA

Description of the Symptom ■ History of head or neck trauma and con-


cerns of:
This chapter describes pathology that may lead ■ Changes in neurological status, as noted
to dizziness. Dizziness is described as a sensa- above
tion of movement of self or surroundings ■ Long tract signs elicited by head or neck
(vertigo), light-headedness (presyncope), or movements
imbalance (disequilibrium). ■ Dizziness with sustained head or neck
positions
Special Concerns ■ Unwillingness to move the neck
Dizziness in the presence of any of the follow- ■ Facial numbness or paresthesias
ing symptoms and signs: ■ Palpitations that are:
■ Chest pain ■ Followed by loss of consciousness
■ Irregular heartbeat ■ Associated with significant shortness of
■ A new onset of, or a change in, neurological breath, chest discomfort, light-headedness,
status, including: or presyncopal symptoms
■ Weakness or paralysis in the face, arm, or ■ Associated with marked hypertension (ie,
leg, especially on one side of the body >220/110) or hypotension (ie, <90/60 in an
■ Tremor individual who is typically normotensive)
■ Change in mental status, such as confu- ■ Of sudden onset and last longer than 15
sion, memory loss, hallucinations to 20 minutes
■ Difficulty talking ■ Occurring in an individual with a family
■ Difficulty understanding speech history of sudden death.
■ Trouble seeing with one or both eyes ■ Fever, chills, accompanied by a very stiff
■ Loss of coordination neck or other signs of infection
■ Loss of balance ■ Unrelenting headache
■ Nystagmus that is spontaneous (resting), ■ Hyperpnea or dyspnea
direction changing, or gaze evoked ■ Anxiety
■ Oculomotor abnormalities including ■ Sudden hearing loss
abnormal smooth pursuit and saccades. ■ Ear pain
■ Loss of sensation

CHAPTER PREVIEW: Conditions That May Lead to Dizziness

T Trauma
COMMON
Benign paroxysmal positional vertigo 532
Cervicogenic dizziness (cervical vertigo) 533
Whiplash injury (whiplash-associated disorder) 546
UNCOMMON
Brainstem–eighth nerve complex injury 533
Labyrinthine concussion 537
Traumatic brain injury 544

526
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Chapter 27 Dizziness 527

Trauma (continued)

DIZZINESS
RARE
Perilymph fistula 542
Superior canal dehiscence syndrome 544

I Inflammation
COMMON
Aseptic
Not applicable
Septic
Labyrinthitis and neuronitis 537
UNCOMMON
Aseptic
Multiple sclerosis 540
Septic
Not applicable
RARE
Aseptic
Autoimmune inner ear disease 532
Paraneoplastic syndromes 542
Septic
Herpes zoster oticus (Ramsey Hunt syndrome) 535
Otosyphilis 541

M Metabolic
COMMON
Adverse effect or side effect of medications 530
Dehydration/hypovolemia 534
Hypoglycemia 536
Pregnancy 543
UNCOMMON
Exposure to toxic chemicals 535
Hyperthyroidism/thyrotoxicosis 535
Hypothyroidism 536
RARE
Vestibular ototoxicity 546

Va Vascular
COMMON
Anemia 530
Orthostatic hypotension 541
UNCOMMON
Dysrhythmias/arrhythmias 535
Mitral valve prolapse 539
Transient ischemic attack 544
(continued)
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528 Chapter 27 Dizziness

Vascular (continued)
DIZZINESS

RARE
Arteriovenous malformation 532
Idiopathic intracranial hypertension 537
Lateral medullary infarction (Wallenberg’s syndrome) 537
Postural tachycardia syndrome 543
Vertebrobasilar artery insufficiency 545

De Degenerative
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Multiple system atrophy with orthostatic hypotension (striatonigral degeneration, olivopontocerebellar
atrophy, Shy-Drager syndrome) 540

Tu Tumor
COMMON
Not applicable
UNCOMMON
Malignant Primary, such as:
• Brain primary tumors 533
Malignant Metastatic, such as:
• Brain metastases 532
Benign:
Not applicable
RARE
Malignant Primary, such as:
• Brain primary tumors 533
• Spinal primary tumors 544
Malignant Metastatic, such as:
• Brain metastases 532
• Spinal metastases 543
Benign, such as:
• Acoustic neuroma (vestibular schwannoma) 530
• Angiomatosis (Von Hippel-Lindau disease) 531
• Neurosarcoidosis 541

Co Congenital
COMMON
Not applicable
UNCOMMON
Not applicable
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Chapter 27 Dizziness 529

Congenital (continued)

DIZZINESS
RARE
Cholesteatoma 534
Hereditary neuropathies 535

Ne Neurogenic/Psychogenic
COMMON
Anxiety disorder/panic attacks 531
Vasovagal syncope 545
UNCOMMON
Ménière’s disease 538
Migraine-related dizziness:
• Basilar migraines 539
• Benign recurrent vertigo 539
• Vestibular migraine 539
RARE
Mal de débarquement 538
Vestibular epilepsy (vestibular seizures) 545

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Dizziness the change in blood pressure between supine


and standing positions will help rule in ortho-
Dizziness manifests itself in many forms and static hypotension; a drop of 20 mm Hg or
includes vertigo, defined as a whirling or tilt- more in systolic blood pressure is significant.
ing sensation with a tendency to fall1,2; dise- This chapter focuses on vertigo or the illusion
quilibrium or imbalance; and light-headedness of movement, typically spinning, either of the
or presyncope. Individuals may use the term individual or of the surrounding environment.
dizziness to describe any of the preceding There are many causes of dizziness, and
symptoms. Individuals may report dizziness while most are benign, serious pathologies
but upon further questioning may describe a must be identified. One study suggests that
feeling of being off-balance, unsteady, light- vertigo or vomiting combined with a positive
headed, spacey, or feeling as if they are rocking Dix-Hallpike test has an 85% positive predic-
or floating. It is important to distinguish be- tive value (PPV) for being benign. In addition,
tween the sensations and determine as well as the presence of vertigo in an individual 69 years
possible what the individual is experiencing, old or younger, with no neurological deficits,
because the description will help narrow down has an 88% negative predictive value for seri-
the diagnostic possibilities.1,3,4 Disequilibrium ous pathology. Over the age of 69, vertigo
or imbalance can be due to multiple factors in- combined with neurological deficits has a
cluding sensory deficits, motor deficits, and 40% PPV for serious pathology. This suggests
impairment in the central nervous system that performing a neurological exam on the
processing of the two. Causes of falling and person presenting with vertigo is essential.5
limited balance are covered in Chapter 28. Once the individual’s concerns have been
Light-headedness such as that felt with ortho- narrowed to vertigo, differentiation must be
static hypotension is typically a sign of presyn- made between central causes (lesions in the
cope or near syncope and suggests impaired central nervous system) and peripheral
central nervous system perfusion. Checking causes (lesions in the vestibular labyrinth or
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530 Chapter 27 Acoustic Neuroma (Vestibular Schwannoma)

nerve or both).1,4 The presence of other headache. If untreated, brainstem compression


DIZZINESS

neurological symptoms such as generalized can occur, with concerns of generalized


weakness, difficulty with speech, problems headache, facial twitch and weakness, visual
with walking, tremor, and so on in addition loss, diplopia, lower cranial nerve dysfunction
to vertigo suggests central involvement. The causing aspiration, hoarseness, dysphagia,
location of the lesion will determine the shoulder weakness, tongue weakness, and long
symptoms of most central vertigo syndromes, tract signs.6
but the etiology of a particular lesion may
Background Information
vary. For example, traumatic, inflammatory,
Acoustic neuromas arise from the Schwann
vascular, or neoplastic lesions will produce
cells of the eighth cranial nerve within the inter-
the same symptoms if they affect the same
nal auditory canal, eventually growing out into
location. The presence of other otologic
and compressing the brainstem. Audiometry,
symptoms, such as hearing loss, tinnitus, and
electronystagmography, auditory brainstem re-
aural fullness, suggests a peripheral cause.
sponse, computed tomography, and magnetic
The intensity of the vertigo in central condi-
resonance imaging with gadolinium contrast
tions is typically more moderate and persist-
confirm the diagnosis, with the latter providing
ent than in peripheral conditions.
the most specificity.6 Treatment is surgical re-
The duration of symptoms can be helpful in
moval of the tumor, with risk of permanent
making the diagnosis. Short episodes of vertigo
changes in hearing, balance and facial sensation,
suggest a peripheral cause such as benign parox-
and motion. Loss of a preoperatively function-
ysmal positional vertigo, the most common
ing vestibular nerve will result in disequilibrium
cause of vertigo. Vertigo lasting 20 to 30 minutes
for which vestibular adaptation exercises and
is suggestive of transient ischemic attacks or mi-
balance rehabilitation are appropriate.11
grainous vertigo. Vertigo lasting hours is typical
of Ménière’s disease, and vestibular neuronitis ■ Adverse Effect or Side Effect
resolves after days or weeks. Peripheral vertigo of Medications
other than benign paroxysmal positional vertigo
Chief Clinical Characteristics
will resolve gradually due to central compensa-
This presentation commonly includes onset
tion, whereas central vertigo will persist for a
of dizziness with use of certain prescription
long time and fluctuates little if at all in intensity.
and over-the-counter medications, as well as
Associated symptoms may assist in making
illicit drugs.
the diagnosis. The presence of headaches sug-
gests migrainous vertigo; panic attacks, anxiety, Background Information
hyperventilation, and agoraphobia suggest psy- The medications, both prescribed and over
chogenic causes. The medical history should the counter, that have dizziness or light-
include questions about drug use; metabolic, headedness as a side effect are too numerous
cardiovascular, or immunologic conditions; to describe here. The prudent clinician will
impaired vision; symptoms of infection; or obtain from the individual a complete list of
generalized neuropathies. prescription and nonprescription drugs the
individual is taking and determine if any of
Description of Conditions them induce dizziness or light-headedness.
That May Lead to Dizziness Consultation with a pharmacist may be
advisable in order to determine drug interac-
■ Acoustic Neuroma (Vestibular tions. Recreational drugs should not be
Schwannoma) overlooked.
Chief Clinical Characteristics
This presentation may include hearing loss, tin- ■ Anemia
nitus, vertigo, or disequilibrium in the early Chief Clinical Characteristics
stages.5–11 Vestibular nerve function may be This presentation can be characterized by light-
gradually lost without much balance distur- headedness, associated with lethargy, cold skin,
bance. Symptoms worsen as the tumor grows, depression, easy fatigability, shortness of breath,
and the individual may also report having a and cognitive impairment.12
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Chapter 27 Anxiety Disorder/Panic Attacks 531

Background Information depend on the size and location of the tu-

DIZZINESS
Comorbid conditions that render the indi- mors. Individuals with this condition are
vidual susceptible to anemia include recent also at a higher risk than normal for certain
major surgery (ie, orthopedic and cardiopul- types of cancer, especially kidney cancer.
monary surgeries), pregnancy, lesions of the Treatment varies according to the location
gastrointestinal tract, sickle cell trait, and and size of the tumor and its associated cyst.
cancer.13 Anemia is defined as a decrease in In general, the objective is to treat the tumors
the oxygen-carrying capacity of blood sec- when they are causing symptoms but are still
ondary to a decrease in the erythrocyte (red small. Treatment of most cases usually in-
blood cell) content of blood, a diminished volves surgical resection. Certain tumors can
content of hemoglobin per erythrocyte, or be treated with focused high-dose irradia-
a combination of both.14 It can arise from tion. Individuals with this condition need
failed synthesis, premature destruction, careful monitoring by a physician and/or
hemorrhage, or deficiencies in iron, B12, or medical team familiar with the condition.
folic acid. The resultant reduction in the
blood’s oxygen-carrying capacity initiates a ■ Anxiety Disorder/Panic Attacks
reflex sinus tachycardia in attempts to main-
Chief Clinical Characteristics
tain adequate tissue oxygenation while blood
This presentation typically includes repeated
pressure is usually within the normal resting
panic or anxiety attacks. Symptoms include
range.15 The pulse is felt to be rapid and reg-
increased heart rate and respiratory rate, pupil
ular upon palpation, supporting the presence
dilation, and trembling and sweating with feel-
of sinus tachycardia. If anemia is suspected,
ings of fear and dread. The symptoms usually
the individual should be referred to his or
subside in 15 to 30 minutes.
her primary care practitioner for further
evaluation. The diagnosis is confirmed by Background Information
routine blood testing, including complete This condition is a nonspecific syndrome and
blood count. can be due to a variety of medical or psychi-
■ Angiomatosis (Von atric syndromes or observed as part of a drug
Hippel-Lindau Disease) withdrawal or drug intoxication effect. The
diagnosis of an anxiety condition is based on
Chief Clinical Characteristics
criteria from the DSM-IV-TR.16 Anxiety con-
This presentation commonly includes headaches,
ditions are classified into specific categories.
problems with balance and walking, dizziness,
One category, panic condition, has dizziness as
weakness of the limbs, vision problems, and high
one of its features. Panic condition is defined
blood pressure.
by recurrent attacks with at least four of the
Background Information following features: increased heart rate, sweat-
This condition is a rare, genetic multisystem ing, trembling or shaking, dyspnea, sensation
condition characterized by the abnormal of choking, chest pain or discomfort, nausea
growth of tumors in certain parts of the body or abdominal distress, feelings of dizziness,
(angiomatosis). The tumors of the central fear of losing control, fear of dying, paresthe-
nervous system are benign and are com- sias, and chills or hot flashes. The etiology of
prised of a nest of blood vessels and are anxiety conditions includes genetic factors,
called hemangioblastomas (or angiomas in social and psychological factors, and physio-
the eye). Hemangioblastomas may develop in logical and biochemical abnormalities.17
the brain, the retina of the eyes, and other Treatment includes cognitive-behavioral ther-
areas of the nervous system. Other types of apy, relaxation exercises, and pharmacologic
tumors develop in the adrenal glands, the treatment.17 The course of this condition is
kidneys, or the pancreas. Cysts (fluid-filled variable; most individuals maintain normal
sacs) and/or tumors (benign or cancerous) social lives. Clinicians are encouraged to con-
may develop around the hemangioblastomas sider referral of individuals suspected of hav-
and cause the symptoms listed above. Spe- ing this condition to a mental health specialist
cific symptoms vary among individuals and for evaluation and treatment.
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532 Chapter 27 Arteriovenous Malformation

■ Arteriovenous Malformation ■ Benign Paroxysmal Positional


DIZZINESS

Chief Clinical Characteristics Vertigo


This presentation may be characterized by Chief Clinical Characteristics
seizures and severe headache. Hemorrhage may This presentation commonly involves dizziness or
result in paresis, ataxia, dyspraxia, dizziness, vi- vertigo (typically described as a spinning sensa-
sual disturbances, aphasia, paresthesias, and tion), light-headedness, imbalance, and nausea.
cognitive deficits.18 Symptoms are precipitated by a position change
of the head relative to gravity, such as getting
Background Information
out of bed, rolling over in bed, or looking up.20,21
This condition is caused by a tangle of arteries
and veins that cause abnormal communication Background Information
within the vasculature. Approximately 12% of This condition is thought to occur when free-
the 300,000 individuals in the United States floating debris becomes trapped in the semicir-
with this condition are symptomatic. This con- cular canal (canalithiasis) or becomes adhered
dition is caused by a developmental abnormal- to the cupula (cupulolithiasis), rendering the
ity that likely arises during embryonic or fetal semicircular canal sensitive to gravity and thus
development. Neurological damage occurs due changes in head position rather than head
to reduction of oxygen delivery, hemorrhage, motion. The etiology of benign paroxysmal
or compression upon nearby structures of the positional vertigo is unknown in most cases,
brain or spinal cord. Computed tomography, but it is associated with head trauma, vestibu-
magnetic resonance imaging, and arteriogra- lar neuritis, and vertebrobasilar ischemia and
phy confirm the diagnosis. Ligation and em- can occur after ear surgery or prolonged bed
bolization may be used to reduce the size of the rest.22 This condition occurs more often in
lesion prior to surgical excision, which is the the elderly, and tends to recur in up to 15% of
preferred method of treatment. Stereotactic ra- cases within 1 year and 50% of cases within
diation and proton beam therapy are alternative 40 months.23 The diagnosis is confirmed with
approaches to invasive methods of interven- the Hallpike maneuver, in which the head is
tion. Up to 90% of individuals who experience rotated 45 degrees and tilted back while hang-
a hemorrhagic arteriovenous malformation ing off the end of the table. This position will
survive.18 elicit torsional and vertical nystagmus when
the vertical semicircular canals are involved.
■ Autoimmune Inner Ear Disease Duration of vertigo while in the Hallpike posi-
tion will determine if the individual has
Chief Clinical Characteristics
canalithiasis (short duration, <1 minute) or
This presentation involves progressive bilateral
cupulolithiasis (long duration, >1 minute).
sensorineural hearing loss often accompanied
When the horizontal (lateral) semicircular
by bilateral loss of vestibular function.19
canals are involved, turning the head while
Background Information the patient is supine will elicit horizontal nys-
The symptoms may occur as a direct assault by tagmus and vertigo. A nonsurgical treatment
the immune system on the inner ear, or may be consisting of a series of positional changes to
related to the deposition of antibody–antigen move the debris out of the canals, known as the
complex in the inner ear structures. This condi- canalith repositioning maneuver, can be very
tion may occur as a result of a concomitant successful.22–28 In recalcitrant cases, the offend-
autoimmune condition, such as rheumatoid ing canal can be plugged surgically.29
arthritis, psoriasis, ulcerative colitis, or Cogan’s
syndrome (iritis accompanied by vertigo and ■ Brain Metastases
sensorineural hearing loss). Treatment is prima- Chief Clinical Characteristics
rily pharmacologic, because this condition rep- This presentation may include headaches,
resents bilateral involvement of the vestibular seizures, dysphagia, weakness, cognitive
system. Controlled physical exercises to promote changes, behavioral changes, dizziness, vomit-
substitution for the lost peripheral vestibular in- ing, alterations in the level of consciousness,
put may promote some clinical benefit. ataxia, aphasia, nystagmus, visual disturbances,
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Chapter 27 Cervicogenic Dizziness (Cervical Vertigo) 533

dysarthria, balance deficits, falls, lethargy, and also variable and depends on the type and grade

DIZZINESS
incoordination.18,30 of tumor, severity of compression, and dura-
tion of compression.
Background Information
The majority of individuals with brain ■ Brainstem–Eighth Nerve
metastases have been previously diagnosed Complex Injury20
with a primary tumor; however, a small per-
Chief Clinical Characteristics
centage of individuals are diagnosed con-
This presentation can be characterized by acute
comitantly with brain metastases and the
signs of vertigo, leading to constant unsteadiness,
primary tumor. The most common cancers
which worsens in darkness and during fatigue
resulting in subsequent brain metastases in-
and contributes to motion intolerance. The
clude lung, breast, melanoma, colorectal, and
symptoms are generally chronic, as the vestibu-
genitourinary tract. The new onset of neuro-
lar disturbance remains uncompensated. Symp-
logical symptoms after a primary tumor war-
toms and signs for this condition are similar to
rants imaging such as magnetic resonance
those for labyrinthine concussion.
imaging or computed tomography to con-
firm the diagnosis. Treatment may include Background Information
corticosteroids, brain irradiation, surgery, A reduced vestibular response is apparent on
chemotherapy, radiotherapy, and rehabilita- caloric testing, and rotary chair tests show
tive therapies. The prognosis is poor with gain asymmetry and an increased phase lag.
death typically occurring within 6 months. Posturography is typically abnormal. Experi-
mental and autopsy reports have described a
■ Brain Primary Tumors shearing effect on the root entry zone of cra-
Chief Clinical Characteristics nial nerves with head trauma. Even mild
This presentation may include headaches, trauma leads to hemorrhages often in the
seizures, dysphagia, weakness, cognitive brainstem and especially in the area of the
changes, behavioral changes, dizziness, vomit- vestibular nuclei. Magnetic resonance imag-
ing, alterations in the level of consciousness, ing confirms the diagnosis when it reveals
ataxia, aphasia, nystagmus, visual disturbances, this anatomical finding. Treatment involves
dysarthria, balance deficits, falls, lethargy, and vestibular habituation exercises.
incoordination.18
■ Cervicogenic Dizziness (Cervical
Background Information Vertigo)
With the excessive proliferation of cells, a tumor
Chief Clinical Characteristics
mass eventually results in compression of the
This presentation typically involves a non-specific
brain. This compression may displace cere-
form of dizziness that is related to neck pain and
brospinal fluid, thereby increasing intracranial
impairments such as decreased cervical spine
pressure and resulting in ischemia to the
range of motion. It can include unsteady gait,
same tissues. Glioblastoma multiforme, astro-
postural disturbances, ataxia, or headaches.
cytoma, oligodendroglioma, metastatic tumors,
Individuals with this condition often will report
primary central nervous system lymphomas,
a history of cervical trauma such as whiplash.
ganglioglioma, neuroblastoma, meningioma,
arachnoid cysts, hemangioblastoma, medul- Background Information
loblastoma, and acoustic neuroma/schwannoma Cervicogenic dizziness is defined as a nonspe-
are some of the more common brain tumors. cific sensation of altered orientation in space
The first test to diagnose brain and spinal col- and dysequilibrium originating from abnormal
umn tumors is a neurological examination. afferent activity from the neck,31 and vertigo
Specific diagnoses for brain tumors may be induced by changes of position of the neck or
confirmed with imaging and biopsy. Treatment vertigo originating from the cervical region.32
is variable depending on the type, size, and The vestibular system is not involved in true cer-
location of the tumor and may include surgical vicogenic dizziness. This condition is thought
resection, chemotherapy, radiation, corticos- to result from malfunction or disturbance in
teroids, and rehabilitative therapies. Prognosis is the afferent flow of impulses from deep cervical
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534 Chapter 27 Cholesteatoma

tissues and cervical proprioceptors.33,34 The perforation, or the formation of a pocket that
DIZZINESS

diagnosis of this condition is by exclusion. accumulates keratin debris. The diagnosis is


When the onset is related to trauma, upper cer- made primarily from the history and physical
vical hypermobility and cervical fractures must examination. Computed tomography can be
be ruled out as both can produce symptoms of used preoperatively to plan surgery. Treatment
dizziness. Physical examination will reveal soft consists of periodic ear cleaning but in almost
tissue tenderness of the cervical spine, limited all cases surgery is necessary to remove the
neck motion, and abnormal intervertebral lesion. Hearing loss, dizziness, facial paralysis,
mobility. The neck torsion nystagmus test, tinnitus, and taste abnormalities are possible
where dizziness and nystagmus are produced postoperative complications.
when the head remains fixed while the neck and
body are rotated, is thought to be a sign of ■ Dehydration/Hypovolemia
cervicogenic dizziness,20 but it is non-specific Chief Clinical Characteristics
for the condition.31 The smooth pursuit neck This presentation can be characterized by
torsion test may be used, which reveals smooth palpitations, lethargy, poor concentration,
pursuit abnormalities when the head is held tremors, light-headedness, constipation, dry
rotated in individuals with whiplash-associated mouth, and syncope. If this condition is severe,
conditions who report dizziness, as well as cyanosis of the lips, sunken eyes, cold extrem-
posturography that is conducted while inducing ities, failure of skin to bounce back when it is
postural perturbances using head and neck lightly pinched and released, confusion, and
vibration.35 Disturbances in balance may be lethargy may be noted.
present36 although not likely enough to produce
falls unless other comorbidities exist. Interven- Background Information
tions such as cervical and thoracic mobilization Dehydration due to sweat loss and/or inade-
and manipulation, traction, exercise including quate fluid replacement during physical
activities to improve cervical joint position error exertion can lead to a significant decrease in
and head control, physical modalities, postural central blood volume. Mild to moderate
re-education, active range of motion, soft tissue dehydration and/or hypovolemia initiates a
mobilization, balance retraining, trigger point sympathetic nervous system reflex mani-
injections, muscle relaxants, and soft collars are fested by an increase in heart rate and
typical treatments.31-34,36-39 contractile force in order to sustain mean
arterial pressure. In addition, the tachycardic
■ Cholesteatoma response is highly correlated with the degree
Chief Clinical Characteristics of hypovolemia. The individual may sense
This presentation typically includes gradual this increase in heart rate and contractility as
onset, persistent intermittent discharge from palpitations. In most cases, the rhythm is
the ear, and, rarely, dizziness and facial sinus tachycardia and the therapist will note
palsy.40,41 While not often painful, infection a rapid but regular pulse that may be slightly
can occasionally occur, causing pain and diminished. Dehydration can be avoided by
swelling behind the ear. reminding the individual to drink fluids dur-
ing exercise sessions in the clinic, especially if
Background Information the exercise is aerobic and will be maintained
This is a rare condition caused by a skin cyst for greater than 20 to 30 minutes. Mild hypo-
behind the eardrum, which grows into the volemia necessitates fluid replacement,
middle ear and mastoid. The cyst gradually which may be accomplished by having the
expands, generally causing destruction of the individual lie semirecumbent and drink
eardrum and ossicles, and can erode the sur- water as the therapist monitors signs and
rounding bone; if it progresses to the point of symptoms. More severe cases of hypovolemia
eroding the bony labyrinth, dizziness will occur. usually require activation of the emergency
Most cases are congenital, and some are ac- medical system and administration of intra-
quired due to metaplastic tissue, or overgrowth venous fluids by appropriate health care
of tympanic membrane epithelial cells after a personnel.
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Chapter 27 Hyperthyroidism/Thyrotoxicosis 535

■ Dysrhythmias/Arrhythmias Background Information

DIZZINESS
Chief Clinical Characteristics This condition includes hereditary motor and
This presentation involves light-headedness, sensory neuropathy, hereditary sensory neu-
and associated symptoms of palpitations, short- ropathy, hereditary motor neuropathy, and
ness of breath, chest discomfort, headache, hereditary sensory and autonomic neuropa-
slurred speech, fatigue, lethargy, and anxiety. thy. The majority of neuropathies composing
this condition are Charcot-Marie-Tooth neu-
Background Information ropathy. This condition is caused by genetic
A variety of pathologies result in this condition, abnormalities. Diagnosis is made by nerve
including myocardial infarction, sick sinus syn- conduction and electromyographic studies.
drome, pacemaker failure, electrolyte imbalance, Prognosis for hereditary sensory neuropathies
pregnancy, dehydration/hypovolemia, Wolff- is poor due to intractable pain.42 Prognosis for
Parkinson-White syndrome, and congestive hereditary motor and sensory neuropathies
heart failure. If palpable, the pulse is abnormal. has also been found to be unfavorable due
Pulse may be either more rapid or slower than to slowing of conduction velocity with age.43
expected, and either regular or irregular in beat. Intervention is typically directed at the under-
Blood pressure may be abnormal. Cardiac aus- lying cause when possible.
cultation may be abnormal. Referral to a physi-
cian or activating the emergency medical system ■ Herpes Zoster Oticus (Ramsay
is warranted, particularly for individuals with a Hunt Syndrome)
new onset or changing signs and symptoms. Chief Clinical Characteristics
This presentation commonly includes intense ear
■ Exposure to Toxic Chemicals pain; a rash around the ear, mouth, face, neck,
Chief Clinical Characteristics and scalp; and paralysis of facial nerves. Other
This presentation includes acute onset of dizzi- symptoms may include hearing loss, vertigo,
ness associated with exposure to chemical and tinnitus. Taste loss in the tongue and dry
irritants. mouth and eyes may also occur. Sensory losses
precede facial paralysis. Vestibular deficits are
Background Information
more frequent than hearing loss (80% vs. 26%,
Any individual who reports dizziness or light-
respectively), although hearing loss may go
headedness should be asked about potential
unnoticed.44
exposure to toxic chemicals. There should be a
temporal association between the exposure Background Information
and the onset of symptoms. Common chemi- This condition is a common complication of
cals include household cleaners, insect sprays, herpes zoster. This condition is an infection
fertilizers, and paints. The most common toxic caused by the spread of varicella-zoster virus,
cause of acute vertigo is ethyl alcohol. Position which is the virus that causes chickenpox, to
changes during a hangover exacerbate vertigo, facial nerves. This condition occurs in people
possibly due to the production of a density who have had chickenpox and represents a
gradient from the different diffusion rates of reactivation of the dormant varicella-zoster
alcohol into the endolymph and the cupula, virus. When treatment is needed, medications
rendering the cupula gravity sensitive. Other such as antiviral drugs or corticosteroids may
toxic agents include organic compounds of be prescribed. Vertigo also may be treated with
heavy metals. Referral to a physician for evalu- the drug diazepam.
ation and treatment is warranted.
■ Hyperthyroidism/Thyrotoxicosis
■ Hereditary Neuropathies Chief Clinical Characteristics
Chief Clinical Characteristics This presentation involves high cardiac output,
This presentation includes distal sensory abnor- hypertension, dyspnea (orthopnea, exertional
malities, such as numbness and tingling of the feet, dyspnea, and paroxysmal nocturnal dyspnea),
and muscle weakness of distal musculature. and dysrhythmias associated with palpitations
Individuals affected with this condition may also that may lead to feelings of dizziness or
report sweating and dizziness upon standing. light-headedness.45–47 Associated signs and
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536 Chapter 27 Hypoglycemia

symptoms include nervousness, heat intoler- from excess ingestion of insulin/oral hypo-
DIZZINESS

ance, fatigue, weight loss despite increased glycemic agents or insufficient food intake in
appetite, sweating, tremors, and exophthalmos. relation to insulin/oral hypoglycemic dose. In
individuals without diabetes mellitus, hypo-
Background Information
glycemia may result from insufficient caloric
A supportive clinical finding is a minimal de-
intake or an abnormal increase in physical
crease in resting heart rate during the individ-
activity or exercise in the absence of proper
ual’s sleeping hours, which may be confirmed
nutrition. The onset of signs and symptoms
by nocturnal heart rate monitoring. Palpita-
typically occurs when blood sugar falls below
tions in these individuals are typically chronic,
50 mg/dL and findings can be divided into two
felt during resting states, and exaggerated with
categories: those related to the activation of
activity. This condition results from overactiv-
the autonomic nervous system and those
ity of the thyroid gland and primarily results
caused by altered cerebral function. The diagno-
in elevated levels of thyroid hormones in the
sis is confirmed by blood glucose testing. Having
bloodstream, while thyrotoxicosis refers to the
the individual immediately ingest a source of
clinical syndrome resulting from hyperthy-
concentrated carbohydrate such as sugar, honey,
roidism. Typical etiologies of hyperthyroidism
candy, or orange juice can readily reverse the
include Graves’ disease, excessive thyroid hor-
condition. The practitioner should monitor vital
mone replacement therapy, toxic adenoma,
signs until they return to normal values. The
thyroiditis, goiter, and hyperthyroidism due to
therapist should be prepared to administer sup-
amiodarone and iodine-containing radi-
portive care or activate the emergency medical
ographic contrast agents. Thyroid hormones
services should the individual lapse into a dia-
are known to enhance myocardial contractility
betic coma (typically preceded by convulsions
and elevate the body’s metabolic rate, leading
and unresponsiveness). Individuals with dia-
to arterial vasodilation and possible hypoten-
betes mellitus should be reminded to check
sion. A reflex tachycardia may ensue to coun-
blood glucose levels periodically and to avoid
teract the hypotension. The most common
exercising in combination with the peak insulin
dysrhythmia associated with hyperthyroidism
effect to avoid episodes of hypoglycemia.
is sinus tachycardia, although supraventricular
dysrhythmias (particularly atrial fibrillation) ■ Hypothyroidism
can occur and may pose a serious health risk Chief Clinical Characteristics
for individuals with known coronary artery This presentation may be characterized by dizzi-
disease or history of stroke. Often, diagnosis is ness in combination with edema of the eyelids,
made by blood test on the basis of elevated thy- face, and dorsum of the hand. Myxedema also
roid hormone levels. If the therapist suspects may be present.
hyperthyroidism in a previously undiagnosed
individual, the individual should be referred to Background Information
a physician for definitive assessment. This condition develops as a result of de-
creased production or levels of T4 and T3 hor-
■ Hypoglycemia mones.3,28 This condition may be suspected if
Chief Clinical Characteristics an individual presents with pretibial edema
This presentation commonly involves headache, and unusual fatigue that does not improve
slurred speech, dizziness, feelings of “vague- with rest.28,29 Lab values for thyroid-stimulating
ness,” palpitations, impaired motor function, hormone are elevated and can be detected
anxiety, sweating, hypotension, and sinus before abnormal plasma levels of T3 and T4
tachycardia.48 hormones are observed. Blood values for
serum T3 and free T4 are elevated, while serum
Background Information TSH is decreased and radioactive iodine up-
The sensation of palpitations is quite often a take is increased. Thyroid hormone replace-
reflex sinus tachycardia in response to the hy- ment therapy is given for individuals with this
poglycemic insult and resolves quickly after condition. In many cases, the thyroid gland is
the condition is corrected. This condition in surgically removed and hormone replacement
individuals with diabetes mellitus may result therapy is required.
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Chapter 27 Lateral Medullary Infarction (Wallenberg’s Syndrome) 537

■ Idiopathic Intracranial form of vestibular habituation exercises is

DIZZINESS
Hypertension warranted postsurgically.
Chief Clinical Characteristics ■ Labyrinthitis and Neuronitis
This presentation includes headaches, visual
disturbances, dizziness, or tinnitus.49 Chief Clinical Characteristics
This presentation may be characterized by a
Background Information relatively sudden onset of severe, constant ro-
Diagnostic criteria include the presence of tary vertigo (made worse by head movement)
symptoms that reflect generalized intracranial that resolves after days or weeks.1,51 This con-
hypertension or papilledema, elevated in- dition is associated with spontaneous nystag-
tracranial pressure (>250 mm H2O per lumbar mus, postural imbalance, and nausea without
puncture), normal cerebrospinal fluid compo- accompanying cochlear or neurological symp-
sition, absence of lesions on magnetic reso- toms.51 The specific presentation varies depend-
nance imaging or computed tomography, and ing on the site of the infection. If the vestibular
no other cause identified.50 Also known as system is affected, the symptoms will include
pseudotumor cerebri or benign intracranial dizziness and difficulty with vision and/or
hypertension, this condition is characterized balance. If the inflammation affects the cochlea,
by increased intracranial pressure without as- this condition will produce disturbances in
sociated space-occupying lesions or hydro- hearing, such as tinnitus or hearing loss. The
cephalus. Approximately one-third of individ- symptoms can be mild or severe, temporary or
uals with idiopathic intracranial hypertension permanent, depending on the severity of the
recover within 6 months following repeated infection.
lumbar punctures and drainage of cere-
brospinal fluid to maintain the pressure at Background Information
near normal or normal levels. Weight reduc- The etiology is a bacterial or viral infection
tion and surgical gastric placation are effective causing inflammation of the vestibular nerve
forms of treatment.18 (neuronitis) or the labyrinth (labyrinthitis).
The terms are often used interchangeably be-
■ Labyrinthine Concussion cause it is difficult to distinguish neuronitis
Chief Clinical Characteristics from labyrinthitis.1 The diagnostic hallmark is
This presentation commonly involves acute signs unilateral hyporesponsiveness with caloric
of vertigo, leading to constant unsteadiness, testing.51 Regardless of the type of infection,
which worsens in darkness and during fatigue, the treatment consists of destroying the bacte-
and results in motion intolerance.20 The symp- ria by means of antibiotics. If the labyrinthitis
toms are generally chronic, because the vestibu- is caused by a break in the membranes separat-
lar disturbance remains uncompensated. There ing the middle and inner ears, surgery may
is significant overlap in clinical presentation also be required to repair the membranes to
between this condition and brainstem–eighth prevent a recurrence of the disease. Residual
nerve complex injury. vertigo can be reduced with vestibular habitu-
ation exercises.52
Background Information
A reduced vestibular response is apparent on ■ Lateral Medullary Infarction
caloric testing and rotary chair tests show (Wallenberg’s Syndrome)
gain asymmetry and an increased phase lag. Chief Clinical Characteristics
Posturography is typically abnormal. The ex- This presentation can include nystagmus, oscil-
act etiology is unknown but damage to lopsia, vertigo, nausea, vomiting, impairment
the semicircular canal epithelium is an ac- of pain and thermal sense over half of the body,
cepted cause. Magnetic resonance imaging ipsilateral Horner syndrome including miosis,
will reveal hemorrhage in the semicircular ptosis, anhidrosis, hoarseness, dysphagia, ipsi-
canals, differentiating this condition from a lateral paralysis of palate and vocal cord with
brainstem–eighth nerve complex injury. Treat- a diminished gag reflex, vertical diplopia or
ment is labyrinthectomy or selective vestibular sensation of tilting vision, ipsilateral ataxia of
nerve section. Vestibular rehabilitation in the limbs, loss of balance to ipsilateral side, and
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538 Chapter 27 Mal de Débarquement

impaired sensation of ipsilateral half of with dizziness, appear to be ineffective. Two-


DIZZINESS

the face.18,53,54 thirds of individuals find small benefit from


vestibular rehabilitation.
Background Information
In 75% of cases, onset was sudden. Main etio- ■ Ménière’s Disease
logic factors associated with this condition Chief Clinical Characteristics
include large-vessel infarction, arterial dissec- This presentation is characterized by recurrent
tion, small-vessel infarction, cardiac embolism, episodic spontaneous attacks of vertigo (exac-
tumor, hemorrhage, and other unknown erbated by head movements and accompanied
factors. The most typical cause is occlusion of by nausea and vomiting), fluctuating sen-
the vertebral artery, with the posterior inferior sorineural hearing loss, aural fullness, and tin-
cerebellar artery being involved to a lesser de- nitus.51,56–63 In the late stages the symptoms are
gree. A thorough history, clinical examination, more severe, hearing loss is less likely to fluctu-
and magnetic resonance imaging may confirm ate, and tinnitus and aural fullness may be
the diagnosis. An emergency response is re- more constant. Sudden unexplained falls with-
quired for individuals experiencing new onset of out loss of consciousness or vertigo (drop
symptoms. Residual symptoms include balance attacks) may occur. Attacks can be preceded by
deficits (most common), dysphagia, dizziness, an aura consisting of a sense of fullness in the
and numbness, which may be addressed with ear, increasing tinnitus, and a decrease in hear-
physical, occupational, and speech therapy. ing,61 but can also occur without warning. The
■ Mal de Débarquement typical duration of an attack is 2 to 3 hours, rang-
ing from minutes to hours in length. Attacks
Chief Clinical Characteristics can be single or multiple, with short or long in-
This presentation may involve vague concerns of tervening periods of remission during which
unsteadiness and disequilibrium, as well as the the individual may be asymptomatic.
illusion of movement such as rocking or swaying.
Tilting, nausea, headache, and jumping or Background Information
blurred vision are also associated concerns.55 The cause of this condition is thought to be
overproduction or underabsorption of en-
Background Information dolymph (endolymphatic hydrops), resulting
Symptoms generally ease during actual move- in distortion of the membranous labyrinth.
ment. Rotational vertigo is not a concern, but Ruptures of the membranous labyrinth, au-
other otologic symptoms such as fullness, tin- toimmune disease processes, and viral infec-
nitus, hyperacusis, otalgia, and decreased hear- tion are among other proposed causes.61
ing may be reported. The symptoms typically Appropriate tests include caloric testing to de-
follow travel by sea, air, or train, space flight, or termine amount of vestibular asymmetry; au-
experience within a slowly rotating room. diometry, which often shows a low-frequency
Duration of symptoms is important to making loss more often than high frequencies; and
the differential diagnosis; although postmo- exclusion of other causes (typically with
tion vertigo lasting up to 48 hours is not gadolinium-enhanced magnetic resonance
uncommon (referred to as “landsickness”), imaging). The head thrust test can be negative
this condition persists for longer periods. despite caloric asymmetry. Restricting salt
Although the cause is unknown, the strong intake and using diuretics may be useful in
predominance of this condition in women of over half of individuals with this condition.
postmenarchal and premenopausal ages sug- Approximately 10% of individuals with this
gests that female hormones may play a role. condition will have persistent vertigo and re-
Another theory is persistent central nervous quire other forms of treatment. These include
system adaptation to the moving environ- surgery to decompress or drain the endolym-
ment. Medications such as benzodiazepines, phatic sac, selective vestibular neurectomy or
amitriptyline hydrochloride, methazolamide, labyrinthectomy, and intratympanic injections
Fioricet, Percocet, and morphine have had of aminoglycosides such as gentamicin58 to
some success at reducing symptoms. Meclizine reduce or abolish vestibular function on the
and scopolamine, typically given to individuals affected side.
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Chapter 27 Mitral Valve Prolapse 539

MIGRAINE-RELATED DIZZINESS posterior fossa tumors, transient ischemic

DIZZINESS
■ Basilar Migraines attacks (shorter duration, lasting only a few
minutes), Ménière’s disease, and vestibular
Chief Clinical Characteristics paroxysmia. In addition to pharmacother-
This presentation consists of two or more neu- apy, vestibular rehabilitation may help, as
rological problems (vertigo, tinnitus, de- may education regarding avoiding mi-
creased hearing, ataxia, dysarthria, visual graine triggers such as stress, nicotine,
symptoms in both hemifields of both eyes, estrogen, and foods known to exacerbate
diplopia, bilateral paresthesia or paresis, de- migraines.
creased level of consciousness) followed by a
throbbing headache. It occurs in individuals ■ Vestibular Migraine
primarily before age 20 years, and the dura- Chief Clinical Characteristics
tion of symptoms is 5 to 60 minutes. This presentation may include episodic ver-
Background Information tigo, primarily rotational but also of the rock-
This condition is a form of migraine with ing type.65–67 The vertigo often occurs before
aura. Audiograms are often normal. Diag- or with a headache but not always (32% to
nostic clues include having been diagnosed 36%). Associated symptoms are phonophobia,
with migraine headaches or having a family photophobia, visual disturbances, imbalance,
history of migraines, but the diagnosis is one nausea, and vomiting. The duration is most
of exclusion. The diagnosis can be substanti- often a few minutes to several hours but can
ated by medical efficacy in treating (ergota- be quite variable.
mines) and preventing (metoprolol, flunar- Background Information
izine) attacks, and exclusion of similar When symptom free, ocular motor signs
diagnoses, such as posterior fossa tumors, (saccadic pursuit, gaze-evoked nystagmus,
transient ischemic attacks (shorter duration, positional nystagmus, and spontaneous nys-
lasting only a few minutes), Ménière’s dis- tagmus) can still be found. Diagnostic clues
ease, and vestibular paroxysmia. In addition include having been diagnosed with mi-
to pharmacotherapy, vestibular rehabilita- graine headaches or having a family history
tion may help, as may education regarding of migraines, but the diagnosis is one of ex-
avoiding migraine triggers such as stress, clusion. The diagnosis can be substantiated
nicotine, estrogen, and foods known to exac- by medical efficacy in treating (ergotamines)
erbate migraines. and preventing (metoprolol, flunarizine) at-
tacks, and exclusion of similar diagnoses,
■ Benign Recurrent Vertigo such as posterior fossa tumors, transient is-
Chief Clinical Characteristics chemic attacks (shorter duration, lasting
This presentation can be characterized by ver- only a few minutes), Ménière’s disease, and
tigo spells, occasionally with tinnitus but with- vestibular paroxysmia. In addition to phar-
out hearing loss, with or without headaches, macotherapy, vestibular rehabilitation may
that last minutes to hours.64 help, as may education regarding avoiding
migraine triggers such as stress, nicotine,
Background Information estrogen, and foods known to exacerbate
If there is no headache, this may be referred migraines.
to as migraine aura without headache.
These spells occur between 20 and 60 years ■ Mitral Valve Prolapse
of age. Diagnostic clues include having Chief Clinical Characteristics
been diagnosed with migraine headaches This presentation may include palpitations,
or having a family history of migraines, but chest pain, shortness of breath, fatigue, and
the diagnosis is one of exclusion. The diag- light-headedness, and the individual may expe-
nosis can be substantiated by medical effi- rience periodic syncopal episodes.68 The great
cacy in treating (ergotamines) and prevent- majority of individuals with this condition are
ing (metoprolol, flunarizine) attacks, and asymptomatic and the condition may go undi-
exclusion of similar diagnoses, such as agnosed for years.
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540 Chapter 27 Multiple Sclerosis

Background Information viral infection, which subsequently targets


DIZZINESS

The palpitations are usually supraventricular myelin.18 The diagnosis may be confirmed by
in origin and occur paroxysmally. If palpable, a thorough history, physical examination,
the pulse is typically rapid and regular. magnetic resonance imaging, analysis of cere-
The palpitation episodes commonly are brospinal fluid, and evoked potentials.18,69,70
self-limiting and last several minutes (less of- Life expectancy and cause of mortality are
ten, for hours), during which the individual similar for all types of this condition.18 Clinical
may experience the other associated symp- characteristics that are associated with a longer
toms. Typically, the associated symptoms time interval for progression of disability include
follow a benign course. Upon cardiac auscul- female sex, younger age of onset, relapsing-
tation, a midsystolic click is often appreciated remitting type, complete recovery after the
best over the fifth intercostal space left of the first relapse, and longer time interval between
sternum. This may be followed by a late sys- first and second exacerbation.71 Medical
tolic murmur. This condition occurs when management may include the use of methyl-
one or both valve leaflets exhibit exaggerated prednisolone, prednisone, cyclophosphamide,
systolic bowing beyond the mitral annulus. immunosuppressant treatment, and beta inter-
This diagnosis is confirmed with echocardio- feron.18 Physical, occupational, and speech
graphy or angiography. Individuals diag- therapy may be indicated to prevent secondary
nosed with this condition may be instructed sequelae and to optimize functional activity
by their physician to cough forcefully or per- and mobility. Some individuals may benefit
form a Valsalva maneuver (bearing down from psychological/psychiatric and social sup-
against a closed glottis) during episodes of port as the disease progresses.
palpitations in an effort to break the abnor-
mal rhythm through vagal mediation. Quite ■ Multiple System Atrophy with
often, these individuals are also prescribed Orthostatic Hypotension
calcium channel blockers or beta blockers to (Striatonigral Degeneration,
suppress the occurrence of palpitations. Oc- Olivopontocerebellar Atrophy,
casionally, the tachycardia may be prolonged Shy-Drager Syndrome)
and immediate medical intervention is usu- Chief Clinical Characteristics
ally warranted, especially if the individual has This presentation involves tremor, rigidity, aki-
underlying coronary artery disease and be- nesia, and/or postural imbalance along with
comes hemodynamically unstable. signs of cerebellar, pyramidal, and autonomic
dysfunction. Autonomic symptoms such as or-
■ Multiple Sclerosis thostatic hypotension, dry mouth, loss of sweat-
Chief Clinical Characteristics ing, impotence, and urinary incontinence or
This presentation may include paresthesias, retention are the initial feature in 41% of in-
weakness, spasticity, hypertonicity, hyperreflexia, dividuals, with 74% to 97% of individuals
positive Babinski, incoordination, optic neuri- developing some degree of autonomic dysfunc-
tis, ataxia, vertigo, dysarthria, diplopia, bladder tion during the course of the disease.72 This
incontinence, tremor, balance deficits, falls, and condition is a combination of parkinsonian
cognitive deficits.18 and non-parkinsonian symptoms and signs.
Background Information Background Information
This condition may present as relapsing- Diagnostic criteria are based on the clinical
remitting, primary progressive, or secondary presentation, which includes poor response to
progressive. The disease occurs most fre- levodopa, presence of autonomic features,
quently in women between the ages of 20 and presence of speech or bulbar problems, absence
40 years. Only a small number of children or of dementia, absence of toxic confusion, and
individuals between 50 and 60 years are diag- presence of falls.73 The disease course ranges
nosed with this condition.18 This condition between 0.5 and 24 years after diagnosis with a
was originally thought to be secondary to mean survival time of 6.2 years.74 This condi-
environmental and genetic factors, but evi- tion is a progressive condition of the central
dence suggests an autoimmune response to a and autonomic nervous systems that rarely
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Chapter 27 Otosyphilis 541

occurs without orthostatic hypotension. severe forms of this condition, individuals may

DIZZINESS
There are three types of this condition. The experience seizures, transient ischemic attacks,
parkinsonian-type includes symptoms of or syncope.
Parkinson’s disease such as slow movement,
Background Information
stiff muscles, and tremor. The cerebellar type
This condition is caused by a sudden decrease
causes problems with coordination and speech.
of greater than 20 mm Hg in systolic blood
The combined type includes symptoms of both
pressure or greater than 10 mm Hg in diastolic
parkinsonism and cerebellar failure. Older age
blood pressure that occurs when a person as-
at onset is associated with a shorter survival
sumes a standing position. It may be caused by
time.74 Average age of onset is 54 years, with
hypovolemia resulting from the excessive use
mean age at death being 60.3 years.72 Most in-
of diuretics, vasodilators, or other types of va-
dividuals with this condition receive a trial of
soactive medications (eg, calcium channel
levodopa although only a minority respond.72
blockers and beta blockers), dehydration, or
Additional treatment addresses symptoms and
prolonged bed rest. Other factors to consider
involves physical and occupational therapy to
include the individual’s neurological status
maintain mobility and address safety issues re-
and hemorrhagic/hypovolemic states. The
lated to the progression of imbalance.
condition may be associated with Addison’s
■ Neurosarcoidosis disease, atherosclerosis, diabetes, and certain
neurological conditions including Shy-Drager
Chief Clinical Characteristics
syndrome and other dysautonomias. Hypov-
This presentation may be characterized
olemia due to medications is reversed by ad-
by facial palsy, impaired taste, sight, smell, or
justing the dosage or by discontinuing the
swallowing, vertigo, loss of sensation in a
medication. If prolonged bed rest is the cause,
stocking/glove pattern, and weakness in a dis-
improvement may occur by sitting up with in-
tal greater than proximal distribution.18
creasing frequency each day. In some cases,
Background Information physical counterpressure such as elastic hose
This condition is a manifestation of sarcoido- or whole-body inflatable suits may be re-
sis with central and/or peripheral nervous quired. Dehydration is treated with salt and
system involvement. It is characterized by for- fluids. Individuals with this condition can be
mation of granulomas in the central nervous instructed to rise slowly from bed in the morn-
system. The lesion consists of lymphocytes ings or when moving from a sitting/squatting
and mononuclear phagocytes surrounding a to standing position. Symptoms usually dissi-
noncaseating epithelioid cell granuloma. pate when the individual is placed in a semire-
These granulomas represent an autoimmune cumbent or supine position, although some
response to central nervous system tissues. individuals may progress to frank syncope. In
This condition includes 5% of individuals this case, the clinician should be prepared to
with sarcoidosis. The diagnosis is established activate the emergency medical system if the
by the presence of clinical features, along with individual fails to regain consciousness with
clinical and biopsy evidence of sarcoid granu- basic life support measures.
lomas in tissues outside the nervous system.
Approximately two-thirds of individuals expe- ■ Otosyphilis
rience this illness only once, whereas the re- Chief Clinical Characteristics
mainder experience chronic relapses. Primary This presentation may involve severe episodic
treatment for neurosarcoidosis is the adminis- vertigo, fluctuating hearing loss, low-
tration of corticosteroids. frequency hearing loss in the early stages of the
disease, and flat audiometric patterns in the
■ Orthostatic Hypotension later stages of the disease.75,76 The hearing
Chief Clinical Characteristics loss is usually bilateral in most individuals; the
This presentation commonly involves dizziness, loss in speech discrimination is usually out of
light-headedness, and blurred vision that gen- proportion to the speech reception threshold.
erally occur after sudden standing. In more Vestibular disturbances could be present in
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542 Chapter 27 Paraneoplastic Syndromes

as many as 80% of individuals with this con- including hypotonia, ataxia, irritability, trun-
DIZZINESS

dition. This condition’s presentation is simi- cal ataxia, gait difficulty, balance deficits, and
lar to that of Ménière’s disease. frequent falls. Stiff-man syndrome presents
with spasms and fluctuating rigidity of axial
Background Information
musculature, legs, and possibly shoulders,
Otosyphilis is caused by the spirochete Tre-
upper extremities, and neck. Paraneoplastic
ponema pallidum. Both congenital and ac-
sensory neuropathy presents with asymmet-
quired forms of syphilis infection can lead to
ric, progressive sensory alterations involving
this condition with subsequent degeneration
the limbs, trunk, and face, sensorineural
of the audiovestibular system. Histopathologi-
hearing loss, autonomic dysfunction, and
cal findings are identical for both the congeni-
pain. Other conditions in this category in-
tal and acquired forms. The underlying
clude vasculitis, Lambert-Eaton myasthenia
syphilis infection causes meningoneuro-
syndrome, myasthenia gravis, dermatomyosi-
labyrinthitis in the early congenital form and
tis, neuromyotonia, and various neu-
in the acute period of the secondary and terti-
ropathies.18,77 These conditions result from
ary acquired forms. It causes temporal bone
an immune-mediated response to the pres-
osteitis with secondary involvement of the
ence of tumor or metastases. Antibodies or
membranous labyrinth in the late congenital,
T cells respond to the presence of the
late latent, and tertiary syphilis stages. En-
tumor, but also attack normal cells of the
dolymphatic hydrops and degenerative
nervous system.78,79 Over 60% of individuals
changes in the sensory and neural structures
present with this condition prior to the dis-
are seen in both the congenital and acquired
covery of the cancer.77 The underlying tumor
forms. Poor prognosis is indicated by en-
is treated according to the type of cancer.
dolymphatic sac obstruction by microgum-
Additional treatment is dependent on this
mata. The goal of treatment is to halt the pro-
condition’s type and may include steroids,
gression of the disease. Treatment includes
plasmapheresis, immunotherapy, chemother-
antibiotic and steroidal anti-inflammatory
apy, radiation, or cyclophosphamide.77 Physi-
medication, and the medication of choice dif-
cal, occupational, and speech therapy may be
fers with the clinical stage of the disease.
indicated to address functional limitations.
■ Paraneoplastic Syndromes ■ Perilymph Fistula
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation commonly includes dizziness This presentation may involve dizziness, vertigo,
in combination with a variety of different imbalance, nausea, and vomiting.51 Some indi-
neurological symptoms and signs in an indi- viduals with this condition experience ringing or
vidual with cancer. Specific neurological fullness in the ears, and many notice a hearing
symptoms and signs depend on the location loss. Most people with fistulas find that their
of involvement of the central or peripheral symptoms get worse with changes in altitude
nervous system. (elevators, airplanes, travel over mountain
passes) or air pressure (weather changes), as well
Background Information
as with exertion and activity.
Paraneoplastic encephalomyelitis and focal
encephalitis may present with ataxia, vertigo, Background Information
balance deficits, nystagmus, nausea, vomit- The cause of this condition is a tear or defect
ing, cranial nerve palsies, seizures, sensory in the oval or round window in one or both
neuropathy, anxiety, depression, cognitive ears, allowing pressure changes in the middle
changes, and hallucinations. For individuals ear to stimulate the inner ear and cause symp-
presenting with ataxia, dysarthria, dysphagia, toms. Head trauma via a direct blow and
and diplopia, paraneoplastic cerebellar de- whiplash injury, barotrauma sustained during
generation may be suspected. Paraneoplastic diving, weightlifting, and childbirth are among
opsoclonus/myoclonus tends to affect both common causes. This condition also may
children and adults with signs and symptoms be present from birth or may result from
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Chapter 27 Spinal Metastases 543

chronic, severe ear infections. Clinical tests emergency medical system if the individual

DIZZINESS
suggestive of a fistula include reproduction of fails to regain consciousness with basic life
symptoms during a Valsalva maneuver or support measures.
when applying pressure to the external audi-
tory canal. The definitive diagnosis is made by ■ Pregnancy
direct visualization during tympanotomy. Sur- Chief Clinical Characteristics
gical repair with grafting is indicated if symp- This condition is commonly characterized by re-
toms persist despite nonsurgical care consist- ports of palpitations associated with shortness
ing of activity restriction aimed at avoiding of breath, dizziness, presyncope, or syncope in
lifting, straining (Valsalva maneuver), bending a pregnant woman.
over, and changes in pressure.
Background Information
■ Postural Tachycardia Syndrome During pregnancy, significant changes occur in
hormonal and hemodynamic function that
Chief Clinical Characteristics
predispose women to the development of dys-
This presentation includes rapid heartbeat,
rhythmias. Changes in hormone levels during
light-headedness or dizziness with prolonged
pregnancy (particularly progesterone) have
standing, headache, chronic fatigue, exercise
been associated with enhanced sympathetic ac-
intolerance, weakness, hyperpnea or dyspnea,
tivity and the precipitation of dysrhythmias.81
tremulousness, nausea/abdominal pain, sweat-
The enhanced maternal blood volume and as-
ing, anxiety/palpitations, chest pain, and other
sociated increase in stroke volume may lead to
nonspecific concerns.
the sensation of a forceful, bounding pulse in
Background Information some individuals. The type of dysrhythmia
This condition is characterized by orthostatic often associated with palpitations is often sinus
intolerance associated with a pulse rate that tachycardia or supraventricular tachycardia.
increases 30 beats per minute or greater when Very rarely is atrial fibrillation or ventricular
the individual moves from a supine to stand- tachycardia the source of the dysrhythmia.82
ing position.80 Causes usually are not identi- The episodes are usually benign and self-
fied but symptoms are related to reduced limiting, can often be associated with shortness
cerebral blood flow associated with inadequate of breath, and there may be an increased occur-
systemic venous return to the right heart. rence of symptoms to term.83 At times, the pal-
Reversible causes such as low blood volume pitations may also be associated with dizziness,
should be ruled out. Treatment depends on presyncope, or frank syncope. If palpitations
the severity of the symptoms. This condition are associated with these latter symptoms, the
is self-limiting, but individuals are usually individual should be referred back to her pri-
advised to increase their fluid and salt intake. mary care physician for evaluation. In the event
Body stockings may provide some relief. Drug of cardiovascular compromise, the therapist
therapy, with fludrocortisone, beta blockers, should be prepared to provide supportive in-
midodrine, or clonidine, can be beneficial. terventions and activate the emergency med-
Physical exercise, such as walking and gluteal ical system if warranted.
and calf muscle resistance training, also may
help.80 Some individuals may require and ■ Spinal Metastases
benefit from insertion of a cardiac pacemaker. Chief Clinical Characteristics
Individuals with this condition can be This presentation can involve spasticity, weak-
instructed to rise slowly from bed in the morn- ness, sensory alterations, bowel and bladder
ings (eg, sitting at the edge of the bed and incontinence, neck pain, back pain, radicular
performing ankle/calf exercises) or when pain, atrophy, cerebellar signs, balance deficits,
going from a sitting/squatting to standing falls, and cranial nerve involvement.18,84,85
position. Symptoms usually dissipate when the
individual is placed in a semirecumbent or Background Information
supine position, although some individuals This condition is the most frequent neoplasm
may progress to frank syncope. In this case, the involving the spine.85 The most common types
therapist should be prepared to activate the and locations of primary tumors that result in
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544 Chapter 27 Spinal Primary Tumors

spinal metastases include breast, lung, lym- transmitted to the canal. Clinical tests suggestive
DIZZINESS

phoma, prostate, kidney, gastrointestinal tract, of superior canal dehiscence are reproduction
and thyroid.18,86 The diagnosis is confirmed of symptoms with coughing, tragal pressure, or
with gadolinium-enhanced magnetic reso- Valsalva maneuver. Computed tomography and
nance imaging and computed tomography.18,85 click-evoked myogenic potentials are important
Treatment is variable depending on the diagnostic tools. Treatment involves plugging
tumor and may include surgical resection, the dehiscence surgically.
chemotherapy, radiation, corticosteroids, and
rehabilitative therapies.18 Although the long- ■ Transient Ischemic Attack
term prognosis is poor, individuals without Chief Clinical Characteristics
paresis or pain and who are still ambulatory This presentation can include numbness or
have longer survival rates.86 weakness in the face, arm, or leg, especially on
one side of the body; confusion or difficulty in
■ Spinal Primary Tumors talking or understanding speech; trouble
Chief Clinical Characteristics seeing with one or both eyes; and difficulty with
This presentation may include spasticity, weak- walking, dizziness, or loss of balance and
ness, sensory alterations, bowel/bladder incon- coordination.
tinence, back pain, radicular pain, atrophy,
Background Information
cerebellar signs, balance deficits, falls, and
This condition is a transient stroke that lasts
cranial nerve involvement.18
only a few minutes. It occurs when the blood
Background Information supply to part of the brain is briefly inter-
Types of this condition include myeloma, neu- rupted. Symptoms of this condition, which
rofibroma, lymphoma, metastasis, menin- usually occur suddenly, are similar to those of
gioma, schwannoma, and astrocytoma. The stroke but do not last as long. Most symptoms
first test to diagnose brain and spinal column disappear within an hour, although they may
tumors is a neurological examination. Special persist for up to 24 hours. Because it is impos-
imaging techniques (computed tomography, sible to differentiate between symptoms from
magnetic resonance imaging, and positron this condition and acute stroke, individuals
emission tomography) are also employed. should assume that all stroke-like symptoms
Specific diagnoses may be confirmed with signal a medical emergency. A prompt evalua-
imaging and biopsy. Treatment is variable de- tion (within 60 minutes) is necessary to iden-
pending on the type, size, and location of the tify the cause of this condition and determine
tumor and may include surgical resection, appropriate therapy. Depending on the indi-
chemotherapy, radiation, corticosteroids, and vidual’s medical history and the results of a
rehabilitative therapies. Prognosis is variable medical examination, the doctor may recom-
and depends on the type and grade of tumor, mend drug therapy or surgery to reduce the
severity of compression, and duration of risk of stroke. Antiplatelet medications, partic-
compression. ularly aspirin, are a standard treatment for
individuals suspected of having this condition
■ Superior Canal Dehiscence and who also are at risk for stroke, including
Syndrome individuals with atrial fibrillation.
Chief Clinical Characteristics
This presentation typically involves recurrent at-
■ Traumatic Brain Injury
tacks of vertigo and oscillopsia (movement of the Chief Clinical Characteristics
visual field) induced by changes in intracra- This presentation typically includes disequilib-
nial or middle ear pressure or by loud noises. rium in the presence of cognitive changes, altered
level of consciousness, seizures, nausea, vomit-
Background Information ing, coma, dizziness, headache, pupillary
This condition is a variant of perilymph fis- changes, tinnitus, weakness, incoordination,
tula. It is caused by a dehiscence of the bone behavioral changes, spasticity, hypertonicity,
overlying the superior (anterior) semicircular cranial nerve lesions, sensory, and motor
canal, which allows pressure changes to be deficits.18,87
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Chapter 27 Vestibular Epilepsy (Vestibular Seizures) 545

Background Information system, usually the anterior inferior cerebellar

DIZZINESS
This condition can be classified as mild, mod- artery.91 Headache or neck pain was found to
erate, or severe based on the Glasgow Coma be the prominent feature in 88% of 26 individ-
Scale, length of coma, and duration of post- uals presenting with vertebral artery dissec-
traumatic amnesia.87 Magnetic resonance tion. The most common focal neurological
imaging may be used to confirm the diagno- symptom was vertigo (57%).92 In cases of stroke
sis.84 Treatment initiated at the scene of the following cervical spine manipulation pur-
accident and during the acute phase is focused ported to be due to vertebral artery dissection,
on medical stabilization. It should be initiated the presenting neurological symptoms were loss
during the acute phase in order to minimize of coordination (52%), dizziness/vertigo/
complications.88 Low Glasgow Coma Scale, nausea/vomiting (50%), speech/swallowing
longer length of coma, longer duration of dysfunction, visual disturbances, and numb-
post-traumatic amnesia, and older age tend to ness, nystagmus, loss of consciousness, hearing
be associated with poor outcomes.89 Optimal deficits/tinnitus, and death.93
rehabilitation is interdisciplinary and cus-
Background Information
tomized to address each specific individual’s
Less severe but similar symptoms may be ex-
disablement.
pected in lesser degrees of vertebrobasilar in-
■ Vasovagal Syncope sufficiency. The circulation to the inner ear
arises from the vertebrobasilar artery system,
Chief Clinical Characteristics
which leads to dizziness when this circula-
This presentation commonly includes dizziness,
tion is impaired.91,94 If the insufficiency is
as well as prodromal symptoms of nausea,
great enough to progress to stroke there
headache, paresthesias, light-headedness, dizzi-
are generally other associated neurological
ness, palpitations, shortness of breath, diaphore-
symptoms typical of central nervous system
sis, and chest pain.90 The therapist will usually
lesions but these can be absent.91,94 A classic
note the individual’s pulse to be rapid and reg-
sequela of stroke related to this condition is
ular, and blood pressure may be hypotensive.
lateral medullary (Wallenberg’s) syndrome.
Background Information Proposed causes of vascular compromise
Individuals susceptible to vasovagal syncope include trauma (including participation in
usually have difficulty standing for prolonged sports or manipulation of the cervical spine),
periods of time and exhibit delayed or dimin- cervical spine rotation and/or extension, and
ished neurocardiovascular responses when complications due to risk factors such as age,
assuming an upright posture. The precise gender, migraine headaches, hypertension,
mechanism responsible for this condition is diabetes, birth control pills, cervical spondy-
not well understood. Predisposing factors losis, and smoking.93,95,96 However, system-
include hypovolemia, anemia, and sympa- atic reviews of the literature suggest there
thetic blocking/antihypertensive medications. is no definitive way to identify who is at risk
The tilt-table test is the diagnostic procedure for vertebrobasilar insufficiency, either by
of choice for confirming vasovagal syncope, history, clinical examination, or special
and the individual should be referred to his or tests.93,95,97,98 Individuals suspected of having
her primary care physician for definitive this condition should be referred for medical
assessment. Treatment involves the individual evaluation urgently.
assuming a more recumbent position and
administering fluids. These treatments will ■ Vestibular Epilepsy (Vestibular
often cause the symptoms to abate. Seizures)
Chief Clinical Characteristics
■ Vertebrobasilar Artery This presentation can be characterized by sen-
Insufficiency sations of horizontal or vertical rotation, or of
Chief Clinical Characteristics falling and rising. Individuals may describe
This presentation commonly involves symp- vertigo and light-headedness as part of the
toms consistent with a cerebrovascular epileptic aura in complex partial seizures with
accident involving the vertebrobasilar artery temporal or extratemporal foci.99
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546 Chapter 27 Vestibular Ototoxicity

Background Information medications such as carboplatin and cisplatin.


DIZZINESS

Associated symptoms may be typical of simple Some medications produce a temporary


partial seizures (no loss of consciousness) or of dysfunction, while others produce permanent
complex partial seizures (loss of conscious- loss. Treatment involves removing exposure
ness) or of generalized tonic-clonic seizures to the medication if possible and post-exposure
(loss of consciousness and movements of the compensatory vestibular habituation and
extremities). There may be associated eye, balance exercises.
head, and body deviations or epileptic nystag-
mus. This condition is caused by focal dis- ■ Whiplash Injury
charge in the thalamus, temporal lobe, or pari- (Whiplash-Associated Disorder)
etal association cortex. Treatment commonly Chief Clinical Characteristics
includes antiepileptic medications. This presentation is characterized by a collection
of symptoms that occur following damage to
■ Vestibular Ototoxicity the neck, usually because of sudden extension and
Chief Clinical Characteristics flexion such as might happen in an automobile
This presentation includes fluctuating or accident. Symptoms commonly include non-
constant tinnitus and hearing loss ranging from specific dizziness in combination with neck pain,
mild to complete deafness if the cochlea is in- stiffness, headache, abnormal sensations such as
volved; vertigo, vomiting, nystagmus, and burning or prickling, or shoulder or back pain.21
imbalance if the vestibular system is involved uni- There may also be complaints of unsteadiness and
laterally; and headache, ear fullness, oscillop- visual disturbances, as well as signs of altered
sia, an inability to tolerate head movement, a postural stability, cervical proprioception, and
wide-based gait, difficulty walking in the dark, head and eye movement control.34
a feeling of unsteadiness and actual unsteadi-
Background Information
ness while moving, imbalance to the point of
Damage to the cervical spine, especially the up-
being unable to walk, light-headedness, and
per cervical region, leads to disruption of cer-
severe fatigue in bilateral involvement. Any
vicocephalic kinesthesia that may be responsi-
combination of the above is possible since either
ble for this condition.31,34,100,101 Sensorimotor
the cochlea or the vestibular system individu-
deficits are common. In addition, some people
ally, or both in combination, can be affected,
experience cognitive, somatic, or psychological
either unilaterally or bilaterally.
conditions such as memory loss, concentration
Background Information impairment, nervousness/irritability, sleep dis-
The severity, type, and particular combina- turbances, fatigue, or depression. Symptoms
tion of symptoms are variable, depending on such as neck pain may be present directly after
the medication exposure, whether it is unilat- the injury or may be delayed for several days.
eral or bilateral, the speed of onset, and the The condition may include injury to interverte-
individual. A slow unilateral loss may pro- bral joints, disks, and ligaments, cervical mus-
duce few symptoms since the brain can com- cles, and nerve roots. The trauma may dislodge
pensate through other mechanisms, whereas otoconia in the inner ear, leading to benign
a fast bilateral loss can produce significant paroxysmal positional vertigo, and complaints
disability. Because these symptoms are simi- of dizziness upon change of position.25 The
lar to many other conditions, the key diag- need for radiographs can be determined
nostic feature is a history of drug or chemical through a careful history; they are not always
exposure. Medications that are known to be necessary.102 Otolaryngologic evaluation may
vestibulotoxic include aspirin and quinine, reveal abnormal findings suggestive of central
loop diuretics (bumetanide [Bumex], ethacrynic and/or peripheral vestibular involvement.103,104
acid [Edecrin], furosemide [Lasix], torsemide Treatment for individuals with an acute
[Demadex]), aminoglycoside antibiotics whiplash may include pain medications, physi-
(amikacin, dihydrostreptomycin, gentamicin, cal therapy modalities, nonsteroidal anti-
kanamycin, Neomycin, netilmicin, ribostamycin, inflammatory drugs, antidepressants, muscle
streptomycin, tobramycin), and anticancer relaxants, and use of a cervical collar. Range of
1528_Ch27_526-549 07/05/12 1:58 PM Page 547

Chapter 27 References 547

motion and strengthening exercises are key 21. Herdman S, ed. Vestibular Rehabilitation. 2nd ed.

DIZZINESS
aspects of long-term management. Treatment Philadelphia, PA: F. A. Davis; 2000.
22. Hilton M, Pinder D. The Epley (canalith repositioning)
for decreased kinesthetic awareness involves manoeuvre for benign paroxysmal positional vertigo
eye–head–neck–trunk coordination exercises.39 [update of Cochrane Database Syst Rev. 2002;(1):
CD003162; PMID: 11869655]. Cochrane Database Syst
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CHAPTER 28
Loss of Balance and Falls
■ Robbin Howard, PT, DPT, NCS ■ Didi Matthews, PT, DPT, NCS

Description of the Symptom an individual coming to rest at a lower


surface.1
This chapter describes pathology that may
lead to a loss of balance and falls. A loss Special Concerns
of balance can be defined as unsteadiness ■ Sudden onset of balance problems or falls
when sitting, standing, or walking that may ■ Balance problems that occur after trauma
result in a stumble, trip, and/or fall. A fall that have not yet been discussed with a
is an unintentional event that results in physician

CHAPTER PREVIEW: Conditions That May Lead to a Loss of Balance and Falls

T Trauma
COMMON
Benign paroxysmal positional vertigo 554
Traumatic brain injury 566
UNCOMMON
Not applicable
RARE
Not applicable

I Inflammation
COMMON
Not applicable
UNCOMMON
Aseptic
Labyrinthitis and neuronitis 558
Multiple sclerosis 560
Septic
Encephalitis 556
Meningitis:
• Bacterial meningitis 559
• Viral meningitis 559
RARE
Aseptic
Behçet’s disease 554
Creutzfeldt-Jakob disease 556
Inflammatory muscle disease 557
Lyme disease (tick paralysis) 558

550
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Chapter 28 Loss of Balance and Falls 551

Inflammation (continued)

LOSS OF BALANCE AND FALLS


RARE
Paraneoplastic syndromes 563
Vasculitis (giant cell arteritis, temporal arteritis, cranial arteritis) 566
Septic
Neurosyphilis (tabes dorsalis, syphilitic spinal sclerosis, progressive locomotor ataxia) 562

M Metabolic
COMMON
Neuropathy secondary to diabetes mellitus 561
UNCOMMON
Not applicable
RARE
Vestibular ototoxicity 567

Va Vascular
COMMON
Orthostatic hypotension 562
Stroke (cerebrovascular accident) 565
UNCOMMON
Cerebral aneurysm 555
Transient ischemic attack 565
RARE
Lateral medullary infarction (Wallenberg’s syndrome) 558
Vasculitis (giant cell arteritis, temporal arteritis, cranial arteritis) 566
Vertebrobasilar artery insufficiency 566

De Degenerative
COMMON
Parkinson’s disease 563
UNCOMMON
Dementia with Lewy bodies 556
RARE
Corticobasal degeneration 555
Fahr’s syndrome (familial idiopathic basal ganglia calcification, bilateral striopallidodentate calcinosis) 557
Multiple system atrophy with orthostatic hypotension (striatonigral degeneration, olivopontocerebellar
atrophy, Shy-Drager syndrome) 560
Progressive supranuclear palsy (Richardson-Steele-Olszewski syndrome) 564
Spinocerebellar ataxia (spinocerebellar atrophy, spinocerebellar degeneration) 565

Tu Tumor
COMMON
Not applicable
(continued)
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552 Chapter 28 Loss of Balance and Falls

Tumor (continued)
LOSS OF BALANCE AND FALLS

UNCOMMON
Malignant Primary, such as:
• Brain primary tumors 554
Malignant Metastatic, such as:
• Brain metastases 554
Benign:
Not applicable
RARE
Malignant Primary, such as:
• Spinal primary tumors 565
Malignant Metastatic, such as:
• Spinal metastases 564
Benign, such as:
• Acoustic neuroma (vestibular schwannoma) 553
• Angiomatosis (Von Hippel-Lindau disease) 553
• Neurofibromatosis 560

Co Congenital
COMMON
Hydrocephalus 557
UNCOMMON
Not applicable
RARE
Not applicable

Ne Neurogenic/Psychogenic
COMMON
Peripheral neuropathy 564
UNCOMMON
Ménière’s disease 559
Neurological complications of acquired immunodeficiency syndrome 561
RARE
Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Loss of Balance balance on a daily basis.2 In addition, approxi-


and Falls mately one-third to one-fourth of the popula-
tion over the age of 65 has experienced a fall in
Any disease affecting cognition, alertness, the past 12 months.1 The etiology of imbalance
motivation, or planning also may affect balance.2 and falls may be associated with a single risk
It is common for a patient or family members factor or may be multifactorial in nature.
to report a history of falls or loss of balance. These risk factors are commonly categorized
Sixty-five percent of the population over the as being either intrinsic (related to the patient)
age of 60 may experience dizziness or loss of or extrinsic (related to the environment).1,3
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Chapter 28 Angiomatosis (Von Hippel-Lindau Disease) 553

Intrinsic risk factors may include: internal auditory canal, eventually growing out

LOSS OF BALANCE AND FALLS


into and compressing the brainstem. Audiome-
● Older age and female sex1,3
try, electronystagmography, auditory brainstem
● Gait deviations1,4 response, computed tomography, and magnetic
● Balance deficits1,4 resonance imaging with gadolinium contrast
● Weakness4,5 confirm the diagnosis, with the latter provid-
● Medications (eg, antidepressants, sedatives, ing the most specificity.9 Treatment is surgical
benzodiazepines, diuretics, analgesics, anti- removal of the tumor, with risk of permanent
hypertensives, anticoagulants, and anticon- changes in hearing, balance, and facial sensa-
vulsants)1,3,4 tion and motion. Loss of a preoperatively
● Diagnosis and possible comorbidities (eg, functioning vestibular nerve will result in
orthostatic hypotension, dizziness, syncope, dysequilibrium for which vestibular adapta-
Parkinson’s disease, polyneuropathy, motor tion exercises and balance rehabilitation are
neuron disease, epilepsy, transient ischemic appropriate.14
attacks, hydrocephalus, bilateral vascular
white matter disease, vestibular and brain- ■ Angiomatosis (Von
stem disease, and emotional conditions)1,4,5 Hippel-Lindau Disease)
● Visual impairments (eg, glaucoma, cataracts, Chief Clinical Characteristics
macular degeneration, and impaired visual This presentation commonly includes headaches,
acuity and depth perception)3,6 problems with balance and walking, dizziness,
● Altered mental status7 weakness of the limbs, vision problems, and high
blood pressure.
● Alcohol use.3
Extrinsic factors may include: Background Information
This condition is a rare, genetic multisystem
● Obstacles1,3 condition characterized by the abnormal
● Inadequate lighting1,3 growth of tumors in certain parts of the body
● Slippery surfaces1,3 (angiomatosis). Tumors of the central nervous
● Inadequate/inappropriate assistive device1 system are benign, are comprised of a nest of
● Poorly fitting shoes or no shoes.1 blood vessels, and are called hemangioblastomas
(or angiomas in the eye). Hemangioblastomas
may develop in the brain, the retina of the eye,
Description of Conditions That May
and other areas of the nervous system. Other
Lead to Loss of Balance and Falls
types of tumors develop in the adrenal glands,
■ Acoustic Neuroma (Vestibular the kidneys, or the pancreas. Cysts (fluid-filled
Schwannoma) sacs) and/or tumors (benign or cancerous)
may develop around the hemangioblastomas
Chief Clinical Characteristics and cause the symptoms listed above. Specific
This presentation may include hearing loss, symptoms vary among individuals and depend
tinnitus, vertigo, or dysequilibrium in the early on the size and location of the tumors. Indi-
stages.8–14 Vestibular nerve function may be viduals with this condition are also at a higher
gradually lost without much balance distur- risk than normal for certain types of cancer,
bance. Symptoms worsen as the tumor grows, and especially kidney cancer. Treatment varies
the individual may also report having a according to the location and size of the tumor
headache. If untreated, brainstem compression and its associated cyst. In general, the objective
can occur, with concerns of generalized headache, is to treat them when they are causing symp-
facial twitch and weakness, visual loss, diplopia, toms but are still small. Treatment of most
lower cranial nerve dysfunction causing aspira- cases usually involves surgical resection.
tion, hoarseness, dysphagia, shoulder weakness, Certain tumors can be treated with focused
tongue weakness, and long tract signs.9 high-dose irradiation. Individuals with this
Background Information condition need careful monitoring by a physi-
Acoustic neuromas arise from the Schwann cian and/or medical team familiar with the
cells of the eighth cranial nerve within the condition.
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554 Chapter 28 Behçet’s Disease

■ Behçet’s Disease rotated 45 degrees and tilted back while hang-


LOSS OF BALANCE AND FALLS

Chief Clinical Characteristics ing off the end of the table. This position will
This presentation may include bilateral pyram- elicit torsional and vertical nystagmus when
idal signs, headache, memory loss, hemiparesis, the vertical semicircular canals are involved.
cerebellar ataxia, balance deficits, sphincter Duration of vertigo while in the Hallpike po-
dysfunction, or cranial nerve palsies. In addi- sition will determine if the individual has
tion to these neurological signs, individuals canalithiasis (short duration, <1 minute) or
with this condition also may present with cupulolithiasis (long duration, >1 minute).
arthritis; renal, gastrointestinal, vascular, Turning the head while supine will elicit hor-
and cardiac diseases; and genital, oral, and izontal nystagmus and vertigo when the hor-
cutaneous ulcerations.15 izontal (lateral) semicircular canals are
involved. Nonsurgical treatment consisting of
Background Information a series of positional changes to move the de-
Mean age of onset is in the third decade of life. bris out of the canals, known as the canalith
Diagnostic criteria according to an interna- repositioning maneuver, can be very success-
tional study group include presence of recurrent ful.19,21–25 In recalcitrant cases, the offending
oral ulceration, recurrent genital ulceration, canal can be plugged surgically.26
eye lesions, skin lesions, papulopustular lesions,
and/or a positive pathergy test.15,16 Medical BRAIN TUMORS
treatment typically consists of corticosteroids ■ Brain Metastases
and immunosuppressants. Neurological symp-
toms tend to clear within weeks, but can some- Chief Clinical Characteristics
times recur or result in permanent deficits.16 This presentation may include headaches,
Onset before the age of 25 and male sex seizures, dysphagia, weakness, cognitive
indicate a poorer prognosis. changes, behavioral changes, dizziness, vom-
iting, alterations in the level of consciousness,
■ Benign Paroxysmal Positional ataxia, aphasia, nystagmus, visual distur-
Vertigo bances, dysarthria, balance deficits, falls,
Chief Clinical Characteristics lethargy, and incoordination.16,27
This presentation commonly involves dizziness Background Information
or vertigo (typically described as a spinning The majority of individuals with brain metas-
sensation), light-headedness, imbalance, and tases have been previously diagnosed with a
nausea. Symptoms are precipitated by a posi- primary tumor; however, a small percentage
tion change of the head relative to gravity, such of individuals are diagnosed concomitantly
as getting out of bed, rolling over in bed, or with brain metastases and the primary tumor.
looking up.17,18 The most common cancers resulting in subse-
Background Information quent brain metastases include lung, breast,
This condition is thought to occur when free- melanoma, colorectal, and genitourinary
floating debris becomes trapped in the semi- tract. The new onset of neurological symp-
circular canal (canalithiasis) or becomes adhered toms after a primary tumor warrants the use
to the cupula (cupulolithiasis), rendering the of imaging such as magnetic resonance imag-
semicircular canal sensitive to gravity and thus ing or computed tomography to confirm
changes in head position rather than head the diagnosis. Treatment may include corticos-
motion. The etiology of benign paroxysmal teroids, brain irradiation, surgery, chemother-
positional vertigo is unknown in most cases, apy, radiotherapy, and rehabilitative therapies.
but is associated with head trauma, vestibular The prognosis is poor with death typically
neuritis, and vertebrobasilar ischemia, and can occurring within 6 months.
occur after ear surgery or prolonged bed rest.19
This condition occurs more often in the ■ Brain Primary Tumors
elderly, and tends to recur in up to 15% Chief Clinical Characteristics
of cases within 1 year and 50% of cases within This presentation may include headaches,
40 months.20 The diagnosis is confirmed with seizures, dysphagia, weakness, cognitive
the Hallpike maneuver, in which the head is changes, behavioral changes, dizziness,
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Chapter 28 Corticobasal Degeneration 555

vomiting, alterations in the level of conscious- subarachnoid hemorrhage.30,31 Definitive

LOSS OF BALANCE AND FALLS


ness, ataxia, aphasia, nystagmus, visual dis- diagnosis is based on catheter angiography;
turbances, dysarthria, balance deficits, falls, however, magnetic resonance angiography,
lethargy, and incoordination.16 magnetic resonance imaging, and computed
tomography may aid in the diagnosis. Unrup-
Background Information tured aneurysms are sometimes surgically
With the excessive proliferation of cells, a treated. Aneurysm size, location, and prior his-
tumor mass eventually results in compres- tory of a subarachnoid hemorrhage help to
sion of the brain. This compression may determine if the risk of surgical treatment is
displace cerebrospinal fluid, thereby in- worth the potential benefits. Most aneurysms
creasing intracranial pressure and resulting that have hemorrhaged must be treated surgi-
in ischemia to the same tissues. Glioblastoma cally. Patients with a previous rupture are
multiforme, astrocytoma, oligodendroglioma, at an 11 times greater risk of having a second
metastatic tumors, primary central nervous intracranial aneurysm rupture. When aneurysms
system lymphomas, ganglioglioma, neurob- do rupture, many patients die within 1 month
lastoma, meningioma, arachnoid cysts, of the rupture, and those who survive often
hemangioblastoma, medulloblastoma, and have residual neurological deficits.28
acoustic neuroma/schwannoma are some of
the more common brain tumors. The first ■ Corticobasal Degeneration
test to diagnose brain and spinal column
Chief Clinical Characteristics
tumors is a neurological examination. Spe-
This presentation typically includes limb
cific diagnoses for brain tumors may be
ideomotor apraxia and unilateral parkinson-
confirmed with imaging and biopsy. Treat-
ism that is unresponsive to levodopa, gait
ment is variable depending on the type, size,
disturbances, tremor, postural instability, and
and location of the tumor and may include
dementia.32,33
surgical resection, chemotherapy, radiation,
corticosteroids, and rehabilitative therapies. Background Information
Prognosis is also variable and depends on A proposed set of criteria for the diagnosis of
the type and grade of tumor, severity of this condition includes core features of:
compression, and duration of compression.
1. Insidious onset and progressive course
2. No identifiable cause
■ Cerebral Aneurysm
3. Cortical dysfunction (ideomotor apraxia,
Chief Clinical Characteristics alien-limb phenomenon, cortical sensory
This presentation may involve loss of balance in loss, visual or sensory hemineglect, construc-
combination with a whole host of other neuro- tional apraxia, focal or asymmetric myoclonus,
logical symptoms and signs that depend on the apraxia of speech, or nonfluent aphasia)
affected cerebral tissue. Any associated signs or 4. Extrapyramidal dysfunction (rigidity that
symptoms may not be reported due to the fact does not respond to levodopa therapy and
that this condition is typically asymptomatic dystonia).34
prior to rupture. However, if the aneurysm
results in a mass effect, ischemia, or hemor- This condition is a sporadic disease with
rhage, then neurological signs and symptoms are an average age of onset of 63 years.33 There
dependent on the affected location.16,28,29 have been reports of familial cases; however,
for most cases there is no known cause.32 Diag-
Background Information nosis is made based on clinical presentation.
There has been some description of genetic A definitive diagnosis can only be made post-
factors in this condition.28 Cigarette smoking, mortem. Medical treatment is not typically
hypertension, and heavy alcohol use have all successful; it has been found that only about
been found to be correlated with increased risk 24% of these patients will respond to levodopa
of aneurysm development.28,29 Factors associ- therapy aimed at addressing the extrapyrami-
ated with increased risk of rupture include dal features of the disease.35 Physical and
size of aneurysm, location in the posterior circu- occupational therapy are used to maintain
lation, and a previous history of aneurismal mobility and address safety issues related to
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556 Chapter 28 Creutzfeldt-Jakob Disease

the progression of imbalance. Mean survival is syncope, transient loss of consciousness, and
LOSS OF BALANCE AND FALLS

7.9 years with a range of 2.5 to 12.5 years.33 sensitivity to antipsychotic and anti-Parkinson
Early presence of bilateral parkinsonism or medications.39
frontal lobe signs indicates a less favorable
prognosis.33 Background Information
This progressive condition is the second most
■ Creutzfeldt-Jakob Disease common dementia after Alzheimer’s disease.39
Chief Clinical Characteristics The specific etiology of this condition is
This presentation may involve rapidly progres- unknown. The characteristic Lewy bodies are
sive dementia, cerebellar ataxia, balance eosinophilic inclusion bodies found within the
deficits, myoclonus, cortical blindness, pyram- cytoplasm of neurons in the cerebral cortex
idal signs, extrapyramidal signs, and akinetic and limbic system.39 A thorough clinical
mutism.36 examination, laboratory screen, and imaging
are important to rule out other causes of
Background Information dementia. The definitive diagnosis for this
Different forms of this condition have been condition is made postmortem; however, it
described including sporadic, iatrogenic, and appears that the use of single-photon emission
variant. Sporadic and iatrogenic forms of this computed tomography and positron emission
condition typically affect older individuals, tomography may be useful in the identifica-
whereas the variant form affects younger tion of occipital hypoperfusion, which may be
individuals.37 The early stages of the variant associated with the visual hallucinations.39,40
form are characterized by psychiatric symp- Management includes caregiver education to
toms, including depression and anxiety.37 The assist in minimizing factors that may contribute
condition is rare and affects only one to two to problematic behaviors. Medication therapy
people per million worldwide per year.36,37 It is may be indicated, but should be monitored
caused by a conformational change of the closely due to potential exacerbation of symp-
normal prion protein, which is encoded by toms.39 Life expectancy for individuals with
human chromosome 20 to a disease-related this condition is similar to that of Alzheimer’s
prion protein. Diagnosis is suggested by a disease. The average survival time is between
thorough history and physical examination, 6 and 8 years from the onset of dementia.39,41
electroencephalography, and cerebrospinal
fluid analysis.37 Computed tomography and ■ Encephalitis
magnetic resonance imaging are typically Chief Clinical Characteristics
normal in sporadic and iatrogenic forms This presentation includes confusion, delirium,
and help to exclude other diagnoses.36,38 convulsions, problems with speech or hearing,
Diagnosis for all forms of this condition is only memory loss, hallucinations, drowsiness, and
confirmed postmortem.38 There is no proven coma. Loss of balance and/or falls may be present.
treatment.37,38 Death in sporadic and iatro-
genic forms occurs in a matter of months with Background Information
death occurring at a mean age of 66 years.39 This condition is an inflammation of nerve
Mean duration of illness in the variant form is cells in the brain. It usually refers to the viral
14 months.37 form, although bacterial, parasitic, and fungal
agents also can cause this condition. Up to
■ Dementia With Lewy Bodies 20,000 new cases of viral forms of this condi-
Chief Clinical Characteristics tion are reported annually in the United States.
This presentation can be characterized by Diagnosis is established by clinical presenta-
fluctuating cognitive dysfunction, particularly tion suggesting dysfunction of the cerebrum,
visuospatial problems and executive dysfunction, brainstem, or cerebellum, cellular reaction and
visual hallucinations, and parkinsonism features elevated protein in spinal fluid, and possible
such as masked facies, autonomic dysfunction, demonstration of diffuse edema or enhance-
rigidity, and bradykinesia. Other signs and ment of the brain on magnetic resonance
symptoms may include postural instability, imaging or computed tomography. Treatment
falls, sleep disturbances, memory problems, is primarily pharmacologic, with drugs such
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Chapter 28 Inflammatory Muscle Disease 557

as corticosteroids, antiviral agents, and anti- volume expansion of cerebrospinal fluid.16

LOSS OF BALANCE AND FALLS


convulsants. The majority of individuals with Magnetic resonance imaging and the presence
encephalitis do recover, but irreversible brain of papilledema are commonly used to estab-
damage and death can result.16 lish the diagnosis of hydrocephalus. Medical
treatment may include restriction of fluid
■ Fahr’s Syndrome (Familial intake and drugs with an osmotic effect, or the
Idiopathic Basal Ganglia addition of diuretics.45 Surgical treatment
Calcification, Bilateral depends on the chronicity of the hydrocephalus.
Striopallidodentate Calcinosis) The acute form of this condition is considered
Chief Clinical Characteristics fatal and is emergently treated via lumbar
This presentation may involve features of puncture or ventricular catheter.45 The
parkinsonism, such as chorea, athetosis, rigidity, chronic form of this condition is treated with
dystonia, and tremor in addition to cognitive placement of a ventricular shunt or with surgi-
impairments, cerebellar impairments, gait and cal removal of a mass if that is the cause of the
balance disorder, psychiatric features, pain, hydrocephalus. Although surgical procedures
pyramidal signs (such as weakness, hyper- for hydrocephalus have a high success rate and
reflexia in the deep tendon reflexes, hypertonia, a good prognosis, it is common to have shunt
clonus, and/or a positive Babinski sign), sensory complications such as infection, occlusion,
changes, and speech disorder.42 and over- or underdrainage. Thus, patients
who have been treated for this condition must
Background Information continue to be medically managed and educated
This condition occurs due to bilateral sym- regarding the indications of shunt compromise.
metric calcification of the basal ganglia with or
without calcification of the dentate nucleus. ■ Inflammatory Muscle Disease
The disease has been described as both famil- Chief Clinical Characteristics
ial and nonfamilial.16 Diagnosis is established This presentation involves asymmetric weakness
using computed tomography or magnetic of the quadriceps, wrist, and finger flexors. In-
resonance imaging of the brain; however, com- dividuals with this condition can present with
puted tomography has been found to be more proximal muscle weakness as well, which may
sensitive for identifying calcium deposits.43 lead to balance deficits and falls. This condition
Treatment aimed at minimizing calcium de- eventually will cause dysphagia in 40% to 60%
posits has been unsuccessful.44 Individuals with of individuals with this condition.46
this condition may be responsive to levodopa
for treatment of their parkinsonian features.16 Background Information
A set of diagnostic criteria has been proposed,
■ Hydrocephalus including duration of illness greater than
Chief Clinical Characteristics 6 months, age over 30 years, and muscle
This presentation commonly includes frontal lobe weakness that must affect proximal and distal
signs such as slowness of mental response, inat- muscles of arms and legs with at least one of
tentiveness, distractibility, perseveration, in- the following groups:
ability to sustain complex cognitive function, and 1. Finger flexor weakness
incontinence. Other symptoms include gait 2. Wrist flexor greater than wrist extensor
deterioration, frequent falls, occipital or frontal weakness
headaches, nausea and vomiting, diplopia, and 3. Quadriceps muscle weakness.47
lethargy. Advanced stages are associated with
coma and extensor posturing. Progression is slow such that patients may
have symptoms for up to 10 years before
Background Information diagnosis. The disease is more common in
Intracranial pressure can be increased due to men over the age of 50 years.46,48 Effective
many mechanisms including a cerebral or treatment for this condition has not been
extracerebral mass, generalized brain swelling, found. Individuals with this condition are
increased venous pressure, obstruction to the generally unresponsive to steroid treatment49
flow and absorption of cerebrospinal fluid, or and controlled studies have found no benefit
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558 Chapter 28 Labyrinthitis and Neuronitis

with intravenous immunoglobulin or immuno- a diminished gag reflex, vertical diplopia or


LOSS OF BALANCE AND FALLS

suppressive treatments.46,49 In individuals over sensation of tilting vision, ipsilateral ataxia of


the age of 60 years, disease progression is limbs, loss of balance to ipsilateral side, and
accelerated, resulting in an early need for an impaired sensation of ipsilateral half of the
assistive device.48 face.16,53,54
■ Labyrinthitis and Neuronitis Background Information
Chief Clinical Characteristics In 75% of cases, onset of this condition was
This presentation may be characterized by a sudden. Main etiologic factors associated
relatively sudden onset of severe, constant with this condition include large-vessel
rotary vertigo (made worse by head move- infarction, arterial dissection, small-vessel
ment) that resolves after days or weeks.50,51 infarction, cardiac embolism, tumor, hemor-
This condition is associated with spontaneous rhage, and other unknown factors. The most
nystagmus, postural imbalance, and nausea typical cause is occlusion of the vertebral
without accompanying cochlear or neurolog- artery, with the posterior inferior cerebellar
ical symptoms.51 The specific presentation artery being involved to a lesser degree. A
varies depending on the site of the infection. thorough history, clinical examination, and
If the vestibular system is affected, the symp- magnetic resonance imaging may confirm
toms will include dizziness and difficulty with the diagnosis. An emergency response is re-
vision and/or balance. If the inflammation quired for individuals experiencing the new
affects the cochlea, this condition will produce onset of symptoms. Residual symptoms in-
disturbances in hearing, such as tinnitus or clude balance deficits (most common), dys-
hearing loss. The symptoms can be mild or phagia, dizziness, and numbness, which may
severe, temporary or permanent, depending be addressed with physical, occupational,
on the severity of the infection. and speech therapy.
Background Information ■ Lyme Disease (Tick Paralysis)
The etiology of this condition is a bacterial or Chief Clinical Characteristics
viral infection causing inflammation of the This presentation can include fluctuating signs
vestibular nerve (neuronitis) or the labyrinth or symptoms such as headache, neck stiffness,
(labyrinthitis). The terms are often used inter- nausea, vomiting, malaise, fever, pain, fatigue,
changeably because it is difficult to distinguish and presence of a “bull’s-eye” rash.16 Over
neuronitis from labyrinthitis.50 The diagnostic time, additional symptoms may include
hallmark is unilateral hyporesponsiveness with sensory changes, irritability, cognitive changes,
caloric testing.51 Regardless of the type of in- depression, behavioral changes, seizures,
fection, the treatment consists of destroying ataxia, chorea movements, pain, weakness,
the bacteria by means of antibiotics. If the balance deficits, arthritis, and cranial nerve
labyrinthitis is caused by a break in the mem- involvement.16,38
branes separating the middle and inner ears,
surgery may also be required to repair the Background Information
membranes to prevent a recurrence of the dis- Borrelia burgdorferi, an organism that infects
ease. Residual vertigo can be reduced with ticks, is responsible for the transmission of this
vestibular habituation exercises.52 condition to a human host.16 The diagnosis is
confirmed with a thorough history, clinical
■ Lateral Medullary Infarction assessment, enzyme-linked immunosorbent
(Wallenberg’s Syndrome) assay, and Western blot or immunoblot analy-
Chief Clinical Characteristics sis. In addition, magnetic resonance imaging
This presentation can include nystagmus, oscil- or computed tomography may reveal multifo-
lopsia, vertigo, nausea, vomiting, impairment cal or periventricular cerebral lesions.16
of pain and thermal sense over half of the body, Medical management includes treatment with
ipsilateral Horner syndrome including miosis, oral tetracycline, penicillin, or intravenous
ptosis, anhidrosis, hoarseness, dysphagia, ipsi- ceftriaxone.16 Many individuals experience full
lateral paralysis of palate and vocal cord with recovery with treatment; however, residual
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Chapter 28 Viral Meningitis 559

deficits may persist for individuals with MENINGITIS

LOSS OF BALANCE AND FALLS


chronic Lyme disease.16 ■ Bacterial Meningitis
■ Ménière’s Disease Chief Clinical Characteristics
Chief Clinical Characteristics This presentation includes acute symptoms
This presentation is characterized by recurrent such as fever, severe headache, neck stiffness,
episodic spontaneous attacks of vertigo (exac- seizures, changes in consciousness, facial and
erbated by head movements and accompa- ocular palsies, positive Kernig and Brudzin-
nied by nausea and vomiting), fluctuating ski signs, and possible hemiparesis, which may
sensorineural hearing loss, aural fullness, and lead to falls. Chronic symptoms include hydro-
tinnitus.51,55–62 In the late stages the symp- cephalus, vomiting, immobility, impaired
toms are more severe, hearing loss is less likely alertness, hemiplegia, decorticate or decere-
to fluctuate, and tinnitus and aural fullness brate posturing, cortical blindness, stupor,
may be more constant. Sudden unexplained or coma.63
falls without loss of consciousness or vertigo Background Information
(drop attacks) may occur. Attacks can be This condition results from an infection
preceded by an aura consisting of a sense of and inflammation of the meninges sur-
fullness in the ear, increasing tinnitus, and a rounding the brain and spinal cord. Lum-
decrease in hearing,60 but can also occur with- bar puncture for spinal fluid pressure and
out warning. The typical duration of an attack cerebrospinal fluid culture, blood cultures,
is 2 to 3 hours, ranging from minutes to hours and radiologic studies confirm the diagno-
in length. Attacks can be single or multiple, sis.63 This condition is considered a medical
with short or long intervening periods of emergency. Medical management includes
remission during which the individual may be maintenance of blood pressure, treatment
asymptomatic. for septic shock, and administration of
Background Information intravenous antibiotics for 10 to 14 days.63
The cause of this condition is thought to Prognosis depends on the strain of bacteria;
be overproduction or underabsorption of approximately 5% to 15% of patients with
endolymph (endolymphatic hydrops), result- bacterial meningitis do not survive.63
ing in distortion of the membranous labyrinth. Residual effects after the infection resolves
Ruptures of the membranous labyrinth, are variable and patients with pneumococ-
autoimmune disease processes, and viral infec- cal and H. influenzae bacterial meningitis
tion are among other proposed causes.60 are more likely to have lasting neurological
Appropriate tests include caloric testing to deficits.63
determine amount of vestibular asymmetry; ■ Viral Meningitis
audiometry, which often shows a low-frequency
loss rather than a high-frequency one; and Chief Clinical Characteristics
exclusion of other causes (typically with This presentation can be characterized
gadolinium-enhanced magnetic resonance by an acute onset of fever, headache, and
imaging). The head thrust test can be negative neck stiffness. Drowsiness, lethargy, and
despite caloric asymmetry. Restricting salt irritability may occur, but overall symp-
intake and using diuretics may be useful in toms tend to be relatively mild.63 Although
over half of individuals with this condition. not a common presenting sign, falls or
Approximately 10% of individuals with this imbalance may occur due to drowsiness
condition will have persistent vertigo and re- and lethargy.
quire other forms of treatment. These include Background Information
surgery to decompress or drain the endolym- This condition is an infection and inflam-
phatic sac, selective vestibular neurectomy or mation of the meninges surrounding the
labyrinthectomy, and intratympanic injections brain and spinal cord. Also known as aseptic
of aminoglycosides such as gentamicin57 to meningitis, this condition is most commonly
reduce or abolish vestibular function on the caused by the echovirus or coxsackie
affected side. virus.63 Cerebrospinal fluid analysis and
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560 Chapter 28 Multiple Sclerosis

blood work are used to determine the diag- ■ Multiple System Atrophy
LOSS OF BALANCE AND FALLS

nosis.63,64 There is no specific treatment, With Orthostatic Hypotension


although supportive care may include (Striatonigral Degeneration,
administration of analgesics. This condition Olivopontocerebellar Atrophy,
is rarely fatal and most patients demonstrate Shy-Drager Syndrome)
a full recovery.63 Chief Clinical Characteristics
■ Multiple Sclerosis This presentation involves tremor, rigidity, aki-
nesia, and/or postural imbalance along with
Chief Clinical Characteristics signs of cerebellar, pyramidal, and autonomic
This presentation may include paresthesias, dysfunction. Autonomic symptoms such as
weakness, spasticity, hypertonicity, hyperreflexia, orthostatic hypotension, dry mouth, loss of
positive Babinski sign, incoordination, optic sweating, impotence, and urinary incontinence
neuritis, ataxia, vertigo, dysarthria, diplopia, or retention are the initial feature in 41% of
bladder incontinence, tremor, balance deficits, individuals, with 74% to 97% of individuals
falls, and cognitive deficits.16 developing some degree of autonomic dysfunc-
Background Information tion during the course of the disease.68 This con-
This condition may present as relapsing- dition is a combination of parkinsonian and
remitting, primary progressive, or second- non-parkinsonian symptoms and signs.
ary progressive. The disease occurs most
frequently in women between the ages of Background Information
20 and 40 years. Only a small number of Diagnostic criteria are based on the clinical pres-
children or individuals between 50 and entation, which includes poor response to lev-
60 years of age are diagnosed with this odopa, presence of autonomic features, presence
condition.16 This condition was originally of speech or bulbar problems, absence of demen-
thought to be secondary to environmental tia, absence of toxic confusion, and presence of
and genetic factors, but evidence suggests falls.69 The disease course ranges between 0.5 and
an autoimmune response to a viral infec- 24 years after diagnosis with a mean survival
tion, which subsequently targets myelin.16 time of 6.2 years.70 This condition is a progres-
The diagnosis may be confirmed by a thor- sive condition of the central and autonomic
ough history, physical examination, mag- nervous systems that rarely occurs without or-
netic resonance imaging, analysis of cere- thostatic hypotension. There are three types of
brospinal fluid, and evoked potentials.16,65,66 this condition. The parkinsonian-type includes
Life expectancy and cause of mortality are symptoms of Parkinson’s disease such as slow
similar for all types of this condition.16 movement, stiff muscles, and tremor. The cere-
Clinical characteristics that are associated bellar type causes problems with coordination
with a longer time interval for progression and speech. The combined type includes symp-
of disability include female sex, younger toms of both parkinsonism and cerebellar failure.
age of onset, relapsing-remitting type, com- Older age at onset is associated with a shorter
plete recovery after the first relapse, and survival time.70 Average age of onset is 54 years,
longer time interval between first and with mean age at death being 60.3 years.68 Most
second exacerbation. 67 Medical manage- individuals with this condition receive a trial of
ment may include the use of methylpred- levodopa although only a minority respond.68
nisolone, prednisone, cyclophosphamide, Additional treatment addresses symptoms and
immunosuppressant treatment, and beta- involves physical and occupational therapy
interferon. 16 Physical, occupational, and to maintain mobility and address safety issues
speech therapy may be indicated to related to the progression of imbalance.
prevent secondary sequelae and to optimize
functional activity and mobility. Some ■ Neurofibromatosis
individuals may benefit from psychology/ Chief Clinical Characteristics
psychiatry and social support as the disease This presentation depends on the type of neu-
progresses. rofibromatosis. Neurofibromatosis type 1 (NF1,
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Chapter 28 Neuropathy Secondary to Diabetes Mellitus 561

Von Recklinghausen’s disease) may include café 3. Diffuse cerebral symptoms that involve cog-

LOSS OF BALANCE AND FALLS


au lait spots, neurofibromas, pathological frac- nitive deficits, altered level of consciousness,
tures, syringomyelia, scoliosis, stroke, neoplasms, hyperreflexia, Babinski sign, presence of
learning difficulties, and hyperactivity. Neu- primitive reflexes (such as secondary to
rofibromatosis type 2 (NF2) is characterized postinfectious encephalomyelitis, acquired
by bilateral vestibular schwannomas, progres- immunodeficiency dementia complex,
sive hearing loss, and possible intracranial or cytomegalovirus encephalitis)
intraspinal neoplasms.16,38 4. Myelopathy associated with gait difficulties,
spasticity, ataxia, balance deficits, and hyper-
Background Information
reflexia (such as secondary to herpes zoster
This condition is an autosomal dominant
myelitis, vacuolar myelopathy that occurs
disorder on chromosome 17 for NF1 and on
with acquired immunodeficiency syndrome
chromosome 22 for NF2. The tumors in NF1
dementia complex)
occur due to the excessive proliferation of
5. Peripheral involvement associated with sen-
cells within the meninges, vascular system,
skin, viscera, peripheral, and central nervous sory changes, weakness, balance deficits, and
systems.16,38 The tumors in NF2 arise from pain (such as secondary to peripheral neu-
ropathy, acute and chronic inflammatory
the posterior nerve roots. Depending on the
demyelinating polyneuropathies).16,71
type of this condition, the diagnosis may be
confirmed by a thorough family history, the Abnormal neurological findings are ob-
presence of six or more café au lait spots, served during a clinical examination in approx-
imaging, and genetic testing. If indicated, imately one-third of patients with acquired
the tumors that result from this condition immunodeficiency syndrome; however, on
may be surgically removed.38 Both forms autopsy, most individuals with this condition
of this condition are progressive and the have abnormalities within the nervous system.16
prognosis varies depending on the severity Diagnosis of the variable neurological complica-
of lesions.16 tions associated with this condition may be con-
firmed with laboratory tests, cerebrospinal fluid
■ Neurological Complications of cultures, imaging, nerve conduction studies, and
Acquired Immunodeficiency physical examination.16,38,71 Treatment appears
Syndrome to be limited primarily to the use of antiviral
Chief Clinical Characteristics medications.71 Physical and occupational ther-
This presentation is variable and dependent on apy may be indicated to address equipment
the affected neuroanatomical structures in an needs and caregiver/patient training related to
individual with acquired immunodeficiency functional mobility.
syndrome.71
■ Neuropathy Secondary
Background Information to Diabetes Mellitus
This condition may be categorized by:
Chief Clinical Characteristics
1. Meningitic symptoms including headache, This presentation is variable, including mani-
malaise, and fever (such as secondary to festations such as acute diabetic mononeu-
meningitis, cryptococcal meningitis, tuber- ropathies, which may include involvement
culous meningitis, and human immunode- of cranial nerves (eg, oculomotor or abducens
ficiency virus headache) nerve involvement) or peripheral nerves16; mul-
2. Focal cerebral symptoms including hemi- tiple mononeuropathies and radiculopathies,
paresis, aphasia, apraxia, sensory deficits, which may include unilateral or asymmetric
homonymous hemianopia, cranial nerve in- pain, low back pain with or without symptoms
volvement, balance deficits, incoordination, in leg, weakness, atrophy, diminished or absent
and/or ataxia (such as secondary to cerebral deep tendon reflexes, and sensory deficits16; dis-
toxoplasmosis, primary central nervous sys- tal polyneuropathy, the most common diabetic
tem lymphoma, and progressive multifocal neuropathy, which consists of chronic, symmet-
leukoencephalopathy) ric, distal sensory deficits (eg, numbness and
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562 Chapter 28 Neurosyphilis

tingling), diminished or absent deep tendon re- T. pallidum is present within the central nervous
LOSS OF BALANCE AND FALLS

flexes, balance deficits, and weakness16; and system the individual is diagnosed with neu-
autonomic neuropathy, which may involve rosyphilis.76 This condition occurs in approxi-
resting tachycardia, orthostatic hypotension, mately 10% of individuals with untreated
sexual impotence, exercise intolerance, abnormal syphilis, and in 81% of these cases it presents
sweating, pupil abnormalities, weakness, sen- as meningovascular, meningeal, and general
sory deficits, and gastroparesis.16,38,72,73 paresis. Treatment includes use of various
forms of penicillin or alternative choices for
Background Information those allergic to penicillin75 and may involve
Approximately 15% to 20% of people with dia- rehabilitative therapies depending on the indi-
betes may present with the signs and symptoms vidual’s activity limitations or participation
of this condition.16,72 However, approximately restrictions. A better prognosis has been
50% will have neuropathic symptoms and may observed for individuals treated during early
have abnormalities in nerve conduction test- neurosyphilis.76
ing.16 Commonly considered a metabolic disor-
der, this condition may be a result of vascular ■ Orthostatic Hypotension
complications disrupting the supply of nutri- Chief Clinical Characteristics
ents to the nerves.38,74 A thorough history, phys- This presentation commonly involves dizziness,
ical examination (specifically including the as- light-headedness, and blurred vision that gen-
sessment of deep tendon reflexes and sensory erally occur after sudden standing. In more
examination), electromyography/nerve conduc- severe forms of this condition, individuals may
tion testing, and laboratory screen helps to experience seizures, transient ischemic attacks,
differentiate other causes of neuropathy.72,73 or syncope.
Treatment consists of maintaining a normal
range of blood glucose levels.16,72,73 In addition, Background Information
individuals with this condition may prevent This condition is caused by a sudden decrease
complications by completing visual inspection of greater than 20 mm Hg in systolic blood
of the skin and routine podiatry care.73 Medica- pressure or greater than 10 mm Hg in dias-
tions may help to control symptoms such as tolic blood pressure, which occurs when a
paresthesias or pain.16,38 Additional manage- person assumes a standing position. It may be
ment may include consultations with an ortho- caused by hypovolemia resulting from the ex-
tist to ensure proper fitting of footwear and cessive use of diuretics, vasodilators, or other
physical therapy to minimize disability by types of vasoactive medications (eg, calcium
addressing impairments associated with limita- channel blockers and beta blockers), dehydra-
tions in functional mobility.74 tion, or prolonged bed rest. Other factors to
consider include the individual’s neurological
■ Neurosyphilis (Tabes Dorsalis, status and hemorrhagic/hypovolemic states.
Syphilitic Spinal Sclerosis, The condition may be associated with Addi-
Progressive Locomotor Ataxia) son’s disease, atherosclerosis, diabetes, and
Chief Clinical Characteristics certain neurological conditions including
This presentation can be characterized by Shy-Drager syndrome and other dysautono-
hemiparesis, ataxia, aphasia, gait instability, mias. Hypovolemia due to medications is re-
falls, neuropathy, personality and cognitive versed by adjusting the dosage or by discon-
changes, seizures, diplopia, visual impairments, tinuing the medication. If prolonged bed rest
hearing loss, psychotic disorders, loss of bowel/ is the cause, improvement may occur by sit-
bladder function, pain, hyporeflexia, and hy- ting up with increasing frequency each day. In
potonia.75,76 some cases, physical counterpressure such as
elastic hose or whole-body inflatable suits
Background Information may be required. Dehydration is treated with
Treponema pallidum infects the human host by salt and fluids. Individuals with this condition
way of contact with contaminated body fluids can be instructed to rise slowly from bed in
or lesions.75 This spirochete is responsible for the mornings or when moving from a
the diagnosis of syphilis; however, when sitting/squatting to standing position. Symptoms
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Chapter 28 Parkinson’s Disease 563

usually dissipate when the individual is type and may include steroids, plasmapheresis,

LOSS OF BALANCE AND FALLS


placed in a semirecumbent or supine position, immunotherapy, chemotherapy, radiation, or
although some individuals may progress to cyclophosphamide.77 Physical, occupational,
frank syncope. In this case, the clinician and speech therapy may be indicated to address
should be prepared to activate the emergency functional limitations.
medical system if the individual fails to re-
gain consciousness with basic life support ■ Parkinson’s Disease
measures. Chief Clinical Characteristics
This presentation commonly involves resting
■ Paraneoplastic Syndromes tremor, bradykinesia, rigidity, and postural
Chief Clinical Characteristics instability. Falls are a common problem in in-
This presentation includes dizziness in combi- dividuals with this condition, with up to
nation with a variety of different neurological 68% falling within a 1-year period and approx-
symptoms and signs in an individual with can- imately 50% of these individuals falling mul-
cer. Specific neurological symptoms and signs tiple times within that same year.80 Other
depend on the location of involvement of the common signs and symptoms include festi-
central or peripheral nervous system. nation, freezing, micrographia, hypophonia
(hypokinetic dysarthria), akinesia, masked
Background Information
facies, drooling, difficulty turning over in bed,
Paraneoplastic encephalomyelitis and focal
dystonia, dyskinesia, falls, dementia, and
encephalitis may present with ataxia, vertigo,
depression.16
balance deficits, nystagmus, nausea, vomiting,
cranial nerve palsies, seizures, sensory neuropa- Background Information
thy, anxiety, depression, cognitive changes, and This condition occurs due to the depletion
hallucinations. For individuals presenting with or injury of dopamine-producing cells in sub-
ataxia, dysarthria, dysphagia, and diplopia, stantia nigra pars compacta. Clinical signs and
paraneoplastic cerebellar degeneration may symptoms are not typically present until after
be suspected. Paraneoplastic opsoclonus/ approximately 80% of dopamine-producing
myoclonus tends to affect both children and cells are lost. The definitive diagnosis is made
adults with signs and symptoms including postmortem. However, a clinically definitive
hypotonia, ataxia, irritability, truncal ataxia, diagnosis may be made with the presence of at
gait difficulty, balance deficits, and frequent least two of three criteria—asymmetric resting
falls. Stiff-man syndrome presents with spasms tremor, bradykinesia, or rigidity—and a positive
and fluctuating rigidity of axial musculature, response to anti-Parkinson medications.16,81
legs and possibly shoulders, upper extremities, Imaging may be useful to exclude vascular
and neck. Paraneoplastic sensory neuropathy involvement. Medical management may in-
presents with asymmetric, progressive sensory clude use of dopamine agonists, levodopa,
alterations involving the limbs, trunk, and and other medications to address nonmotor
face, sensorineural hearing loss, autonomic symptoms, such as depression, constipation,
dysfunction, and pain. Other conditions in autonomic symptoms, and sexual dysfunc-
this category include vasculitis, Lambert- tion. Surgical management also may be
Eaton myasthenia syndrome, myasthenia considered, including deep brain stimulation
gravis, dermatomyositis, neuromyotonia, and or pallidotomy.81 Forced use or higher inten-
various neuropathies.16,77 These conditions sity, challenging activities may provide a neu-
result from an immune-mediated response to roprotective benefit for individuals with early
the presence of tumor or metastases. Antibod- forms of this condition.82 With the high inci-
ies or T-cells respond to the presence of the dence of depression, consultation with a
tumor, but also attack normal cells of the nerv- psychologist or psychiatrist may be warranted.
ous system.78,79 Over 60% of individuals pres- Individuals with a late onset tend to progress
ent with this condition prior to the discovery more rapidly.83,84 Poor prognostic indicators
of the cancer.77 The underlying tumor is treated for disability include initial presentation
according to the type of cancer. Additional without tremor, early dependence, dementia,
treatment is dependent on this condition’s balance impairments, older age, and the
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564 Chapter 28 Peripheral Neuropathy

postural instability/gait difficulty dominant symptom with the majority of falls being back-
LOSS OF BALANCE AND FALLS

type.83,84 ward falls.89 Difficulty with voluntary vertical


eye movements (usually downward) and
■ Peripheral Neuropathy involuntary saccades are relatively early fea-
Chief Clinical Characteristics tures. The disease progresses to the point at
This presentation may be characterized by falls, which all voluntary eye movements are lost.
loss of balance, weakness, and sensory alter- Background Information
ations, as well as diminished or absent deep Some patients may not demonstrate difficul-
tendon reflexes, fasciculations, syncope, abnor- ties with ocular movements for 1 to 3 years
mal sweating, orthostatic hypotension, resting after disease onset. Most cases are sporadic;
tachycardia, and trophic changes.16,85 however, a pattern of inheritance compatible
Background Information with autosomal dominant transmission has
The patterns of peripheral neuropathies are been described.16 Diagnosis is based on clinical
variable and may present as polyneuropathy, presentation, which includes a gradually pro-
polyradiculopathy, neuronopathy, mononeu- gressive disorder with age of onset at 40 years
ropathy, mononeuropathy multiplex, and or older, vertical supranuclear palsy, and pos-
plexopathy.16 Some of the etiologies associated tural instability with falls within the first year
with peripheral neuropathies include trauma, of disease onset.90 Medical treatment is typi-
inflammation (eg, herpes zoster, Lyme disease, cally unsuccessful, because the majority of
human immunodeficiency virus, Guillain- these patients are not responsive to levodopa
Barré syndrome), metabolic causes (eg, dia- therapy aimed at addressing the extrapyrami-
betes mellitus, uremia), nutritional causes (eg, dal features of the disease.35 Physical and occu-
vitamin B deficiencies commonly associated pational therapy are used to maintain mobility
with alcohol abuse, eating disorders, and indi- and address safety issues related to the
viduals with malabsorption syndromes), con- progression of imbalance. The disease course
genital and idiopathic causes (eg, aging or is progressive with a mean survival time of
unknown causes), and toxic etiology (eg, ex- 5.6 years.89 Older age at disease onset, early onset
posure to lead, arsenic, thallium; chemothera- of falls, incontinence, dysarthria, dysphagia,
peutic drugs such as vincristine, cisplatin).85 insertion of a percutaneous gastrostomy, and
Twenty percent of individuals over the age diplopia have all been described as being
of 60 are affected by a type of peripheral neu- predictive of shorter survival time.89
ropathy.86 The diagnosis of the specific disor- ■ Spinal Metastases
der may be differentiated by the pattern of
peripheral neuropathy and temporal features. Chief Clinical Characteristics
The diagnosis may be confirmed after complet- This presentation can involve spasticity, weak-
ing a thorough history, physical examination, ness, sensory alterations, bowel and bladder
gait assessment, balance assessment, laboratory incontinence, neck pain, back pain, radicular
testing, and electromyography/nerve conduc- pain, atrophy, cerebellar signs, balance deficits,
tion testing.16,87 Treatment and prognosis will falls, and cranial nerve involvement.16,38,91
vary depending on the etiology and severity of Background Information
this condition. This condition is the most frequent neoplasm
involving the spine.91 The most common types
■ Progressive Supranuclear Palsy and locations of primary tumors that result in
(Richardson-Steele-Olszewski spinal metastases include breast, lung, lym-
Syndrome) phoma, prostate, kidney, gastrointestinal tract,
Chief Clinical Characteristics and thyroid.16,92 The diagnosis is confirmed
This presentation classically includes vertical gaze with gadolinium-enhanced magnetic resonance
palsy, prominent instability, and falls within imaging and computed tomography.16,91 Treat-
the first year of disease onset.88 Other charac- ment is variable depending on the tumor and
teristics may include rigidity, akinesia, may include surgical resection, chemotherapy,
dysarthria, dysphagia, and mild dementia. Falls radiation, corticosteroids, and rehabilitative
were found to be the most commonly reported therapies.16 Although the long-term prognosis
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Chapter 28 Transient Ischemic Attack 565

is poor, individuals without paresis or pain and condition.93,94 Individuals with this condition

LOSS OF BALANCE AND FALLS


who are still ambulatory have longer survival may benefit from physical, occupational, and
rates.92 speech therapy to address activity limitations
and participation restrictions.
■ Spinal Primary Tumors
Chief Clinical Characteristics ■ Stroke (Cerebrovascular Accident)
This presentation may include spasticity, weak- Chief Clinical Characteristics
ness, sensory alterations, bowel/bladder incon- This presentation may include a wide range
tinence, back pain, radicular pain, atrophy, of symptoms that correspond to specific areas
cerebellar signs, balance deficits, falls, and of the brain that are affected, potentially
cranial nerve involvement.16 including balance deficits or falls. The initial
Background Information symptoms can include numbness or weakness,
Types of this condition include myeloma, neu- especially on one side of the body or face;
rofibroma, lymphoma, metastasis, menin- confusion or aphasia; visual disturbances;
gioma, schwannoma, and astrocytoma. The or sudden severe headache with no known
first test to diagnose brain and spinal column cause.
tumors is a neurological examination. Special Background Information
imaging techniques (computed tomography, This condition occurs when blood flow to
magnetic resonance imaging, and positron the brain is interrupted either by blockage
emission tomography) are also employed. Spe- (ischemia or infarction) or from hemorrhagic
cific diagnoses may be confirmed with imaging disruption. A thrombosis or embolic occlu-
and biopsy. Treatment is variable depending on sion of an artery causes an ischemic type of
the type, size, and location of the tumor and this condition. A hemorrhagic type of this
may include surgical resection, chemotherapy, condition can be caused by arteriovenous
radiation, corticosteroids, and rehabilitative malformation, hypertension, aneurysm,
therapies. Prognosis is variable and depends on neoplasm, drug abuse, and trauma. This
the type and grade of tumor, severity of com- condition is the most common and disabling
pression, and duration of compression. neurological disorder in adults and occurs in
■ Spinocerebellar Ataxia 114 of every 100,000 people.95 This condition
(Spinocerebellar Atrophy, is diagnosed using clinical presentation and
Spinocerebellar Degeneration) positive findings on computed tomography
and magnetic resonance imaging. Medica-
Chief Clinical Characteristics
tion, surgery, and interdisciplinary therapy
This presentation can involve ataxia, incoordi-
are the most common treatments for this
nation, supranuclear ophthalmoplegia, slow
condition. The prognosis for recovery is pre-
saccades, optic atrophy, dysarthria, balance
dicted by the magnitude of initial deficit.
deficits, falls, tremor, myoclonus, chorea, nys-
Factors that are associated with poor
tagmus, dementia, amyotrophy, and periph-
outcomes include coma, poor cognition, se-
eral neuropathy.93,94
vere aphasia, severe hemiparesis with little
Background Information return within 1 month, visual perceptual
This condition refers to a group of progressive, disorders, depression, and incontinence after
neurodegenerative, autosomal dominant dis- 2 weeks.96,97
orders affecting the cerebellum, brainstem,
and spinal cord. Twelve variants have been ■ Transient Ischemic Attack
identified: SCA 1, 2, 3, 6, 7, 8, 10, 12, 14, 17, Chief Clinical Characteristics
FGF10-SCA, and dentatorubral-pallidoluysian This presentation can include numbness or
atrophy.94 The disorders occur due to expan- weakness in the face, arm, or leg, especially on
sions of CAG triplet repeats that are subse- one side of the body; confusion or difficulty in
quently transcribed into long polyglutamine talking or understanding speech; trouble
tracts. Diagnosis is confirmed by deoxyri- seeing in one or both eyes; and difficulty
bonucleic acid testing. There is no current with walking, dizziness, or loss of balance and
medical treatment for the various forms of this coordination.
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566 Chapter 28 Traumatic Brain Injury

Background Information ■ Vasculitis (Giant Cell Arteritis,


LOSS OF BALANCE AND FALLS

This condition is a transient stroke that lasts Temporal Arteritis, Cranial


only a few minutes. It occurs when the blood Arteritis)
supply to part of the brain is briefly inter- Chief Clinical Characteristics
rupted. Symptoms of this condition, which This presentation can be characterized by
usually occur suddenly, are similar to those of headaches, psychiatric syndromes, dementia,
stroke but do not last as long. Most symptoms peripheral or cranial nerve involvement, pain,
disappear within an hour, although they may seizures, hypertension, hemiparesis, balance
persist for up to 24 hours. Because it is impos- deficits, neuropathies, myopathies, organ in-
sible to differentiate between symptoms from volvement, fever, and weight loss.16,38,101
this condition and acute stroke, individuals
should assume that all stroke-like symptoms Background Information
signal a medical emergency. A prompt evalua- This condition is the result of an immune-
tion (within 60 minutes) is necessary to iden- mediated response resulting in the inflamma-
tify the cause of this condition and determine tion of vascular structures.16,101 It includes
appropriate therapy. Depending on the indi- a variety of disorders such as giant cell/
vidual’s medical history and the results of a temporal arteritis (which is the most com-
medical examination, the doctor may recom- mon form), primary angiitis of the central
mend drug therapy or surgery to reduce the nervous system, Takayasu’s disease, periarteri-
risk of stroke. Antiplatelet medications, partic- tis nodosa, Kawasaki disease, Churg-Strauss
ularly aspirin, are a standard treatment for syndrome, Wegener’s granulomatosis, and
individuals suspected of this condition and secondary vasculitis associated with systemic
who also are at risk for stroke, including lupus erythematous, rheumatoid arthritis,
individuals with atrial fibrillation. and scleroderma.101 The diagnosis is con-
firmed through history, physical examina-
■ Traumatic Brain Injury tion, laboratory testing, angiography, biopsy,
Chief Clinical Characteristics and imaging.16,38,101 Corticosteroids, cyto-
This presentation typically includes dysequi- toxic agents, intravenous immunoglobulin,
librium in the presence of cognitive changes, and plasmapheresis may be used in the treat-
altered level of consciousness, seizures, nausea, ment of vasculitis.38,101 Prognosis is variable
vomiting, coma, dizziness, headache, pupillary and depends on the specific underlying disor-
changes, tinnitus, weakness, incoordination, der. For example, giant cell arteritis is typi-
behavioral changes, spasticity, hypertonicity, cally self-limiting within 1 to 2 years; however,
cranial nerve lesions, and sensory and motor death usually occurs within 1 year for indi-
deficits.16,98 viduals with primary angiitis of the central
nervous system.101
Background Information
This condition can be classified as mild, moder- ■ Vertebrobasilar Artery
ate, or severe based on Glasgow Coma Scale, Insufficiency
length of coma, and duration of post-traumatic Chief Clinical Characteristics
amnesia.98 Magnetic resonance imaging may be This presentation commonly involves symptoms
used to confirm the diagnosis.38 Treatment ini- consistent with a cerebrovascular accident
tiated at the scene of the accident and during involving the vertebrobasilar artery system,
the acute phase is focused on medical stabiliza- usually the anterior inferior cerebellar artery.102
tion. It should be initiated during the acute Headache or neck pain was found to be the
phase in order to minimize complications.99 prominent feature in 88% of 26 individuals
Low Glasgow Coma Scale, longer length of presenting with vertebral artery dissection. The
coma, longer duration of post-traumatic amne- most common focal neurological symptom was
sia, and older age tend to be associated with vertigo (57%).103 In cases of stroke following
poor outcomes.100 Optimal rehabilitation is cervical spine manipulation purported to be
interdisciplinary and customized to address the due to vertebral artery dissection, the presenting
specific individuals’ disablement. neurological symptoms were loss of coordination
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Chapter 28 Vestibular Ototoxicity 567

(52%), dizziness/vertigo/nausea/vomiting Background Information

LOSS OF BALANCE AND FALLS


(50%), speech/swallowing dysfunction, visual The severity, type, and particular combination
disturbances, and numbness, nystagmus, loss of symptoms are variable, depending on the
of consciousness, hearing deficits/tinnitus, and medication exposure, whether it is unilateral or
death.104 bilateral, the speed of onset, and the individual.
A slow unilateral loss may produce few symp-
Background Information toms since the brain can compensate through
Less severe but similar symptoms may be other mechanisms, whereas a fast bilateral loss
expected in lesser degrees of vertebrobasilar in- can produce significant disability. Because these
sufficiency. The circulation to the inner ear symptoms are similar to many other conditions,
arises from the vertebrobasilar artery system, the key diagnostic feature is a history of drug
which leads to dizziness when this circulation is or chemical exposure. Medications that are
impaired.102,105 If the insufficiency is great known to be vestibulotoxic include aspirin and
enough to progress to stroke, generally other quinine, loop diuretics (bumetanide [Bumex],
associated neurological symptoms typical of ethacrynic acid [Edecrin], furosemide [Lasix]
central nervous system lesions are present, but torsemide [Demadex]), aminoglycoside antibi-
these can be absent.102,105 A classic sequel of otics (amikacin, dihydrostreptomycin, gen-
stroke related to this condition is lateral tamicin, kanamycin, Neomycin, netilmicin,
medullary (Wallenberg’s) syndrome. Proposed ribostamycin, streptomycin, tobramycin), and
causes of vascular compromise include trauma anticancer medications such as carboplatin and
resulting from participation in sports or ma- cisplatin. Some medications produce a tem-
nipulation of the cervical spine, cervical spine porary dysfunction, while others produce per-
rotation and/or extension, and complications manent loss. Treatment involves removing
due to risk factors such as age, gender, migraine exposure to the medication if possible, and
headaches, hypertension, diabetes, use of birth vestibular habituation and balance exercises.
control pills, cervical spondylosis, and smok-
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Press; 2001.
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CHAPTER 29
Sensory Abnormalities
■ Bernadette M. Currier, PT, DPT, MS, NCS ■ Michelle G. Prettyman, PT, DPT, MS

Description of the Symptom This chapter discusses abnormalities that


may affect each of these sensory systems, ex-
This chapter describes pathology that may lead cept the vestibular system. Conditions affect-
to sensory abnormalities. Sensation refers to ing the vestibular system are discussed in
the registration of an incoming, or afferent, Chapter 30.
nerve impulse. Sensory organs, such as the
eyes, nose, mouth, ears, and skin, transmit in- Special Concerns
formation to the brain to allow us to perceive Sensory abnormalities accompanied by:
diverse stimuli. ■ A new onset of neurological symptoms,
The sensory systems are: including:
■ Loss of balance
● Auditory: the detection of sound or pressure ■ Falls
waves in the air ■ Severe headache
● Gustatory: the sense of taste ■ Impaired speech
● Olfactory: the sense of smell ■ Paresis
● Proprioceptive: the detection of joint or limb ■ Change in mental status
position in space ■ Nausea and vomiting
● Tactile: the detection of changes in pressure, ■ Abnormal blood pressure, heart rate,
temperature, vibration, and other stimuli respiratory rate, or oxygen saturation
for the skin ■ Changes in edema, skin color, and

● Visual: the detection of light temperature


● Vestibular: the perception of movement and
orientation in space.

571
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572 Chapter 29 Sensory Abnormalities

CHAPTER PREVIEW: Conditions That May Lead to Sensory Abnormalities


SENSORY ABNORMALITIES

T Trauma
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Perilymph fistula 596 Not applicable Not applicable

UNCOMMON
Perforated tympanum 596 Not applicable Not applicable

RARE
Not applicable Middle ear surgery 591 Not applicable

I Inflammation
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Not applicable Not applicable Not applicable

UNCOMMON
Not applicable Aseptic Not applicable
Bell’s palsy 583
Septic
Herpes zoster oticus (Ramsay
Hunt syndrome) 588

RARE
Aseptic Not applicable Not applicable
Not applicable
Septic
Neurosyphilis (tabes dorsalis,
syphilitic spinal sclerosis,
progressive locomotor
ataxia) 594
Otosyphilis 595
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Chapter 29 Sensory Abnormalities 573

SENSORY ABNORMALITIES
PROPRIOCEPTIVE TACTILE VISUAL
ABNORMALITIES ABNORMALITIES ABNORMALITIES

Benign paroxysmal positional Brachial plexus injury 584 Detached retina 587
vertigo 583 Carpal tunnel syndrome 584
Ligament sprains 590 Thoracic outlet syndrome 600
Traumatic brain injury 601

Spinal cord injuries: Hydromyelia (syringomyelia) 588 Not applicable


• Brown-Sequard syndrome 598 Spinal cord injuries:
• Central cord syndrome 598 • Brown-Sequard syndrome 598
• Conus medullaris syndrome 598 • Central cord syndrome 598
• Traumatic spinal cord injury 599 • Conus medullaris
syndrome 598
• Traumatic spinal cord injury 599

Not applicable Not applicable Not applicable

PROPRIOCEPTIVE TACTILE VISUAL


ABNORMALITIES ABNORMALITIES ABNORMALITIES

Aseptic Not applicable Not applicable


Not applicable
Septic
Labyrinthitis and neuronitis 589

Not applicable Aseptic Aseptic


Multiple sclerosis 591 Behçet’s disease 583
Progressive multifocal
Septic leukoencephalopathy 597
Herpes zoster 588 Systemic lupus
erythematosus 599
Septic
Not applicable

Not applicable Aseptic Aseptic


Acute demyelinating Neuromyelitis optica 592
polyneuropathy (Guillain-
Barré syndrome) 581 Septic
Transverse myelitis 601 Klüver-Bucy syndrome 589
Rickettsial diseases,
Septic including Rocky Mountain
Tropical spastic paraparesis 601 spotted fever 598
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574 Chapter 29 Sensory Abnormalities


SENSORY ABNORMALITIES

M Metabolic
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Not applicable Not applicable Drug toxicity 587

UNCOMMON
Not applicable Not applicable Not applicable

RARE
Not applicable Not applicable Neurosarcoidosis 594

Va Vascular
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Vasculitis (giant cell arteritis, Vasculitis (giant cell arteritis, Not applicable
temporal arteritis, cranial temporal arteritis, cranial
arteritis) 603 arteritis) 603

UNCOMMON
Not applicable Not applicable Not applicable

RARE
Not applicable Lateral medullary syndrome Not applicable
(Wallenberg’s syndrome) 589

De Degenerative
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Otosclerosis 595 Not applicable Not applicable
Presbycusis 597
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Chapter 29 Sensory Abnormalities 575

SENSORY ABNORMALITIES
PROPRIOCEPTIVE TACTILE VISUAL
ABNORMALITIES ABNORMALITIES ABNORMALITIES

Not applicable Neuropathies: Not applicable


• Diabetes mellitus 593
• Peripheral neuropathy 593
Vitamin B12 deficiency 604

.
Not applicable Not applicable Cerebral beriberi
(Korsakoff’s amnesic
syndrome, Wernicke-
Korsakoff syndrome) 586

Not applicable Paraneoplastic syndromes 596 Neurotoxicity 594

PROPRIOCEPTIVE TACTILE VISUAL


ABNORMALITIES ABNORMALITIES ABNORMALITIES

Vasculitis (giant cell arteritis, Migraine 591 Cerebral aneurysm 585


temporal arteritis, cranial Cerebral arteriosclerosis 586
arteritis) 603 Orthostatic hypotension 594
Stroke (cerebrovascular
accident) 599
Vasculitis (giant cell arteritis,
temporal arteritis, cranial
arteritis) 603

Not applicable Arteriovenous malformation 583 Not applicable


Transient ischemic attack 600

Not applicable Cavernous malformation 585 Idiopathic intracranial


hypertension 588

PROPRIOCEPTIVE TACTILE VISUAL


ABNORMALITIES ABNORMALITIES ABNORMALITIES

Not applicable Not applicable Cataracts 585


Glaucoma 587
Macular degeneration 590
Retinitis 597
(continued)
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576 Chapter 29 Sensory Abnormalities

Degenerative (continued)
SENSORY ABNORMALITIES

AUDITORY GUSTATORY OLFACTORY


ABNORMALITIES ABNORMALITIES ABNORMALITIES
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Tu Tumor
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Malignant Primary, such as: Malignant Primary, such as: Malignant Primary, such as:
• Brain primary tumors 602 • Brain primary tumors 602 • Brain primary tumors 602
Malignant Metastatic: Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable Not applicable
Benign: Benign: Benign:
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Malignant Primary, such as: Malignant Primary, such as: Malignant Primary, such as:
• Brain metastases 602 • Nasopharyngeal • Angiomatosis (Von
• Nasopharyngeal carcinoma 602 carcinoma 602 Hippel-Lindau disease) 581
Malignant Metastatic: • Oropharyngeal carcinoma 602 • Brain metastases 602
Not applicable Malignant Metastatic: • Nasopharyngeal
Benign: Not applicable carcinoma 602
Not applicable Benign: Malignant Metastatic:
Not applicable Not applicable
Benign:
Not applicable

Co Congenital
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
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Chapter 29 Sensory Abnormalities 577

SENSORY ABNORMALITIES
PROPRIOCEPTIVE TACTILE VISUAL
ABNORMALITIES ABNORMALITIES ABNORMALITIES

Not applicable Not applicable Blepharospasm 584

Not applicable Not applicable Progressive supranuclear


palsy (Richardson-Steele-
Olszewski syndrome) 597

PROPRIOCEPTIVE TACTILE VISUAL


ABNORMALITIES ABNORMALITIES ABNORMALITIES

Malignant Primary, such as: Malignant Primary, such as: Malignant Primary, such as:
• Brain primary tumors 602 • Brain primary tumors 602 • Brain primary tumors 602
Malignant Metastatic: Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable Not applicable
Benign: Benign: Benign:
Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Malignant Primary: Malignant Primary: Malignant Primary:


Not applicable Not applicable Not applicable
Malignant Metastatic, such as: Malignant Metastatic, such as: Malignant Metastatic,
• Brain metastases 602 • Brain metastases 602 such as:
• Spinal metastases 603 • Spinal metastases 603 • Brain metastases 602
• Spinal primary tumors 603 • Spinal primary tumors 603 Benign, such as:
Benign: Benign: • Retinoblastoma 603
Not applicable Not applicable

PROPRIOCEPTIVE TACTILE VISUAL


ABNORMALITIES ABNORMALITIES ABNORMALITIES

Not applicable Not applicable Not applicable

Not applicable Neuropathies: Not applicable


• Hereditary motor and sensory
neuropathies 593
Tethered spinal cord
syndrome 600
(continued)
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578 Chapter 29 Sensory Abnormalities

Congenital (continued)
SENSORY ABNORMALITIES

AUDITORY GUSTATORY OLFACTORY


ABNORMALITIES ABNORMALITIES ABNORMALITIES
RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
AUDITORY GUSTATORY OLFACTORY
ABNORMALITIES ABNORMALITIES ABNORMALITIES
COMMON
Not applicable Not applicable Anorexia nervosa 582

UNCOMMON
Ménière’s disease 590 Not applicable Not applicable
Neurological complications of
acquired immunodeficiency
syndrome 592
RARE
Not applicable Not applicable Not applicable

Notes: (1) These are estimates of relative incidence because few data are available for the less common conditions.
(2) See additional characterization of auditory, gustatory, olfactory, proprioceptive, tactile, and visual abnormalities
in the overview section that follows this preview table.

Overview of Sensory and abnormalities in music tones. Reports of


Abnormalities ringing in the ears or tinnitus and other un-
usual sounds, such as buzzing, roaring, hiss-
Auditory Abnormalities ing, pulsating, or chirping sounds, suggest
auditory inner or middle ear problems and
The auditory system is responsible for the recep- nonauditory problems. The presence of tinni-
tion and processing of sound. Although this sys- tus and dizziness following a recent course of
tem is most commonly associated with hearing, antibiotics or exposure to toxins is sympto-
its anatomical proximity to the vestibular system matic of drug toxicity. Finally, symptoms
indicates the need to examine both systems in suggesting an auditory perception problem
the event of hearing loss. (Diagnosis of dizziness may include auditory illusions, hyperacusis
and vertigo is discussed in Chapter 27.) Decline (abnormal sensitivity to sound), and paracusis
in hearing acuity may result from conductive (repeating sounds). A sudden decline in hear-
(external or middle ear), sensorineural (cochlea ing acuity with fever, or change in conscious-
or cochlear nerve), or central (nuclei, auditory ness warrants immediate medical attention.
areas of temporal lobes) pathologies.
Slow-onset loss of high-pitched sounds is
Gustatory Abnormalities
most common in sensorineural problems and
loss of low-pitched sounds in conductive The gustatory system is responsible for the sen-
problems. Additional symptoms suggesting sation of salty, sweet, and bitter qualities of taste
cochlear (sensorineural) problems are height- for caloric nutrition and prevention of spoiled
ened perception of loudness, defects in clarity food ingestion. The sense of smell, additional
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Chapter 29 Sensory Abnormalities 579

SENSORY ABNORMALITIES
PROPRIOCEPTIVE TACTILE VISUAL
ABNORMALITIES ABNORMALITIES ABNORMALITIES

Not applicable Not applicable Arnold-Chiari malformation


(Chiari malformation) 582
Gaucher’s disease 587
Machado-Joseph disease 590

PROPRIOCEPTIVE TACTILE VISUAL


ABNORMALITIES ABNORMALITIES ABNORMALITIES

Not applicable Anxiety disorder/panic Not applicable


attacks 582
Neuropathies:
• Alcohol abuse 592

Not applicable Complex regional pain Not applicable


syndrome 586
Conversion disorder 586

Not applicable Restless legs syndrome 597 Not applicable

taste receptors beyond the tongue (palate, phar- tions of smell and taste are functionally and
ynx, larynx), and somatosensory (temperature, anatomically linked, resulting in equivocal
stinging) sensation serve as further sources of subjective clinical reports. Symptoms of
taste identification, discrimination, and protec- declined or absent sense of smell (hyposmia
tion. Gustatory symptoms necessitate assess- and anosmia, respectively) may be influenced
ment of both taste and smell with careful atten- by peripheral or central processing deficits of
tion to elimination of the redundant system. olfactory and/or gustatory systems. Loss of
Ageusia, the total loss of taste, is infrequent olfactory function is most consistent with a
due to the contributions from olfactory and loss of ability to identify and distinguish
somatosensory systems. More commonly, gus- odors. The loss of taste identification is a qual-
tatory symptoms are described as distorted, itative characteristic most suggestive of cranial
dulled, foul taste to typically pleasurable foods, nerve I (olfactory nerve). The trigeminal nerve
or intensified taste. Disturbed sense of taste also serves a qualitative odor function for irri-
may have a slow onset related to oral and den- tants, such as ammonia. Careful selection of
tal problems or tumor development. More various odors used for clinical testing in the
immediate alterations to taste are experienced absence of cognitive deficits permits differen-
in trauma and inflammatory processes. tiation between cranial nerves.
Neurological pathology and clinical test
Olfactory Abnormalities reliability may be masked in the presence of
The olfactory system participates in the sinus or respiratory infection, and symptoms
chemosensory appreciation of smell for of infection must be ruled out. Pathologies
digestive function and quality of life. Sensa- along the central olfactory pathways may
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580 Chapter 29 Sensory Abnormalities

result in impaired unilateral or bilateral smell, receptors, peripheral sensory nerves, spinal
SENSORY ABNORMALITIES

odorous hallucinations, distorted sense of pathways, and cerebral connections may all
smell (dysosmia), or increased olfactory acuity contribute to clinical symptoms affecting
(hyperosmia). The loss of olfactory discrimi- touch, temperature, pressure, vibration, pain,
nation, olfactory agnosia, is associated with and perception of tactile information. Slow
thalamic nuclei and temporal lobe problems of and fast onset of tactile symptoms may be de-
sudden onset most likely with trauma and scribed as paresthesia (abnormal spontaneous
stroke or slow onset more typical of tumor or sensation, burning, tingling, pins and needles),
autoimmune pathologies. dysesthesia (unpleasant sensation produced by
normally painless stimuli), or numbness. Spe-
Proprioceptive Abnormalities cific touch-related symptoms are anesthesia
(total loss of touch), hypesthesia (partial loss),
Proprioception involves a network of receptors
and hyperesthesia (increased touch sensitivity).
within muscle, tendon, and joint; dorsal column
Anatomical arrangements of peripheral
ascending spinal pathways; and distributed
and spinal paths allow direct relationships
central networks for the interpretation of posi-
between tactile impairments and pathology to
tion and movement. Symptoms of disordered
be made. Patterns of tactile, pain, temperature,
limb movement (incoordination), posture, and
and pressure loss or symptoms that corre-
balance suggest proprioceptive peripheral or
spond to a specific peripheral sensory nerve or
central pathology. Disordered limb movement
spinal-level dermatome permit identification
may present as temporal and spatial problems
of pathological location. Sudden onset of
including movement path, target accuracy, alter-
pattern-specific tactile sensory loss is most
nating movements, and bimanual function.
common in trauma. However, slow-onset sen-
Clinical testing for proprioceptive loss may
sory loss can be described in imprecise bilat-
be performed to identify loss of peripheral
eral distributions that do not correlate with
receptor and sensory nerve distributions as
anatomical distribution and are more sugges-
well as for spinal pathology classification.
tive of metabolic, tumor, inflammatory, or
Clinical tests that indicate failure of position
degenerative pathologies. Sensory loss mapped
and movement awareness confirm involve-
with the medial lemniscal and anterior and
ment of the proprioceptive system without
lateral spinothalamic pathways may reveal pat-
specificity of location. Observed movement
terns of spinal cord pathology and assist in
abnormalities of limbs or trunk with proprio-
classifying the injury. In the presence of cogni-
ceptive clinical test failure may reflect the im-
tive function, clinical tactile tests can reveal
plicit nature of proprioception and add little
problems with two-point discrimination,
to identifying the pathology of the subcortical
stereognosis, graphesthesia and other tactile
processing or the cortical sensory areas. The
perceptions suggesting pathology of the thala-
implicit and intimate nature of proprioception
mic or sensory cortical areas. Intermittent
to movement contributes to a lack of clinical
tactile symptoms may be suggestive of seizure
tests and preference for use of tactile sensation
or transient ischemic episodes. Slow onset of
to assist with diagnosis of pathologies above
sensory loss without Red Flags signifies degen-
the spinal cord. Position and movement
erative, metabolic, autoimmune, or tumor
awareness symptoms that are reported clini-
causes. Sudden onset of tactile symptoms
cally should be considered particularly impor-
with Red Flags indicates a possibly serious
tant if they are characterized by sudden onset,
pathology and medical care is indicated.
accompanied by vital sign abnormalities, or
Tactile symptoms in the presence of motor
coexisting with paresis with hyperreflexia.
symptoms and hyperreflexia suggest pathol-
ogy at or above the spinal cord. Subjective
Tactile Abnormalities
reports of pain, as one aspect of tactile sensa-
Tactile sensation requires function of multiple tion, are covered extensively in other chapters.
receptive fields in the skin, transmission Note, however, that although pain is an impor-
centrally, and interpretation in a distributed tant sensation discussed elsewhere, pain may
network from brainstem to primary sensory also be representative of nervous system
cortex. Pathologies affecting the sensory pathology.
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Chapter 29 Angiomatosis (Von Hippel-Lindau Disease) 581

Visual Abnormalities a distal to proximal pattern are usually affected

SENSORY ABNORMALITIES
first, followed by upper extremities. In the
Vision is a complex sensory system utilized majority of cases, a mild gastrointestinal or res-
and depended on for function. The complexity piratory infection precedes symptoms. Muscles
is represented by the elaborate sensory recep- of the trunk and cranium may be affected
tor arrangement in the eye and its links with following muscles of the limbs later in the
other neural networks governing motor disease process.
control, posture, and equilibrium, as well as
parallel processing pathways. Classification of Background Information
visual symptoms by visual acuity, oculomotor, Symptoms are thought to result from an im-
and visual perceptual categories assists with munologic reaction causing demyelination of
identifying pathology. Abnormality in visual peripheral nerves, and in severe cases, axonal
acuity tested in the presence of cognition and degeneration as well. In addition to clinical
consciousness indicates problems in the visual presentation, differential diagnosis is estab-
receptors. Abnormalities in visual acuity found lished by the presence of only a few lympho-
on the Snellen chart indicate pathology of the cytes and an increase in protein in cerebrospinal
lens or fovea complex, whereas problems with fluid as well as electromyographic findings of
blindness or visual fields represent optic nerve reduction in amplitudes of muscle action
or central pathway pathologies. Oculomotor potentials, slowed conduction velocity, conduc-
signs include reflexes (pupillary, vestibulo- tion block in motor nerves, prolonged distal
ocular, oculocephalic) and eye movement latencies, and prolonged or absent F responses.
(cranial nerve and supranuclear), which if Standard treatment includes administration of
abnormal require further testing to differenti- intravenous immune globulin and plasma
ate cranial nerve, vestibular system, or central exchange. The majority of individuals recover
pathology. Symptoms of unilateral small pupil completely or almost completely within a few
and ptosis indicate Horner’s syndrome. weeks to a few months; however, the presence of
Vision participates in the perception of axonal degeneration increases the regeneration
static and dynamic aspects of objects and the time period to 6 to 18 months. Three percent
environment for function. Ventral temporal to 5% of individuals with this condition do not
and dorsal parietal lobe neural networks from survive.1
the occipital lobe participate in perception and
represent an extensive cortical area at risk for ■ Angiomatosis (Von
pathology and contribution to visual symptoms Hippel-Lindau Disease)
affecting limb and mobility functions. As with Chief Clinical Characteristics
the other sensations, sudden onset of visual dis- This presentation commonly includes headaches,
turbances with other neurological symptoms, problems with balance and walking, dizziness,
unstable vital signs, or fever demands immedi- weakness of the limbs, vision problems, and high
ate medical attention. Conversely, slow onset of blood pressure.
gradual decline in visual acuity or perception is Background Information
less urgent and typical of degenerative, meta- This condition is a rare, genetic multisystem
bolic, and autoimmune processes. condition characterized by the abnormal
growth of tumors in certain parts of the body
Description of Conditions That (angiomatosis). Tumors of the central nervous
May Lead to Sensory Abnormalities system are benign, are comprised of a nest of
blood vessels, and are called hemangioblas-
■ Acute Demyelinating tomas (or angiomas in the eye). Hemangioblas-
Polyneuropathy (Guillain-Barré tomas may develop in the brain, the retina of
Syndrome) the eye, and other areas of the nervous system.
Chief Clinical Characteristics Other types of tumors develop in the adrenal
This presentation typically involves progressive glands, the kidneys, or the pancreas. Cysts
paresthesias described as numbness, tingling, and (fluid-filled sacs) and/or tumors (benign or
prickling, and weakness over the course of sev- cancerous) may develop around the heman-
eral days to a few weeks. Lower extremities in gioblastomas and cause the symptoms listed
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582 Chapter 29 Anorexia Nervosa

above. Specific symptoms vary among individ- diagnosis of an anxiety condition is based on
SENSORY ABNORMALITIES

uals and depend on the size and location of the criteria from the DSM-IV-TR.3 Anxiety condi-
tumors. Individuals with this condition are also tions are classified into specific categories. One
at a higher risk than normal for certain types of category, panic condition, has dizziness as one
cancer, especially kidney cancer. Treatment of its features. Panic condition is defined by
varies according to the location and size of recurrent attacks with at least four of the fol-
the tumor and its associated cyst. In general, lowing features: increased heart rate, sweating,
the objective is to treat the tumors when they trembling or shaking, dyspnea, sensation of
are causing symptoms but are still small. Treat- choking, chest pain or discomfort, nausea or
ment of most cases usually involves surgical abdominal distress, feelings of dizziness, fear of
resection. Certain tumors can be treated with losing control, fear of dying, paresthesias, and
focused high-dose irradiation. Individuals with chills or hot flashes. The etiology of anxiety
this condition need careful monitoring by a conditions includes genetic factors, social and
physician and/or medical team familiar with psychological factors, and physiological and
the condition. biochemical abnormalities.4 Treatment includes
cognitive-behavioral therapy, relaxation exer-
■ Anorexia Nervosa cises and pharmacologic treatment.4 The course
Chief Clinical Characteristics of this condition is variable; most individuals
This presentation involves impaired ability to maintain normal social lives. Clinicians are
smell, decreased rapid visual information pro- encouraged to consider referral of individuals
cessing, abnormal perception of body schema, suspected of having this condition to a mental
cold body temperature, and dryness or yellow- health specialist for evaluation and treatment.
ing of skin. Symptoms of mood disturbance are
often present and are associated with sequelae ■ Arnold-Chiari Malformation
of starvation. (Chiari Malformation)
Background Information Chief Clinical Characteristics
The diagnostic features of anorexia nervosa This presentation may include visual or swal-
are that the individual refuses to maintain a lowing disturbances in combination with
minimally normal body weight, is intensely pain in the occipital or posterior cervical areas,
afraid of gaining weight, and exhibits a signif- downbeating nystagmus, progressive ataxia,
icant disturbance in the perception of the body progressive spastic quadriparesis, or cervical
shape or size. In addition, postmenarchal syringomyelia.5,6
women with this condition are commonly Background Information
amenorrheic. Treatment primarily consists of This condition encompasses a number of con-
psychotherapy to address underlying cognitive genital abnormalities at the base of the brain,
and behavioral correlates of this condition. including extension of the cerebellar tissue or
The standardized mortality ratio is high.2 displacement of the medulla and fourth ven-
■ Anxiety Disorder/Panic Attacks tricle into the cervical canal.5 This condition
has two main types. Individuals with the more
Chief Clinical Characteristics
common form of this condition, Type I, often
This presentation typically includes repeated
remain asymptomatic until adolescence or
panic or anxiety attacks. Symptoms include
adult life.5 Type II is primarily seen in infants
increased heart rate and respiratory rate, pupil
and young children. Please see the pediatric
dilation, and trembling and sweating with feel-
section of this textbook for a more complete
ings of fear and dread. The symptoms usually
description of this type. Diagnosis is made
subside in 15 to 30 minutes.
by magnetic resonance imaging, computed
Background Information tomography, myelography, or some combina-
This condition is a nonspecific syndrome and tion of these tests.6 Treatment varies depend-
can be due to a variety of medical or psychi- ing on clinical progression, and may include
atric syndromes or observed as part of a drug surgical intervention such as an upper cervical
withdrawal or drug intoxication effect. The laminectomy or enlargement of the foramen
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Chapter 29 Benign Paroxysmal Positional Vertigo 583

magnum. Even with surgery symptoms may study group include presence of recurrent

SENSORY ABNORMALITIES
persist or progress.5 oral ulceration, recurrent genital ulceration,
eye lesions, skin lesions, papulopustular le-
■ Arteriovenous Malformation sions, and/or a positive pathergy test.1,7 Med-
Chief Clinical Characteristics ical treatment typically consists of corticos-
This presentation may be characterized by teroids and immunosuppressants. Neurological
seizures and severe headache. Hemorrhage may symptoms tend to clear within weeks, but can
result in paresis, ataxia, dyspraxia, dizziness, sometimes recur or result in permanent
tactile and proprioceptive disturbances, visual deficits.1 Onset before the age of 25 and male
disturbances, aphasia, paresthesias, and cog- sex indicate a poorer prognosis.
nitive deficits.1
■ Bell’s Palsy
Background Information
This condition is caused by a tangle of arteries Chief Clinical Characteristics
and veins that cause abnormal communica- This presentation typically involves unilateral
tion within the vasculature. Approximately facial paralysis and is characterized by acute
12% of the 300,000 individuals in the United drooping of the eyelid and/or corner of the
States with this condition are symptomatic. mouth, drooling, impairment of taste, and
This condition is caused by a developmental dryness of the eye with or without excessive
abnormality that likely arises during embry- tearing that progresses within 48 hours. Tactile
onic or fetal development. Neurological sensation is intact. Facial paralysis is commonly
damage occurs due to reduction of oxygen unilateral, though in rare cases may present
delivery, hemorrhage, or compression of bilaterally. All three quadrants of the face are
nearby structures of the brain or spinal cord. affected. Long-term facial paralysis may lead to
Computed tomography, magnetic resonance synkinesis (imbalance of muscular activation),
imaging, and arteriography confirm the resulting in significant facial distortion.8
diagnosis. Ligation and embolization may Background Information
be used to reduce the size of the lesion prior This condition is caused by an idiopathic
to surgical excision, which is the preferred inflammation of the facial nerve, likely due to
method of treatment. Stereotactic radiation a viral infection, commonly herpes simplex.
and proton beam therapy are alternative Diagnosis utilizes clinical examination to
approaches to invasive methods of interven- rule out other causes of facial weakness; for
tion. Up to 90% of individuals who experi- example, facial weakness due to cortical
ence a hemorrhagic arteriovenous malfor- or subcortical lesions is associated with
mation survive.1 impaired sensation and the frontalis and leva-
■ Behçet’s Disease tor palpebrae muscles are weakened, but not
paralyzed. Treatment involves antiviral and
Chief Clinical Characteristics anti-inflammatory medications and physical
This presentation may include bilateral pyram- therapy to address paralysis and symmetry of
idal signs (signs related to lesions of upper mo- motion and to prevent synkineses.8–11 Natural
tor neurons or descending pyramidal tracts, such recovery of facial motor control occurs within 3
as a positive Babinski sign or hyperreflexia), to 6 months in 94% of patients with incomplete
headache, memory loss, hemiparesis, cerebellar paralysis, but residual synkinesis and weakness
ataxia, balance deficits, sphincter dysfunction, often remain in those with complete palsies.9
or cranial nerve palsies. In addition to these
neurological signs individuals with this condi- ■ Benign Paroxysmal Positional
tion also may present with arthritis; renal, Vertigo
gastrointestinal, vascular, and cardiac diseases;
Chief Clinical Characteristics
and genital, oral, and cutaneous ulcerations.7
This presentation commonly involves dizziness
Background Information or vertigo (typically described as a spinning
Mean age of onset is in the third decade of life. sensation), light-headedness, imbalance, and
Diagnostic criteria according to an international nausea. Symptoms are precipitated by a
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584 Chapter 29 Blepharospasm

position change of the head relative to gravity, ■ Brachial Plexus Injury


SENSORY ABNORMALITIES

such as getting out of bed, rolling over in bed, Chief Clinical Characteristics
or looking up.12,13 This presentation is characterized by upper
Background Information extremity weakness and sensory loss related
This condition is thought to occur when to damage of the brachial plexus, a network
free-floating debris becomes trapped in the of nerves that conducts signals from the
semicircular canal (canalithiasis) or becomes spine to the shoulder, arm, and hand. Associ-
adhered to the cupula (cupulolithiasis), ren- ated symptoms include hyporeflexia and
dering the semicircular canal sensitive to grav- hypotonicity.
ity and thus changes in head position rather Background Information
than head motion. The etiology of benign Injuries are often traumatic; the most com-
paroxysmal positional vertigo is unknown in mon are stretch injuries occurring during the
most cases, but is associated with head trauma, birth process. This condition can be classified
vestibular neuritis, and vertebrobasilar ischemia, in terms of mechanism of injury, closed (mo-
and can occur after ear surgery or prolonged tor vehicle accident) vs. open (intraoperative
bed rest.14 This condition occurs more often in injury and gunshot wounds); location of in-
the elderly, and tends to recur in up to 15% of jury (spinal nerve root, trunk, cord, peripheral
cases within 1 year and 50% of cases within nerve); or type of nerve damage.22 The four
40 months.15 The diagnosis is confirmed with types of nerve damage are avulsion, the most
the Hallpike maneuver, in which the head is severe type, in which the nerve is torn from the
rotated 45 degrees and tilted back while hang- spinal root; rupture, in which the nerve is torn
ing off the end of the table. This position will midsubstance; neuroma, in which the injured
elicit torsional and vertical nystagmus when nerve has scarred, causing a conduction block;
the vertical semicircular canals are involved. and neurapraxia or stretch, in which the nerve
Duration of vertigo while in the Hallpike posi- has been damaged but not torn. Diagnosis of
tion will determine if the individual has injury and localization of the lesion require
canalithiasis (short duration, <1 minute) or clinical investigation, electrodiagnostic study,
cupulolithiasis (long duration, >1 minute). and imaging.22,23 Management differs depend-
Turning the head while supine will elicit ing on type and severity of injury. Open
horizontal nystagmus and vertigo when the injuries with vascular damage should be ex-
horizontal (lateral) semicircular canals are plored operatively immediately. In the absence
involved. Nonsurgical treatment consisting of of clinical or electrophysiological recovery
a series of positional changes to move the after 2 to 4 months, gunshot wounds without
debris out of the canals, known as the canalith vascular compromise should undergo surgical
repositioning maneuver, can be very success- intervention. Spontaneous recovery occurs in
ful.14,16–20 In recalcitrant cases, the offending many closed injures; therefore, surgical inter-
canal can be plugged surgically.21 vention is delayed 4 to 5 months.22 Outcomes
■ Blepharospasm are favorable in patients who have less severe
nerve damage and those who undergo early
Chief Clinical Characteristics operation when indicated.22 Despite residual
This presentation includes excessive involuntary weakness and impaired functional use of the
closure of the eyelids primarily due to spas- extremity, the majority of patients report satis-
modic contraction of the orbicularis oculi mus- faction with their quality of life postinjury
cles. Severity of symptoms ranges from frequent and/or post–surgical repair.24
blinking to functional blindness.
Background Information ■ Carpal Tunnel Syndrome
The majority of cases of this condition are Chief Clinical Characteristics
idiopathic. Diagnosis is made by clinical pres- Presentation of decreased grip strength and
entation. Treatment includes psychotherapy, difficulty performing tasks requiring grasp or
biofeedback, drugs, and surgery. Botulinum manipulation of objects are preceded by initial
toxin A is the most effective form of treatment. report of pain and numbness/tingling in the
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Chapter 29 Cerebral Aneurysm 585

palmar aspect of the thumb, index finger, mid- ■ Cavernous Malformation

SENSORY ABNORMALITIES
dle finger, and radial half of the ring finger with Chief Clinical Characteristics
radiation up the forearm.25 This presentation includes symptoms that are
Background Information dependent on the neuroanatomical location af-
This condition results from compression of the fected. Symptoms often consist of paresthesias,
median nerve as it passes through the carpal visual disturbances, headaches, and seizures.
tunnel. It is associated with repetitive stress, Background Information
such as typing or performing assembly line This condition is a rare disorder of the vascular
tasks. Differential diagnosis includes cervical system of the brain where a blood-filled mass, or
radiculopathy or compression of the median hemangioma, forms. This condition is fre-
nerve proximal to the carpal tunnel. Diagnosis quently inherited in an autosomal dominant
is achieved through use of special tests (eg, pattern. Diagnosis is made on clinical manifes-
Phalen’s sign, monofilament testing, and tations and magnetic resonance imaging find-
provocative tests) and via electromyography and ings of clusters of vessels with a rim of hypoden-
nerve conduction velocity tests.26 Treatment sity on T1-weighted images.1 For individuals
consists of splinting, pharmacologic manage- with this condition who experience neurological
ment of inflammation and pain, modalities, symptoms, treatment is symptomatic and sup-
stretching and strengthening, ergonomic modi- portive. Surgical removal or radiation may be
fications, and surgery if indicated.27 Nonsurgical performed.1 Individuals with prior hemorrhage
care including physical therapy is emphasized and infratentorial location of the hemangioma
first, and it is most effective in those with mild have a poorer prognosis.30
impairment. If nonsurgical management fails,
surgery is usually recommended. In those with ■ Cerebral Aneurysm
severe forms of this condition who have been
properly diagnosed, 70% report complete satis- Chief Clinical Characteristics
faction with pain relief. However, residual weak- This presentation may involve loss of balance in
ness and reoccurrence may occur.25 combination with a whole host of other neuro-
logical symptoms and signs that depend on the
affected cerebral tissue, including visual and
■ Cataracts
proprioceptive loss. Any associated signs or
Chief Clinical Characteristics symptoms may not be reported due to the fact
This presentation can be characterized by that this condition is typically asymptomatic
gradual central greater than peripheral vision prior to rupture. However, if the aneurysm re-
loss that results in myopia, loss of contrast sults in a mass effect, ischemia, or hemorrhage,
sensitivity, and blurred and foggy vision. then neurological signs and symptoms are
dependent on the affected location.1,31,32
Background Information
This condition occurs when the normally clear Background Information
lens opacifies, causing edema and shrinkage.28 There has been some description of genetic
Eventually, the opacification can result in a factors in this condition.31 Cigarette smoking,
milky sac that can dislocate either anteriorly or hypertension, and heavy alcohol use have all
posteriorly through the lens. Posterior disloca- been found to be correlated with increased risk
tions may result in improved light perception. of aneurysm development.31,32 Factors associ-
This condition commonly affects both eyes, ated with increased risk of rupture include size
although one eye is usually more affected. It of aneurysm, location in the posterior circula-
may occur as a primary disease process related tion, and a previous history of aneurismal sub-
to age-related degeneration, secondary to an- arachnoid hemorrhage.33,34 Definitive diagno-
other medical condition, or congenitally. Age sis is based on catheter angiography; however,
and family history are the strongest predictors magnetic resonance angiography, magnetic
of developing the age-related form of the con- resonance imaging, and computed tomogra-
dition.29 Treatment typically involves surgical phy may aid in the diagnosis. Unruptured
interventions to the lens. aneurysms are sometimes surgically treated.
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586 Chapter 29 Cerebral Arteriosclerosis

Aneurysm size, location, and prior history of a administered, the reversal of symptoms should
SENSORY ABNORMALITIES

subarachnoid hemorrhage help to determine if begin to occur within hours to days with vari-
the risk of surgical treatment is worth the able degrees of recovery. Memory has been
potential benefits. Most aneurysms that have shown to have the poorest return, and mortal-
hemorrhaged must be treated surgically. ity rates of up to 17% have been reported.5
Patients with a previous rupture are at an
11 times greater risk of having a second in- ■ Complex Regional Pain Syndrome
tracranial aneurysm rupture. When aneurysms Chief Clinical Characteristics
do rupture, many patients die within 1 month This presentation typically includes a traumatic
of the rupture, and those who survive often onset of severe pain accompanied by allodynia,
have residual neurological deficits.31 hyperalgesia, and trophic, vasomotor, and sudo-
motor changes in later stages. This condition is
■ Cerebral Arteriosclerosis characterized by disproportionate responses to
Chief Clinical Characteristics painful stimuli.
This presentation includes visual disturbances,
Background Information
headache, and facial pain.
This regional neuropathic pain disorder pres-
Background Information ents either without direct nerve trauma (Type I)
Thickening and hardening of the artery walls or with direct nerve trauma (Type II) in any
in the brain lead to the development of this region of the body.35 This condition may pre-
condition. Diagnosis is established by com- cipitate due to an event distant to the affected
puted tomography or magnetic resonance im- area. Thermography may confirm associated
aging of the brain. Treatment includes lifestyle sympathetic dysfunction. Treatment for this
modification, pharmacotherapy, and surgery. condition includes physical therapy for desensi-
Cerebral arteriosclerosis can result in ischemic tization, prevention of secondary complications
or hemorrhagic stroke, thus causing neurolog- and restoration of mobility, pharmacothera-
ical impairments.1 peutics for pain reduction, and surgical inter-
vention such as sympathectomy. Prognosis is
■ Cerebral Beriberi (Korsakoff ’s primarily dependent on timely detection of the
Amnesic Syndrome, Wernicke- disease and rate of progression.1
Korsakoff Syndrome)
Chief Clinical Characteristics ■ Conversion Disorder
This presentation involves ophthalmoparesis, Chief Clinical Characteristics
nystagmus, ataxia, and confusion, as well as This presentation can be characterized by
impaired learning and memory. Other com- motor or sensory deficits, seizures or convul-
mon symptoms include peripheral neuropathy, sions, or blindness or deafness. Symptoms
postural hypotension, syncope, impaired olfac- usually appear suddenly and abate in less than
tory discrimination, mild hypothermia, and 2 weeks.
confabulation.5
Background Information
Background Information This condition is one type of somatoform dis-
This condition is due to a thiamine deficiency order in which psychological stress becomes
that results in a diffuse decrease in cerebral translated into physical problems. The DSM-
glucose utilization. It is most commonly IV-TR defines conversion disorder by symp-
observed in individuals who abuse alcohol and toms that simulate a neurological or other
have nutritional deficiencies, although it is not medical condition that involves voluntary
limited to this population.5 Diagnosis can be muscles or sensory organs excluding pain and
made by blood tests to examine thiamine sexual functions.1 Clinical findings include
levels. Neuroimaging may show slowed brain patterns of sensory loss that do not follow nor-
activity as well as lesions in the medial thala- mal patterns from neurological insults and
mus and periaqueductal region.5 Medical symptoms that disappear when a patient is
treatment involves the immediate administra- distracted or thinks that no one is watching.
tion of thiamine. Once thiamine has been Patients often have a history of emotional
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Chapter 29 Glaucoma 587

disturbance, stress, or traumatic event. Differ- Background Information

SENSORY ABNORMALITIES
ential diagnosis is based on clinical findings. It The severity, type, and particular combination
is helpful to treat the patient as though he or of symptoms are variable, depending on the
she has had an illness and is now in the process drug exposure, whether it is unilateral or bilat-
of recovery.1 Cognitive and behavioral thera- eral, the speed of onset, and the individual. A
pies may be effective in treating underlying slow unilateral loss may produce few symp-
psychological issues. Prognosis is variable toms, since the brain can compensate through
with differing degrees of recovery in days to other mechanisms, whereas a fast bilateral loss
months. Good prognostic factors include can produce significant disability. The key
acute onset of symptoms, short duration of diagnostic feature is a history of drug or chem-
symptoms, healthy premorbid functioning, ical exposure. Treatment involves removing
higher intelligence, absence of coexisting psy- exposure to the medication if possible, and
chopathology, and presence of an identifiable vestibular habituation and balance exercises.
stressor. Poor prognostic symptoms include
pseudoseizures, age greater than 40, and long- ■ Gaucher’s Disease
lasting severe disability.36 Chief Clinical Characteristics
This presentation commonly includes slowly pro-
■ Detached Retina gressive mental decline, seizures, ataxia, and,
Chief Clinical Characteristics upon later development, weakness with spastic-
This presentation typically involves new onset ity and splenomegaly and deficits in lateral gaze.1
of visual disturbances such as flashes or floaters
Background Information
in the visual field, accompanied by central or side
This condition is a rare disorder, although it is
visual field loss and occasionally severe vitreal
prevalent among the Ashkenazi Jewish popula-
hemorrhage.37
tion.38 The disease is an autosomal genetic dis-
Background Information order in which glucocerebroside accumulates
This condition occurs when the retina demon- in the spleen, liver, lungs, bone marrow, and
strates breaks. Breaks commonly occur in brain due to a deficiency in an enzyme.1 There
areas of high adhesion, such as about the are three types of Gaucher’s disease. The most
vasculature. This condition may occur sponta- common, type 1, is characterized by no central
neously, as a result of trauma or other forms of nervous system involvement. In type 2, infants
eye pathology, or as a result of metabolic dis- have extensive and progressive neurological
ease such as diabetes mellitus. Prompt oph- damage.38 Type 3 is less common and is associ-
thalmic evaluation is necessary if this condi- ated with less severe neurological symptoms.38
tion is suspected. Surgical interventions may Diagnosis is established by clinical presenta-
be considered to address this condition, in- tion, laboratory tests that show an increase in
cluding scleral buckling, vitrectomy, and pneu- total acid phosphatase, and biopsy of bone
matic retinopexy. marrow that is positive for Gaucher cells. En-
zyme replacement therapy is standard for most
■ Drug Toxicity patients with types 1 and 3. However, there is
Chief Clinical Characteristics no effective treatment for the severe brain
This presentation involves tinnitus (fluctuating damage that may occur in patients with types 2
or constant) and hearing loss (mild to complete and 3. Prognosis for patients with type 2 dis-
deafness) if the cochlea is involved; vertigo, ease is poor with death within the first 2 years
vomiting, nystagmus, and imbalance if the of life. For type 3 disease, symptoms typically
vestibular system is involved unilaterally; and present in childhood and death occurs by
headache, ear fullness, oscillopsia, an inability age 10 to 15 years.1
to tolerate head movement, a wide-based gait,
difficulty walking in the dark, a feeling of ■ Glaucoma
unsteadiness and actual unsteadiness while Chief Clinical Characteristics
moving, imbalance to the point of being unable This presentation includes gradual loss of visual
to walk, light-headedness, and severe fatigue in acuity, which classically occurs from peripheral
bilateral involvement. to central within the visual field.
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588 Chapter 29 Herpes Zoster

Background Information and tinnitus. Taste loss in the tongue and dry
SENSORY ABNORMALITIES

This condition occurs when optic nerve fibers mouth and eyes may also occur.
are lost in the retinal nerve fiber layer second-
Background Information
ary to elevated intraocular pressure.39 Clini-
This condition is a common complication of
cal assessment of the retina usually confirms
herpes zoster. This condition is an infection
the diagnosis, but many other imaging
caused by the spread of varicella-zoster virus,
modalities are now available to evaluate the
which is the virus that causes chickenpox, to
retina and optic nerve for individuals sus-
facial nerves. This condition occurs in people
pected of having this condition. Management
who have had chickenpox and represents a re-
of this condition currently focuses on con-
activation of the dormant varicella-zoster
trolling intraocular pressure, since this find-
virus. When treatment is needed, medications
ing is the only known risk factor for develop-
such as antiviral drugs or corticosteroids may
ing the condition.
be prescribed. Vertigo also may be treated with
■ Herpes Zoster the drug diazepam.
Chief Clinical Characteristics ■ Hydromyelia (Syringomyelia)
This presentation typically includes an
Chief Clinical Characteristics
exquisitely painful rash or blisters along a
This presentation involves insidious onset of
specific dermatomal pattern accompanied
symptoms including upper and lower extrem-
by flu-like symptoms. Sensory and motor
ity weakness and numbness and, less commonly,
losses within spinal segmental distributions
pain. Trauma usually precedes the onset on
may occur, although this is rare. Individuals
symptoms, but the time frame for subsequent
with this condition also demonstrate a pre -
development of weakness and sensory changes
vious history of varicella exposure or infec-
is variable.
tion. Pain associated with this condition
may be disproportionate to the extent of skin Background Information
irritation. The initial presentation of this This condition is caused by an abnormal widen-
condition may be confused with radiculopa- ing of the central canal of the spinal cord, lead-
thy due to the distribution of symptoms. ing to the accumulation of cerebrospinal fluid
The presence of the rash, extreme pain, and hydrocephalus. Differential diagnosis must
general malaise, and unclear association be made between hydromyelia and other disor-
with spinal movement aids in differential ders such as syringomyelia, spinal cord tumor,
diagnosis. and spinal arteriovenous malformation. Mag-
netic resonance imaging and electromyography
Background Information are used to confirm the diagnosis of this condi-
The virus remains dormant in the spinal gan- tion. Surgery may be indicated to decrease or
glia until its reactivation during a period of eliminate the symptoms. Prognosis is variable.
stress, infection, or physical exhaustion. Treat-
ment includes the administration of antiviral ■ Idiopathic Intracranial
agents as soon as the zoster eruption is noted, Hypertension
ideally within 48 to 72 hours. If timing is
greater than 3 days, treatment is aimed at con- Chief Clinical Characteristics
trolling pain and pruritus and minimizing the This presentation includes headaches, visual
risk of secondary infection.40 disturbances, dizziness, or tinnitus.41
Background Information
■ Herpes Zoster Oticus (Ramsay Diagnostic criteria include presence of symp-
Hunt Syndrome) toms that reflect generalized intracranial
Chief Clinical Characteristics hypertension or papilledema, elevated in-
This presentation commonly includes intense ear tracranial pressure (>250 mm H2O per lumbar
pain; a rash around the ear, mouth, face, neck, puncture), normal cerebrospinal fluid compo-
and scalp; and paralysis of facial nerves. Other sition, absence of lesions on magnetic reso-
symptoms may include hearing loss, vertigo, nance imaging or computed tomography, and
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Chapter 29 Lateral Medullary Syndrome (Wallenberg’s Syndrome) 589

no other cause identified.42 Also known as Background Information

SENSORY ABNORMALITIES
pseudotumor cerebri or benign intracranial The etiology is a bacterial or viral infection
hypertension, this condition is characterized causing inflammation of the vestibular nerve
by increased intracranial pressure without (neuronitis) or the labyrinth (labyrinthitis).
associated space-occupying lesions or hydro- The terms are often used interchangeably be-
cephalus. Approximately one-third of individ- cause it is difficult to distinguish neuronitis
uals with idiopathic intracranial hypertension from labyrinthitis.43 The diagnostic hallmark is
recover within 6 months following repeated unilateral hyporesponsiveness with caloric test-
lumbar punctures and drainage of cere- ing.44 Regardless of the type of infection, the
brospinal fluid to maintain the pressure at near treatment consists of destroying the bacteria by
normal or normal levels. Weight reduction and means of antibiotics. If the labyrinthitis is
surgical gastric placation are effective forms of caused by a break in the membranes separating
treatment.1 the middle and inner ears, surgery may also be
required to repair the membranes to prevent
■ Klüver-Bucy Syndrome a recurrence of the disease. Residual vertigo
Chief Clinical Characteristics can be reduced with vestibular habituation
This presentation typically involves oral ex- exercises.45
ploratory behavior, tactile exploratory behav-
ior, and hypersexuality, with additional symp- ■ Lateral Medullary Syndrome
toms and signs that may include visual agnosia, (Wallenberg’s Syndrome)
decreased attention, seizures, and dementia. Chief Clinical Characteristics
This presentation can include nystagmus, oscil-
Background Information lopsia, vertigo, nausea, vomiting, impairment
This condition arises from medial temporal of pain and thermal sense over half of the body,
lobe dysfunction, and it may be associated ipsilateral Horner syndrome including miosis,
with many different etiologies including her- ptosis, anhidrosis, hoarseness, dysphagia, ipsi-
pes encephalitis, traumatic brain injury, and lateral paralysis of palate and vocal cord with
Pick’s disease. This condition is diagnosed a diminished gag reflex, vertical diplopia or
clinically by the presence of the above cluster sensation of tilting vision, ipsilateral ataxia of
of symptoms. Treatment is symptomatic and limbs, loss of balance to ipsilateral side, and
primarily through pharmacologic means. impaired sensation of ipsilateral half of the
Prognosis is poor.1 face.1,46,47
■ Labyrinthitis and Neuronitis Background Information
Chief Clinical Characteristics In 75% of cases, onset is sudden. Main
This presentation may be characterized by a etiologic factors associated with this condi-
relatively sudden onset of severe, constant ro- tion include large-vessel infarction, arterial
tary vertigo (made worse by head movement) dissection, small-vessel infarction, cardiac
that resolves after days or weeks.43,44 This con- embolism, tumor, hemorrhage, and other
dition is associated with spontaneous nystag- unknown factors. The most typical cause is
mus, postural imbalance, and nausea without occlusion of the vertebral artery, with the
accompanying cochlear or neurological symp- posterior inferior cerebellar artery being
toms.44 The specific presentation varies depend- involved to a lesser degree. A thorough his-
ing on the site of the infection. If the vestibular tory, clinical examination, and magnetic res-
system is affected, the symptoms will include onance imaging may confirm the diagnosis.
dizziness and difficulty with vision and/or An emergency response is required for
balance. If the inflammation affects the cochlea, individuals experiencing the new onset of
this condition will produce disturbances in hear- symptoms. Residual symptoms include
ing, such as tinnitus or hearing loss. The symp- balance deficits (most common), dysphagia,
toms can be mild or severe, temporary or dizziness, and numbness, which may be
permanent, depending on the severity of the addressed with physical, occupational, and
infection. speech therapy.
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590 Chapter 29 Ligament Sprains

■ Ligament Sprains Background Information


SENSORY ABNORMALITIES

Chief Clinical Characteristics This condition consists of wet and dry forms.
This presentation includes decreased proprio- In the dry form, cellular debris (drusen) may
ception and balance following a ligament sprain accumulate between the choroid and retina. In
to the lower extremity. the wet form, blood vessels may grow from be-
hind the choroid, which may lead to retinal
Background Information detachment.50 Examination reveals central sco-
Functional instability is especially common tomata and an alteration of the retina around
following ankle and knee sprains, and propri- the maculae. Treatment to delay progression or
oceptive loss is thought to be a contributory possibly improve vision includes visual aids
factor. Damage to joint capsule and muscle and medication. This condition worsens with
stretch receptors leads to proprioceptive loss. time and eventually may lead to blindness.1
Diagnosis of proprioceptive loss following an
ankle sprain is made by clinical assessment; an ■ Ménière’s Disease
individual’s ability to identify joint position in
space without additional sensory input grossly Chief Clinical Characteristics
determines the degree of proprioceptive loss. This presentation is characterized by recurrent
Proprioception and balance can be restored to episodic spontaneous attacks of vertigo (exac-
varying degrees fusing therapeutic exercises erbated by head movements and accompanied
that challenge balance.48,49 Residual high-level by nausea and vomiting), fluctuating sen-
balance deficits are common.49 sorineural hearing loss, aural fullness, and
tinnitus.44,51–58 In the late stages the symptoms
■ Machado-Joseph Disease are more severe, hearing loss is less likely to fluc-
Chief Clinical Characteristics tuate, and tinnitus and aural fullness may be
This presentation may involve visual impair- more constant. Sudden unexplained falls with-
ments such as nystagmus in combination with out loss of consciousness or vertigo (drop
slowly progressive ataxia, rigidity, dystonia, attacks) may occur. Attacks can be preceded by
weakness in the hands and feet, and difficulty an aura consisting of a sense of fullness in the
with respiration and swallowing. ear, increasing tinnitus and a decrease in hear-
ing,56 but can also occur without warning. The
Background Information typical duration of an attack is 2 to 3 hours, rang-
This condition is genetic, with an autosomal ing from minutes to hours in length. Attacks
dominant pattern of inheritance and onset of can be single or multiple, with short or long
symptoms in adolescence or young adulthood. intervening periods of remission during which
Differential diagnosis includes Parkinson’s dis- the individual may be asymptomatic.
ease and multiple system atrophy. The pres-
ence of ataxia decreases the likelihood of Background Information
Parkinson’s, and the early age of onset and The cause of this condition is thought to be
visual symptoms decrease the likelihood of overproduction or underabsorption of en-
multiple system atrophy. Diagnosis is estab- dolymph (endolymphatic hydrops), resulting
lished by clinical symptoms and magnetic res- in distortion of the membranous labyrinth.
onance imaging findings of reduced width of Ruptures of the membranous labyrinth,
superior and middle cerebellar peduncles, at- autoimmune disease processes, and viral infec-
rophy of the frontal and temporal lobes, and tion are among other proposed causes.58
decreased size of the pons and globus pallidus. Appropriate tests include caloric testing to
There is no treatment for this condition, and determine amount of vestibular asymmetry;
prognosis is poor.1 audiometry, which often shows a low-
frequency loss rather than a high-frequency
■ Macular Degeneration one; and exclusion of other causes (typically
Chief Clinical Characteristics with gadolinium-enhanced magnetic reso-
This presentation involves gradual loss of cen- nance imaging). The head thrust test can be
tral vision, which impairs the ability to read negative despite caloric asymmetry. Restrict-
with preservation of peripheral vision. ing salt intake and using diuretics may be
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Chapter 29 Multiple Sclerosis 591

useful in over half of individuals with this con- factors such as hormonal fluctuations, fatigue,

SENSORY ABNORMALITIES
dition. Approximately 10% of individuals with or anxiety may be triggers that initiate the
this condition will have persistent vertigo and pathophysiological cascade. This condition
require other forms of treatment. These in- also has a strong familial tendency and begins
clude surgery to decompress or drain the at a young age, suggesting that genetic factors
endolymphatic sac, selective vestibular neurec- may predispose individuals to migraine at-
tomy or labyrinthectomy, and intratympanic tacks. The use of neuroimaging is not indi-
injections of aminoglycosides such as gentam- cated in individuals with migraine symptoms
icin53 to reduce or abolish vestibular function and a normal neurological exam. Certain
on the affected side. pharmaceutical agents, such as triptans and
ergots, are effective in preventing migraine
■ Middle Ear Surgery attacks from becoming too severe.63 Other
Chief Clinical Characteristics medications can be considered to prevent mi-
This presentation involves decreased taste graine attacks. A complete discussion of
perception following middle ear surgery. headache diagnosis is included in Chapter 7.
Background Information ■ Multiple Sclerosis
Disorders of taste are related to intraoperative Chief Clinical Characteristics
insult to the facial nerve. Electrogustometry This presentation may include paresthesias,
and chemical taste tests are used to diagnose weakness, spasticity, hypertonicity, hyperreflexia,
taste disorders. No specific treatment is indi- positive Babinski, incoordination, optic neuri-
cated. Approximately 30% of individuals with tis, ataxia, vertigo, dysarthria, diplopia, bladder
taste disorders following middle ear surgery incontinence, tremor, balance deficits, falls, and
recover completely.59 cognitive deficits.1
■ Migraine Background Information
This condition may present as relapsing-
Chief Clinical Characteristics remitting, primary progressive, or secondary
This presentation often includes a severe pulsat- progressive. The disease occurs most frequently
ing and throbbing unilateral headache that may in women between the ages of 20 and 40 years.
switch sides, associated with nausea, vomiting, Only a small number of children or individuals
diarrhea, abdominal cramps, polyuria, sweat- between 50 and 60 years are diagnosed with this
ing, facial pallor, photophobia, or phonophobia. condition.1 This condition was originally
This is a chronic condition of recurring attacks
thought to be secondary to environmental and
of transient focal neurological symptoms,
genetic factors, but evidence suggests an autoim-
headaches, or both.60 Not only is this process re-
mune response to a viral infection, which subse-
sponsible for producing headaches, the condition quently targets myelin.1 The diagnosis may be
can also interfere with the function of other
confirmed by a thorough history, physical exam-
body systems, resulting in the characteristic
ination, magnetic resonance imaging, analysis of
presentation.61 Migraines are preceded by aura
cerebrospinal fluid, and evoked potentials.1,64,65
in 20% of cases.62 Even in individuals who
Life expectancy and cause of mortality are simi-
commonly experience them, auras may not lar for all types of this condition.1 Clinical char-
accompany every headache. Auras can be
acteristics that are associated with a longer time
visual, somatosensory, olfactory, or involve
interval for progression of disability include
speech disturbances. The location of headache
female sex, younger age of onset, relapsing-
may switch sides from episode to episode.
remitting type, complete recovery after the first
Background Information relapse, and longer time interval between first
The pathophysiology of this condition is very and second exacerbation.66 Medical management
complex. Current theories postulate that a cas- may include the use of methylprednisolone,
cade of events occurs involving vasodilation prednisone, cyclophosphamide, immunosup-
of meningeal blood vessels, irritation of pressant treatment, and betainterferon.1 Physi-
perivascular sensory nerves, and stimulation cal, occupational, and speech therapy may be
of brainstem nuclei. Additionally, extrinsic indicated to prevent secondary sequelae and to
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592 Chapter 29 Neurological Complications of Acquired Immunodeficiency Syndrome

optimize functional activity and mobility. Some abnormalities within the nervous system.1 Diag-
SENSORY ABNORMALITIES

individuals may benefit from psychology/ nosis of the variable neurological complications
psychiatry and social support as the disease associated with this condition may be confirmed
progresses. with laboratory tests, cerebrospinal fluid cul-
tures, imaging, nerve conduction studies, and
■ Neurological Complications of physical examination.1,67,68 Treatment appears
Acquired Immunodeficiency to be limited primarily to the use of antiviral
Syndrome medications.67 Physical and occupational ther-
Chief Clinical Characteristics apy may be indicated to address equipment
This presentation is variable and dependent on needs and caregiver/patient training related to
the affected neuroanatomical structures in an functional mobility.
individual with acquired immunodeficiency
syndrome.67 ■ Neuromyelitis Optica
Background Information Chief Clinical Characteristics
This condition may be categorized by: This presentation involves acute to subacute
onset of blindness in one or both eyes and/or
1. Meningitic symptoms including headache, transverse myelitis. Blindness and transverse
malaise, and fever (such as secondary to myelitis can exist in isolation or combination.
meningitis, cryptococcal meningitis, tuber-
culous meningitis, and human immunode- Background Information
ficiency virus headache) This condition occurs when demyelinating
2. Focal cerebral symptoms including hemi- and/or necrotizing lesions form in one or both
paresis, aphasia, apraxia, sensory deficits, optic nerves and in the spinal cord. Differen-
homonymous hemianopia, cranial nerve in- tial diagnosis especially includes acute dissem-
volvement, balance deficits, incoordination, inated encephalomyelitis, subacute necrotic
and/or ataxia (such as secondary to cerebral myelopathy, and a variant form of multiple
toxoplasmosis, primary central nervous sys- sclerosis. High-dose corticosteroids and alky-
tem lymphoma, and progressive multifocal lating agents are used to treat this condition.
leukoencephalopathy) Prognosis is poor; factors placing individuals
3. Diffuse cerebral symptoms that involve cog- at higher risk of earlier morbidity or mortality
nitive deficits, altered level of consciousness, include older age at onset and relapse during
hyperreflexia, Babinski sign, presence of the first 2 years of the disease.69
primitive reflexes (such as secondary to NEUROPATHIES
postinfectious encephalomyelitis, acquired
immunodeficiency dementia complex, ■ Alcohol Abuse
cytomegalovirus encephalitis) Chief Clinical Characteristics
4. Myelopathy associated with gait difficulties, This presentation can be characterized by
spasticity, ataxia, balance deficits, and hyper- slowly progressive sensory disturbances in
reflexia (such as secondary to herpes zoster a distal to proximal pattern in an individ-
myelitis, vacuolar myelopathy that occurs ual with history of alcohol abuse. Loss of
with acquired immunodeficiency syndrome pain sensation and painful burning sensa-
dementia complex) tions are among the most common sensory
5. Peripheral involvement associated with sen- symptoms.
sory changes, weakness, balance deficits, and
Background Information
pain (such as secondary to peripheral neu-
This condition can be caused by the toxic
ropathy, acute and chronic inflammatory
effect of ethanol or its metabolites on the
demyelinating polyneuropathies).1,67
central and peripheral nervous system.
Abnormal neurological findings are ob- Primary treatment is directed to reducing
served during a clinical examination in approx- alcohol abuse behaviors. Prognosis depends
imately one-third of patients with acquired on the course of the disease, in which neu-
immunodeficiency syndrome, however on au- ropathy will continue to progress with con-
topsy most individuals with this condition have tinued alcohol abuse.
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Chapter 29 Peripheral Neuropathy 593

■ Diabetes Mellitus associated with limitations in functional

SENSORY ABNORMALITIES
Chief Clinical Characteristics mobility.72
This presentation is variable, including ■ Hereditary Motor and Sensory
manifestations such as acute diabetic Neuropathies
mononeuropathies, which may include
involvement of peripheral nerves (including Chief Clinical Characteristics
cranial nerves)1; multiple mononeuropathies This presentation includes distal sensory
and radiculopathies, which may include abnormalities, such as numbness and tingling
unilateral or asymmetric pain, low back of the feet, and muscle weakness of distal mus-
pain with or without symptoms in the leg, culature. Individuals affected with heredi-
weakness, atrophy, diminished or absent tary neuropathies may also report sweating
deep tendon reflexes, and sensory deficits1; and dizziness upon standing.
distal polyneuropathy, the most common Background Information
diabetic neuropathy, which consists of Hereditary neuropathies include hereditary
chronic, symmetric, distal sensory deficits motor and sensory neuropathy, hereditary
(eg, numbness and tingling), diminished sensory neuropathy, hereditary motor neu-
or absent deep tendon reflexes, balance ropathy, and hereditary sensory and auto-
deficits, and weakness1; and autonomic nomic neuropathy. The majority of all heredi-
neuropathy, which may involve resting tary neuropathies are Charcot-Marie-Tooth
tachycardia, orthostatic hypotension, sexual neuropathy. Inherited polyneuropathies are
impotence, exercise intolerance, abnormal caused by genetic abnormalities. Diagnosis is
sweating, pupil abnormalities, weakness, made by nerve conduction and electromyo-
sensory deficits, and gastroparesis.1,68,70,71 graphic studies. Prognosis for hereditary sen-
sory neuropathies is poor due to intractable
Background Information pain.73 Prognosis for hereditary motor and
Approximately 15% to 20% of people with sensory neuropathies has also been found to
diabetes may present with the signs and be unfavorable due to slowing of conduction
symptoms of this condition.1,70 However, velocity with age.74
approximately 50% will have neuropathic
symptoms and may have abnormalities in ■ Peripheral Neuropathy
nerve conduction testing. 1 Commonly
Chief Clinical Characteristics
considered a metabolic disorder, this con-
This presentation may be characterized by
dition may be a result of vascular compli-
sensory alterations, falls, loss of balance,
cations disrupting the supply of nutrients
weakness, as well as diminished or absent
to the nerves.68,72 A thorough history, phys-
deep tendon reflexes, fasciculations, syncope,
ical examination (specifically including the
abnormal sweating, orthostatic hypotension,
assessment of deep tendon reflexes and
resting tachycardia, and trophic changes.1,75
sensory examination), electromyography/
nerve conduction testing, and laboratory Background Information
screen helps to differentiate other causes of The patterns of peripheral neuropathies are
neuropathy.70,71 Treatment consists of variable and may present as polyneuropathy,
maintaining a normal range of blood glu- polyradiculopathy, neuronopathy, mononeu-
cose levels.1,69,71 In addition, individuals ropathy, mononeuropathy multiplex, and
with this condition may prevent complica- plexopathy.1 Some of the etiologies associ-
tions by completing visual inspection of ated with peripheral neuropathies include
the skin and routine podiatry care.71 Med- trauma, inflammation (eg, herpes zoster,
ications may help to control symptoms Lyme disease, human immunodeficiency
such as paresthesias or pain.1,68 Additional virus, Guillain-Barré syndrome), metabolic
management may include consultations causes (eg, diabetes mellitus, uremia), nutri-
with an orthotist to ensure proper fitting tional causes (eg, vitamin B deficiencies com-
of foot wear and physical therapy to mini- monly associated with alcohol abuse, eating
mize disability by addressing impairments disorders, and individuals with malabsorption
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594 Chapter 29 Neurosarcoidosis

syndromes), congenital and idiopathic causes seizures, diplopia, visual impairments, hear-
SENSORY ABNORMALITIES

(eg, aging or unknown causes), and toxic eti- ing loss, psychotic disorders, loss of bowel/
ology (eg, exposure to lead, arsenic, thallium; bladder function, pain, hyporeflexia, and
chemotherapeutic drugs such as vincristine, hypotonia.78,79
cisplatin).75 Twenty percent of individuals
Background Information
over the age of 60 are affected by a type of
Treponema pallidum infects the human host by
peripheral neuropathy.76 The diagnosis of the
way of contact with contaminated body fluids
specific disorder may be differentiated by the
or lesions.78 This spirochete is responsible
pattern of peripheral neuropathy and tempo-
for the diagnosis of syphilis; however, when
ral features. The diagnosis may be confirmed
T. pallidum is present within the central nerv-
after completing a thorough history, physical
ous system the individual is diagnosed with
examination, gait assessment, balance assess-
neurosyphilis.79 This condition occurs in ap-
ment, laboratory testing, and electromyogra-
proximately 10% of individuals with untreated
phy/nerve conduction testing.1,77 Treatment
syphilis, and in 81% of these cases it presents as
and prognosis will vary depending on the
meningovascular, meningeal, or general pare-
etiology and severity of this condition.
sis. Treatment includes use of various forms of
■ Neurosarcoidosis penicillin or alternative choices for those aller-
gic to penicillin78 and may involve rehabilita-
Chief Clinical Characteristics
tive therapies depending on the individual’s
This presentation may be characterized by
activity limitations or participation restric-
facial palsy; impaired taste, sight, smell, or swal-
tions. A better prognosis has been observed for
lowing; vertigo; loss of sensation in a
individuals treated during early neurosyphilis.79
stocking/glove pattern; and weakness in a dis-
tal greater than proximal distribution.1
■ Neurotoxicity
Background Information Chief Clinical Characteristics
This condition is a manifestation of sarcoido- This presentation includes limb weakness or
sis with central and/or peripheral nervous sys- numbness; loss of memory, vision, and/or intel-
tem involvement. It is characterized by forma- lect; headache; behavioral problems; and
tion of granulomas in the central nervous sexual dysfunction.
system. The lesion consists of lymphocytes and
mononuclear phagocytes surrounding a non- Background Information
caseating epithelioid cell granuloma. These This condition occurs when exposure to natu-
granulomas represent an autoimmune re- ral or artificial toxins alters the normal activity
sponse to central nervous system tissues. This of the nervous system. This can eventually dis-
condition includes 5% of individuals with sar- rupt or kill neurons. It results from exposure
coidosis. The diagnosis is established by the to substances used in chemotherapy, radiation
presence of clinical features, along with clinical treatment, drug therapies, and organ trans-
and biopsy evidence of sarcoid granulomas in plants, as well as exposure to heavy metals,
tissues outside the nervous system. Approxi- foods, or pesticides. Diagnosis is supported by
mately two-thirds of individuals experience clinical presentation and lab tests for detection
this illness only once, whereas the remainder of the toxic substance. Treatment is prioritized
experience chronic relapses. Primary treat- at removal of the offending toxin. Prognosis
ment for neurosarcoidosis is the administra- varies greatly depending on the level of expo-
tion of corticosteroids. sure and individual’s comorbid medical
conditions.
■ Neurosyphilis (Tabes Dorsalis,
Syphilitic Spinal Sclerosis, ■ Orthostatic Hypotension
Progressive Locomotor Ataxia) Chief Clinical Characteristics
Chief Clinical Characteristics This presentation commonly involves dizziness,
This presentation can be characterized by hemi- light-headedness, and blurred vision that
paresis, ataxia, aphasia, gait instability, falls, generally occur after sudden standing. In more
neuropathy, personality and cognitive changes, severe forms of this condition, individuals may
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Chapter 29 Otosyphilis 595

experience seizures, transient ischemic attacks, fully understood, but it may be hereditary.

SENSORY ABNORMALITIES
or syncope. Audiograms and tympanograms are useful in
the diagnosis of otosclerosis. Surgery is often
Background Information
the form of treatment, in which a stapedec-
This condition is caused by a sudden decrease
tomy will be performed to bypass the dis-
of greater than 20 mm Hg in systolic blood
eased bone with a prosthesis that allows
pressure or greater than 10 mm Hg in dias-
sound waves to be passed to the inner ear. In
tolic blood pressure, which occurs when a
milder cases in which surgery is not indi-
person assumes a standing position. It may be
cated, a hearing aid may be used. With either
caused by hypovolemia resulting from the ex-
form of treatment, there may be residual mild
cessive use of diuretics, vasodilators, or other
hearing loss.
types of vasoactive medications (eg, calcium
channel blockers and beta blockers), dehydra- ■ Otosyphilis
tion, or prolonged bed rest. Other factors to
Chief Clinical Characteristics
consider include the individual’s neurological
This presentation may involve severe episodic
status and hemorrhagic/hypovolemic states.
vertigo, fluctuating hearing loss, low-frequency
The condition may be associated with Addi-
hearing loss in the early stages of the disease, and
son’s disease, atherosclerosis, diabetes, and
flat audiometric patterns in the later stages of
certain neurological conditions including
the disease.80,81 The hearing loss is usually
Shy-Drager syndrome and other dysautono-
bilateral in most individuals; the loss in speech
mias. Hypovolemia due to medications is re-
discrimination is usually out of proportion to
versed by adjusting the dosage or by discon-
the speech reception threshold. Vestibular dis-
tinuing the medication. If prolonged bed rest
turbances could be present in as many as 80%
is the cause, improvement may occur by sit-
of individuals with this condition. This condi-
ting up with increasing frequency each day. In
tion’s presentation is similar to that of Ménière’s
some cases, physical counterpressure such as
disease.
compression hose or whole-body inflatable
suits may be required. Dehydration is treated Background Information
with salt and fluids. Individuals with this con- Otosyphilis is caused by the spirochete
dition can be instructed to rise slowly from Treponema pallidum. Both congenital and
bed in the mornings or when moving from a acquired forms of syphilis infection can
sitting to standing position. Symptoms usu- lead to this condition with subsequent de-
ally dissipate when the individual is placed generation of the audiovestibular system.
in a semirecumbent or supine position, Histopathological findings are identical for
although some individuals may progress to both the congenital and acquired forms.
frank syncope. In this case, the clinician The underlying syphilis infection causes
should be prepared to activate the emergency meningoneurolabyrinthitis in the early con-
medical system. genital form and in the acute period of the
secondary and tertiary acquired forms. It
■ Otosclerosis causes temporal bone osteitis with secondary
Chief Clinical Characteristics involvement of the membranous labyrinth in
This presentation includes hearing loss as the the late congenital, late latent, and tertiary
most frequent symptom. The loss may appear syphilis stages. Endolymphatic hydrops and
very gradually. Many people with otosclero- degenerative changes in the sensory and neu-
sis first notice that they cannot hear low- ral structures are seen in both the congenital
pitched sounds or a whisper. In addition to and acquired forms. Poor prognosis is indi-
hearing loss, some people with otosclerosis may cated by endolymphatic sac obstruction
experience dizziness, balance problems, or by microgummata. The goal of treatment is
tinnitus. to halt the progression of the disease. Treat-
ment includes antibiotic and steroidal anti-
Background Information inflammatory medication, and the medica-
Otosclerosis is the abnormal growth of bone of tion of choice differs with the clinical stage of
the middle ear. The cause of otosclerosis is not the disease.
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596 Chapter 29 Paraneoplastic Syndromes

■ Paraneoplastic Syndromes ■ Perforated Tympanum


SENSORY ABNORMALITIES

Chief Clinical Characteristics Chief Clinical Characteristics


This presentation commonly includes visual, This presentation can be characterized by loss
tactile, and hearing deficits in combination of hearing, tinnitus, and occasionally clear, pus-
with a variety of different neurological symp- tular, or bloody discharge.
toms and signs in an individual with cancer.
Specific neurological symptoms and signs depend Background Information
on the location of involvement of the central or This condition can result from a temporal
peripheral nervous system. skull fracture, foreign body obstruction, or
loud explosion. Diagnosis is made by visual
Background Information inspection through an otoscope. Spontaneous
Paraneoplastic encephalomyelitis and focal closure often occurs within 2 months. If the
encephalitis may present with ataxia, vertigo, tympanum does not close spontaneously, sur-
balance deficits, nystagmus, nausea, vomit- gical repair, in which thin paper patches are
ing, cranial nerve palsies, seizures, sensory placed over the tympanum to support healing,
neuropathy, anxiety, depression, cognitive is indicated. Complete restoration of hearing
changes, and hallucinations. For individuals following surgical intervention is probable.
presenting with ataxia, dysarthria, dysphagia,
and diplopia, paraneoplastic cerebellar de- ■ Perilymph Fistula
generation may be suspected. Paraneoplastic Chief Clinical Characteristics
opsoclonus/myoclonus tends to affect both This presentation may involve dizziness, ver-
children and adults with signs and symptoms tigo, imbalance, nausea, and vomiting.44 Some
including hypotonia, ataxia, irritability, trun- individuals with this condition experience
cal ataxia, gait difficulty, balance deficits, and ringing or fullness in the ears, and many
frequent falls. Stiff-man syndrome presents notice a hearing loss. Most people with
with spasms and fluctuating rigidity of axial fistulas find that their symptoms get worse
musculature, legs and possibly shoulders, with changes in altitude (elevators, airplanes,
upper extremities, and neck. Paraneoplastic travel over mountain passes) or air pressure
sensory neuropathy presents with asymmet- (weather changes), as well as with exertion and
ric, progressive sensory alterations involving activity.
the limbs, trunk, and face, sensorineural
hearing loss, autonomic dysfunction, and Background Information
pain. Other conditions in this category in- The cause of this condition is a tear or defect
clude vasculitis, Lambert-Eaton myasthenia in the oval or round window in one or both
syndrome, myasthenia gravis, dermato- ears, allowing pressure changes in the middle
myositis, neuromyotonia, and various neu- ear to stimulate the inner ear and cause
ropathies.1,82 These conditions result from symptoms. Head trauma via a direct blow
an immune-mediated response to the pres- and whiplash injury, barotrauma sustained
ence of tumor or metastases. Antibodies or during diving, weightlifting, and childbirth
T-cells respond to the presence of the tumor, are among common causes. This condition
but also attack normal cells of the nervous also may be present from birth or may result
system.83,84 Over 60% of individuals present from chronic, severe ear infections. Clinical
with this condition prior to the discovery tests suggestive of a fistula include reproduc-
of the cancer. 82 The underlying tumor tion of symptoms during a Valsalva maneuver
is treated according to the type of cancer. or when applying pressure to the external au-
Additional treatment is dependent on ditory canal. The definitive diagnosis is made
this condition’s type and may include by direct visualization during tympanotomy.
steroids, plasmapheresis, immunotherapy, Surgical repair with grafting is indicated if
chemotherapy, radiation, or cyclophos- symptoms persist despite nonsurgical care
phamide.82 Physical, occupational, and consisting of activity restriction aimed at
speech therapy may be indicated to address avoiding lifting, straining (Valsalva maneu-
functional limitations. ver), bending over, and changes in pressure.
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Chapter 29 Retinitis 597

■ Presbycusis involuntary saccades are relatively early fea-

SENSORY ABNORMALITIES
Chief Clinical Characteristics tures. The disease progresses to the point at
This presentation includes the loss of hearing that which all voluntary eye movements are lost.
gradually occurs in most individuals as they Background Information
grow older. The loss associated with presbycu- Some patients may not demonstrate difficul-
sis is usually greater for high-pitched sounds. ties with ocular movements for 1 to 3 years
Background Information after disease onset. Most cases are sporadic;
This condition is most often due to a gradual however, a pattern of inheritance compatible
loss of sensory receptors in the inner ear. with autosomal dominant transmission has
Decreasing exposure to loud noises is the best been described.1 Diagnosis is based on clinical
way to prevent this condition. Using a hearing presentation, which includes a gradually
aid after the development of this condition is progressive disorder with age of onset at
often helpful. The degree to which hearing can 40 years or older, vertical supranuclear palsy,
be restored is dependent on the extent of loss. and postural instability with falls within the
first year of disease onset.87 Medical treatment
■ Progressive Multifocal is typically unsuccessful, because the majority
Leukoencephalopathy of these patients are not responsive to lev-
Chief Clinical Characteristics odopa therapy aimed at addressing the ex-
This presentation commonly involves cortical trapyramidal features of the disease.88 Physical
blindness, visual field defects, hemiparesis with and occupational therapy are used to maintain
progression to quadriparesis, aphasia, ataxia, mobility and address safety issues related to
dysarthria, personality changes, and impaired the progression of imbalance. The disease
intellect evolving over a period of days to weeks. course is progressive with a mean survival time
of 5.6 years.86 Older age at disease onset, early
Background Information onset of falls, incontinence, dysarthria, dys-
This condition is most likely due to viral infec- phagia, insertion of a percutaneous gastros-
tion of the central nervous system, which then tomy, and diplopia have all been described as
causes widespread demyelinative lesions prima- being predictive of shorter survival time.86
rily of the cerebral hemispheres. Diagnosis is
made by computed tomography and magnetic ■ Restless Legs Syndrome
resonance imaging to localize the lesions. Treat- Chief Clinical Characteristics
ment for individuals with acquired immunode- This presentation includes complaints of un-
ficiency syndrome consists of antiretroviral pleasant sensations located in the legs, which
drug combinations and can lead to slower pro- commonly leads to additional problems such as
gression or even remission. Currently, no treat- sleep disturbances and depression.
ment exists to impair disease progression in in-
dividuals with this condition who do not have Background Information
acquired immunodeficiency syndrome.1 Etiology of restless legs syndrome is unknown,
but commonly occurs with pregnancy, iron
■ Progressive Supranuclear Palsy depletion, uremia, polyneuropathy, spinal dis-
(Richardson-Steele-Olszewski orders, and rheumatoid arthritis. Diagnosis is
Syndrome) based on subjective reports. Active movement,
Chief Clinical Characteristics massage, or cold compressions can provide
This presentation classically includes vertical temporary relief, and dopaminergic treatment
gaze palsy, prominent instability, and falls within can provide longer term relief. There is no cure
the first year of disease onset.85 Other character- however, and symptoms progressively worsen
istics may include rigidity, akinesia, dysarthria, with age.89
dysphagia, and mild dementia. Falls were found
to be the most commonly reported symptom ■ Retinitis
with the majority of falls occurring in a back- Chief Clinical Characteristics
ward direction.86 Difficulty with voluntary ver- This presentation typically involves decreased
tical eye movements (usually downward) and vision at night or in reduced light, loss of
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598 Chapter 29 Rickettsial Diseases, Including Rocky Mountain Spotted Fever

peripheral vision, and near blindness in the cervical enlargement, disrupting ipsilateral
SENSORY ABNORMALITIES

advanced cases. corticospinal and dorsal column tracts and


contralateral projections of the spinothalamic
Background Information tract.5 Medical management consists of diag-
This condition is a progressive degeneration of nosis via imaging, appropriate medical care in
the retina, affecting rods more frequently than the acute care setting, surgical decompression
cones. Tests to determine the integrity of the of spinal cord if indicated, and multidiscipli-
retina confirm the diagnosis, including slit nary rehabilitation.94 Outcome is favorable
lamp examination, intraocular pressure deter- in most patients following surgical decom-
mination, and eye ultrasound. There is no pression of disk herniation or tumor, demon-
known effective treatment for this slowly strating vast improvement in motor function
progressive condition, but sunglasses may pro- with minimal residual sensory deficits.93
tect the retina and antioxidants may have a
preventive effect. ■ Central Cord Syndrome
■ Rickettsial Diseases, Including Chief Clinical Characteristics
Rocky Mountain Spotted Fever This presentation commonly involves pres-
entation of profound weakness of the arms and
Chief Clinical Characteristics hands, and to a lesser extent the legs, commonly
This presentation typically includes visual dis- due to traumatic spinal cord injury. Associ-
turbances, a rash that occurs on the palms of ated sensory loss below the level of the lesion
hands and soles of feet, headache, nausea, fever, and/or sphincter dysfunction may occur.
and myalgias.90 Initial symptoms begin within
2 to 14 days of infection and last approximately Background Information
2 to 3 weeks. Damage to the more centrally located as-
cending and descending spinal tracts results
Background Information in this characteristic presentation of motor
Rocky Mountain spotted fever is the most and sensory loss. Medical management con-
common type of this condition in the United sists of diagnosis via imaging, appropriate
States. It is transmitted by a variety of tick, and medical care in the acute care setting, reduc-
is common in Long Island, Tennessee, Virginia, tion of fracture and/or surgical decompres-
North Carolina, and Maryland.1 Like malaria, sion of spinal cord, and multidisciplinary re-
rickettsiae in the blood vessels cause vascular habilitation.95 Many individuals with central
injury, which forms the basis for central nerv- cord syndrome recover the ability to walk,
ous system damage. Diagnosis is based on clin- but impairment of fine motor control in the
ical signs and symptoms and confirmed by skin hands often remains.96
biopsy. Treatment consists of the use of doxy-
cycline or chloramphenicol. The mortality rate ■ Conus Medullaris Syndrome
in untreated cases is 20% to 25%.91
Chief Clinical Characteristics
SPINAL CORD INJURIES This presentation typically involves weakness
of the lower extremities in association with
■ Brown-Sequard Syndrome
hyperreflexia, bowel/bladder dysfunction,
Chief Clinical Characteristics sexual dysfunction, and sensory loss in a
This presentation can be characterized by dermatomal pattern of the sacral segments.5
ipsilateral loss of strength and tactile discrim-
ination, position, and vibration sense, with Background Information
contralateral hemianesthesia to pain and The presentation of weakness with upper
temperature secondary to spinal cord injury, motor neuron symptoms is secondary to in-
disk herniation, tumor, ischemia, or inflam- jury to the conus medullaris, most commonly
matory disorder.92,93 due to trauma (vertebral body fracture of
acute disk herniations of the thoracolumbar
Background Information junction).97 Diagnosis and treatment involve
This condition occurs secondary to lateral clinical examination, imaging, and surgical
hemisection of the spinal cord, usually below investigation, decompression, fusion, and
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Chapter 29 Systemic Lupus Erythematosus 599

fixation if indicated. Improvement in spinal equipment. Individuals with this condition

SENSORY ABNORMALITIES
cord function, bladder function, and nerve require adequate follow-up medical care to
root recovery following surgical intervention prevent secondary impairments.102 Acute
occurs in more than half of the patients forms of this condition constitute a medical
following surgical intervention.98 emergency.

■ Traumatic Spinal Cord Injury ■ Stroke (Cerebrovascular Accident)


Chief Clinical Characteristics Chief Clinical Characteristics
This presentation is characterized by loss of sen- This presentation may include a wide range of
sation in a dermatomal pattern, hypertonicity, symptoms that correspond to specific areas of the
spasticity, hyperreflexia, and sphincter dysfunc- brain that are affected. The initial symptoms
tion in combination with muscle weakness. can include numbness or weakness, especially on
Specific syndromes (discussed below) have one side of the body or face; visual disturbances,
characteristic presentations secondary to the confusion or aphasia; balance deficits or falls; or
location of the lesion. sudden severe headache with no known cause.
Background Information
Background Information This condition occurs when blood flow to
Insult to the spinal cord results from frac- the brain is interrupted either by blockage
tured bone, displaced disk material, or a (ischemia or infarction) or from hemorrhagic
foreign object transecting or injuring the disruption. A thrombosis or embolic occlusion
cord. Imaging is used to identify the cause of of an artery causes an ischemic type of this
injury and direct surgical stabilization or in- condition. A hemorrhagic type of this condi-
tervention. Motor vehicle accidents and falls tion can be caused by arteriovenous malfor-
are the most common etiology of this condi- mation, hypertension, aneurysm, neoplasm,
tion.99 Physical examination of strength and drug abuse and trauma. This condition is the
sensation is used to assign a score from the most common and disabling neurological dis-
American Spinal Injury Association.100 In- order in adults and occurs in 114 of every
juries are classified in terms of neurological 100,000 people.103 This condition is diagnosed
level (ie, most rostral segment where my- using clinical presentation and positive find-
otomal and dermatomal function is spared) ings on computed tomography and magnetic
and extent as either complete (total lack of resonance imaging. Medication, surgery, and
sensory or motor function below level of interdisciplinary therapy are the most com-
injury) or incomplete (some motor or sen- mon treatments for this condition. The prog-
sory function spared below the level of nosis for recovery is predicted by the magni-
injury). Cervical locations of injury result in tude of initial deficit. Factors that are
tetraplegia and may cause paralysis or weak- associated with poor outcomes include coma,
ness of the respiratory musculature, requir- poor cognition, severe aphasia, severe hemi-
ing mechanical ventilation and/or respira- paresis with little return within 1 month,
tory strengthening.101 Thoracic or lumbar visual perceptual disorders, depression, and
locations of injury result in paraplegia. incontinence after 2 weeks.104,105
Cauda equina syndrome occurs following
injury inferior to the conus medullaris. ■ Systemic Lupus Erythematosus
Treatment consists of medical management, Chief Clinical Characteristics
multidisciplinary rehabilitation, equipment This presentation can include abnormal vision,
prescription, and prevention of pressure swallowing, taste, hearing, changes in mood or
ulcers, contractures, and further complica- thinking, and seizures in combination with
tions. Individuals with incomplete forms fatigue, joint pain, and swelling affecting the
of this condition may continue to recover hands, feet, knees, and shoulders.
strength and function, while individuals
with complete forms of this condition Background Information
have a poor prognosis for recovery and in- This condition affects mostly women of child-
stead use compensatory techniques and bearing age. It is a chronic autoimmune disorder
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600 Chapter 29 Tethered Spinal Cord Syndrome

that can affect any organ system, including and the palm; muscle weakness with difficulty
SENSORY ABNORMALITIES

skin, joints, kidneys, brain, heart, lungs, and gripping and performing fine motor tasks of
blood. Microinfarcts in the cerebral cortex and the hand; atrophy of the muscles of the palm;
brainstem, which lead to destructive and pro- cramps of the muscles on the inner forearm;
liferative changes in capillaries and arterioles, pain in the arm and hand; and tingling and
are primarily responsible for central nervous numbness in the neck, shoulder region, arm,
system manifestations. Hypertension and and hand.
endocarditis can also predispose an affected
Background Information
individual to development of neurological ab-
There are three types of this condition, which
normalities. Multiple sclerosis is a disease that
can coexist or occur independently: compres-
may be mistaken for this condition, especially
sion of the subclavian vein, compression of the
if the central nervous system manifestations
subclavian artery, and a primary neurological
include visual dysfunction. The diagnosis is
syndrome. Multiple anatomical anomalies can
confirmed by the presence of skin lesions;
lead to thoracic outlet syndrome, including an
heart, lung, or kidney involvement; laboratory
incomplete cervical rib, a taut fibrous band
abnormalities including low red or white cell
passing from the transverse process of C7 to
counts, low platelet counts, or positive ANA
the first rib, and a complete rib that articulates
and anti-DNA antibody tests.106 Treatment in-
with the first rib, or anomalies of the position
volves corticosteroid medication.
and insertion of the anterior and medial
■ Tethered Spinal Cord Syndrome scalene muscles. Diagnosis includes physical
examination tests (eg, Adson’s test, extremity
Chief Clinical Characteristics
abducted stress test, costoclavicular sign), ra-
This presentation typically includes back pain
diology of the cervical spine, and nerve con-
associated with neurological deficits and bowel
duction and electromyography studies. Non-
and bladder dysfunction.107 The most common
surgical approaches to treatment include
manifestations of this condition are worsening
exercise, stretches, modalities, and analgesic
in motor function of the lower extremities (and
medication. Surgery is indicated if pain is
less likely the upper extremities), changes in
persistent and severe neurogenic or vascular
muscle tone and deep tendon reflexes, progres-
features of the syndrome exist. Prognosis for
sive loss of articular dexterity, progressively
decreased pain and improved function is
worsening scoliosis or kyphosis, and back or
good for the majority of individuals with this
leg pain.108
condition.
Background Information
This condition occurs commonly in children, ■ Transient Ischemic Attack
but also can present in undiagnosed adults. Chief Clinical Characteristics
Magnetic resonance imaging confirms the di- This presentation can include numbness or
agnosis, with a low-lying (caudally positioned) weakness in the face, arm, or leg, especially on
conus medullaris present. Surgical resection one side of the body; confusion or difficulty
of a thickened filum terminale is a common in talking or understanding speech; trouble
treatment. seeing in one or both eyes; and difficulty with
walking, dizziness, or loss of balance and
■ Thoracic Outlet Syndrome coordination.
Chief Clinical Characteristics Background Information
This presentation can be characterized by This condition is a transient stroke that lasts
swelling or puffiness in the arm or hand; bluish only a few minutes. It occurs when the blood
discoloration of the hand; a feeling of heaviness supply to part of the brain is briefly inter-
in the arm or hand; deep, boring toothache- rupted. Symptoms of this condition, which
like pain in the neck and shoulder region that usually occur suddenly, are similar to those of
seems to increase at night; easily fatigued arms stroke but do not last as long. Most symp-
and hands; superficial vein distention in the toms disappear within an hour, although they
hand; paresthesias along the inside forearm may persist for up to 24 hours. Because it is
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Chapter 29 Tropical Spastic Paraparesis 601

impossible to differentiate between symp- the midthoracic region or an abrupt, severe

SENSORY ABNORMALITIES
toms from this condition and acute stroke, onset of symptoms.1
individuals should assume that all stroke-like
symptoms signal a medical emergency. A ■ Traumatic Brain Injury
prompt evaluation (within 60 minutes) is Chief Clinical Characteristics
necessary to identify the cause of this condi- This presentation typically includes dysequi-
tion and determine appropriate therapy. librium in the presence of cognitive changes,
Depending on the individual’s medical his- altered level of consciousness, seizures, nausea,
tory and the results of a medical examination, vomiting, coma, dizziness, headache, pupillary
the doctor may recommend drug therapy or changes, tinnitus, weakness, incoordination,
surgery to reduce the risk of stroke. Antiplatelet behavioral changes, spasticity, hypertonicity,
medications, particularly aspirin, are a stan- cranial nerve lesions, and sensory and motor
dard treatment for individuals suspected of deficits.1,109
having this condition and who also are at risk
for stroke, including individuals with atrial Background Information
fibrillation. This condition can be classified as mild,
moderate, or severe based on Glasgow Coma
■ Transverse Myelitis Scale, length of coma, and duration of post-
Chief Clinical Characteristics traumatic amnesia.109 Magnetic resonance
This presentation involves the gradual devel- imaging may be used to confirm the diagno-
opment of sensory changes, back or neck sis.68 Treatment initiated at the scene of the
pain, weakness, and/or bowel and bladder accident and during the acute phase is focused
dysfunction over the course of several hours to on medical stabilization.110 Low Glasgow
weeks. Coma Scale, longer length of coma, longer du-
ration of post-traumatic amnesia, and older
Background Information age tend to be associated with poor out-
This condition occurs when inflammation comes.111 Optimal rehabilitation is interdisci-
affects the spinal cord, but the brain can be plinary and customized to address the specific
affected as well. Inflammation can result from individuals’ disablement.
viral infections, abnormal immune reactions,
or ischemia, or present as an idiopathic form. ■ Tropical Spastic Paraparesis
Diagnosis is established by exclusion through Chief Clinical Characteristics
imaging and blood tests. The first line of treat- This presentation commonly involves slowly
ment requires accurate diagnosis of the under- progressive paresis of the lower extremities,
lying pathology and decreasing inflammation sphincter dysfunction early in the disease course,
in the acute stage, usually by way of corticos- paresthesias, and uncoordinated movements.1
teroid medication. Physical therapy is indi-
cated to address secondary impairments and Background Information
provide supportive therapy. Recovery from The retrovirus human T-cell leukemia virus
transverse myelitis usually begins within 2 to type 1 causes a chronic infective-inflammatory
12 weeks of the onset of symptoms and may disease of the spinal cord, which results in the
continue for up to 2 years. The majority of re- symptoms of this condition. Diagnosis is
covery occurs within the first 3 to 6 months. confirmed by the presence of the serum of
About one-third of people affected with trans- the antibodies to human T-cell leukemia
verse myelitis experience good or full recovery virus type 1 in the cerebrospinal fluid. Mag-
from their symptoms, regaining the ability to netic resonance imaging also reveals thinness
ambulate. Another one-third is left with signi- of the spinal cord. Treatment is primarily
ficant deficits, while the remaining one-third symptomatic with focus on improved urinary
demonstrates no recovery at all. Prognosis function and decreased spasticity. Steroidal
varies between recovery without relapse to a medications and gamma globulin may be
permanent presence of symptoms, with the used. The majority of individuals with this
primary poor prognostic factors being pain in disease survive.112
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602 Chapter 29 Brain Metastases

TUMORS corticosteroids, and rehabilitative therapies.


SENSORY ABNORMALITIES

■ Brain Metastases Prognosis is also variable and depends on the


type and grade of tumor, severity of compres-
Chief Clinical Characteristics sion, and duration of compression.
This presentation may include headaches,
seizures, dysphagia, weakness, cognitive
■ Nasopharyngeal Carcinoma
changes, behavioral changes, dizziness, vom-
iting, alterations in the level of consciousness, Chief Clinical Characteristics
ataxia, aphasia, nystagmus, visual distur- This presentation may include facial pain
bances, dysarthria, balance deficits, falls, combined with headaches, palpable mass in
lethargy, and incoordination.1,113 the head or neck, bloody nasal discharge,
chronic unilateral nasal congestion, unilateral
Background Information hearing loss or frequent ear infections, or
The majority of individuals with brain metas- cranial nerve signs.
tases have been previously diagnosed with a
primary tumor; however, a small percentage Background Information
of individuals are diagnosed concomitantly Nasopharyngeal tumors usually develop in
with brain metastases and the primary tumor. the wall of the nasopharynx. The relatively
The most common cancers resulting in subse- large amount of space to occupy in this
quent brain metastases include lung, breast, region of the body results in a late onset of
melanoma, colorectal, and genitourinary presenting symptoms and diagnostic delay.
tract. The new onset of neurological symp- This may result in a poorer prognosis related
toms after a primary tumor warrants the use to advanced tumor development and in-
of imaging such as magnetic resonance imag- creased potential for metastatic spread. The
ing or computed tomography to confirm the diagnosis is confirmed with fused positron
diagnosis. Treatment may include corticos- emission tomography–computed tomogra-
teroids, brain irradiation, surgery, chemother- phy and head/neck magnetic resonance
apy, radiotherapy, and rehabilitative thera- imaging. Treatment typically depends on
pies. The prognosis is poor with death cancer staging, ranging from surgical resec-
typically occurring within 6 months. tion and radiation therapy to chemotherapy.
■ Brain Primary Tumors ■ Oropharyngeal Carcinoma
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation may include headaches, This presentation may involve facial pain
seizures, dysphagia, weakness, cognitive combined with hoarseness, throat pain,
changes, behavioral changes, dizziness, vom- change in tongue sensation, pain in the
iting, alterations in the level of consciousness, tongue, and lump in the neck region, dyspha-
ataxia, aphasia, nystagmus, visual distur- gia, dyspnea, coughing, or hemoptysis.
bances, dysarthria, balance deficits, falls, Background Information
lethargy, and incoordination.1,69 Up to 90% of oropharyngeal carcinomas are
Background Information squamous cell carcinomas, or abnormal col-
Glioblastoma multiforme, astrocytoma, oligo- lections of squamous cells on histological
dendroglioma, metastatic tumors, primary observation. Lower patient socioeconomic
central nervous system lymphomas, gangli- status, patient and clinician delay in recogniz-
oglioma, neuroblastoma, meningioma, arach- ing the health condition, lack of indirect
noid cysts, hemangioblastoma, medulloblas- laryngoscopy performed on physical exami-
toma, and acoustic neuroma/schwannoma nation, failure to inspect the site of the tumor,
are some of the more common brain tumors. and clinician failure to consider tumor or in-
Specific diagnoses for brain tumors may be fection as potential causes of symptoms are
confirmed with imaging and biopsy. Treat- associated with overlooking this disease
ment is variable depending on the type, size, process. In turn, overlooking this disease
and location of the tumor and may include process results in reduced prognosis.114 Com-
surgical resection, chemotherapy, radiation, puted tomography and magnetic resonance
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Chapter 29 Vasculitis (Giant Cell Arteritis, Temporal Arteritis, Cranial Arteritis) 603

imaging can further confirm the diagnosis. incontinence, back pain, radicular pain,

SENSORY ABNORMALITIES
Treatment typically depends on cancer stag- atrophy, ataxia, balance deficits, and falls.7
ing, ranging from surgical resection and radi-
ation therapy to chemotherapy.115 Background Information
Types of this condition include astrocytomas,
ependymomas, hemangioblastoma, myeloma,
■ Retinoblastoma
neurofibroma, lymphoma, metastasis, menin-
Chief Clinical Characteristics gioma, schwannoma, and astrocytoma.
This presentation may include leukocoria Extradural tumors, such as meningiomas,
(white reflection from the retina of the eye) and produce a rapid onset of symptoms, with
strabismus (more commonly known as “cross weakness being predominant. Intramedullary
eyes”). tumors, or ependymomas, astrocytomas, and
Background Information hemangioblastomas, present with slowly pro-
Retinoblastoma is a rare childhood tumor that gressive symptoms, of which loss of pain and
is diagnosed by funduscopy most commonly temperature sensation is usually the first. The
between 1 and 2 years of age. Sixty percent of first test to diagnose brain and spinal column
cases are unilateral, while the remainder are tumors is a neurological examination. Special
bilateral. Treatment typically involves laser imaging techniques (computed tomography,
therapy, chemotherapy, cryotherapy, and magnetic resonance imaging, positron emis-
brachytherapy. More severe cases occasionally sion tomography) are also employed. Specific
require external beam radiotherapy. Prognosis diagnoses may be confirmed with imaging
is excellent.116 and biopsy. Treatment is variable depending
on the type, size, and location of the tumor
■ Spinal Metastases and may include surgical resection, chemother-
Chief Clinical Characteristics apy, radiation, corticosteroids, and rehabilita-
This presentation can involve spasticity, tive therapies. Prognosis is variable and
weakness, sensory alterations, bowel and depends on the type and grade of tumor,
bladder incontinence, neck pain, back pain, severity of compression, and duration of
radicular pain, atrophy, cerebellar signs, compression.
balance deficits, falls, and cranial nerve
involvement.1,69,117 ■ Vasculitis (Giant Cell Arteritis,
Temporal Arteritis, Cranial
Background Information Arteritis)
This condition is the most frequent neoplasm Chief Clinical Characteristics
involving the spine.117 The most common
This presentation can be characterized by
types and locations of primary tumors that headaches, psychiatric syndromes, dementia,
result in spinal metastases include breast, peripheral or cranial nerve involvement, pain,
lung, lymphoma, prostate, kidney, gastroin- seizures, hypertension, hemiparesis, balance
testinal tract, and thyroid.1,118 The diagnosis deficits, neuropathies, myopathies, organ in-
is confirmed with gadolinium enhanced mag- volvement, fever, and weight loss.1,69,119
netic resonance imaging and computed to-
mography.1,117 Treatment is variable depend- Background Information
ing on the tumor and may include surgical This condition is the result of an immune-
resection, chemotherapy, radiation, corticos- mediated response resulting in the inflamma-
teroids, and rehabilitative therapies.1 Although tion of vascular structures.1,119 It includes a
the long-term prognosis is poor, individuals variety of disorders such as giant cell/temporal
without paresis and pain and who are still arteritis (which is the most common form),
ambulatory have longer survival rates.118 primary angiitis of the central nervous system,
Takayasu’s disease, periarteritis nodosa,
■ Spinal Primary Tumors Kawasaki disease, Churg-Strauss syndrome, We-
Chief Clinical Characteristics gener‘s granulomatosis, and secondary vasculitis
This presentation may include spasticity, associated with systemic lupus erythematous,
weakness, sensory alterations, bowel/bladder rheumatoid arthritis, and scleroderma.119 The
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604 Chapter 29 Vitamin B12 Deficiency

diagnosis is confirmed through history, physical synkinesis after facial palsy. Otolaryngol Head Neck Surg.
SENSORY ABNORMALITIES

examination, laboratory testing, angiography, Apr 2003;128(4):539–543.


9. Allen D, Dunn L. Acyclovir or valacyclovir for Bell’s
biopsy, and imaging.1,69,119 Corticosteroids, palsy (idiopathic facial paralysis). Cochrane Database
cytotoxic agents, intravenous immunoglobulin, Syst Rev. 2004(3):CD001869.
and plasmapheresis may be used in the treat- 10. VanSwearingen JM, Brach JS. Validation of a treatment-
ment of vasculitis.69,119 Prognosis is variable and based classification system for individuals with facial
neuromotor disorders. Phys Ther. Jul 1998;78(7):
depends on the specific underlying disorder. 678–689.
For example, giant cell arteritis is typically 11. Ramsey MJ, DerSimonian R, Holtel MR, Burgess LP.
self-limiting within 1 to 2 years; however, Corticosteroid treatment for idiopathic facial nerve
death usually occurs within 1 year for individu- paralysis: a meta-analysis. Laryngoscope. Mar 2000;110
(3 pt 1):335–341.
als with primary angiitis of the central nervous 12. Fitzgerald DC. Head trauma: hearing loss and dizziness
system.119 [see comment]. J Trauma-Injury Infect Crit Care.
1996;40(3):488–496.
13. Herdman S, ed. Vestibular Rehabilitation. 2nd ed.
■ Vitamin B12 Deficiency Philadelphia, PA: F. A. Davis; 2000.
Chief Clinical Characteristics 14. Hilton M, Pinder D. The Epley (canalith repositioning)
manoeuvre for benign paroxysmal positional vertigo.
This presentation involves paresthesias in [update of Cochrane Database Syst Rev. 2002;(1):
a stocking and glove distribution, weakness, CD003162; PMID: 11869655]. Cochrane Database Syst
impaired memory, irritability, and depression. Rev. 2004(2):CD003162.
15. Nunez RA, Cass SP, Furman JM. Short- and long-term
Background Information outcomes of canalith repositioning for benign paroxys-
Nutritional deficiency and gastrointestinal mal positional vertigo. Otolaryngol Head Neck Surg.
dysfunction cause this condition. Clinical evi- 2000;122:647–652.
16. Froehling DA, Bowen JM, Mohr DN, et al. The canalith
dence of disease, low serum vitamin B12 levels, repositioning procedure for the treatment of benign
and measurements of metabolites such as paroxysmal positional vertigo: a randomized controlled
methylmalonic acid and homocysteine con- trial. Mayo Clinic Proc. Jul 2000;75(7):695–700.
firm the diagnosis. Anemia is often associated 17. Parnes LS, Agrawal SK, Atlas J. Diagnosis and manage-
ment of benign paroxysmal positional vertigo (BPPV).
with this condition. Treatment includes oral or CMAJ. Sep 30 2003;169(7):681–693.
parenteral vitamin B12 replacement. Repeated 18. Tusa RJ. Benign paroxysmal positional vertigo. Curr
measurement of serum vitamin B12, methyl- Neurol Neurosci Rep. Sep 2001;1(5):478–485.
malonic acid, and homocysteine levels should 19. Dornhoffer JL, Colvin GB. Benign paroxysmal posi-
tional vertigo and canalith repositioning: clinical corre-
be performed every 2 to 3 months after initiat- lations. Am J Otol. Mar 2000;21(2):230–233.
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30
CHAPTER

Abnormal Movement
■ Claire Smith, PT, DPT, NCS ■ Beth E. Fisher, PT, PhD

Description of the Symptom are incompatible with the performance of


normal activities.
The chapter describes pathology that may lead
to “abnormal movement.” Because abnormal Special Concerns
movement is associated with a wide spectrum ■ A change in one’s prior abnormal move-
of movement abnormalities, it is most appro- ment presentation, including but not
priate to first describe normal movement. Nor- limited to:
mal movement is characterized as accurate/ ■ Increase or decrease of tremor
precise, coordinated, smooth, effortless, and ■ Worsening incoordination
purposeful/intentional. Additionally, normal ■ Decrease in accuracy of movement
movement is manifested as a countless variety ■ Decrease in movement speed
of possible movements. Simply stated, abnor- ■ A new onset of abnormal movement not
mal movement then is any movement that associated with the original purpose of the
lacks any or all of those characteristics. physical therapy visit. This may include:
With brain injury or disease, the areas of ■ Change in motor control of face, eyes,
the brain that control the cognitive, visual, and arm, leg, trunk
motor functions involved in movement may ■ Tremor
be injured, resulting in a weakening or absence ■ Difficulty talking
of the many functions required for purposeful ■ Loss of coordination
movement and/or the development of abnor- ■ Loss of balance
mal patterns of posture and movement that

CHAPTER PREVIEW: Conditions That May Lead to Abnormal Movement

T Trauma
COMMON
Encephalopathy 618
Traumatic brain injury 628
UNCOMMON
Not applicable
RARE
Not applicable

I Inflammation
COMMON
Aseptic
Vasculitis (giant cell arteritis, temporal arteritis, cranial arteritis) 629
Septic
Not applicable
608
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Chapter 30 Abnormal Movement 609

Inflammation (continued)

ABNORMAL MOVEMENT
UNCOMMON
Aseptic
Behçet’s disease 613
Multiple sclerosis 621
Septic
Not applicable
RARE
Aseptic
Miller Fisher syndrome 620
Multifocal motor neuropathy 621
Neuromyotonia (Isaac syndrome, Isaac-Merten syndrome,
continuous muscle fiber activity syndrome, quantal squander syndrome) 623
Opsoclonus myoclonus (Kinsbourne syndrome, myoclonic encephalopathy
of infants, dancing eyes–dancing feet syndrome, opsoclonus-myoclonus-ataxia syndrome) 624
Paraneoplastic syndromes 624
Progressive multifocal leukoencephalopathy 626
Septic
Acute disseminated encephalomyelitis 612
Encephalitis 617
Neurosyphilis (tabes dorsalis, syphilitic spinal sclerosis, progressive locomotor ataxia) 623

M Metabolic
COMMON
Drug toxicity 616
UNCOMMON
Not applicable
RARE
Cerebral beriberi (Korsakoff’s amnesic syndrome, Wernicke-Korsakoff syndrome) 614
Fahr’s syndrome (familial idiopathic basal ganglia calcification,
bilateral striopallidodentate calcinosis) 618
Niemann-Pick disease (types C and D only) 623
Tardive dyskinesia 627
Whipple’s disease (intestinal lipodystrophy) 629
Wilson’s disease 630

Va Vascular
COMMON
Binswanger’s disease (subcortical arteriosclerotic encephalopathy, subcortical dementia) 613
Hypoxia (cerebral hypoxia, anoxia) 619
Stroke (cerebrovascular accident) 627
UNCOMMON
Arteriovenous malformation 612
Cerebral aneurysm 614
Cerebral arteriosclerosis 614
RARE
Moyamoya disease 621
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610 Chapter 30 Abnormal Movement


ABNORMAL MOVEMENT

De Degenerative
COMMON
Dementia with Lewy bodies 615
Dysgraphia 616
UNCOMMON
Blepharospasm 613
Huntington’s disease 619
Mitochondrial myopathies 620
Parkinson’s disease 625
RARE
Choreoacanthocytosis (Levine-Critchley syndrome, neuroacanthocytosis) 614
Corticobasal degeneration 615
Creutzfeldt-Jakob disease 615
Dyssynergia cerebellaris myoclonica (Ramsey Hunt syndrome I, dyssynergia cerebellaris progressiva,
dentate cerebellar ataxia, dentatorubral atrophy, primary dentatum atrophy) 617
Friedreich’s ataxia 618
Hallervorden-Spatz syndrome (neurodegeneration with brain iron accumulation, pantothenate
kinase-associated neurodegeneration) 618
Multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy, Shy-Drager
syndrome) 622
Primary lateral sclerosis 626
Progressive supranuclear palsy (Richardson-Steele-Olszewski syndrome) 626
Spinocerebellar ataxia (spinocerebellar atrophy, spinocerebellar degeneration) 627

Tu Tumor
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Malignant Primary, such as:
• Brain primary tumors 628
• Spinal primary tumors 629
Malignant Metastatic, such as:
• Brain metastases 628
• Spinal metastases 628
Benign:
Not applicable

Co Congenital
COMMON
Hydrocephalus 619
UNCOMMON
Dystonia 617
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Chapter 30 Abnormal Movement 611

Congenital (continued)

ABNORMAL MOVEMENT
RARE
Arnold-Chiari malformation (Chiari malformation) 612
Bulbospinal muscular atrophy (Kennedy’s disease, X-linked bulbospinal neuronopathy) 613
Kearns-Sayre syndrome 620
Myoclonus 622
Paroxysmal choreoathetosis 625
Pelizaeus-Merzbacher disease 626

Ne Neurogenic/Psychogenic
COMMON
Depression 616
UNCOMMON
Neurological complications of acquired immunodeficiency syndrome 622
RARE
Hemifacial spasm 619
Normal pressure hydrocephalus 624

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Abnormal Movement inability to coordinate muscle activity during


voluntary movement, so that smooth move-
The term movement disorders refers to neuro- ments occur. It is most often due to disorders
logical syndromes involving abnormal excess of the cerebellum or the posterior columns of
or paucity of movement, often due to basal the spinal cord and may involve the limbs,
ganglia dysfunction. Phenotypically, they head, or trunk. Chorea involves involuntary,
appear as involuntary or semivoluntary move- purposeless, arrhythmic movements of a
ments. A first-pass approach to distinguishing forcible, rapid, jerky type, while athetosis is the
movement disorders of the basal ganglia is to inability to sustain any body part in one posi-
subdivide them into: tion. Relatively slow, sinuous, purposeless
movements that have a tendency to flow into
1. Hypokinesias (paucity of movement) one another interrupt the maintained postures
2. Hyperkinesias (excess of movement). in athetosis. Ballismus includes an uncontrol-
Hypokinesias include bradykinesia and lable, poorly patterned flinging movement on
akinesia. Also, apraxia is the inability to an entire limb.
perform learned movements to command Dystonia is a persistent attitude or posture
or imitation. The group of hyperkinesias is in an extreme of athetoid movement, pro-
diverse. Incoordination involves abnormalities duced by cocontraction of agonist and
of intended (volitional) movement, while antagonist muscles in one region of the body.
tremor includes more or less involuntary and Dystonias may be further classified based on
rhythmic oscillatory movement produced by the number and innervation of the muscles
alternating or irregularly synchronous con- involved. Focal dystonia occurs in one body
tractions of reciprocally innervated muscles. part, segmental dystonia occurs in muscles
Tremors are characterized by whether they innervated by a single segmental level of the
occur with movement (intention tremors) or at spinal cord, and generalized dystonia involves
rest (resting tremors). Ataxia is defined as an most or all body parts. Finally, myoclonus is
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612 Chapter 30 Acute Disseminated Encephalomyelitis

the shock-like contraction(s) of a group of ■ Arnold-Chiari Malformation


ABNORMAL MOVEMENT

muscles, irregular in rhythm and amplitude, (Chiari Malformation)


and, with few exceptions, asynchronous and Chief Clinical Characteristics
asymmetrical in distribution. This presentation may include visual or swal-
The impairments of motor function that lowing disturbances in combination with
result from lesions in various parts of the pain in the occipital or posterior cervical areas,
nervous system may be subdivided into: downbeating nystagmus, progressive ataxia,
1. Paralysis (or paresis) due to involvement of progressive spastic quadriparesis, or cervical
lower motor neurons syringomyelia.4,5
2. Paralysis due to involvement of upper Background Information
motor (corticospinal) neurons This condition encompasses a number of con-
3. Apraxic or nonparalytic disturbances of genital abnormalities at the base of the brain,
purposeful movement due to involvement including extension of the cerebellar tissue or
of association pathways in the cerebrum displacement of the medulla and fourth ven-
4. Involuntary movements and abnormalities tricle into the cervical canal.4 This condition
of posture due to disease of the basal has two main types. Individuals with the more
ganglia common form of this condition, Type I, often
5. Abnormalities of coordination due to lesions remain asymptomatic until adolescence or
of the cerebellum.1 Careful characterization adult life.4 Type II is primarily seen in infants
of the type of movement abnormality is crit- and young children. Please see the pediatric
ical to arrive at a diagnosis of its underlying section of this textbook for a more complete
cause and to accurately communicate the description of this type of the disease. Diagno-
observed movement abnormality with an- sis is made by magnetic resonance imaging,
other health care provider. computed tomography, myelography, or some
combination of these tests.5 Treatment varies
Description of Conditions That depending on clinical progression and may
May Lead to Abnormal Movement include surgical intervention such as an upper
cervical laminectomy or enlargement of the
■ Acute Disseminated foramen magnum. Even with surgery symp-
Encephalomyelitis toms may persist or progress.4
Chief Clinical Characteristics
This presentation may involve confusion, som- ■ Arteriovenous Malformation
nolence, and convulsion in its encephalitic form. Chief Clinical Characteristics
Initial symptoms of the myelitic form include This presentation may be characterized by
weakness and sensory impairments.1 seizures and severe headache. Hemorrhage may
result in paresis, ataxia, dyspraxia, dizziness, tac-
Background Information
tile and proprioceptive disturbances, visual dis-
This condition is a demyelinating disease of
turbances, aphasia, paresthesias, and cognitive
the central nervous system,2 which may be due
deficits.1
to an immune-mediated complication of
infection.1 The presence of upper motor neu- Background Information
ron signs, cerebrospinal fluid pleocytosis and This condition is caused by a tangle of arter-
elevated protein, and multiple white matter ies and veins that cause abnormal communi-
lesions demonstrated on magnetic resonance cation within the vasculature. Approximately
imaging supports the diagnosis.3 Definitive 12% of the 300,000 individuals in the United
diagnosis requires a brain biopsy. The primary States with this condition are symptomatic.
goal of treatment is to suppress the immune This condition is caused by a developmental
response; thus high-dose corticosteroids are abnormality that likely arises during embry-
generally administered over a course of 3 to onic or fetal development. Neurological
5 days.3 The prognosis is variable depending damage occurs due to reduction of oxygen
on the severity of the disease and acuity of the delivery, hemorrhage, or compression of
diagnosis. nearby structures of the brain or spinal cord.
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Chapter 30 Bulbospinal Muscular Atrophy 613

Computed tomography, magnetic resonance Background Information

ABNORMAL MOVEMENT
imaging, and arteriography confirm the di- This condition is a type of vascular dementia
agnosis. Ligation and embolization may be that results from multiple strokes and demyeli-
used to reduce the size of the lesion prior to nation of the central white matter.4,7 Diagnosis
surgical excision, which is the preferred is made by neuroimaging, specifically com-
method of treatment. Stereotactic radiation puted tomography and magnetic resonance
and proton beam therapy are alternative imaging.7 Medical management includes drug
approaches to invasive methods of interven- therapy targeted at improving core symptoms
tion. Up to 90% of individuals who experi- and delaying disease progression, as well as
ence a hemorrhagic arteriovenous malfor- secondary prevention of stroke by decreasing
mation survive.1 hypertension.8,9
■ Behçet’s Disease ■ Blepharospasm
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation may include bilateral py- This presentation includes excessive involuntary
ramidal signs (signs related to lesions of upper closure of the eyelids primarily due to spas-
motor neurons or descending pyramidal modic contraction of the orbicularis oculi mus-
tracts, such as a positive Babinski sign or cles. Severity of symptoms ranges from frequent
hyperreflexia), headache, memory loss, hemi- blinking to functional blindness.
paresis, cerebellar ataxia, balance deficits,
Background Information
sphincter dysfunction, or cranial nerve palsies.
The majority of cases of this condition
In addition to these neurological signs indi-
are idiopathic. Diagnosis is made by clinical
viduals with this condition also may present
presentation. Treatment includes psychother-
with arthritis; renal, gastrointestinal, vascular,
apy, biofeedback, drugs, and surgery. Botu-
and cardiac diseases; and genital, oral, and cu-
linum toxin A is the most effective form of
taneous ulcerations.6
treatment.
Background Information
Mean age of onset is in the third decade of ■ Bulbospinal Muscular Atrophy
life. Diagnostic criteria according to an inter- (Kennedy’s Disease, X-Linked
national study group include presence of Bulbospinal Neuronopathy)
recurrent oral ulceration, recurrent genital Chief Clinical Characteristics
ulceration, eye lesions, skin lesions, papulo- This presentation can be characterized
pustular lesions, and/or a positive pathergy by severe, diffuse muscle cramping and
test.1,6 Medical treatment typically consists of fasciculations, muscle weakness in a limb-
corticosteroids and immunosuppressants. girdle distribution, and postural hand tremor.
Neurological symptoms tend to clear within Other symptoms include variable bulbar
weeks, but can sometimes recur or result in muscle weakness, gynecomastia, premature
permanent deficits.1 Negative prognostic muscle exhaustion, and hyporeflexia or
factors include onset before the age of 25 and areflexia.10,11
male sex.
Background Information
■ Binswanger’s Disease (Subcortical This condition is a rare, x-linked, progressive
Arteriosclerotic Encephalopathy, neuromuscular disorder that is usually seen
Subcortical Dementia) in males between 30 and 50 years old. Diag-
Chief Clinical Characteristics nosis is made by clinical features, electro-
This presentation involves small-stepped gait, physiological study, and genetic testing.10
slowed motor function with perseveration, There is currently no proven treatment for
deficits in executive function, slow informa- this disease, but genetic counseling is recom-
tion processing, and impaired memory. Other mended upon diagnosis.12 This condition is
symptoms include dysarthria, dysphagia, uri- usually associated with a normal life span,
nary disturbances, and lateral homonymous but individuals may experience significant
hemianopias.4,7,8 disability.10
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614 Chapter 30 Cerebral Aneurysm

■ Cerebral Aneurysm lifestyle modification, pharmacotherapy, and


ABNORMAL MOVEMENT

Chief Clinical Characteristics surgery. Cerebral arteriosclerosis can result in


This presentation may involve loss of balance in ischemic or hemorrhagic stroke, thus causing
combination with a whole host of other neuro- neurological impairments.1
logical symptoms and signs that depend on the
■ Cerebral Beriberi (Korsakoff ’s
affected cerebral tissue, including visual and
Amnesic Syndrome,
proprioceptive loss. Any associated signs or
Wernicke-Korsakoff Syndrome)
symptoms may not be reported due to the fact
that this condition is typically asymptomatic Chief Clinical Characteristics
prior to rupture. However, if the aneurysm This presentation involves ophthalmoparesis,
results in a mass effect, ischemia, or hemor- nystagmus, ataxia, and confusion, as well as
rhage, then neurological signs and symptoms are impaired learning and memory. Other com-
dependent on the affected location.1,13,14 mon symptoms include peripheral neuropathy,
postural hypotension, syncope, impaired olfac-
Background Information tory discrimination, mild hypothermia, and
There has been some description of genetic confabulation.4
factors in this condition.13 Cigarette smoking,
hypertension, and heavy alcohol use have all Background Information
been found to be correlated with increased risk This condition is due to a thiamine deficiency
of aneurysm development.13,14 Factors associ- that results in a diffuse decrease in cerebral
ated with increased risk of rupture include size glucose utilization. It is most commonly
of aneurysm, location in the posterior circula- observed in individuals who abuse alcohol and
tion, and a previous history of aneurismal have nutritional deficiencies, although it is not
subarachnoid hemorrhage.15,16 Definitive di- limited to this population.4 Diagnosis can
agnosis is based on catheter angiography; be made by blood tests to examine thiamine
however, magnetic resonance angiography, levels. Neuroimaging may show slowed brain
magnetic resonance imaging, and computed activity as well as lesions in the medial thala-
tomography may aid in the diagnosis. Unrup- mus and periaqueductal region.4 Medical
tured aneurysms are sometimes surgically treatment involves the immediate administra-
treated. Aneurysm size, location, and prior tion of thiamine. Once thiamine has been
history of a subarachnoid hemorrhage help to administered, the reversal of symptoms should
determine if the risk of surgical treatment is begin to occur within hours to days with vari-
worth the potential benefits. Most aneurysms able degrees of recovery. Memory has been
that have hemorrhaged must be treated shown to have the poorest return, and mortal-
surgically. Patients with a previous rupture ity rates of up to 17% have been reported.4
are at an 11 times greater risk of having a
second intracranial aneurysm rupture. When ■ Choreoacanthocytosis
aneurysms do rupture, many patients die (Levine-Critchley Syndrome,
within one month of the rupture, and those Neuroacanthocytosis)
who survive often have residual neurological Chief Clinical Characteristics
deficits.13 This presentation commonly includes chorea,
motor or vocal tics, dystonia, orofacial dyskine-
■ Cerebral Arteriosclerosis sias, and parkinsonism. Seizures, cognitive im-
Chief Clinical Characteristics pairment, psychosis, paranoia, and personality
This presentation includes visual disturbances, changes are also seen with this diagnosis, along
headache, and facial pain. with hyporeflexia and distal myopathy due to
denervation of muscles.17–19
Background Information
Thickening and hardening of the artery Background Information
walls in the brain leads to the development of This condition is a rare, autosomal recessive
this condition. Diagnosis is established by disorder that typically has its onset during the
computed tomography or magnetic resonance third and fourth decades of life.19 This neu-
imaging of the brain. Treatment includes rodegenerative disorder is associated with
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Chapter 30 Dementia with Lewy Bodies 615

acanthocytes, aberrant spiky or thorny red ■ Creutzfeldt-Jakob Disease

ABNORMAL MOVEMENT
blood cells, as well as atrophy and gliosis of the Chief Clinical Characteristics
caudate, putamen, and globus pallidus.18 This presentation may involve rapidly progres-
Diagnosis is made by a combination of tests, sive dementia, cerebellar ataxia, balance
including clinical features, lab work demon- deficits, myoclonus, cortical blindness, pyram-
strating acanthocytosis, neuroimaging, and idal signs, extrapyramidal signs, and akinetic
genetic testing to rule out Huntington’s dis- mutism.24
ease.17 There is currently no effective, long-
term treatment, although verapamil has been Background Information
found to provide temporary reduction of Different forms of this condition have been
symptoms. Life expectancy is reduced, and described including sporadic, iatrogenic, and
suicidal action or ideation is not uncommon variant. Sporadic and iatrogenic forms of this
due to cognitive impairments.19 condition typically affect older individuals,
whereas the variant form affects younger indi-
■ Corticobasal Degeneration viduals.25 The early stages of the variant form
Chief Clinical Characteristics are characterized by psychiatric symptoms,
This presentation typically includes limb ideo- including depression and anxiety.25 The con-
motor apraxia and unilateral parkinsonism that dition is rare and affects only one to two peo-
is unresponsive to levodopa, gait disturbances, ple per million worldwide per year.24,25 It is
tremor, postural instability, and dementia.20,21 caused by a conformational change of the
normal prion protein, which is encoded by
Background Information human chromosome 20, to a disease-related
A proposed set of criteria for the diagnosis of prion protein. Diagnosis is suggested by a
this condition includes core features of: thorough history and physical examination,
1. Insidious onset and progressive course electroencephalography, and cerebrospinal
2. No identifiable cause fluid analysis.25 Computed tomography and
3. Cortical dysfunction (ideomotor apraxia, magnetic resonance imaging are typically nor-
alien-limb phenomenon, cortical sensory loss, mal in sporadic and iatrogenic forms and help
visual or sensory hemineglect, constructional to exclude other diagnoses.24,26 Diagnosis for
apraxia, focal or asymmetric myoclonus, all forms of this condition is confirmed post-
apraxia of speech or nonfluent aphasia) mortem.26 There is no proven treatment.25,26
4. Extrapyramidal dysfunction (rigidity which Death in the sporadic and iatrogenic forms
does not respond to levodopa therapy and occurs in a matter of months, at a mean age of
dystonia).22 66 years.25 Mean duration of illness in the vari-
ant form is 14 months.25
This condition is a sporadic disease with
an average age of onset of 63 years.21 There ■ Dementia with Lewy Bodies
have been reports of familial cases; however, Chief Clinical Characteristics
for most cases there is no known cause.20 Diag- This presentation can be characterized by fluc-
nosis is suggested based on clinical presenta- tuating cognitive dysfunction, particularly
tion. A definitive diagnosis can only be made visuospatial problems and executive dysfunc-
postmortem. Medical treatment is not typi- tion, visual hallucinations, and parkinsonism
cally successful; it has been found that only features such as masked facies, autonomic dys-
about 24% of these patients will respond to function, rigidity, and bradykinesia. Other signs
levodopa therapy aimed at addressing the and symptoms may include postural instabil-
extrapyramidal features of the disease.23 Phys- ity, falls, sleep disturbances, memory problems,
ical and occupational therapy are used to syncope, transient loss of consciousness, and
maintain mobility and address safety issues sensitivity to antipsychotic and anti-Parkinson
related to the progression of imbalance. medications.27
Mean survival is 7.9 years with a range of 2.5
to 12.5 years.21 Early presence of bilateral Background Information
parkinsonism or frontal lobe signs indicates a This progressive condition is the second most
less favorable prognosis.21 common dementia after Alzheimer’s disease.27
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616 Chapter 30 Depression

The specific etiology of this condition is functioning and are not better accounted for
ABNORMAL MOVEMENT

unknown. The characteristic Lewy bodies are by bereavement. The best approach to treat-
eosinophilic inclusion bodies found within the ment for depression is a combination of psy-
cytoplasm of neurons in the cerebral cortex chotherapy and antidepressant medications.32
and limbic system.27 A thorough clinical This condition responds well to treatment—
examination, laboratory screen, and imaging approximately 70% to 80% of treated patients
are important to rule out other causes of have significant reduction in symptoms. How-
dementia. The definitive diagnosis for this ever, approximately 20% of patients who are
condition is made postmortem; however, it chronically depressed have recurrent and se-
appears that the use of single-photon emission vere depressive episodes.33
computed tomography and positron emission
■ Drug Toxicity
tomography may be useful in the identifica-
tion of occipital hypoperfusion, which may be Chief Clinical Characteristics
associated with the visual hallucinations.27,28 This presentation involves tinnitus (fluctuating
Management includes caregiver education to or constant) and hearing loss (mild to complete
assist in minimizing factors that may con- deafness) if the cochlea is involved; vertigo,
tribute to problematic behaviors. Medication vomiting, nystagmus and imbalance if the
therapy may be indicated, but should be mon- vestibular system is involved unilaterally; and
itored closely due to potential exacerbation headache, ear fullness, oscillopsia, an inability
of symptoms.27 Life expectancy for individuals to tolerate head movement, a wide-based gait,
with this condition is similar to that of difficulty walking in the dark, a feeling of un-
Alzheimer’s disease. The average survival steadiness and actual unsteadiness while mov-
time is between 6 and 8 years from the onset of ing, imbalance to the point of being unable to
dementia.27,29 walk, lightheadedness, and severe fatigue in
bilateral involvement.
■ Depression Background Information
Chief Clinical Characteristics The severity, type, and particular combination
This presentation may involve slowness of move- of symptoms are variable, depending on the
ment that can progress to the point of catato- drug exposure, whether it is unilateral or bilat-
nia, intensely dysphoric mood, appetite loss, eral, the speed of onset, and the individual.
insomnia or hypersomnia, social withdrawal, A slow unilateral loss may produce minimal
loss of motivation, helplessness, hostility, and symptoms, since the brain can compensate
agitation.4 through other mechanisms, whereas a fast
Background Information bilateral loss can produce significant disability.
The origin of this condition is not fully under- The key diagnostic feature is a history of drug
stood and genetic, biochemical, neuroanatom- or chemical exposure. Treatment involves
ical and psychosocial factors all appear to play removing exposure to the medication, if possi-
a role.30 For a clinical diagnosis, the following ble, and vestibular habituation and balance
criteria from the DSM-IV-TR31 must be met: exercises.
at least five of the following symptoms, during ■ Dysgraphia
the same 2-week period, representing a change
Chief Clinical Characteristics
from previous functioning: depressed mood;
This presentation typically includes distorted or
diminished interest or pleasure; significant
incorrect writing, including inappropriately
weight loss or gain; insomnia or hypersomnia;
sized or spaced letters. Individuals with this
psychomotor agitation or delay; fatigue or loss
condition may also demonstrate incorrect or
of energy; feelings of worthlessness; dimin-
odd spelling.4,34
ished ability to think or concentrate, indeci-
siveness; recurrent thoughts of death, suicidal Background Information
ideation, suicide attempt, or specific plan for In adults, dysgraphia is frequently seen in, but
suicide. In addition symptoms must cause not limited to, patients with delirium, demen-
clinically significant distress or impairment of tia of Alzheimer’s type, or after traumatic
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Chapter 30 Encephalitis 617

brain injury or stroke.34,35 Assessment and idiopathic, due to an autosomal dominant

ABNORMAL MOVEMENT
diagnosis can be made by having patients write trait, or secondary to a vascular, traumatic,
to dictation and perform letter copying; how- infectious, or toxic brain insult.4,38 Diagnosis
ever, the underlying cause of the dysgraphia is made by clinical presentation or genetic
should also be determined by clinical exam and testing in the case of hereditary forms of this
ancillary testing.34 Treatment varies, but may condition. Treatment for generalized forms of
include therapy for motoric control of writing this condition has shown only fair success
movements. Some patients may also benefit and includes levodopa therapy, calcium chan-
from computer training to allow communica- nel blockers, anticonvulsants, and anxiolytics.
tion without handwriting. Prognosis is variable Positive results have been reported for the
due to the wide array of possible causes. implantation of deep brain stimulation to the
globus pallidus in severe, generalized forms
■ Dyssynergia Cerebellaris of this condition.38 In focal forms of this
Myoclonica (Ramsay Hunt condition, the most effective treatment is
Syndrome I, Dyssynergia botulinum toxin injections to the affected
Cerebellaris Progressiva, Dentate muscles.4,39 Rehabilitation strategies should
Cerebellar Ataxia, Dentatorubral focus on the orthopedic and neurological
Atrophy, Primary Dentatum complications that can occur as a result of
Atrophy) this condition.40 Improvement is usually lim-
Chief Clinical Characteristics ited, although a small percentage have sponta-
This presentation involves the clinical triad of neous remission.39 Occasionally, this condi-
action myoclonus, progressive ataxia, and tion can increase in intensity or progress to
epilepsy with cognitive impairment. Tremor other body parts.4
typically begins in one extremity and progresses
■ Encephalitis
throughout the body.36,37
Chief Clinical Characteristics
Background Information This presentation includes confusion, delirium,
This is a rare syndrome that is caused by convulsions, problems with speech or hearing,
degeneration of the olivodentatorubral system memory loss, hallucinations, drowsiness,
with a typical onset in early adulthood.37 Diag- and coma. Loss of balance and/or falls may be
nosis is made by clinical presentation as well as present.
neurophysiological and radiologic findings,
including atrophy of the dentate nucleus.4,37 Background Information
Treatment is symptomatic for myoclonus and Encephalitis is an inflammation of nerve
seizures, and may include the prescription of cells in the brain. This term usually refers to
drugs such as valproate and acetazolamid.36 the viral form, although bacterial, parasitic,
The disease is progressive and ataxic symp- and fungal agents also can cause this condi-
toms may not be present for up to 20 years tion. Up to 20,000 new cases of viral forms of
after onset.4 this condition are reported annually in the
United States. Diagnosis is established by
■ Dystonia clinical presentation suggesting dysfunction
Chief Clinical Characteristics of the cerebrum, brainstem, or cerebellum,
This presentation can be characterized by invol- cellular reaction and elevated protein in
untary muscle contractions causing repetitive spinal fluid, and possible demonstration of
movements or abnormal postures. The abnor- diffuse edema or enhancement of the brain
mal posturing can be generalized, occurring in on magnetic resonance imaging or computed
the hands, feet, head, trunk, and/or face; or tomography. Treatment is primarily pharma-
focal, limited to one area of the body. cologic, with drugs such as corticosteroids,
antiviral agents, and anticonvulsants. The
Background Information majority of individuals with encephalitis do
The most frequent cause of this condition recover, but irreversible brain damage and
is drug intoxication; however, it can also be death can result.1
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618 Chapter 30 Encephalopathy

■ Encephalopathy sensitive for identifying calcium deposits.44


ABNORMAL MOVEMENT

Chief Clinical Characteristics Treatment aimed at minimizing calcium


This presentation commonly includes neuromy- deposits has been unsuccessful.45 Individuals
oclonus, nystagmus, ataxia, and tremor. with this condition may be responsive to
Altered mental state, loss of memory, personal- levodopa for treatment of their parkinsonian
ity changes, dementia, and seizures are possi- features.1
ble signs. Additional symptoms include muscle ■ Friedreich’s Ataxia
atrophy and weakness. Patients may also pres-
ent with a progressive loss of consciousness Chief Clinical Characteristics
resulting in coma.4,41 This presentation can be characterized by the
onset of progressive gait and limb ataxia
Background Information in childhood to early adulthood. Associated
Encephalopathy involves diffuse disease of the symptoms include loss of vibration sense and
brain that alters brain function or structure. proprioception, absent deep tendon reflexes,
The numerous causes of encephalopathy weakness, dysarthria, and sensorineural hear-
include cirrhosis of the liver, severe hyperten- ing loss. Often, cardiac hypertrophy, diabetes mel-
sion, thiamine deficiency, infection, metabolic litus, and optic atrophy are also present.4,46,47
or mitochondrial dysfunction, toxin exposure,
trauma, or lack of oxygen to the brain.41 Diag- Background Information
nosis is made by numerous studies due to the This autosomal recessive condition is caused
variable causes of encephalopathy. These tests by a genetic mutation, resulting in a progres-
include blood work, cerebrospinal fluid exam- sive loss of large myelinated sensory axons,
ination, electroencephalography, and neu- followed by degeneration of the posterior
roimaging studies.42 Treatment is sympto- columns and spinocerebellar and pyramidal
matic and varies according to the cause of tracts.4,48 Magnetic resonance imaging may
encephalopathy. Even with treatment, en- show atrophic changes characteristic of the
cephalopathy can cause permanent brain disorder, and should be performed along
damage and, in some cases, may be fatal.4 with genetic testing and electrocardiography
for definitive diagnosis. There is currently no
■ Fahr’s Syndrome (Familial medical treatment to slow or stop disease
Idiopathic Basal Ganglia progression, but individuals with this condi-
Calcification, Bilateral tion may benefit from surgical intervention to
Striopallidodentate Calcinosis) correct foot and spine deformities and allow
Chief Clinical Characteristics for improved mobility. Average lifetime sur-
This presentation may involve features of vival is 25 to 30 years of age.4,48
parkinsonism, such as chorea, athetosis, rigid-
ity, dystonia, and tremor in addition to cogni- ■ Hallervorden-Spatz Syndrome
tive impairments, cerebellar impairments, gait (Neurodegeneration With Brain
and balance disorder, psychiatric features, pain, Iron Accumulation, Pantothenate
pyramidal signs (such as weakness, hyper- Kinase-Associated
reflexia in the deep tendon reflexes, hypertonia, Neurodegeneration)
clonus, and/or a positive Babinski sign), sensory Chief Clinical Characteristics
changes, and speech disorder.43 This presentation includes dystonia, parkin-
sonism, choreoathetosis, spasticity, cognitive
Background Information
impairment, corticospinal tract involvement,
This condition occurs due to bilateral sym-
optic atrophy, and pigmentary retinopathy.49
metric calcification of the basal ganglia with or
without calcification of the dentate nucleus. Background Information
The disease has been described as both famil- This rare, inherited disorder results from a
ial and nonfamilial.1 Diagnosis is established genetic mutation in the iron regulatory path-
using computed tomography or magnetic ways, resulting in excessive iron accumulation
resonance imaging of the brain; however, com- in the basal ganglia.49,50 Diagnosis is made by
puted tomography has been found to be more magnetic resonance imaging, which shows
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Chapter 30 Hypoxia (Cerebral Hypoxia, Anoxia) 619

characteristic abnormalities in the basal but require high levels of care and supervision

ABNORMAL MOVEMENT
ganglia, known as the “eye of the tiger” sign. during those years.53
Pathological studies may also show brown dis-
coloration, iron pigmentation, and gliosis in ■ Hydrocephalus
the globus pallidus and substantia nigra.49 Chief Clinical Characteristics
There is no cure or effective treatment for This presentation commonly includes frontal lobe
the condition, but patients may benefit from signs such as slowness of mental response, inat-
rehabilitation therapies to decrease disability tentiveness, distractibility, perseveration, in-
as the disease follows its progressive course of ability to sustain complex cognitive function, and
degeneration.50 incontinence. Other symptoms include gait
deterioration, frequent falls, occipital or frontal
■ Hemifacial Spasm headaches, nausea and vomiting, diplopia, and
Chief Clinical Characteristics lethargy. Advanced stages are associated with
This presentation typically involves involun- coma and extensor posturing.
tary paroxysmal contractions of the muscles
innervated by the facial nerve. These contrac- Background Information
tions can range in severity and may affect the Intracranial pressure can be increased due to
orbicularis oculi, orbicularis oris, platysma, many mechanisms including a cerebral or
and/or other superficial muscles of the ipsilat- extracerebral mass, generalized brain swelling,
eral hemiface.39 increased venous pressure, obstruction to the
flow and absorption of cerebrospinal fluid, or
Background Information volume expansion of cerebrospinal fluid.1
The exact pathophysiology of this condition is Magnetic resonance imaging and the presence
currently unknown, but it may be due to a hy- of papilledema are commonly used to estab-
perexcitable facial motor nucleus or compres- lish the diagnosis of hydrocephalus. Medical
sion of the facial nerve.39 Diagnosis is made treatment may include restriction of fluid
by observation and clinical history. Currently, intake and drugs with an osmotic effect, or the
the two most widely used treatments are addition of diuretics.54 Surgical treatment
microvascular decompression of the facial depends on the chronicity of the hydro-
nerve at the pons and intramuscular injections cephalus. The acute form of this condition is
of botulinum toxin type A.39,51 This condition considered fatal and is emergently treated via
is a chronic disease and spontaneous recovery lumbar puncture or ventricular catheter.54 The
rarely occurs.39 chronic form of this condition is treated with
placement of a ventricular shunt or with surgi-
■ Huntington’s Disease
cal removal of a mass if that is the cause of the
Chief Clinical Characteristics hydrocephalus. Although surgical procedures
This presentation involves progressive chorea for hydrocephalus have a high success rate and
of the entire body, emotional disturbances a good prognosis, it is common to have shunt
such as behavior and personality changes, and complications such as infection, occlusion,
dementia.4,52,53 and over- or underdrainage. Thus, patients
Background Information who have been treated for this condition
This autosomal dominant genetic disorder must continue to be medically managed and
causes selective neurodegeneration, most educated regarding the indications of shunt
commonly in the neostriatum. Diagnosis is compromise.
made by genetic testing.53 There is currently
no treatment to slow or stop the progression of ■ Hypoxia (Cerebral Hypoxia,
this condition; care is focused on symptom Anoxia)
management and optimization of function- Chief Clinical Characteristics
ing.53 The prognosis for Huntington’s disease This presentation includes a wide variety of
is poor, and individuals usually experience symptoms that depend on the condition’s sever-
very rapid decline. On average, patients sur- ity. Mild hypoxia without loss of consciousness
vive for 15 to 20 years after initial diagnosis, may present with inattentiveness, poor judgment,
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620 Chapter 30 Kearns-Sayre Syndrome

and motor incoordination. More severe levels of mostly symptomatic and supportive, al-
ABNORMAL MOVEMENT

hypoxia can result in seizures and/or coma. though cardiac symptoms may be managed
Post-hypoxic neurological symptoms include with medication. This condition is a slowly
dementia, parkinsonian syndrome, choreoa- progressive disorder and prognosis is often
thetosis, cerebellar ataxia, intention or action determined by the degree of heart conduc-
myoclonus, or a Korsakoff amnesic state.4 tion impairment.58
Background Information ■ Miller Fisher Syndrome
This condition occurs as a result of a decrease
of oxygen supply to the brain. It has numerous Chief Clinical Characteristics
causes, including cardiac arrest, drowning, This presentation can be characterized by
strangulation, aspiration, choking, carbon an acute onset of the classic triad of ophthalmo-
monoxide poisoning, and complications of plegia, ataxia, and areflexia. Additional
general anesthesia. Pure hypoxia produces symptoms include mydriasis, sensory loss, facial
damage in areas susceptible to reduced oxygen palsy, bulbar palsy, dysesthesia, weakness, and
delivery such as the hippocampi and the cere- urinary incontinence.59
bellum. This condition is often seen along with Background Information
ischemia, producing complex patterns of cere- This condition is thought to be a variant
bral damage.4 Diagnosis and determination of form of acute demyelinating polyneuropathy
the cause of hypoxia may require magnetic (Guillain-Barré syndrome) and is usually pre-
resonance imaging, electrocardiography, labo- ceded by infectious gastrointestinal or respira-
ratory studies, electroencephalography, and tory symptoms approximately 8 days before
evoked potentials.4,55 Treatment is directed at onset of symptoms.59 Diagnosis is based on
prevention of further hypoxic injury. Out- clinical presentation. In addition, elevated cere-
comes vary, depending on cause and severity brospinal fluid protein values and electrophys-
of hypoxia, and range from full recovery to iological examination demonstrating conduc-
coma or even death. The longer an individual tion block or axonal damage on limbs of
with this condition is unconscious, the lower normal strength can help reinforce the diagno-
the likelihood of a meaningful recovery.4 sis.60 Plasmapheresis and administration of
intravenous immunoglobulins have both been
■ Kearns-Sayre Syndrome
found to be helpful in decreasing recovery
Chief Clinical Characteristics time.60 The natural course of recovery in indi-
This presentation commonly includes a classic viduals with this condition is good with mini-
triad of symptoms, involving progressive exter- mal disability seen 6 months after onset.59
nal ophthalmoplegia, retinal pigmentary degen-
eration, and heart block. Common additional ■ Mitochondrial Myopathies
findings include cerebellar dysfunction, my-
opathy, ataxia, sensorineural hearing loss, men- Chief Clinical Characteristics
tal retardation, growth hormone deficit with This presentation typically involves a combina-
dwarfism, hypoparathyroidism, and diabetes tion of exercise intolerance, ataxia, seizures,
mellitus.56,57 myoclonus, headaches, small strokes, ophthal-
moplegia, deafness, muscle cramps and/
Background Information or slowly progressive myopathy with proximal
Etiology has not been established; however greater than distal involvement. Other less
this rare, sporadic mitochondrial disorder is common symptoms that may be seen include
thought to occur via a mutation in either dementia, lactic acidosis, ptosis, and cardiac
the ovum or zygote.56 Diagnosis is established conduction defects.4,61
with a combination of clinical, radiologic,
pathological, biochemical, and molecular Background Information
studies. Mitochondrial deoxyribonucleic acid This condition refers to a large group of disor-
analysis and histological verification of the ders that result from a mutation in the mito-
presence of ragged red fibers may be helpful chondrial genome, resulting in damage to
in determining diagnosis.56 Treatment is the mitochondria. These disorders include
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Chapter 30 Multiple Sclerosis 621

Kearns-Sayre syndrome, myoclonus epilepsy Background Information

ABNORMAL MOVEMENT
with ragged red fibers, mitochondrial en- This condition is thought to be immunologi-
cephalomyopathy with lactic acidosis and cally mediated, although the exact mechanism
stroke-like episodes, as well as other childhood- is unknown.64 Diagnosis is made by the pres-
onset disorders.4 A combination of clinical ence of definite motor conduction block with
picture, histological findings of ragged red normal sensory nerve conduction on electro-
fibers, elevated serum lactate, and possible physiological study.65 Medical treatment is
family history contribute to the diagnosis of intravenous immunoglobulin therapy.65,66
this condition.4 There is no specific treatment, This condition has a slow, progressive course
but new research shows that patients may of deterioration of muscle strength, but the
benefit from physical therapy for submaximal disease generally does not cause severe disabil-
exercise training.63 Most patients experience ity or death.65,66
lifelong progression of the disease and prog-
nosis varies according to the type of disease ■ Multiple Sclerosis
and amount of involvement.4 Chief Clinical Characteristics
This presentation may include paresthesias,
■ Moyamoya Disease weakness, spasticity, hypertonicity, hyper-
Chief Clinical Characteristics reflexia, positive Babinski sign, incoordination,
This presentation may include unsteady gait, in- optic neuritis, ataxia, vertigo, dysarthria,
voluntary movement, weakness, speech and diplopia, bladder incontinence, tremor,
sensory impairments, headache, seizures, balance deficits, falls, and cognitive deficits.1
impaired mental development, visual distur-
bances, and nystagmus.4 Background Information
This condition may present as relapsing-
Background Information remitting, primary progressive, or secondary
This rare condition results from progressive progressive. The disease occurs most fre-
occlusion of the arteries of the circle of Willis.4 quently in women between the ages of 20 and
Diagnosis is based on clinical findings and 40 years. Only a small number of children or
results of magnetic resonance imaging and individuals between 50 and 60 years of age are
magnetic resonance angiography. Images will diagnosed with this condition.1 This condition
demonstrate the occlusion of the circle of was originally thought to be secondary to en-
Willis as well as secondary cerebral infarction, vironmental and genetic factors, but evidence
white matter lesions, atrophy, and hemor- suggests an autoimmune response to a viral in-
rhage.62 Treatment options include revascular- fection, which subsequently targets myelin.1
ization surgery and medical treatment to The diagnosis may be confirmed by a thor-
prevent hypertension and further strokes.63 ough history, physical examination, magnetic
Rehabilitative therapies are used to treat func- resonance imaging, analysis of cerebrospinal
tional deficits that the patient may incur from fluid, and evoked potentials.1,67,68 Life ex-
a stroke, secondary to the progression of the pectancy and cause of mortality are similar
disease. Outcome depends on the severity of for all types of this condition.1 Clinical charac-
secondary complications and presence of teristics that are associated with a longer time
subsequent occlusion. interval for progression of disability include
female sex, younger age of onset, relapsing-
■ Multifocal Motor Neuropathy remitting type, complete recovery after the
Chief Clinical Characteristics first relapse, and longer time interval between
This presentation includes progressive, asymmet- first and second exacerbation.69 Medical
rical weakness, muscle atrophy, cramps, and management may include the use of methyl-
fasciculations that develop slowly over several prednisolone, prednisone, cyclophosphamide,
years. Other symptoms include wrist and foot immunosuppressant treatment, and betainter-
drop, grip weakness, reduced tendon reflexes feron.1 Physical, occupational, and speech
in affected areas, and occasional cranial or therapy may be indicated to prevent secondary
phrenic nerve involvement. sequelae and to optimize functional activity
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622 Chapter 30 Multiple System Atrophy

and mobility. Some individuals may benefit ■ Myoclonus


ABNORMAL MOVEMENT

from psychology/psychiatry and social sup- Chief Clinical Characteristics


port as the disease progresses. This presentation includes sudden, brief, shock-
like, involuntary movements that are caused
■ Multiple System Atrophy
by muscular contraction or inhibition.73,74
(Striatonigral Degeneration,
Olivopontocerebellar Atrophy, Background Information
Shy-Drager Syndrome) This condition may be caused by many differ-
Chief Clinical Characteristics ent processes, including epileptic forms, essen-
This presentation involves tremor, rigidity, tial and heredofamilial forms, myoclonic
akinesia, and/or postural imbalance along dementias, myoclonus with cerebellar disease,
with signs of cerebellar, pyramidal, and auto- metabolic and toxic disorders, and focal and
nomic dysfunction. Autonomic symptoms such spinal forms of myoclonus.4,73 This condition
as orthostatic hypotension, dry mouth, loss of may originate from numerous structures, in-
sweating, impotence, and urinary inconti- cluding the cerebral cortex, subcortical struc-
nence or retention are the initial feature in 41% tures, brainstem, spinal cord, and peripheral
of individuals, with 74% to 97% of individ- nerves.74 Diagnosis is made by a combination
uals developing some degree of autonomic of clinical picture, clinical neurophysiology
dysfunction during the course of the disease.70 testing, basic ancillary testing, and, if necessary,
This condition is a combination of parkin- advanced testing for rare and specific diag-
sonian and non-parkinsonian symptoms and noses.73 Treatment varies according to the etiol-
signs. ogy and primarily includes treatment of the un-
derlying cause or antimyoclonic pharmacologic
Background Information treatment.73,74 Prognosis also varies depending
Diagnostic criteria are based on the clinical on the cause of this condition, with some cases
presentation, which includes poor response being very transient in nature and others result-
to levodopa, presence of autonomic features, ing in progressive, significant disability.4,73
presence of speech or bulbar problems,
absence of dementia, absence of toxic confusion, ■ Neurological Complications of
and presence of falls.71 The disease course Acquired Immunodeficiency
ranges between 0.5 and 24 years after diagno- Syndrome
sis with a mean survival time of 6.2 years.72 Chief Clinical Characteristics
This condition is a progressive condition of This presentation is variable and dependent on
the central and autonomic nervous systems the affected neuroanatomical structures in an
that rarely occurs without orthostatic hy- individual with acquired immunodeficiency
potension. There are three types of this condi- syndrome.75
tion. The parkinsonian-type includes symp-
Background Information
toms of Parkinson’s disease such as slow
This condition may be categorized by:
movement, stiff muscles, and tremor. The
cerebellar type causes problems with coordi- 1. Meningitic symptoms including headache,
nation and speech. The combined type in- malaise, and fever (such as secondary to
cludes symptoms of both parkinsonism and meningitis, cryptococcal meningitis, tuber-
cerebellar failure. Older age at onset is associ- culous meningitis, and human immunode-
ated with a shorter survival time.72 Average age ficiency virus headache)
of onset is 54 years, with mean age at death be- 2. Focal cerebral symptoms including hemi-
ing 60.3 years.70 Most individuals with this paresis, aphasia, apraxia, sensory deficits,
condition receive a trial of levodopa although homonymous hemianopia, cranial nerve in-
only a minority respond.70 Additional treat- volvement, balance deficits, incoordination,
ment addresses symptoms and involves physi- and/or ataxia (such as secondary to cerebral
cal and occupational therapy to maintain mo- toxoplasmosis, primary central nervous sys-
bility and address safety issues related to the tem lymphoma, and progressive multifocal
progression of imbalance. leukoencephalopathy)
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Chapter 30 Niemann-Pick Disease (Types C and D Only) 623

3. Diffuse cerebral symptoms that involve cog- hyperexcitability of the peripheral nerve.76 It is

ABNORMAL MOVEMENT
nitive deficits, altered level of consciousness, a rare, usually acquired disease that is often
hyperreflexia, Babinski sign, presence of seen with myasthenia gravis and is most likely
primitive reflexes (such as secondary to due to an autoimmune or paraneoplastic ori-
postinfectious encephalomyelitis, acquired gin.77 Electrophysiological study is used in di-
immunodeficiency dementia complex, agnosis and will show myokymic and neu-
cytomegalovirus encephalitis) romyotonic discharges.76 Muscle activity
4. Myelopathy associated with gait difficulties, persists throughout sleep and anesthesia, but
spasticity, ataxia, balance deficits, and hyper- can be blocked by curare.4 Symptomatic relief
reflexia (such as secondary to herpes zoster has been demonstrated with anticonvulsant
myelitis, vacuolar myelopathy that occurs drugs such as phenytoin and carbamazepine,
with acquired immunodeficiency syndrome and research has also shown successful out-
dementia complex) comes with plasmapharesis.78
5. Peripheral involvement associated with
sensory changes, weakness, balance deficits, ■ Neurosyphilis (Tabes Dorsalis,
and pain (such as secondary to peripheral Syphilitic Spinal Sclerosis,
neuropathy, acute and chronic inflamma- Progressive Locomotor Ataxia)
tory demyelinating polyneuropathies).1,75 Chief Clinical Characteristics
Abnormal neurological findings are ob- This presentation can be characterized by hemi-
served during a clinical examination in approx- paresis, ataxia, aphasia, gait instability, falls,
imately one-third of patients with acquired im- neuropathy, personality and cognitive changes,
munodeficiency syndrome; however, on seizures, diplopia, visual impairments, hearing
autopsy most individuals with this condition loss, psychotic disorders, loss of bowel/bladder
have abnormalities within the nervous system.1 function, pain, hyporeflexia, and hypotonia.79,80
Diagnosis of the variable neurological compli- Background Information
cations associated with this condition may be Treponema pallidum infects the human host by
confirmed with laboratory tests, cerebrospinal way of contact with contaminated body fluids
fluid cultures, imaging, nerve conduction stud- or lesions.79 This spirochete is responsible for
ies, and physical examination.1,26,75 Treatment the diagnosis of syphilis; however, when T. pal-
appears to be limited primarily to the use of an- lidum is present within the central nervous
tiviral medications.75 Physical and occupational system the individual is diagnosed with neu-
therapy may be indicated to address equipment rosyphilis.80 This condition occurs in approxi-
needs and caregiver/patient training related to mately 10% of individuals with untreated
functional mobility. syphilis, and in 81% of these cases it presents
■ Neuromyotonia (Isaac Syndrome, as meningovascular, meningeal, and general
Isaac-Merten Syndrome, paresis. Treatment includes use of various
Continuous Muscle Fiber Activity forms of penicillin or alternative choices for
Syndrome, Quantal Squander those allergic to penicillin79 and may involve
Syndrome) rehabilitative therapies depending on the indi-
vidual’s activity limitations or participation
Chief Clinical Characteristics restrictions. A better prognosis has been
This presentation commonly involves intermit- observed for individuals treated during early
tent or continuous muscle contraction, slow re- neurosyphilis.80
laxation following muscle contraction, cramps,
stiffness, and hyperhidrosis. These symptoms ■ Niemann-Pick Disease
are commonly seen in conjunction with periph- (Types C and D Only)
eral neuropathy.76,77 Chief Clinical Characteristics
Background Information This presentation includes ataxia, dystonia,
This condition is an antibody-mediated potas- vertical gaze palsy, dysarthria, and seizures.
sium channel disorder, in which the suppres- Often the presenting feature is a psychotic
sion of these voltage-gated channels results in episode.4,81
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624 Chapter 30 Normal Pressure Hydrocephalus

Background Information of individuals with this condition improve sig-


ABNORMAL MOVEMENT

This condition is an autosomal recessive lipid nificantly, and 30% will completely recover
storage disorder that results in impaired cho- and return to premorbid levels of function.
lesterol transport and excessive glycosphin- Others have residual gait, sphincteric, and
golipid storage.82 There are four types of cognitive deficits.84
Niemann-Pick disease; types A and B are seen
only in children and are not discussed here. ■ Opsoclonus Myoclonus
Types C and D have a variable age of onset (Kinsbourne Syndrome,
ranging from childhood to late teens or even Myoclonic Encephalopathy of
early adulthood. Individuals with types C and Infants, Dancing Eyes–Dancing
D demonstrate an enlarged spleen and liver, as Feet Syndrome, Opsoclonus-
well as progressive neurological dysfunction.81 Myoclonus-Ataxia Syndrome)
Diagnosis is made by a combination of studies, Chief Clinical Characteristics
including filipin staining of cultured fibrob- This presentation includes irregular, rapid eye
lasts, cholesterol esterification studies, and movements (opsoclonus) and brief, shock-like
DNA mutation analysis.81 Unfortunately, there muscle spasms (myoclonus), as well as stagger-
is no effective treatment for types C and D of ing, falling, ataxia, drooling, decreased muscle
this condition. Many patients who are diag- tone, and an inability to sleep.85
nosed in childhood live well into adult years,
and overall prognosis depends on the severity Background Information
of the disease.81 This autoimmune disorder is commonly
found in association with the presence of neo-
■ Normal Pressure Hydrocephalus plasm, the most common types being neurob-
Chief Clinical Characteristics lastoma in children, and breast and small-cell
This presentation is characterized by progres- lung cancer in adults.4 Diagnosis is made by a
sive difficulty in walking characterized by dimin- combination of clinical presentation, the pos-
ished cadence, widened base, short steps, and en sible presence of neoplasm, the presence of
bloc turning (requiring more than 3 steps to anti-Hu antibodies, mild pleocytosis on cere-
make a 90-degree turn). Movements involving brospinal fluid, and positive serologic tests in
the axial musculature appear awkward and children without tumors.4 Treatment includes
apraxic. Progression involves impairment in adrenocorticotropic hormone, corticosteroid,
mental function and sphincter incontinence. and intravenous immunoglobulin therapy,
and, when present, tumor resection and adju-
Background Information vant treatment such as chemotherapy and
Symptoms are caused by increased pressure on radiation.4,85 There is good response to drug
the brain, specifically the frontal lobe, due to and surgical treatment, but relapse is common
an abnormal increase in cerebrospinal fluid and many patients report residual neurologi-
secondary to trauma, infection, space-occupying cal symptoms.4,85
lesion, or unknown cause. It is most common
in the elderly, but can occur in people of any ■ Paraneoplastic Syndromes
age. Symptoms must be differentiated from
Chief Clinical Characteristics
disorders with similar presentation such as
This presentation includes dizziness in combi-
Alzheimer’s, Parkinson’s, and Creutzfeldt-
nation with a variety of different neurological
Jakob diseases.83 Diagnosis involves the clini-
symptoms and signs in an individual with can-
cal presentation combined with imaging to
cer. Specific neurological symptoms and signs
identify ventricular enlargement, intracranial
depend on the location of involvement of the
pressure monitoring, and neuropsychological
central or peripheral nervous system.
testing.83 Treatment entails shunt placement
to drain cerebrospinal fluid and regular Background Information
follow-up by a physician to monitor shunt Paraneoplastic encephalomyelitis and focal
function. Without treatment, symptoms con- encephalitis may present with ataxia, vertigo,
tinue to worsen. With shunt placement, 60% balance deficits, nystagmus, nausea, vomiting,
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Chapter 30 Paroxysmal Choreoathetosis 625

cranial nerve palsies, seizures, sensory neu- Background Information

ABNORMAL MOVEMENT
ropathy, anxiety, depression, cognitive changes, This condition occurs due to the depletion or
and hallucinations. For individuals presenting injury of dopamine-producing cells in sub-
with ataxia, dysarthria, dysphagia, and diplopia, stantia nigra pars compacta. Clinical signs and
paraneoplastic cerebellar degeneration may symptoms are not typically present until after
be suspected. Paraneoplastic opsoclonus/ approximately 80% of dopamine-producing
myoclonus tends to affect both children and cells are lost. The definitive diagnosis is made
adults with signs and symptoms including post-mortem. However, a clinically definitive
hypotonia, ataxia, irritability, truncal ataxia, diagnosis may be made with the presence of at
gait difficulty, balance deficits, and frequent least two of the three criteria—asymmetric
falls. Stiff-man syndrome presents with spasms resting tremor, bradykinesia, or rigidity—and
and fluctuating rigidity of axial musculature, a positive response to anti-Parkinson medica-
legs and possibly shoulders, upper extremities, tions.1,90 Imaging may be useful to exclude
and neck. Paraneoplastic sensory neuropathy vascular involvement. Medical management
presents with asymmetric, progressive sensory may include use of dopamine agonists, lev-
alterations involving the limbs, trunk, and odopa, and other medications to address
face, sensorineural hearing loss, autonomic nonmotor symptoms, such as depression, con-
dysfunction, and pain. Other conditions in stipation, autonomic symptoms, and sexual
this category include vasculitis, Lambert- dysfunction. Surgical management also may
Eaton myasthenia syndrome, myasthenia be considered, including deep brain stimula-
gravis, dermatomyositis, neuromyotonia, and tion or pallidotomy.90 Forced use or higher in-
various neuropathies.1,86 These conditions tensity, challenging activities may provide a
result from an immune-mediated response to neuroprotective benefit for individuals with
the presence of tumor or metastases. Antibod- early forms of this condition.91 With the high
ies or T-cells respond to the presence of the incidence of depression, consultation with a
tumor, but also attack normal cells of the psychologist or psychiatrist may be warranted.
nervous system.87,88 Over 60% of individuals Individuals with a late onset tend to progress
present with this condition prior to the discovery more rapidly.92,93 Poor prognostic indicators
of the cancer.86 The underlying tumor is treated for disability include initial presentation
according to the type of cancer. Additional without tremor, early dependence, dementia,
treatment is dependent on this condition’s balance impairments, older age, and the
type and may include steroids, plasmapheresis, postural instability/gait difficulty dominant
immunotherapy, chemotherapy, radiation, or type.92,93
cyclophosphamide.86 Physical, occupational,
and speech therapy may be indicated to ■ Paroxysmal Choreoathetosis
address functional limitations. Chief Clinical Characteristics
This presentation includes discrete episodes of
■ Parkinson’s Disease abnormal involuntary movements of the limbs,
trunk, and facial muscles. Some patients report
Chief Clinical Characteristics
lingering muscle stiffness following the attacks.94
This presentation commonly involves resting
The episodes of choreoathetosis may be pro-
tremor, bradykinesia, rigidity, and postural
voked by startle, sudden movement, hyperven-
instability. Falls are a common problem in indi-
tilation, alcohol, coffee, fatigue, or prolonged
viduals with this condition, with up to 68%
exercise. They vary in duration from 10 seconds
falling within a 1-year period and approximately
to up to 4 hours at a time, and may occur dozens
50% of these individuals falling multiple times
of times per day or only occasionally.4
within that same year.89 Other common signs and
symptoms include festination, freezing, micro- Background Information
graphia, hypophonia (hypokinetic dysarthria), The exact pathological mechanism of this con-
akinesia, masked facies, drooling, difficulty turn- dition is unknown, but it is thought that
ing over in bed, dystonia, dyskinesia, dementia, the disorder may be genetically linked, or due
and depression.1 to a secondary cause such as neurological or
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626 Chapter 30 Pelizaeus-Merzbacher Disease

metabolic disease.4,94 Individuals with this indicated to prevent immobility. This condi-
ABNORMAL MOVEMENT

condition tend to respond well to antiepileptic tion is not fatal and progression of symptoms
drugs and, overall, this condition has been varies; some individuals maintain ambulatory
shown to spontaneously improve as patients status throughout life while others become
move into adulthood.94 wheelchair-bound.
■ Pelizaeus-Merzbacher Disease ■ Progressive Multifocal
Chief Clinical Characteristics Leukoencephalopathy
This presentation commonly includes deterio- Chief Clinical Characteristics
ration of coordination, motor abilities, and This presentation commonly involves cortical
intellectual function. blindness, visual field defects, hemiparesis
with progression to quadriparesis, aphasia,
Background Information
ataxia, dysarthria, personality changes and im-
Severity and onset of the disease range widely,
paired intellect evolving over a period of days
depending on the type of genetic mutation,
to weeks.
and extend from the mild, adult-onset spastic
paraplegia to the severe form with onset at Background Information
infancy and death in early childhood.95 This This condition is most likely due to viral in-
condition is an X-linked disease caused by a fection of the central nervous system, which
mutation in the gene that controls the produc- then causes widespread demyelinative lesions
tion of a myelin protein called proteolipid primarily of the cerebral hemispheres. Diag-
protein.95 Genetic diagnostic testing is the nosis is made by computed tomography and
definitive method for diagnosing this condi- magnetic resonance imaging to localize the
tion.96 There is no cure for this condition. lesions. Treatment for individuals with ac-
Therefore, treatment is based on symptoms quired immunodeficiency syndrome consists
and includes physical therapy, orthotics, and of antiretroviral drug combinations and can
antispasticity agents with a goal of minimizing lead to slower progression or even remission.
the development of joint contractures, disloca- Currently, no treatment exists to impair dis-
tions, and kyphoscoliosis.97 Individuals with ease progression in individuals with this con-
severe forms of this condition experience dition but without acquired immunodefi-
progressive deterioration until death. Individuals ciency syndrome.1
with the adult-onset form with spasticity may
have a nearly normal life span.4 ■ Progressive Supranuclear Palsy
(Richardson-Steele-Olszewski
■ Primary Lateral Sclerosis Syndrome)
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes spastic paraparesis This presentation classically includes vertical gaze
of voluntary muscles with associated upper mo- palsy, prominent instability, and falls within
tor neuron signs in the absence of lower motor the first year of disease onset.98 Other charac-
neuron signs. Onset of difficulty with gait, bal- teristics may include rigidity, akinesia,
ance, and leg weakness appears in the fifth to dysarthria, dysphagia, and mild dementia. Falls
sixth decade of life and may progress to affect were found to be the most commonly reported
upper extremity and facial musculature. symptom with the majority of falls being
backwards falls.99 Difficulty with voluntary
Background Information
vertical eye movements (usually downward)
Imaging, cerebrospinal fluid analysis, and elec-
and involuntary saccades are relatively early
tromyographic studies confirm the diagnosis.
features. The disease generally progresses to the
In particular, this condition is differentiated
point at which all voluntary eye movements
from the more severe amyotrophic lateral scle-
are lost.
rosis only after a period of 3 years from onset
to ensure the absence of lower motor neuron Background Information
signs.1 Treatment for this condition addresses Some patients may not demonstrate difficul-
symptom management. Physical therapy is ties with ocular movements for 1 to 3 years
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Chapter 30 Tardive Dyskinesia 627

after disease onset. Most cases are sporadic; ■ Stroke (Cerebrovascular Accident)

ABNORMAL MOVEMENT
however, a pattern of inheritance compatible Chief Clinical Characteristics
with autosomal dominant transmission has This presentation may include a wide range of
been described.1 Diagnosis is based on clinical symptoms that correspond to specific areas of
presentation, which includes a gradually pro- the brain that are affected, potentially includ-
gressive disorder with age of onset at 40 years ing visual disturbances. The initial symptoms
or older, vertical supranuclear palsy, and pos- can include numbness or weakness, especially
tural instability with falls within the first year on one side of the body or face; confusion or apha-
of disease onset.100 Medical treatment is typi- sia; balance deficits or falls; or sudden severe
cally unsuccessful, because the majority of headache with no known cause.
patients are not responsive to the levodopa
therapy aimed at addressing the extrapyrami- Background Information
dal features of the disease.23 Physical and occu- This condition occurs when blood flow to
pational therapy are used to maintain mobility the brain is interrupted either by blockage
and address safety issues related to the pro- (ischemia or infarction) or from hemorrhagic
gression of imbalance. The disease course is disruption. A thrombosis or embolic occlusion
progressive with a mean survival time of 5.6 of an artery causes an ischemic type of this
years.99 Older age at disease onset, early onset condition. A hemorrhagic type of this condi-
of falls, incontinence, dysarthria, dysphagia, tion can be caused by arteriovenous malfor-
insertion of a percutaneous gastrostomy, and mation, hypertension, aneurysm, neoplasm,
diplopia have all been described as being pre- drug abuse, and trauma. This condition is the
dictive of shorter survival time.99 most common and disabling neurological dis-
order in adults and occurs in 114 of every
■ Spinocerebellar Ataxia 100,000 people.30 This condition is diagnosed
(Spinocerebellar Atrophy, using clinical presentation and positive find-
Spinocerebellar Degeneration) ings on computed tomography and magnetic
Chief Clinical Characteristics resonance imaging. Medication, surgery,
This presentation can be characterized by ataxia, and interdisciplinary therapy are the most
incoordination, supranuclear ophthalmoplegia, common treatments for this condition. The
slow saccades, optic atrophy, dysarthria, bal- prognosis for recovery is predicted by the mag-
ance deficits, falls, tremor, myoclonus, chorea, nitude of initial deficit. Factors that are associ-
nystagmus, dementia, amyotrophy, and periph- ated with poor outcomes include coma, poor
eral neuropathy.101,102 cognition, severe aphasia, severe hemiparesis
with little return within 1 month, visual per-
Background Information ceptual disorders, depression, and inconti-
This condition refers to a group of progres- nence after 2 weeks.103,104
sive, neurodegenerative, autosomal domi-
nant disorders affecting the cerebellum, ■ Tardive Dyskinesia
brainstem, and spinal cord. Twelve variants Chief Clinical Characteristics
have been identified including SCA 1, 2, 3, 6, This presentation includes choreoathetoid dysk-
7, 8, 10, 12, 14, 17, FGF10-SCA, and denta- inesia, which can occur in the face, trunk, and
torubral-pallidoluysian atrophy.102 The dis- limbs. These dyskinesias have been described as
orders occur due to expansions of CAG repetitive, stereotyped, rhythmic movements,
triplet repeats that are subsequently tran- and are most commonly seen in the mouth or
scribed into long polyglutamine tracts. Diag- tongue.4
nosis is confirmed by deoxyribonucleic acid
testing. There is no current medical treat- Background Information
ment for the various forms of this condi- These symptoms develop as a side effect of
tion.101,102 Individuals with this condition exposure to or withdrawal from neuroleptic-
may benefit from physical, occupational, and antipsychotic drugs. This condition develops
speech therapy to address activity limitations in 20% of patients treated with neuroleptic
and participation restrictions. medications, with a higher risk in the elderly
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628 Chapter 30 Traumatic Brain Injury

population.105 Excess dopamine is thought to with a primary tumor; however, a small


ABNORMAL MOVEMENT

play a role in etiology. Diagnosis is based on percentage of individuals are diagnosed


clinical examination and history of prior treat- concomitantly with brain metastases and the
ment with neuroleptic medications. No treat- primary tumor. The most common cancers
ment for this condition has been proven to be resulting in subsequent brain metastases in-
effective and the best current treatment is pre- clude lung, breast, melanoma, colorectal, and
vention.106,107 Improvement or resolution of the genitourinary tract. The new onset of neuro-
symptoms is more favorable when there is early logical symptoms after a primary tumor war-
detection, lower drug exposure, use of atypical rants the use of imaging such as magnetic
instead of typical antipsychotic medications, resonance imaging or computed tomography
younger age, and longer follow-up.105,108 to confirm the diagnosis. Treatment may
include corticosteroids, brain irradiation, sur-
■ Traumatic Brain Injury gery, chemotherapy, radiotherapy, and reha-
Chief Clinical Characteristics bilitative therapies. The prognosis is poor with
This presentation typically includes disequilibrium death typically occurring within 6 months.
in the presence of cognitive changes, altered level
of consciousness, seizures, nausea, vomiting, ■ Brain Primary Tumors
coma, dizziness, headache, pupillary changes, Chief Clinical Characteristics
tinnitus, weakness, incoordination, behavioral This presentation may include headaches,
changes, spasticity, hypertonicity, cranial nerve seizures, dysphagia, weakness, cognitive
lesions, and sensory and motor deficits.1,109 changes, behavioral changes, dizziness, vomit-
ing, alterations in the level of consciousness,
Background Information
ataxia, aphasia, nystagmus, visual disturbances,
This condition can be classified as mild, mod-
dysarthria, balance deficits, falls, lethargy, and
erate, or severe based on Glasgow Coma Scale,
incoordination.1,26
length of coma, and duration of post-traumatic
amnesia.109 Magnetic resonance imaging may Background Information
be used to confirm the diagnosis.26 Treatment Glioblastoma multiforme, astrocytoma,
initiated at the scene of the accident and during oligodendroglioma, metastatic tumors, pri-
the acute phase is focused on medical stabiliza- mary central nervous system lymphomas,
tion. It should be initiated during the acute ganglioglioma, neuroblastoma, menin-
phase in order to minimize complications.110 gioma, arachnoid cysts, hemangioblastoma,
Low Glasgow Coma Scale, longer length of medulloblastoma, and acoustic neuroma/
coma, longer duration of post-traumatic am- schwannoma are some of the more common
nesia, and older age tend to be associated with brain tumors. Specific diagnoses for brain
poor outcomes.111 Optimal rehabilitation is in- tumors may be confirmed with imaging and
terdisciplinary and customized to address the biopsy. Treatment is variable depending on
specific individuals’ needs the type, size, and location of the tumor and
may include surgical resection, chemother-
TUMORS apy, radiation, corticosteroids, and rehabili-
■ Brain Metastases tative therapies. Prognosis is also variable
and depends on the type and grade of tumor,
Chief Clinical Characteristics severity of compression, and duration of
This presentation may include headaches, compression.
seizures, dysphagia, weakness, cognitive changes,
behavioral changes, dizziness, vomiting, alter- ■ Spinal Metastases
ations in the level of consciousness, ataxia,
Chief Clinical Characteristics
aphasia, nystagmus, visual disturbances,
This presentation can involve spasticity, weak-
dysarthria, balance deficits, falls, lethargy, and
ness, sensory alterations, bowel and bladder
incoordination.1,112
incontinence, neck pain, back pain, radicular
Background Information pain, atrophy, cerebellar signs, balance
The majority of individuals with brain deficits, falls, and cranial nerve involve-
metastases have been previously diagnosed ment.1,26,113
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Chapter 30 Whipple’s Disease (Intestinal Lipodystrophy) 629

Background Information ■ Vasculitis (Giant Cell Arteritis,

ABNORMAL MOVEMENT
This condition is the most frequent neo- Temporal Arteritis, Cranial
plasm involving the spine.113 The most com- Arteritis)
mon types and locations of primary tumors Chief Clinical Characteristics
that result in spinal metastases include This presentation can be characterized by
breast, lung, lymphoma, prostate, kidney, headaches, psychiatric syndromes, dementia,
gastrointestinal tract, and thyroid.1,114 The peripheral or cranial nerve involvement, pain,
diagnosis is confirmed with gadolinium en- seizures, hypertension, hemiparesis, balance
hanced magnetic resonance imaging and deficits, neuropathies, myopathies, organ
computed tomography.1,113 Treatment is involvement, fever, and weight loss.1,26,115
variable depending on the tumor and may
include surgical resection, chemotherapy, ra- Background Information
diation, corticosteroids, and rehabilitative This condition is the result of an immune-
therapies.1 Although the long-term progno- mediated response resulting in the inflamma-
sis is poor, individuals without paresis and tion of vascular structures.1,115 It includes
pain and who are still ambulatory have a variety of disorders such as giant cell/
longer survival rates.114 temporal arteritis (which is the most common
form), primary angiitis of the central nervous
■ Spinal Primary Tumors system, Takayasu’s disease, periarteritis no-
Chief Clinical Characteristics dosa, Kawasaki disease, Churg-Strauss syn-
This presentation may include spasticity, drome, Wegener’s granulomatosis, and sec-
weakness, sensory alterations, bowel/bladder ondary vasculitis associated with systemic
incontinence, back pain, radicular pain, at- lupus erythematous, rheumatoid arthritis,
rophy, cerebellar signs, balance deficits, falls, and scleroderma.115 The diagnosis is con-
and cranial nerve involvement.1 firmed through history, physical examination,
laboratory testing, angiography, biopsy, and
Background Information
imaging.1,26,115 Corticosteroids, cytotoxic
Types of this condition include astrocy-
agents, intravenous immunoglobulin, and
tomas, ependymomas, hemangioblastoma,
plasmapheresis may be used in the treatment
myeloma, neurofibroma, lymphoma, metas-
of vasculitis.26,115 Prognosis is variable and
tasis, meningioma, schwannoma, and
depends on the specific underlying disorder.
astrocytoma. Extradural tumors, such as
For example, giant cell arteritis is typically self-
meningiomas, produce a rapid onset of
limiting within 1 to 2 years; however, death
symptoms, with weakness being predomi-
usually occurs within 1 year for individuals
nant. Intramedullary tumors, or ependymo-
with primary angiitis of the central nervous
mas, astrocytomas and hemangioblastomas,
system.115
present with slowly progressive symptoms,
of which loss of pain and temperature ■ Whipple’s Disease (Intestinal
sensation is usually the first. The first test to Lipodystrophy)
diagnose brain and spinal column tumors is
Chief Clinical Characteristics
a neurological examination. Special imaging
This presentation includes dementia, personal-
techniques (computed tomography, mag-
ity changes, ataxia, myoclonus, nystagmus, and
netic resonance imaging, and positron emis-
visual loss. The presentation also includes
sion tomography) are also employed. Spe-
weight loss, arthropathy, diarrhea, and abdom-
cific diagnoses may be confirmed with
inal pain. Some individuals may experience a
imaging and biopsy. Treatment is variable
low-grade fever, lymphadenopathy, hyperpig-
depending on the type, size, and location of
mentation, hypotension, peripheral edema, car-
the tumor and may include surgical resec-
diac murmurs, occult bleeding, and myalgia.116
tion, chemotherapy, radiation, corticos-
teroids, and rehabilitative therapies. Progno- Background Information
sis is variable and depends on the type and This systemic disease is rare and is thought to
grade of tumor, severity of compression, and be caused by the bacteria Tropheryma whip-
duration of compression. plei.116 Diagnosis is determined by biopsy of
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630 Chapter 30 Wilson’s Disease

the duodenum, cerebrospinal fluid, cardiac- 4. Ropper AH, Brown RJ. Adams and Victor’s Principles of
ABNORMAL MOVEMENT

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CHAPTER31
Problems of Cognition, Communication,
and Behavior
■ Julie Hershberg, PT, DPT, NCS

Description of the Symptom Frontal Motor cortex Parietal


lobe lobe
This chapter describes pathology that may lead
to problems with cognition, communication,
and behavior. These types of problems are of-
ten observed together with central nervous
system pathology. Disturbances of cognition
include difficulty with problem solving and
making sound judgments, organizing infor-
mation, and memory. Cognitive problems can
range from difficulty attending to a task to be-
ing in a comatose state.

Temporal Occipital
lobe lobe

Functions of Selected Brain Structures


BRAIN STRUCTURE FUNCTION(S)
Frontal lobe Initiation, judgment, memory, impulse control,
Broca’s area (left hemisphere) sequencing, social behavior
Expressive language
Parietal lobe Sensory integration, awareness of body image and
environment
Temporal lobe Long-term memory, auditory processing
Wernicke’s area (left hemisphere) Processing of speech
Occipital lobe Visual field, color discrimination
Motor cortex (motor homunculus) Contralateral control of movement
Somatosensory cortex (sensory homunculus) Contralateral perception of sensation
Visual cortex Contralateral perception of vision
Auditory cortex Bilateral reception of auditory input

Special Concerns bacterial infection that have not yet been


■ Problems with cognition, communication, or discussed with a physician
behavior of sudden onset or rapid progression ■ Problems with behavior that cause poten-
■ Any problems that occur after trauma or tial harm to the patient or others
are present during or soon after a viral or

634
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Chapter 31 Problems of Cognition, Communication, and Behavior 635

CHAPTER PREVIEW: Conditions That May Lead to Problems with Cognition,

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


Communication, and Behavior

T Trauma
COMMON
Epilepsy/seizure disorder 645
Traumatic brain injury 658
UNCOMMON
Hypoxia (cerebral hypoxia, anoxia) 648
RARE
Not applicable

I Inflammation
COMMON
Aseptic
Multiple sclerosis 650
Septic
Encephalitis 644
Encephalopathy 645
Meningitis:
• Bacterial meningitis 649
• Viral meningitis 649
UNCOMMON
Aseptic
Systemic lupus erythematosus 657
Septic
Acute disseminated encephalomyelitis 638
Cysticercosis 643
Lyme disease (tick paralysis) 649
Neurological complications of acquired immunodeficiency syndrome 652
Reye’s syndrome 655
Rickettsial diseases, including Rocky Mountain spotted fever 656
RARE
Aseptic
Behçet’s disease 640
Paraneoplastic syndromes 653
Septic
Klüver-Bucy syndrome 648
Malaria 649
Neurosyphilis (tabes dorsalis, syphilitic spinal sclerosis, progressive locomotor ataxia) 652
Progressive multifocal leukoencephalopathy 655
Subacute sclerosing panencephalitis (Dawson’s disease) 657
Whipple’s disease (intestinal lipodystrophy) 660
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636 Chapter 31 Problems of Cognition, Communication, and Behavior


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

M Metabolic
COMMON
Cushing’s syndrome (hypercortisolism) 643
Neurotoxicity 653
UNCOMMON
Not applicable
RARE
Cerebral beriberi (Korsakoff’s amnesic syndrome, Wernicke-Korsakoff syndrome) 641
Mitochondrial myopathies 650

Va Vascular
COMMON
Stroke (cerebrovascular accident) 657
Transient ischemic attack 658
UNCOMMON
Arteriovenous malformation 639
Cerebral aneurysm 641
Multi-infarct dementia/vascular dementia 650
RARE
Cavernous malformation 641
Gerstmann’s syndrome 646
Moyamoya disease 650

De Degenerative
COMMON
Alzheimer’s disease 638
Dementia with Lewy bodies 643
Parkinson’s disease 654
UNCOMMON
Binswanger’s disease (subcortical arteriosclerotic encephalopathy, subcortical dementia) 640
Corticobasal degeneration 642
Frontotemporal dementia 646
Progressive supranuclear palsy (Richardson-Steele-Olszewski syndrome) 655
RARE
Creutzfeldt-Jakob disease 643
Multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy, Shy-Drager syndrome) 651
Pick’s disease 655

Tu Tumor
COMMON
Malignant Primary:
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Neurofibromatosis 651
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Chapter 31 Problems of Cognition, Communication, and Behavior 637

Tumor (continued)

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


UNCOMMON
Malignant Primary, such as:
• Brain primary tumors 659
Malignant Metastatic, such as:
• Brain metastases 659
Benign:
Not applicable
RARE
Malignant Primary, such as:
• Spinal primary tumors 659
Malignant Metastatic, such as:
• Spinal metastases 659
Benign:
Not applicable

Co Congenital
COMMON
Attention deficit hyperactivity disorder 639
Dyslexia (developmental reading disorder) 644
Huntington’s disease 647
UNCOMMON
Choreoacanthocytosis (Levine-Critchley syndrome, neuroacanthocytosis) 642
RARE
Fahr’s syndrome (familial idiopathic basal ganglia calcification, bilateral striopallidodentate calcinosis) 645
Gaucher’s disease 646
Hallervorden-Spatz syndrome (neurodegeneration with brain iron accumulation, pantothenate
kinase-associated neurodegeneration) 647
Hereditary spastic paraplegia (familial spastic paralysis) 647
Niemann-Pick disease (types C and D only) 653
Pelizaeus-Merzbacher disease 654
Tuberous sclerosis 658
Wilson’s disease 660

Ne Neurogenic/Psychogenic
COMMON
Anxiety disorder/panic attacks 639
Bipolar disorder (manic depression) 640
Depression 644
Hydrocephalus 647
Sleep apnea 656
UNCOMMON
Paranoia, delusional disorder 654
Schizophrenia 656
(continued)
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638 Chapter 31 Acute Disseminated Encephalomyelitis

Neurogenic/Psychogenic (continued)
PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

RARE
Conversion disorder 642
Hypersomnia 648
Narcolepsy 651
Tourette’s syndrome 658

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Problems with Common problems with behavior or mood


Cognition, Communication, and include anxiety, depression, and impaired con-
Behavior trol of emotion.2 A patient or family member
may report the patient has decreased initiative,
Though often not the primary symptoms lead- personality changes, emotional lability, re-
ing to a physical therapy visit, problems with duced range of mental activity, aggression, or
cognition, communication, and behavior often social inappropriateness.3
are the first indicators of neurological insult and
are thus important in the differential diagnosis Description of Conditions That May
process. Problems with cognition, communica- Lead to Problems with Cognition,
tion, and behavior invariably are caused by Communication, and Behavior
cerebral cortex pathology. Symptoms are dis-
tinctly related to the part of the cortex involved ■ Acute Disseminated
and assist in the differential diagnosis process. If Encephalomyelitis
the frontal lobes are involved, prevalent signs and
Chief Clinical Characteristics
symptoms include hemiparesis, apathy, alter-
This presentation may involve confusion, som-
ations in personality and behavior, inability to
nolence, and convulsion in its encephalitic form.
sequence motor tasks, perseveration, inattention,
Initial symptoms of the myelitic form include
abulia, Broca’s aphasia, and prominent grasp and
weakness and sensory impairments.2
suck reflexes. Lesions in the temporal cortex may
cause memory and learning impairments and le- Background Information
sions on the left side also often cause Wernicke’s This condition is a demyelinating disease of the
aphasia and visual agnosia. Parietal lobe dysfunc- central nervous system,4 which may be due to
tion includes hemisensory loss, agnosia, visual- an immune-mediated complication of infec-
perceptual disorders, and neglect.1 tion.2 The presence of upper motor neuron
Problems with communication include signs, cerebrospinal fluid pleocytosis and ele-
difficulties with comprehension or production vated protein, and multiple white matter lesions
of spoken or written language. Receptive or demonstrated on magnetic resonance imaging
Wernicke’s aphasia denotes a difficulty with supports the diagnosis.5 Definitive diagnosis
comprehension characterized by frequent requires a brain biopsy. The primary goal of
word substitutions, impaired repetition and treatment is to suppress the immune response;
naming, and excessive talking. Expressive or thus high-dose corticosteroids are generally
Broca’s aphasia is used to describe difficulty administered over a course of 3 to 5 days.5 The
with conveying thoughts or ideas through prognosis is variable depending on the severity
words. Global aphasia results from damage to of the disease and acuity of the diagnosis.
all language areas of the brain and includes
impairments in both comprehension and ex- ■ Alzheimer’s Disease
pression. The most common cause of aphasia Chief Clinical Characteristics
is a stroke. It can also result from traumatic This presentation includes progressive mem-
brain injury, brain tumor, or any other insult ory loss, language deterioration, poor judgment,
affecting the language areas of the brain.2 confusion, restlessness, and mood swings.
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Chapter 31 Attention Deficit Hyperactivity Disorder 639

Background Information cognitive-behavioral therapy, relaxation ex-

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


This condition is the most common neu- ercises and pharmacologic treatment.10 The
rodegenerative disorder that causes cognitive course of this condition is variable; most in-
decline in persons over the age of 60 years6 dividuals maintain normal social lives. Clini-
with prevalence rates from 3% to nearly 50% cians are encouraged to consider referral of
in persons between ages 65 and 85 years.7 individuals suspected of having this condi-
The anatomical pathology of this condition tion to a mental health specialist for evalua-
is characterized by progressive accumulation tion and treatment.
of amyloid plaques and neurofibrillary tan-
gles in the cortex that lead to destruction of ■ Arteriovenous Malformation
nervous tissue.2 Diagnosis is made based on Chief Clinical Characteristics
clinical presentation and cognitive testing. This presentation may be characterized by
There is no cure for this condition at this seizures and severe headache. Hemorrhage may
time and medical treatment is largely symp- result in paresis, ataxia, dyspraxia, dizziness,
tomatic using psychotropic medications and tactile and proprioceptive disturbances, visual
behavioral interventions; cholinesterase in- disturbances, aphasia, paresthesias, and cog-
hibitors for early stages of the disease; and nitive deficits.2
N-methyl-D-aspartate antagonists for later
stages of the disease. This condition is a Background Information
progressive disease, but its course can vary This condition is caused by a tangle of arteries
from 5 to 20 years.8 Most deaths result from and veins that cause abnormal communica-
respiratory or cardiovascular compromise.2 tion within the vasculature and approximately
12% of the 300,000 individuals in the United
■ Anxiety Disorder/Panic Attacks States with this condition are symptomatic.
Chief Clinical Characteristics This condition is caused by a developmental
This presentation typically includes repeated abnormality that likely arises during embry-
panic or anxiety attacks. Symptoms include onic or fetal development. Neurological dam-
increased heart rate and respiratory rate, pupil age occurs due to reduction of oxygen delivery,
dilation, and trembling and sweating with feel- hemorrhage, or compression of nearby struc-
ings of fear and dread. The symptoms usually tures of the brain or spinal cord. Computed
subside in 15 to 30 minutes. tomography, magnetic resonance imaging,
and arteriography confirm the diagnosis. Liga-
Background Information tion and embolization may be used to reduce
This condition is a nonspecific syndrome the size of the lesion prior to surgical excision,
and can be due to a variety of medical or psy- which is the preferred method of treatment.
chiatric syndromes or observed as part of a Stereotactic radiation and proton beam ther-
drug withdrawal or drug intoxication effect. apy are alternative approaches to invasive
The diagnosis of an anxiety condition is methods of intervention. Up to 90% of indi-
based on criteria from the DSM-IV-TR.9 viduals who experience a hemorrhagic arteri-
Anxiety conditions are classified into specific ovenous malformation survive.2
categories. One category, panic condition,
has dizziness as one of its features. Panic con- ■ Attention Deficit Hyperactivity
dition is defined by recurrent attacks with at Disorder
least four of the following features: increased
Chief Clinical Characteristics
heart rate, sweating, trembling or shaking,
This presentation includes distractibility,
dyspnea, sensation of choking, chest pain or
forgetfulness, restlessness, and impulsivity.11
discomfort, nausea or abdominal distress,
feelings of dizziness, fear of losing control, Background Information
fear of dying, paresthesias, and chills or hot Diagnosis is based on DSM-IV criteria12 and a
flashes. The etiology of anxiety conditions thorough patient and family history. DSM-
includes genetic factors, social and psycho- IV-TR criteria include having inattention and
logical factors, and physiological and bio- hyperactivity-impulsivity symptoms for at
chemical abnormalities.10 Treatment includes least 6 months; symptoms present in at least
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640 Chapter 31 Behçet’s Disease

two settings; some symptoms present before ■ Binswanger’s Disease (Subcortical


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

age 7; and clinically significant impairment in Arteriosclerotic Encephalopathy,


education, work, or socially. This condition is Subcortical Dementia)
prevalent in 1.7% to 16% of the pediatric pop- Chief Clinical Characteristics
ulation.13 The etiology of this condition in- This presentation involves small-stepped gait,
volves genetic and environmental factors. slowed motor function with perseveration,
Several genes appear to be associated because deficits in executive function, slow informa-
of their affect on dopamine receptors, tion processing, and impaired memory. Other
dopamine transport, and dopamine beta- symptoms include dysarthria, dysphagia, uri-
hydroxylase. Environmental factors may also nary disturbances, and lateral homonymous
play a role, including pregnancy and delivery hemianopias.15–17
problems, family dysfunction, and poorer
socioeconomic status. Treatment includes Background Information
medication and behavioral intervention. This This condition is a type of vascular dementia
condition can be well controlled with treat- that results from multiple strokes and demyeli-
ment and the prognosis is excellent for indi- nation of the central white matter.15,17 Diagno-
viduals who have no major comorbidities, sis is made by neuroimaging, specifically com-
have individualized medication management, puted tomography and magnetic resonance
have received family education regarding the imaging.15 Medical management includes
diagnosis and treatment, and whose associated drug therapy targeted at improving core
learning disorders or emotional problems have symptoms and delaying disease progression,
been addressed.13 as well as secondary prevention of stroke by
decreasing hypertension.16,18
■ Behçet’s Disease
Chief Clinical Characteristics ■ Bipolar Disorder (Manic
This presentation may include bilateral Depression)
pyramidal sign (signs related to lesions of Chief Clinical Characteristics
upper motor neurons or descending pyramidal This presentation typically involves episodes
tracts, such as a positive Babinski sign or of clinical depression and episodes of mania. The
hyperreflexia), headache, memory loss, hemi- episodes of mania involve flight of ideas, hyper-
paresis, cerebellar ataxia, balance deficits, activity, increased appetite, and poor judgment.
sphincter dysfunction, or cranial nerve palsies.
In addition to these neurological signs indi- Background Information
viduals with this condition also may present Diagnosis is based on criteria from the DSM-
with arthritis; renal, gastrointestinal, vascular, IV-TR.12 Manic episodes are characterized as
and cardiac diseases; and genital, oral, and having at least 1 week of profound mood
cutaneous ulcerations.14 disturbance with elation, irritability, or expan-
siveness. In addition, three or more of the
Background Information following symptoms are present: grandiosity;
Mean age of onset is in the third decade of diminished need for sleep; racing thoughts or
life. Diagnostic criteria according to an inter- flight of ideas; distractibility; increased level of
national study group include presence of goal-focused activity at home, at work, or sex-
recurrent oral ulceration, recurrent genital ually; and excessive pleasurable activities, often
ulceration, eye lesions, skin lesions, papulo- with painful consequences. The disturbance in
pustular lesions, and/or a positive pathergy mood must be sufficient to cause impairment
test.2,14 Medical treatment typically consists at work or danger to the patients or others.
of corticosteroids and immunosuppressants. Estimates of prevalence range from 1% to
Neurological symptoms tend to clear within 1.6% of the population in the United States.19
weeks, but can sometimes recur or result The cause of this condition is incompletely
in permanent deficits.2 Onset before the understood, although it is acknowledged that
age of 25 and male sex indicate a poorer genetic and environmental factors are respon-
prognosis. sible. Pharmacologic treatment is the mainstay
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Chapter 31 Cerebral Beriberi 641

of managing bipolar disorder. This condition been found to be correlated with increased risk

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


is associated with 80% to 90% relapse and re- of aneurysm development.22,23 Factors associ-
currence rates.19 Poor prognosis is associated ated with increased risk of rupture include size
with high degrees of neuroticism, long dura- of aneurysm, location in the posterior circula-
tion before treatment, positive family history, tion, and a previous history of aneurismal sub-
and the presence of depression-provoking cir- arachnoid hemorrhage.24,25 Definitive diagno-
cumstances.2 Suicide is a risk for all patients sis is based on catheter angiography; however,
with bipolar disorder with approximately 25% magnetic resonance angiography, magnetic
of patients attempting suicide and 11% of pa- resonance imaging, and computed tomogra-
tients dying by suicide.19 phy may aid in the diagnosis. Unruptured
aneurysms are sometimes surgically treated.
■ Cavernous Malformation Aneurysm size, location, and prior history of a
Chief Clinical Characteristics subarachnoid hemorrhage help to determine if
This presentation includes symptoms that are the risk of surgical treatment is worth the po-
dependent on the neuroanatomical location af- tential benefits. Most aneurysms that have
fected. Symptoms often consist of paresthesias, hemorrhaged must be treated surgically.
mood and cognitive changes, visual distur- Patients with a previous rupture are at an
bances, headaches, and seizures. 11 times greater risk of having a second in-
tracranial aneurysm rupture. When aneurysms
Background Information do rupture, many patients die within one
This condition is a rare disorder of the vascular month of the rupture, and those who survive
system of the brain where a blood-filled mass, often have residual neurological deficits.22
or hemangioma, forms. This condition is
frequently inherited in an autosomal dominant ■ Cerebral Beriberi (Korsakoff ’s
pattern. Diagnosis is made on clinical manifes- Amnesic Syndrome,
tations and magnetic resonance imaging find- Wernicke-Korsakoff Syndrome)
ings of clusters of vessels with a rim of hyp Chief Clinical Characteristics
density on T1-weighted images.2 For individu- This presentation involves ophthalmoparesis,
als with this condition who experience neuro- nystagmus, ataxia, and confusion, as well
logical symptoms, treatment is symptomatic as impaired learning and memory. Other com-
and supportive. Surgical removal or radiation mon symptoms include peripheral neuropathy,
may be performed.2 Individuals with prior postural hypotension, syncope, impaired olfac-
hemorrhage and infratentorial location of the tory discrimination, mild hypothermia, and
hemangioma have a poorer prognosis.21 confabulation.17
■ Cerebral Aneurysm Background Information
Chief Clinical Characteristics This condition is due to a thiamine deficiency
This presentation may involve mood and that results in a diffuse decrease in cerebral
cognitive changes in combination with other glucose utilization. It is most commonly ob-
neurological symptoms and signs that depend served in individuals who abuse alcohol and
on the affected cerebral tissue, including visual have nutritional deficiencies, although it is not
and proprioceptive loss. Any associated signs limited to this population.17 Diagnosis can be
or symptoms may not be reported due to the fact made by blood tests to examine thiamine
that this condition is typically asymptomatic levels. Neuroimaging may show slowed brain
prior to rupture. However, if the aneurysm activity as well as lesions in the medial thala-
results in a mass effect, ischemia, or hemor- mus and periaqueductal region.17 Medical
rhage, then neurological signs and symptoms are treatment involves the immediate administra-
dependent on the affected location.2,22,23 tion of thiamine. Once thiamine has been
administered, the reversal of symptoms should
Background Information begin to occur within hours to days with vari-
There has been some description of genetic able degrees of recovery. Memory has been
factors in this condition.22 Cigarette smoking, shown to have the poorest return, and mortal-
hypertension, and heavy alcohol use have all ity rates of up to 17% have been reported.17
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642 Chapter 31 Choreoacanthocytosis (Levine-Critchley Syndrome, Neuroacanthocytosis)

■ Choreoacanthocytosis event. Differential diagnosis is based on clinical


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

(Levine-Critchley Syndrome, findings. It is helpful to treat the patient as


Neuroacanthocytosis) though he or she has had an illness and is now
Chief Clinical Characteristics in the process of recovery.2 Cognitive and be-
This presentation commonly includes chorea, havioral therapies may be effective in treating
motor or vocal tics, dystonia, orofacial dyskine- underlying psychological issues. Prognosis is
sias, and parkinsonism. Seizures, cognitive im- variable with differing degrees of recovery in
pairment, psychosis, paranoia, and personality days to months. Good prognostic factors in-
changes are also seen with this diagnosis, along clude acute onset of symptoms, short duration
with hyporeflexia and distal myopathy due to of symptoms, healthy premorbid functioning,
denervation of muscles.26–28 higher intelligence, absence of coexisting psy-
chopathology, and presence of an identifiable
Background Information stressor. Poor prognostic symptoms include
This condition is a rare, autosomal recessive pseudoseizures, age greater than 40, and long-
disorder that typically has its onset during the lasting severe disability.29
third and fourth decades of life.28 This neu-
rodegenerative disorder is associated with ■ Corticobasal Degeneration
acanthocytes, aberrant spiky or thorny red Chief Clinical Characteristics
blood cells, as well as atrophy and gliosis of This presentation typically includes limb
the caudate, putamen, and globus pallidus.27 ideomotor apraxia and unilateral parkinson-
Diagnosis is made by a combination of tests, ism that is unresponsive to levodopa, gait
including clinical features, lab work demon- disturbances, tremor, postural instability, and
strating acanthocytosis, neuroimaging, and dementia.30,31
genetic testing to rule out Huntington’s dis-
Background Information
ease.26 There is currently no effective, long-
A proposed set of criteria for the diagnosis of
term treatment, although verapamil has been
this condition includes core features of:
found to provide temporary reduction of
symptoms. Life expectancy is reduced, and 1. Insidious onset and progressive course
suicidal action or ideation is not uncommon 2. No identifiable cause
due to cognitive impairments.28 3. Cortical dysfunction (ideomotor apraxia,
alien-limb phenomenon, cortical sensory
■ Conversion Disorder loss, visual or sensory hemineglect, construc-
Chief Clinical Characteristics tional apraxia, focal or asymmetric my-
This presentation can be characterized by oclonus, apraxia of speech or nonfluent
motor or sensory deficits, seizures or convul- aphasia), and
sions, or blindness or deafness. Symptoms 4. Extrapyramidal dysfunction (rigidity which
usually appear suddenly and abate in less than does not respond to levodopa therapy and
2 weeks. dystonia).32
Background Information This condition is a sporadic disease with
This condition is one type of somatoform disor- an average age of onset of 63 years.31 There
der in which psychological stress becomes trans- have been reports of familial cases; however,
lated into physical problems. The DSM-IV-TR for most cases there is no known cause.30 Diag-
defines conversion disorder by symptoms that nosis is made based on clinical presentation.
simulate a neurological or other medical condi- A definitive diagnosis can only be made post-
tion that involves voluntary muscles or sensory mortem. Medical treatment is not typically
organs excluding pain and sexual functions.2 successful; it has been found that only about
Clinical findings include patterns of sensory loss 24% of these patients will respond to levodopa
that do not follow normal patterns from neuro- therapy aimed at addressing the extrapyrami-
logical insults and symptoms that disappear dal features of the disease.33 Physical and occu-
when a patient is distracted or thinks that no pational therapy are used to maintain mobility
one is watching. Patients often have a history of and address safety issues related to the pro-
emotional disturbance, stress, or traumatic gression of imbalance. Mean survival is
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Chapter 31 Dementia with Lewy Bodies 643

7.9 years with a range of 2.5 to 12.5 years.31 Women with this condition may show an in-

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


Early presence of bilateral parkinsonism or creased growth of facial and body hair and
frontal lobe signs indicates a less favorable irregularity of menstruation.
prognosis.31
Background Information
■ Creutzfeldt-Jakob Disease This rare endocrine disorder is caused by chronic
Chief Clinical Characteristics exposure of the body’s tissues to excess levels of
This presentation may involve rapidly progres- cortisol due to medications or tumors of the pi-
sive dementia, cerebellar ataxia, balance tuitary or adrenal gland. Diagnosis is confirmed
deficits, myoclonus, cortical blindness, pyram- by laboratory evidence of excessive cortisol in the
idal signs, extrapyramidal signs, and akinetic urine and blood.36,37 Treatment is achieved
mutism.34 through decreasing cortisol levels via medica-
tion, surgery, or radiation. Most individuals with
Background Information this condition show significant improvement in
Different forms of this condition have been de- symptom presentation with treatment. However,
scribed including sporadic, iatrogenic, and in some individuals, residual symptoms may
variant. Sporadic and iatrogenic forms of this persist and some kinds of tumors may recur.
condition typically affect older individuals;
whereas the variant form affects younger indi- ■ Cysticercosis
viduals.35 The early stages of the variant form Chief Clinical Characteristics
are characterized by psychiatric symptoms, in- This presentation commonly involves seizures,
cluding depression and anxiety.35 The condi- headache, motor deficits, and psychiatric symp-
tion is rare and affects only one to two people toms.38 In children headache and vomiting are
per million worldwide per year.34,35 It is caused seen in about a third of cases.39
by a conformational change of the normal
prion protein which is encoded by human Background Information
chromosome 20 to a disease-related prion pro- Cysticercosis is more prevalent in immigrants
tein. Diagnosis is suggested by a thorough in California, New Mexico, and Texas than
history and physical examination, electroen- elsewhere in the United States. This condition
cephalography, and cerebrospinal fluid analy- is the most frequent parasitosis of the central
sis.35 Computed tomography and magnetic nervous system and occurs when a person in-
resonance imaging are typically normal in spo- gests Taenia solium eggs by fecal-oral contam-
radic and iatrogenic forms and help to exclude ination. The larvae become established as
other diagnoses.1,34 Diagnosis for all forms of viable cysts, which are eventually destroyed by
this condition is only confirmed postmortem.1 cellular response and later appear as a residual
There is no proven treatment.1,35 Death in calcification.2 The diagnosis is confirmed with
sporadic and iatrogenic forms occurs in a mat- the presence of multiple calcified lesions in the
ter of months with death occurring at a mean cortex as shown in computed tomography or
age of 66 years.35 Mean duration of illness in magnetic resonance imaging, positive histo-
the variant form is 14 months.35 logical findings, and direct visualization of the
parasite by funduscopic examination. Treat-
■ Cushing’s Syndrome ment for cysticercosis includes anticysticercal
(Hypercortisolism) drugs and corticosteroids for cerebral edema.
Chief Clinical Characteristics If a large cyst obstructs the flow of cere-
This presentation includes severe weakness of brospinal fluid, surgical removal of the cyst or
the proximal limb and girdle muscles often a shunt becomes necessary.2 Prognosis is most
manifested by difficulty climbing stairs or favorable for patients with one lesion.
rising from a low chair. Additional signs and
symptoms include fatigue, obesity of the upper ■ Dementia with Lewy Bodies
body (including the hallmark feature of a moon- Chief Clinical Characteristics
shaped face), easy bruising and bluish-red This presentation can be characterized by
stretch marks, high blood pressure, elevated fluctuating cognitive dysfunction, particularly
blood glucose, memory loss, and depression.36,37 visuospatial problems and executive dysfunction,
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644 Chapter 31 Depression

visual hallucinations, and parkinsonism fea- diminished interest or pleasure; significant


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

tures such as masked facies, autonomic dysfunc- weight loss or gain; insomnia or hypersomnia;
tion, rigidity, and bradykinesia. Other signs psychomotor agitation or delay; fatigue or loss
and symptoms may include postural instability, of energy; feelings of worthlessness; dimin-
falls, sleep disturbances, memory problems, ished ability to think or concentrate, indeci-
syncope, transient loss of consciousness, and siveness; recurrent thoughts of death, suicidal
sensitivity to antipsychotic and anti-Parkinson ideation, suicide attempt, or specific plan for
medications.40 suicide. In addition symptoms must cause
clinically significant distress or impairment of
Background Information
functioning and are not better accounted for
This progressive condition is the second most
by bereavement. The best approach to treat-
common dementia after Alzheimer’s disease.40
ment for depression is a combination of psy-
The specific etiology of this condition is un-
chotherapy and antidepressant medications.44
known. The characteristic Lewy bodies are
This condition responds well to treatment—
eosinophilic inclusion bodies found within the
approximately 70% to 80% of treated patients
cytoplasm of neurons in the cerebral cortex
have significant reduction in symptoms. How-
and limbic system.40 A thorough clinical ex-
ever, approximately 20% of patients who are
amination, laboratory screen, and imaging are
chronically depressed have recurrent and se-
important to rule out other causes of demen-
vere depressive episodes.11
tia. The definitive diagnosis for this condition
is made postmortem; however, it appears that ■ Dyslexia (Developmental Reading
the use of single-photon emission computed Disorder)
tomography and positron emission tomogra-
Chief Clinical Characteristics
phy may be useful in the identification of oc-
This presentation is characterized by an inabil-
cipital hypoperfusion which may be associated
ity to read, write, and spell words with the
with the visual hallucinations.40,41 Manage-
preservation of intelligence, motivation, and
ment includes caregiver education to assist in
schooling necessary to accomplish these
minimizing factors that may contribute to
tasks.45 During early school years individuals
problematic behaviors. Medication therapy
with dyslexia may experience difficulty copy-
may be indicated, but should be monitored
ing and naming colors and frequent reversal
closely due to potential exacerbation of symp-
of letters.2
toms.40 Life expectancy for individuals with
this condition is similar that of Alzheimer’s Background Information
disease. The average survival time is between 6 This condition occurs in approximately 5% to
and 8 years from the onset of dementia.40,42 10% of the school-age population46 and is
commonly familial with a sex-linked recessive
■ Depression pattern. This condition may also be present
Chief Clinical Characteristics in children with congenital developmental ab-
This presentation may involve slowness of move- normalities or after brain injury.2 Neurophysi-
ment that can progress to the point of catatonia, ological studies suggest abnormal cortical
intensely dysphoric mood, appetite loss, insom- organization in the language areas of the brain.46
nia or hypersomnia, social withdrawal, loss of The diagnosis is based on clinical presentation
motivation, helplessness, hostility, and agitation. of symptoms, family history, and school-based
testing. Treatment for dyslexia includes refer-
Background Information ral to special education or special tutoring for
The origin of this condition is not fully under- intensive individualized training.46 Most chil-
stood and genetic, biochemical, neuroanatom- dren with dyslexia do not spontaneously remit
ical and psychosocial factors all appear to play or catch up to peers in reading ability.47
a role.43 For a clinical diagnosis, the following
criteria from the DSM-IV-TR12 must be met: ■ Encephalitis
at least five of the following symptoms, during Chief Clinical Characteristics
the same 2-week period, representing a change This presentation includes confusion, delir-
from previous functioning: depressed mood; ium, convulsions, problems with speech or
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Chapter 31 Fahr’s Syndrome 645

hearing, memory loss, hallucinations, drowsi- ■ Epilepsy/Seizure Disorder

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


ness, and coma. Loss of balance and/or falls Chief Clinical Characteristics
may be present. This presentation is variable and can range
Background Information from abnormal sensations or behavior to
Encephalitis is an inflammation of nerve convulsions, muscles spasms, and loss of con-
cells in the brain. This term usually refers to sciousness. Specific cognitive symptoms include
the viral form, although bacterial, parasitic, problems with perception, attention, emotion,
and fungal agents also can cause this condi- and memory.50
tion. Up to 20,000 new cases of viral forms of Background Information
this condition are reported annually in the According to the International League
United States. Diagnosis is established by Against Epilepsy an epileptic seizure is de-
clinical presentation suggesting dysfunction fined as “a transient occurrence of signs
of the cerebrum, brainstem, or cerebellum, and/or symptoms due to abnormal excessive
cellular reaction and elevated protein in or synchronous neuronal activity in the
spinal fluid, and possible demonstration of brain.”50 The diagnosis of epilepsy includes a
diffuse edema or enhancement of the brain history of at least one seizure with the “asso-
on magnetic resonance imaging or computed ciated neurobiologic, cognitive, psychologi-
tomography. Treatment is primarily pharma- cal, and social disturbances.”50 Seizures are
cologic, with drugs such as corticosteroids, also classified into different categories based
antiviral agents, and anticonvulsants. The on presentation and electroencephalogram
majority of individuals with encephalitis do features. Approximately 1% of the U.S. pop-
recover, but irreversible brain damage and ulation will have epilepsy before 20 years of
death can result.2 age, although the incidence increases slightly
after age 60.2 Most seizures can be controlled
■ Encephalopathy pharmacologically with antiepileptic drugs.
Chief Clinical Characteristics Surgical treatment is indicated in patients
This presentation commonly includes neuromy- with recurrent seizures that arise from a sin-
oclonus, nystagmus, ataxia, and tremor. Al- gle focus and prevent a normal lifestyle.51
tered mental state, loss of memory, personality The prognosis for a patient with epilepsy
changes, dementia, and seizures are possible depends on the cause of the seizures and
signs. Additional symptoms include muscle treatment. Remission occurs in only 16% to
atrophy and weakness. Patients may also pres- 43% of patients with partial seizures.55 Less
ent with a progressive loss of consciousness favorable prognosis is also associated with
resulting in coma.17,48 multiple seizure types, associated neurologi-
Background Information cal deficits, and behavioral or psychiatric
Encephalopathy involves diffuse disease of the disturbances.52
brain that alters brain function or structure.
The numerous causes of encephalopathy in- ■ Fahr’s Syndrome (Familial
clude cirrhosis of the liver, severe hyperten- Idiopathic Basal Ganglia
sion, thiamine deficiency, infection, metabolic Calcification, Bilateral
or mitochondrial dysfunction, toxin expo- Striopallidodentate Calcinosis)
sure, trauma, or lack of oxygen to the brain.48 Chief Clinical Characteristics
Diagnosis is made by numerous studies due to This presentation may involve features of
the variable causes of encephalopathy. These parkinsonism, such as chorea, athetosis, rigid-
tests include blood work, cerebrospinal fluid ity, dystonia, and tremor in addition to cogni-
examination, electroencephalography, and tive impairments, cerebellar impairments, gait
neuroimaging studies.49 Treatment is sympto- and balance disorders, psychiatric features,
matic and varies according to the cause of pain, pyramidal signs (such as weakness, hyper-
encephalopathy. Even with treatment, en- reflexia in the deep tendon reflexes, hypertonia,
cephalopathy can cause permanent brain clonus, and/or a positive Babinski sign), sensory
damage and, in some cases, may be fatal.17 changes and speech disorders.53
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646 Chapter 31 Frontotemporal Dementia

Background Information spasticity and splenomegaly and deficits in


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

This condition occurs due to bilateral sym- lateral gaze.2


metric calcification of the basal ganglia with or
Background Information
without calcification of the dentate nucleus.
This condition is a rare disorder, although it is
The disease has been described as both famil-
prevalent among the Ashkenazi Jewish popula-
ial and nonfamilial.2 Diagnosis is established
tion.59 The disease is an autosomal genetic dis-
using computed tomography or magnetic
order in which glucocerebroside accumulates
resonance imaging of the brain; however, com-
in the spleen, liver, lungs, bone marrow, and the
puted tomography has been found to be more
brain due to a deficiency in an enzyme.2 There
sensitive for identifying calcium deposits.54
are three types of Gaucher’s disease. The most
Treatment aimed at minimizing calcium de-
common, type 1, is characterized by no central
posits has been unsuccessful.55 Individuals
nervous system involvement. In type 2, infants
with this condition may be responsive to
have extensive and progressive neurological
levodopa for treatment of their parkinsonian
damage.59 Type 3 is less common and is associ-
features.2
ated with less severe neurological symptoms.59
Diagnosis is established by clinical presenta-
■ Frontotemporal Dementia
tion, laboratory tests that show an increase in
Chief Clinical Characteristics total acid phosphatase, and biopsy of bone
This presentation can be characterized by ap- marrow that is positive for Gaucher cells. En-
athy, perseveration, poor judgment, aphasia, zyme replacement therapy is standard for most
bizarre affect, and disengagement.2 patients with types 1 and 3. However, there is
no effective treatment for the severe brain
Background Information
damage that may occur in patients with types 2
With frontotemporal dementia, progression of
and 3. Prognosis for patients with type 2 dis-
symptoms ranges from a few years to 10 years
ease is poor with death within the first 2 years
and the average age at onset is 59 years. This
of life. For type 3 disease, symptoms typically
condition is distinguished from Alzheimer’s
present in childhood and death occurs by age
disease by earlier onset and more prominent
10 to 15 years.2
behavioral rather than cognitive impairments.7
Prevalence is 81 per 100,000 persons ages 45 ■ Gerstmann’s Syndrome
to 64 years.56 The cause of this disease is
unknown, but recent evidence has suggested Chief Clinical Characteristics
that an abnormality of chromosome 17 may This presentation commonly includes a distinct
be responsible.57 Diagnosis is based on clinical collection of symptoms including agraphia, dys-
presentation, and magnetic resonance imaging graphia, dyscalculia, finger agnosia, and apha-
and computed tomography are useful to dis- sia.2 Children may also manifest behavioral
tinguish it from focal neurological insults. problems.60
Effective treatment is not well characterized. Background Information
Speech language therapy may be helpful to The cause of the developmental form of this
maintain or improve spared function and disorder in children is not known. In adults,
most pharmacologic treatments are investiga- this syndrome may result from damage to the
tive at this time. Prognosis is poor for those dominant or left parietal lobe caused by
patients with this condition who also develop stroke, tumor, or trauma. Magnetic resonance
motor neuron diseases in which dysphagia is imaging will usually demonstrate a lesion of
present in late stages.58 the angular gyrus in the left parietal lobe.61
The underlying cause in adults should be
■ Gaucher’s Disease treated appropriately; rehabilitation may help
Chief Clinical Characteristics diminish symptoms. Prognosis for adults with
This presentation commonly includes slowly this condition is dependent on the underlying
progressive mental decline, seizures, ataxia, cause and usually improves with time and
and, upon later development, weakness with therapy.62
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Chapter 31 Hydrocephalus 647

■ Hallervorden-Spatz Syndrome findings of corticospinal tract deficits in the

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


(Neurodegeneration with lower extremities, family history or genetic
Brain Iron Accumulation, testing, and exclusion of any other disorders
Pantothenate Kinase-Associated that could account for the symptoms.65 Cur-
Neurodegeneration) rent treatment is limited to decreasing muscle
Chief Clinical Characteristics spasticity through exercise and medication.
This presentation includes dystonia, parkin- Physical therapy is beneficial for the mainte-
sonism, choreoathetosis, spasticity, cognitive nance and improvement of muscle flexibility,
impairment, corticospinal tract involvement, muscle strength, gait, and cardiovascular fit-
optic atrophy, and pigmentary retinopathy.63 ness.65 Prognosis varies, and those with an
onset after adolescence are more likely to have
Background Information insidious worsening.65
This rare, inherited disorder results from a
genetic mutation in the iron regulatory path- ■ Huntington’s Disease
ways, resulting in excessive iron accumulation Chief Clinical Characteristics
in the basal ganglia.63,64 Diagnosis is made by This presentation involves progressive chorea
magnetic resonance imaging, which shows of the entire body, emotional disturbances
characteristic abnormalities in the basal ganglia, such as behavior and personality changes, and
known as the “eye of the tiger” sign. Pathologi- dementia.17,67,68
cal studies may also show brown discoloration,
iron pigmentation, and gliosis in the globus Background Information
pallidus and substantia nigra.63 There is no cure This autosomal dominant genetic disorder
or effective treatment for the condition, but causes selective neurodegeneration, most com-
patients may benefit from rehabilitation thera- monly in the neostriatum. Diagnosis is made by
pies to decrease disability as the disease follows genetic testing.68 There is currently no treatment
its progressive course of degeneration.64 to slow or stop the progression of this condition;
care is focused on symptom management and
■ Hereditary Spastic Paraplegia optimization of functioning.68 The prognosis
(Familial Spastic Paralysis) for Huntington’s disease is poor, and individuals
Chief Clinical Characteristics usually experience very rapid decline. On aver-
This presentation includes insidious, progres- age, patients survive for 15 to 20 years after ini-
sive difficulty with walking due to bilateral, tial diagnosis, but require high levels of care and
symmetric lower extremity spastic weakness. supervision during those years.68
Patients may report tripping, stumbling, and
falling, as well as urinary urgency.65 ■ Hydrocephalus
Background Information Chief Clinical Characteristics
This condition can be divided into two types. This presentation commonly includes frontal lobe
The complicated form of this condition is signs such as slowness of mental response, inat-
indicated by the presence of neurological tentiveness, distractibility, perseveration, in-
abnormalities such as dementia, mental retar- ability to sustain complex cognitive function, and
dation, epilepsy, extrapyramidal disturbance, incontinence. Other symptoms include gait
ataxia, deafness, retinopathy, optic neuropathy, deterioration, frequent falls, occipital or frontal
peripheral neuropathy, and skin lesions. The headaches, nausea and vomiting, diplopia, and
uncomplicated form is indicated by the absence lethargy. Advanced stages are associated with
of these features.66 This condition is a group of coma and extensor posturing.
inherited disorders with a primary pathologi- Background Information
cal feature of axonal degeneration of the distal Intracranial pressure can be increased due to
ends of the longest ascending and descending many mechanisms including a cerebral or ex-
tracts, resulting in the characteristic spastic- tracerebral mass, generalized brain swelling,
ity.65,66 Diagnosis is made by the presence of increased venous pressure, obstruction to the
symptoms of gait disturbance, neurological flow and absorption of cerebrospinal fluid, or
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648 Chapter 31 Hypersomnia

volume expansion of cerebrospinal fluid.2 ■ Hypoxia (Cerebral Hypoxia,


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

Magnetic resonance imaging and the presence Anoxia)


of papilledema are commonly used to estab- Chief Clinical Characteristics
lish the diagnosis of hydrocephalus. Medical This presentation includes a wide variety of
treatment may include restriction of fluid symptoms that depend on the condition’s sever-
intake and drugs with an osmotic effect, or ity. Mild hypoxia without loss of consciousness
the addition of diuretics.69 Surgical treatment may present with inattentiveness, poor judgment,
depends on the chronicity of the hydro- and motor incoordination. More severe levels of
cephalus. The acute form of this condition is hypoxia can result in seizures and/or coma.
considered fatal and is emergently treated via Post-hypoxic neurological symptoms include
lumbar puncture or ventricular catheter.69 The dementia, parkinsonian syndrome, choreoa-
chronic form of this condition is treated with thetosis, cerebellar ataxia, intention or action
placement of a ventricular shunt or with surgi- myoclonus, or a Korsakoff amnesic state.17
cal removal of a mass if that is the cause of the
hydrocephalus. Although surgical procedures Background Information
for hydrocephalus have a high success rate and This condition occurs as a result of a decrease
a good prognosis, it is common to have shunt of oxygen supply to the brain. It has numerous
complications such as infection, occlusion, causes, including cardiac arrest, drowning,
and over- or underdrainage. Thus, patients strangulation, aspiration, choking, carbon
who have been treated for this condition must monoxide poisoning, and complications of
continue to be medically managed and edu- general anesthesia. Pure hypoxia produces
cated regarding the indications of shunt damage in areas susceptible to reduced oxygen
compromise. delivery such as the hippocampi and the cere-
bellum. This condition is often seen along with
■ Hypersomnia ischemia, producing complex patterns of cere-
Chief Clinical Characteristics bral damage.17 Diagnosis and determination
This presentation can involve recurrent episodes of the cause of hypoxia may require magnetic
of excessive daytime sleepiness or prolonged resonance imaging, electrocardiography, labo-
nighttime sleep. Individuals with this condi- ratory studies, electroencephalography, and
tion nap repeatedly during the day and may evoked potentials.17,71 Treatment is directed at
demonstrate anxiety, increased irritation, de- prevention of further hypoxic injury. Out-
creased energy, restlessness, slow thinking, slow comes vary, depending on cause and severity
speech, loss of appetite, hallucinations, and of hypoxia, and range from full recovery to
memory difficulty. coma or even death. The longer an individual
with this condition is unconscious, the lower
Background Information
the likelihood of a meaningful recovery.17
This condition typically affects adolescents
and young adults.70 It may be caused by an- ■ Klüver-Bucy Syndrome
other sleep disorder (such as narcolepsy or
Chief Clinical Characteristics
sleep apnea), dysfunction of the autonomic
This presentation typically involves oral
nervous system, or drug or alcohol abuse. In
exploratory behavior, tactile exploratory
some cases this condition results from a tu-
behavior, and hypersexuality, with additional
mor, head trauma, or other injury to the cen-
symptoms and signs that may include visual
tral nervous system, especially localized to the
agnosia, decreased attention, seizures, and
mesencephalon and the floor and walls of the
dementia.
third ventricle.1 Diagnosis is based on clinical
presentation and classified according to Background Information
DSM-IV criteria.12 Treatment involves med- This condition arises from medial temporal
ication. The prognosis for persons with hyper- lobe dysfunction, and it may be associated
somnia depends on the cause of the disorder. with many different etiologies including her-
This condition can have serious consequences, pes encephalitis, traumatic brain injury, and
such as automobile accidents caused by falling Pick’s disease. This condition is diagnosed
asleep while driving.70 clinically by the presence of the above cluster
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Chapter 31 Viral Meningitis 649

of symptoms. Treatment is symptomatic and managed easily if diagnosed promptly; it fol-

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


primarily through pharmacologic means. lows a serious course in 12% of patients.72
Prognosis is poor.2 Once coma is present, 20% to 30% of patients
do not survive.2
■ Lyme Disease (Tick Paralysis)
Chief Clinical Characteristics MENINGITIS
This presentation can include fluctuating signs ■ Bacterial Meningitis
or symptoms such as headache, neck stiffness, Chief Clinical Characteristics
nausea, vomiting, malaise, fever, pain, fatigue, This presentation includes acute symptoms
and presence of a “bull’s-eye” rash.2 Over time, such as fever, severe headache, neck stiffness,
additional symptoms may include sensory seizures, changes in consciousness, facial and
changes, irritability, cognitive changes, depres- ocular palsies, positive Kernig and Brudzin-
sion, behavioral changes, seizures, ataxia, chorea ski signs, and possible hemiparesis, which may
movements, pain, weakness, balance deficits, lead to falls. Chronic symptoms include hydro-
arthritis, and cranial nerve involvement.1,2 cephalus, vomiting, immobility, impaired
Background Information alertness, hemiplegia, decorticate or decere-
Borrelia burgdorferi, an organism that infects brate posturing, cortical blindness, stupor,
ticks, is responsible for the transmission of this or coma.17
condition to a human host.2 The diagnosis is Background Information
confirmed with a thorough history, clinical Bacterial meningitis results from an infec-
assessment, enzyme-linked immunosorbent tion and inflammation of the meninges sur-
assay, and Western blot or immunoblot analy- rounding the brain and spinal cord. Lumbar
sis. In addition, magnetic resonance imaging puncture for spinal fluid pressure and cere-
or computed tomography may reveal multifo- brospinal fluid culture, blood cultures, and
cal or periventricular cerebral lesions.2 Medical radiologic studies confirm the diagnosis.17
management includes treatment with oral This condition is considered a medical emer-
tetracycline, penicillin, or intravenous ceftri- gency. Medical management includes main-
axone.2 Many individuals experience full re- tenance of blood pressure, treatment for
covery with treatment; however, residual septic shock, and administration of intra-
deficits may persist for individuals with venous antibiotics for 10 to 14 days.17 Prog-
chronic Lyme disease.2 nosis depends on the strain of bacteria;
approximately 5% to 15% of patients with
■ Malaria bacterial meningitis do not survive.17 Resid-
Chief Clinical Characteristics ual effects after the infection resolves are
This presentation commonly involves fever, variable and patients with pneumococcal
tachycardia, and sweating. Neurological symp- and H. influenzae bacterial meningitis are
toms that characterize cerebral malaria present more likely to have lasting neurological
in about 2% to 6% of cases and may include con- deficits.17
fusion, focal neurological deficits, convulsions,
and coma.2,72 ■ Viral Meningitis
Chief Clinical Characteristics
Background Information
This presentation can be characterized by an
Cases of malaria are most common in tropical
acute onset of fever, headache, and neck stiff-
areas.72 Malaria is transmitted by bites from
ness. Drowsiness, lethargy, and irritability
infected female mosquitoes usually at dusk
may occur, but overall symptoms tend to be
and during the night. The protozoan infects
relatively mild.17 Although not a common
red blood cells, which then adhere to blood
presenting sign, falls or imbalance may occur
vessels and block capillaries. Diagnosis is made
due to drowsiness and lethargy.
by clinical presentation and the presence of
plasmodia in red blood cells.7 Treatment for Background Information
malaria involves quinine, chloroquine, and re- This condition is an infection and inflamma-
lated antimalarial drugs.2 This condition is tion of the meninges surrounding the brain
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650 Chapter 31 Mitochondrial Myopathies

and spinal cord. Also known as aseptic Willis.17 Diagnosis is based upon clinical find-
PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

meningitis, this condition is most commonly ings and results of magnetic resonance imag-
caused by the echovirus or coxsackie virus.17 ing and magnetic resonance angiography.
Cerebrospinal fluid analysis and blood work Images will demonstrate the occlusion of the
are used to determine the diagnosis.73 There circle of Willis as well as secondary cerebral
is no specific treatment, although supportive infarction, white matter lesions, atrophy, and
care may include administration of anal- hemorrhage.75 Treatment options include
gesics. This condition is rarely fatal and most revascularization surgery and medical treat-
patients demonstrate a full recovery.17 ment to prevent hypertension and further
strokes.76 Rehabilitative therapies are used to
■ Mitochondrial Myopathies treat functional deficits that the patient may
Chief Clinical Characteristics incur from a stroke secondary to the progres-
This presentation typically involves a combination sion of the disease. Outcome depends on the
of exercise intolerance, ataxia, seizures, myoclonus, severity of secondary complications and pres-
headaches, small strokes, ophthalmoplegia, deaf- ence of subsequent occlusion.
ness, muscle cramps and/or slowly progressive
myopathy with proximal greater than distal in- ■ Multi-Infarct Dementia/Vascular
volvement. Other less common symptoms that Dementia
may be seen include dementia, lactic acidosis, Chief Clinical Characteristics
ptosis, and cardiac conduction defects.17,74 This presentation includes confusion, memory
impairment, aphasia, and agnosia that occur in
Background Information
a progressive stepwise and patchy pattern.11,77
This condition refers to a large group of disor-
ders that result from a mutation in the mito- Background Information
chondrial genome, resulting in damage to More men than women are affected with peak
the mitochondria. These disorders include incidence between the ages of 60 and 75 years.
Kearns-Sayre syndrome, myoclonus epilepsy This condition occurs when a series of small
with ragged red fibers, mitochondrial en- strokes gradually leads to mental decline.1 Di-
cephalomyopathy with lactic acidosis and agnosis is obtained from the history and con-
stroke-like episodes, as well as other child- firmed by magnetic resonance imaging or
hood-onset disorders.17 A combination of clin- computed tomography. Treatment emphasizes
ical picture, histological findings of ragged red prevention of additional brain damage by con-
fibers, elevated serum lactate, and possible trolling high blood pressure. Prognosis is gen-
family history contribute to the diagnosis of erally poor. Early treatment and management
this condition.17 There is no specific treatment, of blood pressure may prevent progression of
but new research shows that patients may the disorder.77
benefit from physical therapy for submaximal
exercise training.74 Most patients experience ■ Multiple Sclerosis
lifelong progression of the disease, and progno- Chief Clinical Characteristics
sis varies according to the type of disease and This presentation may include paresthesias,
amount of involvement.17 weakness, spasticity, hypertonicity, hyperreflexia,
positive Babinski sign, incoordination, optic
■ Moyamoya Disease neuritis, ataxia, vertigo, dysarthria, diplopia,
Chief Clinical Characteristics bladder incontinence, tremor, balance deficits,
This presentation may include unsteady gait, falls, and cognitive deficits.2
involuntary movement, weakness, speech and
Background Information
sensory impairments, headache, seizures, im-
This condition may present as relapsing-
paired mental development, visual distur-
remitting, primary progressive, or secondary
bances, and nystagmus.17
progressive. The disease occurs most fre-
Background Information quently in women between the ages of 20 and
This rare condition results from progressive 40 years. Only a small number of children or
occlusion of the arteries of the circle of individuals between 50 and 60 years of age are
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Chapter 31 Neurofibromatosis 651

diagnosed with this condition.2 This condition mean survival time of 6.2 years.83 This condi-

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


was originally thought to be secondary to en- tion is a progressive condition of the central
vironmental and genetic factors, but evidence and autonomic nervous systems that rarely
suggests an autoimmune response to a viral occurs without orthostatic hypotension. There
infection, which subsequently targets myelin.2 are three types of this condition. The parkin-
The diagnosis may be confirmed by a thor- sonian-type includes symptoms of Parkinson’s
ough history, physical examination, magnetic disease such as slow movement, stiff muscles,
resonance imaging, analysis of cerebrospinal and tremor. The cerebellar type causes prob-
fluid, and evoked potentials.2,78,79 Life ex- lems with coordination and speech. The
pectancy and cause of mortality are similar for combined type includes symptoms of both
all types of this condition.2 Clinical character- parkinsonism and cerebellar failure. Older age
istics that are associated with a longer time at onset is associated with a shorter survival
interval for progression of disability include time.83 Average age of onset is 54 years, with
female sex, younger age of onset, relapsing- mean age at death being 60.3 years.81 Most in-
remitting type, complete recovery after the dividuals with this condition receive a trial of
first relapse, and longer time interval between levodopa although only a minority respond.81
first and second exacerbation.80 Medical man- Additional treatment addresses symptoms and
agement may include the use of methylpred- involves physical and occupational therapy to
nisolone, prednisone, cyclophosphamide, im- maintain mobility and address safety issues
munosuppressant treatment, and betainterferon.2 related to the progression of imbalance.
Physical, occupational, and speech therapy
may be indicated to prevent secondary seque- ■ Narcolepsy
lae and to optimize functional activity and Chief Clinical Characteristics
mobility. Some individuals may benefit from This presentation includes excessive daytime
psychology/psychiatry and social support as sleepiness; cataplexy, or the sudden loss of
the disease progresses. voluntary muscle tone; vivid hallucinations
during sleep onset or upon awakening; and
■ Multiple System Atrophy brief episodes of total paralysis at the beginning
(Striatonigral Degeneration, or end of sleep.
Olivopontocerebellar Atrophy,
Shy-Drager Syndrome) Background Information
Chief Clinical Characteristics This condition is hypothesized to result from
This presentation involves tremor, rigidity, a genetic predisposition, abnormal neuro-
akinesia, and/or postural imbalance along with transmitter functioning and sensitivity, and
signs of cerebellar, pyramidal, and autonomic abnormal immune predisposition.2 Preva-
dysfunction. Autonomic symptoms such as lence is 1:20001 with onset between the ages
orthostatic hypotension, dry mouth, loss of of 15 and 35.2 Diagnosis is based on the clin-
sweating, impotence, and urinary incontinence ical presentation. Treatment for narcolepsy
or retention are the initial feature in 41% of includes strategically placed naps and med-
individuals, with 74% to 97% of individuals de- ication.84 With individualized treatment,
veloping some degree of autonomic dysfunc- most patients are able to function in daily
tion during the course of the disease.81 This activities.
condition is a combination of parkinsonian ■ Neurofibromatosis
and non-parkinsonian symptoms and signs.
Chief Clinical Characteristics
Background Information This presentation depends on the type of neu-
Diagnostic criteria are based on the clinical rofibromatosis. Neurofibromatosis type 1 (NF1,
presentation, which includes poor response to Von Recklinghausen’s disease) may include café
levodopa, presence of autonomic features, au lait spots, neurofibromas, pathological frac-
presence of speech or bulbar problems, absence tures, syringomyelia, scoliosis, stroke, neoplasms,
of dementia, absence of toxic confusion, and learning difficulties, and hyperactivity. Neu-
presence of falls.82 The disease course ranges rofibromatosis type 2 (NF2) is characterized by
between 0.5 and 24 years after diagnosis with a bilateral vestibular schwannomas, progressive
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652 Chapter 31 Neurological Complications of Acquired Immunodeficiency Syndrome

hearing loss, and possible intracranial or myelitis, vacuolar myelopathy that occurs
PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

intraspinal neoplasms.1,2 with acquired immunodeficiency syndrome


dementia complex)
Background Information
5. Peripheral involvement associated with
This condition is an autosomal dominant
sensory changes, weakness, balance deficits,
disorder on chromosome 17 (NF1) and 22
and pain (such as secondary to peripheral
(NF2), respectively. The tumors in NF1 occur
neuropathy, acute and chronic inflamma-
due to the excessive proliferation of cells within
tory demyelinating polyneuropathies).2,85
the meninges, vascular system, skin, viscera,
peripheral, and central nervous systems.1,2 The Abnormal neurological findings are ob-
tumors in NF2 arise from the posterior nerve served during a clinical examination in approx-
roots. Depending on the type of this condition imately one-third of patients with acquired
the diagnosis may be confirmed by a thorough immunodeficiency syndrome; however, on au-
family history, the presence of six or more café topsy most individuals with this condition have
au lait spots, imaging, and genetic testing. If in- abnormalities within the nervous system.2 Di-
dicated, the tumors that result from this condi- agnosis of the variable neurological complica-
tion may be surgically removed.1 Both forms of tions associated with this condition may be
this condition are progressive and the progno- confirmed with laboratory tests, cerebrospinal
sis varies depending on the severity of lesions.2 fluid cultures, imaging, nerve conduction stud-
ies, and physical examination.1,2,85 Treatment
■ Neurological Complications of appears to be limited primarily to the use of
Acquired Immunodeficiency antiviral medications.85 Physical and occupa-
Syndrome tional therapy may be indicated to address
Chief Clinical Characteristics equipment needs and caregiver/patient train-
This presentation is variable and dependent ing related to functional mobility.
upon the affected neuroanatomical structures
in an individual with acquired immunodefi- ■ Neurosyphilis (Tabes Dorsalis,
ciency syndrome.85 Syphilitic Spinal Sclerosis,
Progressive Locomotor Ataxia)
Background Information Chief Clinical Characteristics
This condition may be categorized by: This presentation can be characterized by hemi-
1. Meningitic symptoms including headache, paresis, ataxia, aphasia, gait instability, falls,
malaise, and fever (such as secondary to neuropathy, personality and cognitive changes,
meningitis, cryptococcal meningitis, tuber- seizures, diplopia, visual impairments, hearing
culous meningitis, and human immunodefi- loss, psychotic disorders, loss of bowel/bladder
ciency virus headache) function, pain, hyporeflexia, and hypotonia.86,87
2. Focal cerebral symptoms including hemipare-
sis, aphasia, apraxia, sensory deficits, homony- Background Information
mous hemianopia, cranial nerve involvement, Treponema pallidum infects the human host by
balance deficits, incoordination, and/or ataxia way of contact with contaminated body fluids
(such as secondary to cerebral toxoplasmosis, or lesions.86 This spirochete is responsible for
primary central nervous system lymphoma, and the diagnosis of syphilis; however, when T. pal-
progressive multifocal leukoencephalopathy) lidum is present within the central nervous
3. Diffuse cerebral symptoms that involve cog- system the individual is diagnosed with neu-
nitive deficits, altered level of consciousness, rosyphilis.87 This condition occurs in approxi-
hyperreflexia, Babinski sign, presence of mately 10% of individuals with untreated
primitive reflexes (such as secondary to syphilis, and in 81% of these cases it presents
postinfectious encephalomyelitis, acquired as meningovascular, meningeal, or general
immunodeficiency dementia complex, cy- paresis. Treatment includes use of various
tomegalovirus encephalitis) forms of penicillin or alternative choices for
4. Myelopathy associated with gait difficulties, those allergic to penicillin86 and may involve
spasticity, ataxia, balance deficits, and hyper- rehabilitative therapies depending on the indi-
reflexia (such as secondary to herpes zoster vidual’s activity limitations or participation
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Chapter 31 Paraneoplastic Syndromes 653

restrictions. A better prognosis has been ob- who are diagnosed in childhood live well into

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


served for individuals treated during early adult years, and overall prognosis depends on
neurosyphilis.87 the severity of the disease.88

■ Neurotoxicity ■ Paraneoplastic Syndromes


Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes limb weakness or This presentation includes dizziness in combi-
numbness; loss of memory, vision, and/or intel- nation with a variety of different neurological
lect; headache; behavioral problems; and symptoms and signs in an individual with
sexual dysfunction. cancer. Specific neurological symptoms and
signs depend on the location of involvement of
Background Information the central or peripheral nervous system.
This condition occurs when exposure to
natural or artificial toxins alters the normal Background Information
activity of the nervous system, eventually lead- Paraneoplastic encephalomyelitis and focal
ing to disruption or death of neurons. It results encephalitis may present with ataxia, vertigo,
from exposure to substances use inchemother- balance deficits, nystagmus, nausea, vomiting,
apy, radiation treatment, drug therapies, and cranial nerve palsies, seizures, sensory neu-
organ transplants, as well as eposure to heavy ropathy, anxiety, depression, cognitive changes,
metals, foods, or pesticides. Diagnosis is sup- and hallucinations. For individuals presenting
ported by clinical presentation and lab tests for with ataxia, dysarthria, dysphagia, and diplopia,
detection of the toxic substance. Treatment is paraneoplastic cerebellar degeneration may
prioritized at removal of the offending toxin. be suspected. Paraneoplastic opsoclonus/my-
Prognosis varies greatly depending upon the oclonus tends to affect both children and
level of exposure and individual’s comorbid adults with signs and symptoms including
medical conditions. hypotonia, ataxia, irritability, truncal ataxia,
gait difficulty, balance deficits, and frequent
■ Niemann-Pick Disease (Types C falls. Stiff-man syndrome presents with spasms
and D Only) and fluctuating rigidity of axial musculature,
legs and possibly shoulders, upper extremities,
Chief Clinical Characteristics
and neck. Paraneoplastic sensory neuropathy
This presentation includes ataxia, dystonia,
presents with asymmetric, progressive sensory
vertical gaze palsy, dysarthria, and seizures.
alterations involving the limbs, trunk, and face,
Often the presenting feature is a psychotic
sensorineural hearing loss, autonomic dysfunc-
episode.17,88
tion, and pain. Other conditions in this
Background Information category include vasculitis, Lambert-Eaton
This condition is an autosomal recessive lipid myasthenia syndrome, myasthenia gravis, der-
storage disorder that results in impaired cho- matomyositis, neuromyotonia, and various
lesterol transport and excessive glycosphin- neuropathies.2,90 These conditions result from
golipid storage.89 There are four types of an immune-mediated response to the presence
Niemann-Pick disease: types A and B are seen of tumor or metastases. Antibodies or T-cells
only in children and are not discussed here. respond to the presence of the tumor, but also
Types C and D have a variable age of onset attack normal cells of the nervous system.91,92
ranging from childhood to late teens or even Over 60% of individuals present with this con-
early adulthood. Individuals with types C dition prior to the discovery of the cancer.90
and D demonstrate an enlarged spleen and The underlying tumor is treated according to
liver, as well as progressive neurological dys- the type of cancer. Additional treatment is
function.88 Diagnosis is made by a combina- dependent on this condition’s type and may
tion of studies, including filipin staining of include steroids, plasmapheresis, immunother-
cultured fibroblasts, cholesterol esterification apy, chemotherapy, radiation, or cyclophos-
studies, and DNA mutation analysis.91 Unfor- phamide.90 Physical, occupational, and speech
tunately, there is no effective treatment for therapy may be indicated to address functional
types C and D of this condition. Many patients limitations.
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654 Chapter 31 Paranoia, Delusional Disorder

■ Paranoia, Delusional Disorder post-mortem. However, a clinically definitive


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

Chief Clinical Characteristics diagnosis may be made with the presence of at


This presentation is characterized by delusions. least two of the three criteria—asymmetric
The delusions can be one of several types such resting tremor, bradykinesia, or rigidity—and
as being followed (persecutory type), having a a positive response to anti-Parkinson medica-
disease (somatic type), being loved at a dis- tions.2,95 Imaging may be useful to exclude
tance (erotomanic type), having an unfaithful vascular involvement. Medical management
sexual partner (jealous type), and possessing may include use of dopamine agonists,
inflated worth, power, identity, or knowledge levodopa, or other medications to address
(grandiose type).2 Age at onset is usually mid- nonmotor symptoms, such as depression, con-
dle or late adulthood, and the course is variable. stipation, autonomic symptoms, and sexual
dysfunction. Surgical management also may
Background Information be considered, including deep brain stimula-
The cause of this condition is unknown, but tion or pallidotomy.95 Forced use or higher in-
familial transmission is suspected. Diagnosis tensity, challenging activities may provide a
is defined by the DSM-IV-TR12 with the fol- neuroprotective benefit for individuals with
lowing criteria: one or more nonbizarre early forms of this condition.96 With the high
(could occur in everyday life) delusions for at incidence of depression, consultation with a
least 1 month; hallucinations not as promi- psychologist or psychiatrist may be warranted.
nent as in schizophrenia; functioning is not Individuals with a late onset tend to progress
markedly impaired and behavior is not obvi- more rapidly.97,98 Poor prognostic indicators
ously bizarre; and mood episodes are brief. for disability include initial presentation with-
Psychotherapy and medication are often indi- out tremor, early dependence, dementia, bal-
cated for treatment of this condition. The ance impairments, older age, and the postural
course of this condition is variable, with im- instability/gait difficulty dominant type.97,98
provement of delusional symptoms in 10%,
remission in 33% to 50%, and persisting ■ Pelizaeus-Merzbacher Disease
symptoms in 30% to 40% of cases. Patients Chief Clinical Characteristics
with the acute and jealous subtypes may have This presentation commonly includes deterio-
a better prognosis.93 ration of coordination, motor abilities, and
intellectual function.
■ Parkinson’s Disease
Background Information
Chief Clinical Characteristics
Severity and onset of the disease range widely,
This presentation commonly involves resting
depending on the type of genetic mutation,
tremor, bradykinesia, rigidity, and postural
and extend from the mild, adult-onset spastic
instability. Falls are a common problem in
paraplegia to the severe form with onset at in-
individuals with this condition, with up to 68%
fancy and death in early childhood.99 This
falling within a 1-year period and approxi-
condition is an X-linked disease caused by a
mately 50% of these individuals falling
mutation in the gene that controls the produc-
multiple times within that same year.94 Other
tion of a myelin protein called proteolipid pro-
common signs and symptoms include festina-
tein.99 Genetic diagnostic testing is the defini-
tion, freezing, micrographia, hypophonia (hy-
tive method for diagnosing this condition.100
pokinetic dysarthria), akinesia, masked facies,
There is no cure for this condition. Therefore,
drooling, difficulty turning over in bed, dysto-
treatment is based on symptoms and includes
nia, dyskinesia, dementia, and depression.2
physical therapy, orthotics, and antispasticity
Background Information agents with goals to minimize the develop-
This condition occurs due to the depletion or ment of joint contractures, dislocations, and
injury of dopamine-producing cells in sub- kyphoscoliosis.101 Individuals with severe
stantia nigra pars compacta. Clinical signs and forms of this condition experience progressive
symptoms are not typically present until after deterioration until death. Individuals with the
approximately 80% of dopamine-producing adult-onset form with spasticity may have
cells are lost. The definitive diagnosis is made nearly normal life span.17
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Chapter 31 Reye’s Syndrome 655

■ Pick’s Disease but without acquired immunodeficiency

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


Chief Clinical Characteristics syndrome.2
This presentation includes a gradual onset of dif- ■ Progressive Supranuclear Palsy
ficulty with memory, apathy, poor concentra- (Richardson-Steele-Olszewski
tion, personality changes, and disturbances of Syndrome)
speech. The disease usually affects individuals,
more commonly women, between the ages of Chief Clinical Characteristics
40 and 60.1 This presentation classically includes vertical
gaze palsy, prominent instability, and falls within
Background Information the first year of disease onset.104 Other charac-
Individuals with this condition typically have teristics may include rigidity, akinesia,
atrophy of the frontal and temporal lobes, as dysarthria, dysphagia, and mild dementia. Falls
evidenced on magnetic resonance imaging. were found to be the most commonly reported
Computed tomography and single photon symptom with the majority of falls being back-
emission computed tomography reveal ante- wards falls.105 Difficulty with voluntary verti-
rior hypoperfusion.1 On microscopic inspec- cal eye movements (usually downward) and in-
tion, argyrophilic inclusion bodies (Pick bod- voluntary saccades are relatively early features.
ies) are found within the cytoplasm of cells The disease generally progresses to the point at
taken from the frontotemporal cortex.1 The which all voluntary eye movements are lost.
cause of the disease is unknown, though ge-
netics are thought to play a role. Often, this Background Information
condition is described as a subtype of fron- Some patients may not demonstrate difficul-
totemporal dementia.102 There is no cure or ties with ocular movements for 1 to 3 years
specific treatment for this condition and its after disease onset. Most cases are sporadic;
progression cannot be slowed. However, some however, a pattern of inheritance compatible
of the symptoms of the disease may be treated with autosomal dominant transmission has
effectively pharmacologically.103 The course of been described.2 Diagnosis is based on clinical
this condition involves an inevitable progres- presentation, which includes a gradually pro-
sive deterioration that lasts approximately 2 to gressive disorder with age of onset at 40 years
5 years.2 or older, vertical supranuclear palsy, and pos-
tural instability with falls within the first year
■ Progressive Multifocal of disease onset.106 Medical treatment is typi-
Leukoencephalopathy cally unsuccessful, because the majority of
Chief Clinical Characteristics these patients are not responsive to levodopa
This presentation commonly involves cortical therapy aimed at addressing the extrapyrami-
blindness, visual field defects, hemiparesis with dal features of the disease.33 Physical and occu-
progression to quadriparesis, aphasia, ataxia, pational therapy are used to maintain mobility
dysarthria, personality changes and impaired and address safety issues related to the pro-
intellect evolving over a period of days to weeks. gression of imbalance. The disease course is
progressive with a mean survival time of
Background Information 5.6 years.105 Older age at disease onset, early
This condition is most likely due to viral infec- onset of falls, incontinence, dysarthria, dys-
tion of the central nervous system, which then phagia, insertion of a percutaneous gastros-
causes widespread demyelinative lesions pri- tomy, and diplopia have all been described as
marily of the cerebral hemispheres. Diagnosis being predictive of shorter survival time.105
is made by computed tomography and mag-
netic resonance imaging to localize the lesions. ■ Reye’s Syndrome
Treatment for individuals with acquired im- Chief Clinical Characteristics
munodeficiency syndrome consists of anti- This presentation involves persistent or recur-
retroviral drug combinations and can lead to rent vomiting, listlessness, personality changes
slower progression or even remission. Cur- such as irritability or combativeness, disorien-
rently, no treatment exists to impair disease tation or confusion, delirium, convulsions, and
progression in individuals with this condition loss of consciousness.8
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656 Chapter 31 Rickettsial Diseases, Including Rocky Mountain Spotted Fever

Background Information the disease followed by a time of relative stabil-


PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

Reye’s syndrome primarily presents in chil- ity (though not often a return to baseline).
dren, although it can occur at any age.107 The
Background Information
cause of this condition is unclear, but it com-
Diagnosis of schizophrenia is made on clinical
monly occurs during recovery from a viral in-
presentation and according to DSM-IV-TR
fection or 3 to 5 days after the onset. There is
criteria12: The patient must have experienced
an increased risk of the disease if aspirin was
at least two of the following symptoms for at
taken during the course of the illness.107 This
least 1 month of a 6-month period causing
condition causes an acute increase of pressure
significant social or occupational problems:
within the brain and massive accumulations of
delusions, hallucinations, disorganized speech,
fat in the liver and other organs. Diagnosis is
disorganized or catatonic behavior, or negative
made on clinical presentation and by the pres-
symptoms. The incidence peaks during ado-
ence of cerebral edema.107 Treatment is prima-
lescent years with a 1% worldwide lifetime
rily aimed at reducing brain swelling, reversing
prevalence.110 The cause of this condition is
the metabolic injury, preventing complica-
not known, but genetic and neurodevelop-
tions in the lungs, and anticipating cardiac
mental factors may play a role, as well as neu-
arrest. The fatality rate for this condition is
rotransmitter and metabolic activity in differ-
approximately 30%, with the worst prognosis
ent areas of the cortex.2 Treatment involves use
for children under 5 years of age.107
of antipsychotic medications. The prognosis is
■ Rickettsial Diseases, Including guarded, because full recovery is unusual. Poor
Rocky Mountain Spotted Fever prognosis is associated with early onset of
Chief Clinical Characteristics symptoms, family history, and prominent neg-
This presentation typically includes visual dis- ative symptoms. Individuals with this condi-
turbances, a rash that occurs on the palms of tion also have a 4% to 10% risk of committing
hands and soles of feet, headache, nausea, fever suicide.110 With pharmacologic treatment and
and myalgias.108 Initial symptoms begin within psychiatric management, 60% of patients
2 to 14 days of infections and last approximately return to home and some social activity.2
2 to 3 weeks.
■ Sleep Apnea
Background Information Chief Clinical Characteristics
Rocky Mountain spotted fever is the most This presentation is characterized by brief
common type of this condition in the United breathing disturbances during sleep that occur
States. It is transmitted by a variety of tick, and repeatedly throughout the night. The hallmark
is common in Long Island, Tennessee, Vir- symptom of the disorder is excessive daytime
ginia, North Carolina and Maryland.2 Like sleepiness. Additional symptoms of sleep
malaria, rickettsiae in the blood vessels cause apnea include loud snoring, irritability, forget-
vascular injury, which forms the basis for cen- fulness, mood or behavior changes, anxiety, and
tral nervous system damage. Diagnosis is depression.111
based on clinical signs and symptoms and
confirmed by skin biopsy. Treatment consists Background Information
of the use of doxycycline or chloramphenicol. The most common type of sleep apnea is ob-
The mortality rate in untreated cases is 20% structive sleep apnea, with mechanical causes
to 25%.109 such as obesity or enlarged tonsils.1 Central
sleep apnea is the result of impairment in res-
■ Schizophrenia piratory control of breathing often caused
Chief Clinical Characteristics by brainstem medullary infarction or follow-
This presentation can be characterized by hal- ing cervical/foramen magnum surgery.1 The
lucinations, delusions, disorganized speech, and prevalence of sleep apnea is between approxi-
negative symptoms such as flat affect, decreased mately 2% and 4%, with higher incidence in
initiation, and decreased spontaneity in the elderly.111 Diagnosis is made based on
conversation. Most marked deterioration in clinical presentation. Most treatment regimens
patients occurs within the first 5 to 10 years of begin with lifestyle changes, such as avoiding
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Chapter 31 Systemic Lupus Erythematosus 657

alcohol and medications that relax the central deterioration, and memory loss, followed by

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


nervous system, weight management, and involuntary movements and seizures.
quitting smoking. If these conservative meth-
Background Information
ods are inadequate, continuous positive airway
This condition primarily presents in children
pressure is recommended. In extreme cases
younger than 10 years, and since the introduc-
surgical procedures can be used to remove tis-
tion of the measles vaccine it has become very
sue and widen the airway.2 If untreated, sleep
rare.2 It is an infection of the central nervous
apnea can lead to hypertension with right
system caused by an altered form of the
heart failure secondary to pulmonary hyper-
measles virus that triggers changes in the white
tension, which can become life threatening.1
and gray matter primarily of the hemispheres
■ Stroke (Cerebrovascular Accident) and brainstem.2 The annual incidence is be-
tween 0.1 and 5 per one million children.114
Chief Clinical Characteristics
Diagnosis is made using the clinical presenta-
This presentation may include a wide range of
tion, elevated measles antibody titers, and
symptoms that correspond to specific areas of
typical electroencephalographic changes.2
the brain that are affected, potentially includ-
Patients with this condition have shown stabi-
ing visual disturbances. The initial symptoms
lization of disease and delay in clinical pro-
can include numbness or weakness, especially
gression after therapy with immunomodulator
on one side of the body or face; confusion or apha-
and antiviral medications. When not treated,
sia; balance deficits or falls; or sudden severe
this condition is almost always fatal. Death
headache with no known cause.
usually occurs between 1 and 3 years after on-
Background Information set, although some spontaneous remissions
This condition occurs when blood flow to (up to 5%) have been reported.115
the brain is interrupted either by blockage (is-
chemia or infarction) or from hemorrhagic ■ Systemic Lupus Erythematosus
disruption. A thrombosis or embolic occlusion Chief Clinical Characteristics
of an artery causes an ischemic type of this con- This presentation can include abnormal vision,
dition. A hemorrhagic type of this condition swallowing, taste, hearing, or changes in mood
can be caused by arteriovenous malformation, or thinking, and seizures in combination with
hypertension, aneurysm, neoplasm, drug abuse fatigue, joint pain, and swelling affecting the
and trauma. This condition is the most com- hands, feet, knees, and shoulders.
mon and disabling neurological disorder in
adults and occurs in 114 of every 100,000 peo- Background Information
ple.43 This condition is diagnosed using clinical This condition affects mostly women of child-
presentation and positive findings on com- bearing age. It is a chronic autoimmune disor-
puted tomography and magnetic resonance der that can affect any organ system, including
imaging. Medication, surgery and interdiscipli- skin, joints, kidneys, brain, heart, lungs, and
nary therapy are the most common treatments blood. Microinfarcts in the cerebral cortex and
for this condition. The prognosis for recovery is brainstem, which lead to destructive and pro-
predicted by the magnitude of initial deficit. liferative changes in capillaries and arterioles,
Factors that are associated with poor outcomes are primarily responsible for central nervous
include coma, poor cognition, severe aphasia, system manifestations. Hypertension and en-
severe hemiparesis with little return within 1 docarditis can also predispose an affected
month, visual perceptual disorders, depression, individual to development of neurological
and incontinence after 2 weeks.112,113 abnormalities. Multiple sclerosis is a disease
that may be mistaken for this condition, espe-
■ Subacute Sclerosing cially if the central nervous system manifesta-
Panencephalitis (Dawson’s tions include visual dysfunction. The diagnosis
Disease) is confirmed by the presence of skin lesions,
Chief Clinical Characteristics heart, lung, or kidney involvement, laboratory
This presentation may involve progressive stages abnormalities including low red or white cell
of abnormal behavior, irritability, intellectual counts, low platelet counts, or positive ANA
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658 Chapter 31 Tourette’s Syndrome

and anti-DNA antibody tests.116 Treatment drug therapy or surgery to reduce the risk of
PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

involves corticosteroid medication. stroke. Antiplatelet medications, particularly


aspirin, are a standard treatment for individu-
■ Tourette’s Syndrome als suspected of this condition and who also
Chief Clinical Characteristics are at risk for stroke, including individuals
This presentation involves motor and vocal tics, with atrial fibrillation.
inappropriate and involuntary obscene speech
and gestures, and anger control problems that ■ Traumatic Brain Injury
begin before 21 years of age.7 Many children with Chief Clinical Characteristics
this condition experience additional neurobe- This presentation typically includes dysequi-
havioral problems including inattention and librium in the presence of cognitive changes,
obsessive-compulsive symptoms.117 altered level of consciousness, seizures, nausea,
vomiting, coma, dizziness, headache, pupillary
Background Information
changes, tinnitus, weakness, incoordination,
This condition may be inherited and appears
behavioral changes, spasticity, hypertonicity,
to be due to a neurotransmitter disturbance.
cranial nerve lesions, and sensory and motor
Its incidence is 0.5 in 1,000 and occurs more
deficits.2,119
commonly in males than females.117 It is diag-
nosed by presentation of tics for over a year Background Information
and onset prior to age 18 according to DSM- This condition can be classified as mild, moder-
IV-TR guidelines.12 Currently all treatment is ate, or severe based on Glasgow Coma Scale,
symptomatic with medication and behavioral length of coma, and duration of post-traumatic
therapy.7 This condition is considered chronic, amnesia.119 Magnetic resonance imaging may
but tic severity on average peaks in adoles- be used to confirm the diagnosis.1 Treatment
cence and wanes thereafter.118 initiated at the scene of the accident and during
the acute phase is focused on medical stabiliza-
■ Transient Ischemic Attack tion. It should be initiated during the acute
Chief Clinical Characteristics phase in order to minimize complications.120
This presentation can include numbness or Low Glasgow Coma Scale, longer length of
weakness in the face, arm, or leg, especially on coma, longer duration of post-traumatic amne-
one side of the body; confusion or difficulty in sia, and older age tend to be associated with
talking or understanding speech; trouble seeing poor outcomes.121 Optimal rehabilitation is in-
in one or both eyes; and difficulty with walking, terdisciplinary and customized to address the
dizziness, or loss of balance and coordination. specific individuals’ disablement.
Background Information ■ Tuberous Sclerosis
This condition is a transient stroke that lasts
Chief Clinical Characteristics
only a few minutes. It occurs when the blood
This presentation includes seizures, mental
supply to part of the brain is briefly interrupted.
retardation, behavior problems, and skin
Symptoms of this condition, which usually
abnormalities.
occur suddenly, are similar to those of stroke
but do not last as long. Most symptoms disap- Background Information
pear within an hour, although they may persist This condition is a genetic disease that causes
for up to 24 hours. Because it is impossible benign tumors to grow in the brain and on
to differentiate between symptoms from this other vital organs such as the kidneys, heart,
condition and acute stroke, individuals should eyes, lungs, and skin.122 Limited hyperplasia of
assume that all stroke-like symptoms signal ectodermal and mesodermal cells during devel-
a medical emergency. A prompt evaluation opment causes the tumors.2 The inheritance is
(within 60 minutes) is necessary to identify autosomal dominant in approximately 50% of
the cause of this condition and determine cases and due to mutations in the other cases.
appropriate therapy. Depending on the individ- The estimated prevalence of the disease is
ual’s medical history and the results of a med- 1:10,000.123 The presence of seizures and an-
ical examination, the doctor may recommend giofibromas is diagnostic. Angiofibromas are
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Chapter 31 Spinal Primary Tumors 659

pink or skin-colored papules commonly ob- dysarthria, balance deficits, falls, lethargy, and

PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR


served in the nasolabial folds and on the cheeks incoordination.1,2
and chin.2 In addition, computed tomography
Background Information
may show subependymal areas of calcium de-
Glioblastoma multiforme, astrocytoma,
posits, and magnetic resonance imaging will
oligodendroglioma, metastatic tumors, pri-
reveal uncalcified subependymal tubers. Treat-
mary central nervous system lymphomas,
ment includes anticonvulsant therapy for
ganglioglioma, neuroblastoma, menin-
seizures and surgical removal of symptomatic
gioma, arachnoid cysts, hemangioblastoma,
lesions. This condition shows a wide variety of
medulloblastoma, and acoustic neuroma/
clinical expressions depending on the location
schwannoma are some of the more common
of the tumors. Approximately 30% of severely
brain tumors. Specific diagnoses for brain
affected infants are thought to die before age 5,
tumors may be confirmed with imaging and
and 50% to 75% die before attaining adult age.2
biopsy. Treatment is variable depending on
TUMORS the type, size, and location of the tumor and
may include surgical resection, chemother-
■ Brain Metastases
apy, radiation, corticosteroids, and rehabili-
Chief Clinical Characteristics tative therapies. Prognosis is also variable
This presentation may include headaches, and depends on the type and grade of tumor,
seizures, dysphagia, weakness, cognitive severity of compression, and duration of
changes, behavioral changes, dizziness, vom- compression.
iting, alterations in the level of consciousness,
ataxia, aphasia, nystagmus, visual distur- ■ Spinal Metastases
bances, dysarthria, balance deficits, falls, Chief Clinical Characteristics
lethargy, and incoordination.2,20 This presentation can involve spasticity, weak-
Background Information ness, sensory alterations, bowel and bladder in-
The majority of individuals with brain continence, neck pain, back pain, radicular
metastases have been previously diagnosed pain, atrophy, cerebellar signs, balance deficits,
with a primary tumor; however, a small per- falls, and cranial nerve involvement.1,2,124
centage of individuals are diagnosed con- Background Information
comitantly with brain metastases and the This condition is the most frequent neo-
primary tumor. The most common cancers plasm involving the spine.124 The most com-
resulting in subsequent brain metastases mon types and locations of primary tumors
include lung, breast, melanoma, colorectal, that result in spinal metastases include
and genitourinary tract. The new onset of breast, lung, lymphoma, prostate, kidney,
neurological symptoms after a primary tu- gastrointestinal tract, and thyroid.2,125 The
mor warrants the use of imaging such as diagnosis is confirmed with gadolinium-
magnetic resonance imaging or computed enhanced magnetic resonance imaging and
tomography to confirm the diagnosis. Treat- computed tomography.2,124 Treatment is
ment may include corticosteroids, brain variable depending on the tumor and may
irradiation, surgical resection, chemother- include surgical resection, chemotherapy,
apy, radiotherapy, and rehabilitative thera- radiation, corticosteroids, and rehabilitative
pies. The prognosis is poor with death therapies.2 Although the long-term progno-
typically occurring within 6 months. sis is poor, individuals without paresis, pain,
and who are still ambulatory have longer
■ Brain Primary Tumors survival rates.125
Chief Clinical Characteristics
This presentation may include headaches, ■ Spinal Primary Tumors
seizures, dysphagia, weakness, cognitive Chief Clinical Characteristics
changes, behavioral changes, dizziness, vomit- This presentation may include spasticity,
ing, alterations in the level of consciousness, weakness, sensory alterations, bowel/bladder
ataxia, aphasia, nystagmus, visual disturbances, incontinence, back pain, radicular pain,
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660 Chapter 31 Whipple’s Disease (Intestinal Lipodystrophy)

atrophy, cerebellar signs, balance deficits, falls, such tremor, rigidity, drooling, difficulty with
PROBLEMS OF COGNITION, COMMUNICATION, AND BEHAVIOR

and cranial nerve involvement.2 speech, and abrupt personality change.


Background Information Background Information
Types of this condition include myeloma, Symptoms of Wilson’s disease usually appear
neurofibroma, lymphoma, metastasis, menin- in childhood or young adult life7 with a fre-
gioma, schwannoma, and astrocytoma. The quency of 1 in 30,000.128 This condition is an
first test to diagnose brain and spinal column autosomal recessive disorder in which exces-
tumors is a neurological examination. Special sive amounts of copper accumulate in the
imaging techniques (computed tomography, body especially in eye membranes (causing a
magnetic resonance imaging, and positron golden brown Kayser-Fleischer ring), nailbeds,
emission tomography) are also employed. and kidney.1 The gene abnormality has been
Specific diagnoses may be confirmed with located on chromosome 13, which causes a de-
imaging and biopsy. Treatment is variable ficiency in ceruloplasmin. In the central nerv-
depending on the type, size, and location of ous system, cavitation and neuronal loss occur
the tumor and may include surgical resec- within the putamen and globus pallidus. This
tion, chemotherapy, radiation, corticos- condition is diagnosed on clinical findings
teroids, and rehabilitative therapies. Progno- supported by biochemical evidence of low
sis is variable and depends on the type and ceruloplasmin, elevated unbound serum cop-
grade of tumor, severity of compression, and per, high urinary copper excretion, positive
duration of compression. liver biopsy and copper metabolism tests, and
T2-weighted magnetic resonance imaging that
■ Whipple’s Disease (Intestinal shows thalamus and putamen hyperintensity.1
Lipodystrophy) Treatment consists of low-copper diet and a
Chief Clinical Characteristics chelating agent (usually penicillamine).1 With
This presentation includes dementia, personal- adequate treatment, the life span is normal. If
ity changes, ataxia, myoclonus, nystagmus, and left untreated in children, death is predicted in
visual loss. The presentation also includes 2 years from hepatic and renal failure. In un-
weight loss, arthropathy, diarrhea, and abdom- treated adults, death is predicted in 10 years.1
inal pain. Some individuals may experience a
low-grade fever, lymphadenopathy, hyperpig- References
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CHAPTER 32
Stiffness
■ Claire Smith, PT, DPT, NCS ■ Beth E. Fisher, PT, PhD

Description of the Symptom Special Concerns


■ A change in prior level of stiffness, including
This chapter describes pathology that may lead but not limited to:
to stiffness of the body. Stiffness is both an ob- ■ Increase in spasticity
jective event that can be perceived by a clinician ■ Change in resting muscle tone
and a subjective phenomenon that is perceived ■ A new onset of stiffness not associated with
by the individual. The increased resistance to original purpose of the physical therapy
movement a therapist feels while passively mov- visit. This may include:
ing a limb of his or her patient might be ■ Spasticity
described as stiffness. Additionally, stiff might ■ Clonus
refer to a patient’s description of how the limbs ■ Rigidity
feel when he or she attempts to actively move or ■ Hypertonicity
bend. Subjective stiffness may arise from issues ■ Hypotonicity
with motor sequencing and recruitment, coor- ■ Stiffness that is not of musculoskeletal
dination, and the length of passive structures. origin

Conceptual overview of conditions that lead to stiffness

“Stiffness”

Passive Active

Increased Abnormal Abnormal


Response Timing Recruitment

Clonus Rigidity Spasticity Hypertonicity

665
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666 Chapter 32 Stiffness

CHAPTER PREVIEW: Conditions That May Lead to Stiffness


STIFFNESS

T Trauma
COMMON
Overuse syndromes (repetitive motion disorders, cumulative trauma disorders, repetitive stress
injuries) 675
Traumatic brain injury 678
Traumatic spinal cord injury 678
Whiplash injury (whiplash-associated disorder) 680
UNCOMMON
Not applicable
RARE
Not applicable

I Inflammation
COMMON
Aseptic
Encephalitis 670
Meningitis:
• Bacterial meningitis 673
• Viral meningitis 674
Vasculitis (giant cell arteritis, temporal arteritis, cranial arteritis) 679
Septic
Not applicable
UNCOMMON
Aseptic
Behçet’s disease 669
Septic
Not applicable
RARE
Aseptic
Neuromyotonia (Isaac syndrome, Isaac-Merten syndrome, continuous muscle fiber activity syndrome,
quantal squander syndrome) 675
Opsoclonus myoclonus (Kinsbourne syndrome, myoclonic encephalopathy of infants, dancing
eyes–dancing feet syndrome, opsoclonus-myoclonus-ataxia syndrome) 675
Septic
Acute disseminated encephalomyelitis 669
Lyme disease (tick paralysis) 673

M Metabolic
COMMON
Not applicable
UNCOMMON
Neuroleptic malignant syndrome 675
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Chapter 32 Stiffness 667

Metabolic (continued)

STIFFNESS
RARE
Fahr’s syndrome (familial idiopathic basal ganglia calcification, bilateral striopallidodentate calcinosis) 671
Gaucher’s disease 671
Wilson’s disease 680

Va Vascular
COMMON
Stroke (cerebrovascular accident) 677
UNCOMMON
Cerebral aneurysm 669
Spinal cord infarction (vascular myelopathy) 677
RARE
Not applicable

De Degenerative
COMMON
Dementia with Lewy bodies 670
Parkinson’s disease 676
UNCOMMON
Huntington’s disease 672
RARE
Choreoacanthocytosis (Levine-Critchley syndrome, neuroacanthocytosis) 670
Hallervorden-Spatz syndrome (neurodegeneration with brain iron accumulation, pantothenate
kinase-associated neurodegeneration) 672
Hereditary spastic paraplegia (familial spastic paralysis) 672
Machado-Joseph disease 673
Multiple system atrophy (striatonigral degenerative, olivopontocerebellar atrophy, Shy-Drager
syndrome) 674
Primary lateral sclerosis 676
Stiff-person syndrome 677

Tu Tumor
COMMON
Not applicable
UNCOMMON
Malignant Primary:
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Hydromyelia (syringomyelia) 672
(continued)
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668 Chapter 32 Stiffness

Tumor (continued)
STIFFNESS

RARE
Malignant Primary, such as:
• Brain primary tumors 679
• Spinal primary tumors 679
Malignant Metastatic, such as:
• Brain metastases 678
• Spinal metastases 679
Benign:
Not applicable

Co Congenital
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Paroxysmal choreoathetosis 676

Ne Neurogenic/Psychogenic
COMMON
Epilepsy/seizure disorder 671
UNCOMMON
Myotonia congenital 674
RARE
Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Stiffness The pathophysiological basis of these


passive processes is incompletely understood.
Clinical assessment of stretch reflex dysfunc- The changes in muscle tone (hypertonicity)
tion has generally been a key assessment of probably result from alterations in the balance
“stiffness” and has focused on spasticity. The of inputs from reticulospinal and other descend-
precise definition of spasticity is a velocity- ing pathways to the motor and interneuronal
dependent increase in the tonic stretch reflex circuits of the spinal cord, and the absence of
with exaggerated tendon jerks resulting from an intact corticospinal system. Loss of descend-
hyperexcitability of the stretch reflex as one ing tonic or phasic excitatory and inhibitory
component of the upper motor neuron syn- inputs to the spinal motor apparatus, alter-
drome.1,2 In addition to increased stretch ations in the segmental balance of excitatory
reflexes, hypertonicity, defined as increased and inhibitory control, denervation supersen-
resistance to passive movement, and paresis sitivity, and neuronal sprouting may be
are other signs collectively called the upper observed. Once hypertonicity is established,
motor neuron syndrome. Upper motor neuron the chronically shortened muscle may develop
syndrome results from damage to descending physical changes, such as shortening and con-
motor pathways at cortical, brainstem, or tracture, that further contribute to muscle
spinal cord levels. stiffness. The pathophysiological mechanisms
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Chapter 32 Cerebral Aneurysm 669

causing the increase in stretch reflexes in spas- administered over a course of 3 to 5 days.5 The

STIFFNESS
ticity also are not well understood.3 prognosis is variable depending on the severity
Spasticity is a form of muscle overactivity. of the disease and acuity of the diagnosis.
Spasticity is often used as an umbrella term to
describe other forms of muscle overactivity ■ Behçet’s Disease
that often occur. However, each of the follow- Chief Clinical Characteristics
ing represents a distinct pathophysiological This presentation may include bilateral pyram-
mechanism and it is inaccurate to interchange idal signs (eg, weakness, hyperreflexia in the
these terms even though it is commonly done deep tendon reflexes, hypertonia, clonus, and/or
in the clinic (particularly the terms spasticity a positive Babinski sign), headache, memory
and hypertonicity).2 Other forms of muscle loss, hemiparesis, cerebellar ataxia, balance
overactivity that may be part of the upper mo- deficits, sphincter dysfunction, or cranial nerve
tor neuron syndrome are: palsies. In addition to these neurological signs
● Spasms, or strong and sustained contrac- individuals with this condition also may pres-
tions of muscles, which are often painful ent with arthritis; renal, gastrointestinal, vas-
● Increased reflexes, in which the normal cular, and cardiac diseases; and genital, oral, and
reflexes (such as knee extension in response cutaneous ulcerations.6
to tapping) are greatly exaggerated Background Information
● Clonus, which refers to self-sustaining oscil- Mean age of onset is in the third decade of life.
lating movements around a joint elicited Diagnostic criteria according to an international
with quick stretch study group include presence of recurrent
● Hypertonicity, or an abnormal increase in oral ulceration, recurrent genital ulceration, eye
resting muscle tension lesions, skin lesions, papulopustular lesions,
● Rigidity, which describes involuntary bidi- and/or a positive pathergy test.2,6 Medical treat-
rectional non–velocity-dependent resistance ment typically consists of corticosteroids and
to movement. immunosuppressants. Neurological symptoms
tend to clear within weeks, but can sometimes
Description of Conditions That recur or result in permanent deficits.2 Negative
May Lead to Stiffness prognostic factors include onset before the age
of 25 and male sex.
■ Acute Disseminated
Encephalomyelitis ■ Cerebral Aneurysm
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation may involve confusion, som- This presentation may involve loss of balance in
nolence, and convulsion in its encephalitic form. combination with a whole host of other neuro-
Individuals with this condition may report logical symptoms and signs that depend on the
headache and neck stiffness. Initial symptoms affected cerebral tissue, including visual and
of the myelitic form include weakness and proprioceptive loss. Any associated signs or
sensory impairments.2 symptoms may not be reported due to the fact
that this condition is typically asymptomatic
Background Information prior to rupture. However, if the aneurysm
This condition is a demyelinating disease of the results in a mass effect, ischemia, or hemor-
central nervous system,4 which may be due to an rhage, then neurological signs and symptoms are
immune-mediated complication of infection.2 dependent on the affected location.2,7,8
The presence of upper motor neuron signs,
cerebrospinal fluid pleocytosis and elevated Background Information
protein, and multiple white matter lesions There has been some description of genetic
demonstrated on magnetic resonance imaging factors in this condition.7 Cigarette smoking,
supports the diagnosis.5 Definitive diagnosis hypertension, and heavy alcohol use have all
requires a brain biopsy. The primary goal of been found to be correlated with increased risk
treatment is to suppress the immune response; of aneurysm development.7,8 Factors associated
thus high-dose corticosteroids are generally with increased risk of rupture include size of
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670 Chapter 32 Choreoacanthocytosis (Levine-Critchley Syndrome, Neuroacanthocytosis)

aneurysm, location in the posterior circulation, visuospatial problems and executive dysfunc-
STIFFNESS

and a previous history of aneurismal subarach- tion, visual hallucinations, and parkinsonism
noid hemorrhage.9,10 Definitive diagnosis is features such as masked facies, autonomic dys-
based on catheter angiography; however, mag- function, rigidity, and bradykinesia. Other signs
netic resonance angiography, magnetic reso- and symptoms may include postural instabil-
nance imaging, and computed tomography may ity, falls, sleep disturbances, memory problems,
aid in the diagnosis. Unruptured aneurysms are syncope, transient loss of consciousness, and
sometimes surgically treated. Aneurysm size, sensitivity to antipsychotic and anti-Parkinson
location, and prior history of a subarachnoid medications.14
hemorrhage help to determine if the risk of sur-
gical treatment is worth the potential benefits. Background Information
Most aneurysms that have hemorrhaged must This progressive condition is the second
be treated surgically. Patients with a previous most common dementia after Alzheimer’s
rupture are at an 11 times greater risk of having disease.14 The specific etiology of this condi-
a second intracranial aneurysm rupture. When tion is unknown. The characteristic Lewy
aneurysms do rupture, many patients die within bodies are eosinophilic inclusion bodies
one month of the rupture, and those who sur- found within the cytoplasm of neurons in
vive often have residual neurological deficits.7 the cerebral cortex and limbic system.14
A thorough clinical examination, laboratory
■ Choreoacanthocytosis screen, and imaging are important to rule
(Levine-Critchley Syndrome, out other causes of dementia. The definitive
Neuroacanthocytosis) diagnosis for this condition is made post-
Chief Clinical Characteristics mortem; however, it appears that the use of
This presentation commonly includes chorea, single-photon emission computed tomogra-
motor or vocal tics, dystonia, orofacial dyskine- phy and positron emission tomography may
sias, and parkinsonism. Seizures, cognitive im- be useful in the identification of occipital
pairment, psychosis, paranoia, and personality hypoperfusion which may be associated with
changes are also seen with this diagnosis, along the visual hallucinations.14,15 Management
with hyporeflexia and distal myopathy due to includes caregiver education to assist in
denervation of muscles.11–13 minimizing factors that may contribute to
problematic behaviors. Medication therapy
Background Information may be indicated, but should be monitored
This condition is a rare, autosomal recessive dis- closely due to potential exacerbation of
order that typically has its onset during the third symptoms.14 Life expectancy for individuals
and fourth decades of life.13 This neurodegener- with this condition is similar to that of
ative disorder is associated with acanthocytes, Alzheimer’s disease. The average survival
aberrant spiky or thorny red blood cells, as time is between 6 and 8 years from the onset
well as atrophy and gliosis of the caudate, puta- of dementia.14,16
men, and globus pallidus.12 Diagnosis is made
by a combination of tests, including clinical ■ Encephalitis
features, lab work demonstrating acanthocyto- Chief Clinical Characteristics
sis, neuroimaging, and genetic testing to rule out This presentation includes confusion, delirium,
Huntington’s disease.11 There is currently no convulsions, problems with speech or hearing,
effective, long-term treatment, although vera- memory loss, hallucinations, drowsiness,
pamil has been found to provide temporary and coma. Headache or back stiffness may
reduction of symptoms. Life expectancy is be present.
reduced, and suicidal action or ideation is not
uncommon due to cognitive impairments.13 Background Information
Encephalitis is an inflammation of nerve
■ Dementia with Lewy Bodies cells in the brain. This term usually refers to
Chief Clinical Characteristics the viral form, although bacterial, parasitic,
This presentation can be characterized by fluc- and fungal agents also can cause this condition.
tuating cognitive dysfunction, particularly Up to 20,000 new cases of viral forms of this
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Chapter 32 Gaucher’s Disease 671

condition are reported annually in the ■ Fahr’s Syndrome (Familial

STIFFNESS
United States. Diagnosis is established by Idiopathic Basal Ganglia
clinical presentation suggesting dysfunction Calcification, Bilateral
of the cerebrum, brainstem, or cerebellum, Striopallidodentate Calcinosis)
cellular reaction and elevated protein in Chief Clinical Characteristics
spinal fluid, and possible demonstration of This presentation may involve features of
diffuse edema or enhancement of the brain parkinsonism, such as chorea, athetosis, rigid-
on magnetic resonance imaging or computed ity, dystonia, and tremor in addition to cogni-
tomography. Treatment is primarily pharma- tive impairments, cerebellar impairments, gait
cologic, with drugs such as corticosteroids, and balance disorder, psychiatric features, pain,
antiviral agents, and anticonvulsants. The pyramidal signs, such as weakness, hyperreflexia
majority of individuals with encephalitis do in the deep tendon reflexes, hypertonia, clonus,
recover, but irreversible brain damage and and/or a positive Babinski, sensory changes and
death can result.2 speech disorder.20

■ Epilepsy/Seizure Disorder Background Information


This condition occurs due to bilateral sym-
Chief Clinical Characteristics metric calcification of the basal ganglia with or
This presentation is variable and can range without calcification of the dentate nucleus.
from abnormal sensations or behavior to The disease has been described as both famil-
convulsions, muscles spasms, and loss of ial and nonfamilial.2 Diagnosis is established
consciousness. Specific cognitive symptoms using computed tomography or magnetic
include problems with perception, attention, resonance imaging of the brain; however, com-
emotion, and memory.17 puted tomography has been found to be more
sensitive for identifying calcium deposits.21
Background Information
Treatment aimed at minimizing calcium
According to the International League Against
deposits has been unsuccessful.22 Individuals
Epilepsy an epileptic seizure is defined as “a
with this condition may be responsive to
transient occurrence of signs and/or symp-
levodopa for treatment of their parkinsonian
toms due to abnormal excessive or synchro-
features.2
nous neuronal activity in the brain.”17 The
diagnosis of epilepsy includes a history of ■ Gaucher’s Disease
at least one seizure with the “associated
Chief Clinical Characteristics
neurobiological, cognitive, psychological,
This presentation commonly includes slowly
and social disturbances.”17 Seizures are also
progressive mental decline, seizures, ataxia,
classified into different categories based on
and, upon later development, weakness with
presentation and electroencephalogram fea-
spasticity and splenomegaly and deficits in
tures. Approximately 1% of the U.S. popula-
lateral gaze.2
tion will have epilepsy before 20 years of age,
although the incidence increases slightly Background Information
after age 60.2 Most seizures can be controlled This condition is a rare disorder, although it is
pharmacologically with antiepileptic drugs. prevalent among the Ashkenazi Jewish popu-
Surgical treatment is indicated in patients lation.23 The disease is an autosomal genetic
with recurrent seizures that arise from a sin- disorder in which glucocerebroside accumulates
gle focus and prevent a normal lifestyle.18 in the spleen, liver, lungs, bone marrow, and
The prognosis for a patient with epilepsy brain due to a deficiency in an enzyme.2 There
depends on the cause of the seizures and are three types of Gaucher’s disease.
treatment. Remission occurs in only 16% to The most common, type 1, is characterized by
43% of patients with partial seizures.19 Less no central nervous system involvement. In
favorable prognosis is also associated with type 2, infants have extensive and progressive
multiple seizure types, associated neurologi- neurological damage.23 Type 3 is less common
cal deficits, and behavioral or psychiatric and is associated with less severe neurological
disturbance.19 symptoms.23 Diagnosis is established by
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672 Chapter 32 Hallervorden-Spatz Syndrome

clinical presentation, laboratory tests that abnormalities such as dementia, mental retar-
STIFFNESS

show increase in total acid phosphatase and dation, epilepsy, extrapyramidal disturbance,
biopsy of bone marrow that is positive for ataxia, deafness, retinopathy, optic neuropathy,
Gaucher cells. Enzyme replacement therapy is peripheral neuropathy, and skin lesions. The
standard for most patients with types 1 and 3. uncomplicated form is indicated by the absence
However, there is no effective treatment for of these features.27 This condition is a group of
the severe brain damage that may occur in inherited disorders with a primary pathologi-
patients with types 2 and 3. Prognosis for cal feature of axonal degeneration of the distal
patients with type 2 disease is poor with death ends of the longest ascending and descending
within the first 2 years of life. For type 3 dis- tracts, resulting in the characteristic spastic-
ease, symptoms typically present in childhood ity.26,27 Diagnosis is made by the presence of
and death occurs by age 10 to 15 years.2 symptoms of gait disturbance, neurological
findings of corticospinal tract deficits in the
■ Hallervorden-Spatz Syndrome lower extremities, family history or genetic
(Neurodegeneration with Brain testing, and exclusion of any other disorders
Iron Accumulation, Pantothenate that could account for the symptoms.26
Kinase-Associated Current treatment is limited to decreasing
Neurodegeneration) muscle spasticity through exercise and med-
Chief Clinical Characteristics ication. Physical therapy is beneficial for
This presentation includes dystonia, parkin- the maintenance and improvement of muscle
sonism, choreoathetosis, spasticity, cognitive flexibility, muscle strength, gait, and cardiovas-
impairment, corticospinal tract involvement, cular fitness.26 Prognosis varies, and those
optic atrophy, and pigmentary retinopathy.24 with an onset after adolescence are more likely
to have insidious worsening.26
Background Information
This rare, inherited disorder results from a ■ Huntington’s Disease
genetic mutation in the iron regulatory path-
ways, resulting in excessive iron accumulation Chief Clinical Characteristics
in the basal ganglia.24,25 Diagnosis is made by This presentation involves progressive chorea
magnetic resonance imaging, which shows of the entire body, emotional disturbances such
characteristic abnormalities in the basal ganglia, as behavior and personality changes, and
known as the “eye of the tiger” sign. Pathologi- dementia.28–30
cal studies may also show brown discoloration, Background Information
iron pigmentation, and gliosis in the globus This autosomal dominant genetic disorder
pallidus and substantia nigra.24 There is no cure causes selective neurodegeneration, most com-
or effective treatment for the condition, but monly in the neostriatum. Diagnosis is made
patients may benefit from rehabilitation thera- by genetic testing.30 There is currently no treat-
pies to decrease disability as the disease follows ment to slow or stop the progression of this
its progressive course of degeneration.25 condition; care is focused on symptom man-
■ Hereditary Spastic Paraplegia agement and optimization of functioning.30
(Familial Spastic Paralysis) The prognosis for Huntington’s disease is poor,
and individuals usually experience very rapid
Chief Clinical Characteristics decline. On average, patients survive for 15 to
This presentation includes insidious, progres- 20 years after initial diagnosis, but require high
sive difficulty with walking due to bilateral, levels of care and supervision during those
symmetric lower extremity spastic weakness. years.30
Patients may report tripping, stumbling, and
falling, as well as urinary urgency.26 ■ Hydromyelia (Syringomyelia)
Background Information Chief Clinical Characteristics
This condition can be divided into two types. This presentation involves insidious onset of
The complicated form of this condition is symptoms including upper and lower extrem-
indicated by the presence of neurological ity weakness and numbness, lower extremity
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Chapter 32 Bacterial Meningitis 673

stiffness, and less commonly, pain. Trauma Background Information

STIFFNESS
usually precedes the onset of symptoms, but the This condition is genetic, with an autosomal
time frame for subsequent development of these dominant pattern of inheritance and onset of
symptoms is variable. symptoms in adolescence or young adulthood.
Differential diagnosis includes Parkinson’s
Background Information
disease and multiple system atrophy. The pres-
This condition is caused by an abnormal widen-
ence of ataxia decreases the likelihood of
ing of the central canal of the spinal cord, lead-
Parkinson’s, and the early age of onset and
ing to the accumulation of cerebrospinal fluid
visual symptoms decrease the likelihood of
and hydrocephalus. Differential diagnosis must
multiple system atrophy. Diagnosis is established
be made between hydromyelia and other disor-
by clinical symptoms and magnetic resonance
ders such as syringomyelia, spinal cord tumor,
imaging findings of reduced width of superior
and spinal arteriovenous malformation. Mag-
and middle cerebellar peduncles, atrophy of
netic resonance imaging and electromyography
the frontal and temporal lobes, and decreased
are used to confirm the diagnosis of this condi-
size of the pons and globus pallidus. There is
tion. Surgery may be indicated to decrease or
no treatment for this condition, and prognosis
eliminate the symptoms. Prognosis is variable.
is poor.2
■ Lyme Disease (Tick Paralysis)
MENINGITIS
Chief Clinical Characteristics
This presentation can include fluctuating signs ■ Bacterial Meningitis
or symptoms such as headache, neck stiffness, Chief Clinical Characteristics
nausea, vomiting, malaise, fever, pain, fatigue, This presentation includes acute symptoms
and presence of a “bull’s-eye” rash.2 Over time, such as fever, severe headache, neck stiffness,
additional symptoms may include sensory seizures, changes in consciousness, facial and
changes, irritability, cognitive changes, depres- ocular palsies, positive Kernig and Brudzin-
sion, behavioral changes, seizures, ataxia, chorea ski signs, and possible hemiparesis, which may
movements, pain, weakness, balance deficits, lead to falls. Chronic symptoms include hydro-
arthritis, and cranial nerve involvement.2,31 cephalus, vomiting, immobility, impaired
alertness, hemiplegia, decorticate or decere-
Background Information brate posturing, cortical blindness, stupor,
Borrelia burgdorferi, an organism that infects or coma.29
ticks, is responsible for the transmission of this
condition to a human host.2 The diagnosis is Background Information
confirmed with a thorough history, clinical Bacterial meningitis results from an infec-
assessment, enzyme-linked immunosorbent tion and inflammation of the meninges sur-
assay, and Western blot or immunoblot analysis. rounding the brain and spinal cord. Lumbar
In addition, magnetic resonance imaging or puncture for spinal fluid pressure and cere-
computed tomography may reveal multifocal or brospinal fluid culture, blood cultures, and
periventricular cerebral lesions.2 Medical man- radiologic studies confirm the diagnosis.29
agement includes treatment with oral tetracy- This condition is considered a medical
cline, penicillin, or intravenous ceftriaxone.2 emergency. Medical management includes
Many individuals experience full recovery with maintenance of blood pressure, treatment
treatment; however, residual deficits may persist for septic shock, and administration of in-
for individuals with chronic Lyme disease.2 travenous antibiotics for 10 to 14 days 29
Prognosis depends on the strain of bacteria;
■ Machado-Joseph Disease approximately 5% to 15% of patients with
Chief Clinical Characteristics bacterial meningitis do not survive.29 Resid-
This presentation may involve visual impair- ual effects after the infection resolves are
ments such as nystagmus in combination with variable and patients with pneumococcal
slowly progressive ataxia, rigidity, dystonia, and H. influenzae bacterial meningitis are
weakness in the hands and feet, and difficulty more likely to have lasting neurological
with respiration and swallowing. deficits.29
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674 Chapter 32 Viral Meningitis

■ Viral Meningitis that rarely occurs without orthostatic hypoten-


STIFFNESS

Chief Clinical Characteristics sion. There are three types of this condition.
This presentation can be characterized by an The parkinsonian-type includes symptoms of
acute onset of fever, headache, and neck stiff- Parkinson’s disease such as slow movement,
ness. Drowsiness, lethargy, and irritability stiff muscles, and tremor. The cerebellar type
may occur, but overall symptoms tend to be causes problems with coordination and
relatively mild.29 Although not a common speech. The combined type includes symp-
presenting sign, falls or imbalance may occur toms of both parkinsonism and cerebellar
due to drowsiness and lethargy. failure. Older age at onset is associated with
a shorter survival time.35 Average age of onset
Background Information is 54 years, with mean age at death being
This condition is an infection and inflamma- 60.3 years.33 Most individuals with this condi-
tion of the meninges surrounding the brain tion receive a trial of levodopa although only a
and spinal cord. Also known as aseptic minority respond.33 Additional treatment
meningitis, this condition is most commonly addresses symptoms and involves physical
caused by the echovirus or coxsackie virus.29 and occupational therapy to maintain mobil-
Cerebrospinal fluid analysis and blood work ity and address safety issues related to the
are used to determine the diagnosis.32 There progression of imbalance.
is no specific treatment, although supportive
care may include administration of anal- ■ Myotonia Congenital
gesics. This condition is rarely fatal and most Chief Clinical Characteristics
patients demonstrate a full recovery.29 This presentation includes impaired relaxation
of a muscle after forceful voluntary contrac-
■ Multiple System Atrophy tion. Individuals with this condition may also
(Striatonigral Degeneration, experience some degree of hypertrophy of
Olivopontocerebellar Atrophy, involved muscle groups.29
Shy-Drager Syndrome)
Chief Clinical Characteristics Background Information
This presentation involves tremor, rigidity, There are many different variants of this
akinesia, and/or postural imbalance along condition, all of which are thought to be the
with signs of cerebellar, pyramidal, and auto- result of mutations in the genes coding for
nomic dysfunction. Autonomic symptoms such skeletal muscle sodium channels and chlo-
as orthostatic hypotension, dry mouth, loss of ride channels.36 Of the different types, the
sweating, impotence, and urinary inconti- two most common are Thomsen’s disease
nence or retention are the initial feature in and Becker’s disease. Thomsen’s disease
41% of individuals, with 74% to 97% of in- begins early in life and is characterized by
dividuals developing some degree of auto- myotonia, muscular hypertrophy, a nonpro-
nomic dysfunction during the course of the gressive course, and dominant inheritance.
disease.33 This condition is a combination of Becker’s disease is recessive, with a later
parkinsonian and non-parkinsonian symp- onset in life, and patients present with more
toms and signs. severe myotonia and hypertrophy, and often
with weakness and atrophy of the forearm
Background Information and neck muscles.29 Becker’s disease typi-
Diagnostic criteria are based on the clinical cally has a progressive course until about
presentation, which includes poor response 30 years of age. Medical management may
to levodopa, presence of autonomic features, include quinidine sulfate, procainamide, or
presence of speech or bulbar problems, ab- mexiletine to relieve myotonia. Patients may
sence of dementia, absence of toxic confusion, also benefit from what is known as the
and presence of falls.34 The disease course “warm-up effect,” which refers to repeated
ranges between 0.5 and 24 years after diagno- contractions of a muscle that can reduce or
sis with a mean survival time of 6.2 years.35 abolish myotonia. This effect is short lived
This condition is a progressive condition of and wears off after approximately 5 minutes
the central and autonomic nervous systems of rest.29
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Chapter 32 Overuse Syndromes 675

■ Neuroleptic Malignant Syndrome autoimmune or paraneoplastic origin.39 Elec-

STIFFNESS
Chief Clinical Characteristics trophysiological study is used in diagnosis
This presentation can be characterized by severe and will show myokymic and neuromyotonic
muscle rigidity, changes in mental status such discharges.38 Muscle activity persists throughout
as confusion and delirium, high fever, diaphore- sleep and anesthesia, but can be blocked by
sis, dysphagia, incontinence, and autonomic curare.29 Symptomatic relief has been demon-
dysfunction. strated with anticonvulsant drugs such as
phenytoin and carbamazepine, and research
Background Information has also shown successful outcomes with
This condition is a rare, but potentially fatal plasmapharesis.40
disorder that is caused by a complication of
treatment with neuroleptic medication.37 The ■ Opsoclonus Myoclonus
pathophysiology of this condition is unknown, (Kinsbourne Syndrome,
but is thought to be related to a dopaminergic Myoclonic Encephalopathy of
transmission block in the basal ganglia and Infants, Dancing Eyes–Dancing
hypothalamus.37 Diagnosis is made by a tem- Feet Syndrome, Opsoclonus-
perature of 38ºC or higher, rigidity, mental sta- Myoclonus-Ataxia Syndrome)
tus and autonomic changes, and laboratory Chief Clinical Characteristics
findings, as well as a recent history of exposure This presentation includes irregular, rapid eye
to antipsychotic medication. The best treat- movements (opsoclonus) and brief, shock-like
ment for this condition is prevention, including muscle spasms (myoclonus), as well as stagger-
early recognition of symptoms and prompt ing, falling, ataxia, drooling, decreased muscle
discontinuation of the causal agent. Medical tone, and an inability to sleep.41
management of this condition in its acute form
includes fluid replacement, reduction of tem- Background Information
perature, and monitoring of cardiac, pulmonary, This autoimmune disorder is commonly
and renal functions. Patients may also benefit found in association with the presence of neo-
from electroconvulsive therapy and the intro- plasm, the most common types being neurob-
duction of dopamine agonists and/or dantro- lastoma in children, and breast and small-cell
lene. The clinical course varies, and most lung cancer in adults.29 Diagnosis is made by a
patients eventually experience a total resolution combination of clinical presentation, the pos-
of symptoms. sible presence of neoplasm, the presence of
anti-Hu antibodies, mild pleocytosis on cere-
■ Neuromyotonia (Isaac Syndrome, brospinal fluid, and positive serologic tests in
Isaac-Merten Syndrome, children without tumors.29 Treatment includes
Continuous Muscle Fiber Activity adrenocorticotropic hormone, corticosteroid,
Syndrome, Quantal Squander and intravenous immunoglobulin therapy,
Syndrome) and, when present, tumor resection and adju-
Chief Clinical Characteristics vant treatment such as chemotherapy and
This presentation commonly involves intermit- radiation.29,41 There is good response to drug
tent or continuous muscle contraction, slow and surgical treatment, but relapse is common
relaxation following muscle contraction, and many patients report residual neurological
cramps, stiffness, and hyperhidrosis. These symptoms.29,41
symptoms are commonly seen in conjunction ■ Overuse Syndromes (Repetitive
with peripheral neuropathy.38,39 Motion Disorders, Cumulative
Background Information Trauma Disorders, Repetitive
This condition is an antibody-mediated potas- Stress Injuries)
sium channel disorder, in which the suppression Chief Clinical Characteristics
of these voltage-gated channels results in hyper- This presentation includes pain, tingling,
excitability of the peripheral nerve.38 It is a rare, numbness, swelling, erythema, loss of flexibil-
usually acquired disease that is often seen with ity, and/or weakness in the affected body
myasthenia gravis and is most likely due to an area.42
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676 Chapter 32 Parkinson’s Disease

Background Information autonomic symptoms, and sexual dysfunction.


STIFFNESS

Overuse syndromes are a group of disorders Surgical management also may be considered,
that result from the performance of repetitive including deep brain stimulation or pallido-
motions. Common pathologies that are associ- tomy.44 Forced use or higher intensity, challeng-
ated with this condition include carpal tunnel ing activities may provide a neuroprotective
syndrome, trigger finger, and epicondylitis.42 In benefit for individuals with early forms of this
addition, bursitis, tendonitis, and tenosynovitis condition.45 With the high incidence of depres-
may occur at numerous different locations sion, consultation with a psychologist or psychi-
throughout the body, depending on the type of atrist may be warranted. Individuals with a late
activity performed. Treatment varies, but usu- onset tend to progress more rapidly.46,47 Poor
ally begins with stopping the motions or activi- prognostic indicators for disability include
ties that cause the symptoms. Physical therapy initial presentation without tremor, early
is often indicated for stretching, strengthening, dependence, dementia, balance impairments,
splinting, and therapeutic modalities such as ice older age, and the postural instability/gait
and ultrasound. Further medical management difficulty dominant type.46,47
may include analgesics, corticosteroids, and
even surgery.42 If this condition is suspected, ■ Paroxysmal Choreoathetosis
the reader is encouraged to review the appro- Chief Clinical Characteristics
priate chapter discussing diagnosis of pain This presentation includes discrete episodes of
within the affected body region. abnormal involuntary movements of the limbs,
trunk, and facial muscles. Some patients report
■ Parkinson’s Disease lingering muscle stiffness following the attacks.48
Chief Clinical Characteristics The episodes of choreoathetosis may be pro-
This presentation commonly involves resting voked by startle, sudden movement, hyperven-
tremor, bradykinesia, rigidity, and postural tilation, alcohol, coffee, fatigue, or prolonged
instability. Falls are a common problem in exercise. They vary in duration from 10 seconds
individuals with this condition, with up to to up to 4 hours at a time, and may occur dozens
68% falling within a 1-year period and approx- of times per day or only occasionally.29
imately 50% of these individuals falling Background Information
multiple times within that same year.43 Other The exact pathological mechanism of this con-
common signs and symptoms include festi- dition is unknown, but it is thought that the
nation, freezing, micrographia, hypophonia disorder may be genetically linked, or due to a
(hypokinetic dysarthria), akinesia, masked secondary cause such as neurological or meta-
facies, drooling, difficulty turning over in bed, bolic disease.29,48 Individuals with this condi-
dystonia, dyskinesia, dementia, and depression.2 tion tend to respond well to antiepileptic drugs
Background Information and, overall, this condition has been shown to
This condition occurs due to the depletion or spontaneously improve as patients move into
injury of dopamine-producing cells in substan- adulthood.48
tia nigra pars compacta. Clinical signs and ■ Primary Lateral Sclerosis
symptoms are not typically present until after
approximately 80% of dopamine-producing Chief Clinical Characteristics
cells are lost. The definitive diagnosis is made This presentation includes spastic paraparesis of
post-mortem. However, a clinically definitive voluntary muscles with associated upper motor
diagnosis may be made with the presence of at neuron signs in the absence of lower motor neu-
least two of the three criteria—asymmetric ron signs. Onset of difficulty with gait, balance,
resting tremor, bradykinesia, or rigidity—and a and leg weakness appears in the fifth to sixth
positive response to anti-Parkinson medica- decade of life and may progress to affect upper
tions.2,44 Imaging may be useful to exclude extremity and facial musculature.
vascular involvement. Medical management Background Information
may include use of dopamine agonists, levodopa, Imaging, cerebrospinal fluid analysis, and elec-
and other medications to address nonmotor tromyographic studies confirm the diagnosis.
symptoms, such as depression, constipation, In particular, this condition is differentiated
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Chapter 32 Stroke (Cerebrovascular Accident) 677

from the more severe amyotrophic lateral scle- Background Information

STIFFNESS
rosis only after a period of 3 years from onset This rare, chronic condition is thought to be the
to ensure the absence of lower motor neuron result of an autoimmune process that
signs.2 Treatment for this condition addresses results in continuous motor unit activity.2 Stiff-
symptom management. Physical therapy is person syndrome is characterized by an insidi-
indicated to prevent immobility. This condi- ous onset and follows a progressive course for
tion is not fatal and progression of symptoms months to years before it stabilizes. Diagnosis of
varies; some individuals maintain ambulatory this condition is made by clinical picture, the
status throughout life while others become demonstration of continuous muscle activity in
wheelchair-bound. affected muscles despite an effort to relax, and
enhanced exteroceptive reflexes. Many patients
■ Spinal Cord Infarction (Vascular also present with antibodies to glutamic acid
Myelopathy) decarboxylase, but the role of these antibodies is
Chief Clinical Characteristics not yet fully understood. Treatment options
This presentation depends on the location of include diazepam or baclofen, as well as immune-
the infarction. The most common anterior spinal directed therapy such as plasma exchange, corti-
artery syndrome presents with bilateral, incom- costeroids, or intravenous immunoglobulin.29,51
plete motor paralysis and loss of pinprick and
temperature sensation below the level of the ■ Stroke (Cerebrovascular Accident)
lesion, loss of deep tendon reflexes, sphincter Chief Clinical Characteristics
paralysis, acute complaint of back pain, and This presentation may include a wide range of
spared proprioception. Onset of symptoms symptoms that correspond to specific areas of
occurs over the course of minutes to hours. the brain that are affected, potentially includ-
ing visual disturbances. The initial symptoms
Background Information
can include numbness or weakness, especially
Most forms of this condition are insidious,
on one side of the body or face; confusion or apha-
or secondary to aortic surgery or rupture,
sia; balance deficits or falls; or sudden severe
atherosclerosis, aneurysm, or an acute deficit
headache with no known cause.
of perfusion. Diagnosis is often difficult at
initial presentation, because magnetic reso- Background Information
nance imaging may be negative. Repeat This condition occurs when blood flow to the
magnetic resonance imaging may show T2 brain is interrupted either by blockage (ischemia
hyperintensity secondary to edema.49 This or infarction) or from hemorrhagic disruption.
condition has a severe prognosis with often A thrombosis or embolic occlusion of an artery
permanent disability. Prognosis for recovery causes an ischemic type of this condition.
of ambulation is inversely correlated with the A hemorrhagic type of this condition can be
severity of motor deficit at onset.50 Physical caused by arteriovenous malformation, hyper-
therapy interventions address weakness, tension, aneurysm, neoplasm, drug abuse and
spasticity, and mobility and ambulation trauma. This condition is the most common
deficits. and disabling neurological disorder in adults
and occurs in 114 of every 100,000 people.52
■ Stiff-Person Syndrome This condition is diagnosed using clinical pres-
Chief Clinical Characteristics entation and positive findings on computed
This presentation typically involves progres- tomography and magnetic resonance imaging.
sive, painful muscle stiffness and rigidity along Medication, surgery and interdisciplinary ther-
with spontaneous and reflex muscle spasms. apy are the most common treatments for this
These symptoms are seen primarily in the condition. The prognosis for recovery is pre-
axial and proximal limb muscles and can cause dicted by the magnitude of initial deficit. Factors
walking to become very slow and awkward. that are associated with poor outcomes include
Individuals with this condition also commonly coma, poor cognition, severe aphasia, severe
present with exaggerated lumbar lordosis sec- hemiparesis with little return within 1 month,
ondary to involvement of the lumbar paraspinal visual perceptual disorders, depression, and
muscles.51 incontinence after 2 weeks.53,54
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678 Chapter 32 Traumatic Brain Injury

■ Traumatic Brain Injury (total lack of sensory or motor function below


STIFFNESS

Chief Clinical Characteristics level of injury) or incomplete (some motor or


This presentation typically includes disequilib- sensory function spared below the level of
rium in the presence of cognitive changes, altered injury). Cervical locations of injury result in
level of consciousness, seizures, nausea, vomit- tetraplegia and may cause paralysis or weak-
ing, coma, dizziness, headache, pupillary ness of the respiratory musculature, requiring
changes, tinnitus, weakness, incoordination, mechanical ventilation and/or respiratory
behavioral changes, spasticity, hypertonicity, strengthening.60 Thoracic or lumbar locations
cranial nerve lesions, and sensory and motor of injury result in paraplegia. Treatment con-
deficits.2,55 sists of medical management, multidisciplinary
rehabilitation, equipment prescription, and
Background Information prevention of pressure ulcers, contractures, and
This condition can be classified as mild, mod- further complications. Individuals with incom-
erate, or severe based on Glasgow Coma plete forms of this condition may continue to
Scale, length of coma, and duration of post- recover strength and function, while individu-
traumatic amnesia.55 Magnetic resonance als with complete forms of this condition have
imaging may be used to confirm the diagno- a poor prognosis for recovery and instead use
sis.31 Treatment initiated at the scene of compensatory techniques and equipment.
the accident and during the acute phase is Individuals with this condition require adequate
focused on medical stabilization. It should follow-up medical care to prevent secondary
be initiated during the acute phase in order impairments.61 Acute forms of this condition
to minimize complications.56 Low Glasgow constitute a medical emergency.
Coma Scale, longer length of coma, longer
duration of post-traumatic amnesia, and older TUMORS
age tend to be associated with poor outcomes.57
■ Brain Metastases
Optimal rehabilitation is interdisciplinary
and customized to address the specific indi- Chief Clinical Characteristics
viduals’ needs. This presentation may include headaches,
seizures, dysphagia, weakness, cognitive
■ Traumatic Spinal Cord Injury changes, behavioral changes, dizziness, vom-
Chief Clinical Characteristics iting, alterations in the level of consciousness,
This presentation is characterized by loss of ataxia, aphasia, nystagmus, visual distur-
sensation in a dermatomal pattern, hypertonic- bances, dysarthria, balance deficits, falls,
ity, spasticity, hyperreflexia, and sphincter lethargy, and incoordination.2,62
dysfunction in combination with muscle weak-
Background Information
ness. Specific syndromes (discussed below) have
The majority of individuals with brain metas-
characteristic presentations secondary to the
tases have been previously diagnosed with a
location of the lesion.
primary tumor; however, a small percentage
Background Information of individuals are diagnosed concomitantly
Insult to the spinal cord results from fractured with brain metastases and the primary tumor.
bone, displaced disk material, or a foreign The most common cancers resulting in subse-
object transecting or injuring the cord. Imag- quent brain metastases include lung, breast,
ing is used to identify the cause of injury and melanoma, colorectal, and genitourinary
direct surgical stabilization or intervention. tract. The new onset of neurological symp-
Motor vehicle accidents and falls are the most toms after a primary tumor warrants the use
common etiology of this condition.58 Physical of imaging such as magnetic resonance imag-
examination of strength and sensation is used ing or computed tomography to confirm the
to assign a score from the American Spinal diagnosis. Treatment may include corticos-
Injury Association.59 Injuries are classified in teroids, brain irradiation, surgery, chemother-
terms of neurological level (ie, most rostral apy, radiotherapy, and rehabilitative thera-
segment where myotomal and dermatomal pies. The prognosis is poor with death
function is spared) and extent as either complete typically occurring within 6 months.
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Chapter 32 Vasculitis (Giant Cell Arteritis, Temporal Arteritis, Cranial Arteritis) 679

■ Brain Primary Tumors ■ Spinal Primary Tumors

STIFFNESS
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation may include headaches, This presentation may include spasticity,
seizures, dysphagia, weakness, cognitive weakness, sensory alterations, bowel/bladder
changes, behavioral changes, dizziness, vom- incontinence, back pain, radicular pain,
iting, alterations in the level of consciousness, atrophy, cerebellar signs, balance deficits, falls,
ataxia, aphasia, nystagmus, visual distur- and cranial nerve involvement.56
bances, dysarthria, balance deficits, falls,
Background Information
lethargy, and incoordination.2,31
Types of this condition include ependymoma,
Background Information hemangioblastoma, myeloma, neurofibroma,
Glioblastoma multiforme, astrocytoma, lymphoma, metastasis, meningioma, schwan-
oligodendroglioma, metastatic tumors, pri- noma, and astrocytoma. Extradural tumors,
mary central nervous system lymphomas, such as meningiomas, produce a rapid on-
ganglioglioma, neuroblastoma, menin- set of symptoms, with weakness being pre-
gioma, arachnoid cysts, hemangioblastoma, dominant. Intramedullary tumors, or
medulloblastoma, and acoustic neuroma/ ependymomas, astrocytomas and heman-
schwannoma are some of the more common gioblastomas, present with slowly progres-
brain tumors. Specific diagnoses for brain sive symptoms, of which loss of pain and
tumors may be confirmed with imaging and temperature sensation is usually the first.
biopsy. Treatment is variable depending on The first test to diagnose brain and spinal
the type, size, and location of the tumor and column tumors is a neurological examina-
may include surgical resection, chemother- tion. Special imaging techniques (com-
apy, radiation, corticosteroids, and rehabilita- puted tomography, magnetic resonance im-
tive therapies. Prognosis is also variable and aging, and positron emission tomography)
depends on the type and grade of tumor, are also employed. Specific diagnoses may
severity of compression, and duration of be confirmed with imaging and biopsy.
compression. Treatment is variable depending on the
type, size, and location of the tumor and
■ Spinal Metastases may include surgical resection, chemother-
Chief Clinical Characteristics apy, radiation, corticosteroids, and rehabili-
This presentation can involve spasticity, weak- tative therapies. Prognosis is variable and
ness, sensory alterations, bowel and bladder depends on the type and grade of tumor,
incontinence, neck pain, back pain, radicular severity of compression, and duration of
pain, atrophy, cerebellar signs, balance deficits, compression.
falls, and cranial nerve involvement.2,31,63
■ Vasculitis (Giant Cell Arteritis,
Background Information Temporal Arteritis, Cranial
This condition is the most frequent neoplasm Arteritis)
involving the spine.63 The most common
Chief Clinical Characteristics
types and locations of primary tumors that
This presentation can be characterized by
result in spinal metastases include breast,
headaches, psychiatric syndromes, dementia,
lung, lymphoma, prostate, kidney, gastroin-
peripheral or cranial nerve involvement, pain,
testinal tract, and thyroid.2,64 The diagnosis
seizures, hypertension, hemiparesis, balance
is confirmed with gadolinium enhanced mag-
deficits, neuropathies, myopathies, organ
netic resonance imaging and computed
involvement, fever, and weight loss.2,31,65
tomography.2,63 Treatment is variable depend-
ing on the tumor and may include surgical Background Information
resection, chemotherapy, radiation, corticos- This condition is the result of an immune-
teroids, and rehabilitative therapies.2 Although mediated response resulting in the inflamma-
the long-term prognosis is poor, individuals tion of vascular structures.2,65 It includes a
without paresis and pain and who are still variety of disorders such as giant cell/temporal
ambulatory have longer survival rates.64 arteritis (which is the most common form),
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680 Chapter 32 Whiplash Injury (Whiplash-Associated Disorder)

primary angiitis of the central nervous sys- peripheral vestibular involvement.72,73 Treat-
STIFFNESS

tem, Takayasu’s disease, periarteritis nodosa, ment for individuals with an acute whiplash
Kawasaki disease, Churg-Strauss syndrome, may include pain medications, physical therapy
Wegener’s granulomatosis, and secondary modalities, nonsteroidal anti-inflammatory
vasculitis associated with systemic lupus drugs, antidepressants, muscle relaxants, and
erythematous, rheumatoid arthritis, and scle- use of a cervical collar. Range of motion
roderma.65 The diagnosis is confirmed and strengthening exercises are key aspects
through history, physical examination, labo- of long-term management. Treatment for
ratory testing, angiography, biopsy, and imag- decreased kinesthetic awareness involves
ing.2,31,65 Corticosteroids, cytotoxic agents, eye–head–neck–trunk coordination exercises.74
intravenous immunoglobulin, and plasma-
pheresis may be used in the treatment of ■ Wilson’s Disease
vasculitis.31,65 Prognosis is variable and depends Chief Clinical Characteristics
on the specific underlying disorder. For This presentation commonly involves liver dys-
example, giant cell arteritis is typically self- function followed by neurological symptoms
limiting within 1 to 2 years; however, death such as tremor, rigidity, drooling, difficulty with
usually occurs within 1 year for individuals speech and abrupt personality change.
with primary angiitis of the central nervous
Background Information
system.65
Symptoms of Wilson’s disease usually appear
■ Whiplash Injury in childhood or young adult life75 with a
(Whiplash-Associated Disorder) frequency of 1 in 30,000.76 This condition is
an autosomal recessive disorder in which
Chief Clinical Characteristics
excessive amounts of copper accumulate in
This presentation commonly involves dizziness
the body especially in eye membranes (caus-
in combination with neck pain, stiffness,
ing a golden brown Kayser-Fleischer ring),
headache, abnormal sensations such as burn-
nailbeds, and kidney.31 The gene abnormality
ing or prickling, or shoulder or back pain.66
has been located on chromosome 13, which
Whiplash is characterized by a collection of
causes a deficiency in ceruloplasmin. In the
symptoms that occur following damage to the
central nervous system, cavitation and neu-
neck, usually because of sudden extension and
ronal loss occur within the putamen and
flexion such as might happen in an automobile
globus pallidus. This condition is diagnosed
accident.
based on clinical findings supported by bio-
Background Information chemical evidence of low ceruloplasmin, ele-
Cervicocephalic kinesthesia is adversely affected vated unbound serum copper, high urinary
and may be responsible for this condition.67–69 copper excretion, positive liver biopsy and
In addition, some people experience cognitive, copper metabolism tests, and T2-weighted
somatic, or psychological conditions such as magnetic resonance imaging that shows
memory loss, concentration impairment, thalamus and putamen hyperintensity.31
nervousness/irritability, sleep disturbances, Treatment consists of low-copper diet and a
fatigue, or depression. Symptoms such as neck chelating agent (usually penicillamine).31
pain may be present directly after the injury or With adequate treatment, the life span is
may be delayed for several days. The condition normal. If left untreated in children, death
may include injury to intervertebral joints, is predicted in 2 years from hepatic and renal
disks, and ligaments, cervical muscles, and failure. In untreated adults, death is predicted
nerve roots. The trauma may dislodge otoconia in 10 years.31
in the inner ear, leading to benign paroxysmal
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57. Hukkelhoven CWPM, Steyerberg EW, Rampen AJJ, et Rehabil. 1994;75(8):895–899.
al. Patient age and outcome following severe traumatic 75. Greenberg DA, Aminoff MJ, Simon RP. Clinical Neurol-
brain injury: an analysis of 5600 patients. J Neurosurg. ogy. 5th ed. New York, NY: McGraw-Hill; 2002.
2003;99:666–673. 76. Gow PJ, Smallwood RA, Angus PW, Smith AL, Wall AJ,
58. Jackson AB, Dijkers M, Devivo MJ, Poczatek RB. A Sewell RB. Diagnosis of Wilson’s disease: an experience
demographic profile of new traumatic spinal cord over three decades. Gut. Mar 2000;46(3):415–419.
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33
CHAPTER

Weakness
■ Katherine J. Sullivan, PT, PhD, FAHA ■ Katherine M. Weimer, PT, DPT, NCS

Description of the Symptom ■ Visual disturbances


■ Language disturbances
This chapter describes pathology that may ■ Changes in level of consciousness
lead to weakness. Weakness is the inability ■ Confusion or other cognitive changes
of muscles to generate force to generate the ■ Weakness accompanied by numbness or
age-related functional activities. Strength is tingling
the capacity of skeletal muscle to develop ■ Weakness with paresthesias that are pro-
the amount of force needed to provide the gressive and in a radicular pattern
musculoskeletal mobility and stability re- ■ Weakness accompanied by changes in
quired for functional performance.1 Impair- bowel and bladder function
ment in strength result in the symptom of ■ Weakness accompanied by signs or symp-
weakness. toms of systemic illness such as fever, di-
aphoresis, and anxiousness
Special Concerns ■ Weakness accompanied by lower motor
■ A sudden onset of rapidly evolving weak- neuron disease signs such as fasciculations
ness or progressive change in strength in and noticeable atrophy
combination with any of the following: ■ Weakness accompanied by severe, localized
■ Abnormal reflexes pain or generalized muscle pain
■ Cranial nerve signs

CHAPTER PREVIEW: Conditions That May Lead to Weakness

T Trauma
COMMON
Brachial plexus injuries:
• Dejerine-Klumpke palsy (Klumpke’s palsy) 690
• Erb-Duchenne palsy (Erb’s palsy) 690
• Traumatic brachial plexus injury 690
Cervical spondylotic myelopathy 691
Neuropathies:
• Carpal tunnel syndrome 700
• Compression neuropathy 700
• Peripheral neuropathies 702
Spinal cord injuries:
• Brown-Sequard syndrome 705
• Central cord syndrome 706
• Conus medullaris syndrome 706
• Traumatic spinal cord injury 706
Thoracic outlet syndrome 708
Traumatic brain injury 710
(continued)

683
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684 Chapter 33 Weakness

Trauma (continued)
WEAKNESS

UNCOMMON
Bell’s palsy 689
Brachial plexus injuries:
• Dejerine-Klumpke palsy (Klumpke’s palsy) 690
• Erb-Duchenne palsy (Erb’s palsy) 690
Hydromyelia (syringomyelia) 693
RARE
Locked-in syndrome 696

I Inflammation
COMMON
Aseptic
Not applicable
Septic
Poliomyelitis (undeveloped countries) 704
Rheumatoid arthritis 705
UNCOMMON
Aseptic
Behçet’s disease 689
Multiple sclerosis 698
Neuropathies:
• Chronic inflammatory demyelinating polyneuropathy 700
• Multifocal motor neuropathy 701
Systemic lupus erythematosus 707
Septic
Neurological complications of acquired immunodeficiency syndrome 699
Poliomyelitis (most developed countries) 704
RARE
Aseptic
Acute demyelinating polyneuropathy (Guillain-Barré syndrome) 687
Critical illness polyneuropathy 691
Inflammatory myopathies:
• Dermatomyositis 694
• Inclusion body myositis 694
• Polymyositis 694
Lambert-Eaton myasthenic syndrome 695
Lyme disease (tick paralysis) 696
Miller Fisher syndrome 697
Neurosarcoidosis 702
Transverse myelitis 709
Septic
Botulism 689
Neurosyphilis (tabes dorsalis, syphilitic spinal sclerosis, progressive locomotor ataxia) 703
Tropical spastic paralysis 710
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Chapter 33 Weakness 685

WEAKNESS
M Metabolic
COMMON
Neuropathies:
• Diabetes mellitus-induced neuropathies 701
UNCOMMON
Mitochondrial myopathies 697
Toxic myopathy 709
RARE
Cushing’s syndrome (hypercortisolism) 691
Glycogen storage diseases (glycogenosis, Pompe’s disease, McArdle’s disease, Cori’s disease) 693
Lipid storage diseases of muscle 696
Myasthenia gravis 699
Neuropathies:
• Toxic neuropathy 702
Neurotoxicity 703
Thyrotoxic myopathy (Graves’ disease) 708

Va Vascular
COMMON
Stroke (cerebrovascular accident) 707
UNCOMMON
Arteriovenous malformation 688
Spinal cord infarction (vascular myelopathy) 705
Transient ischemic attack 709
RARE
Moyamoya disease 697
Normal pressure hydrocephalus 703
Vasculitis (giant cell arteritis, temporal arteritis, cranial arteritis) 711

De Degenerative
COMMON
Deconditioning 692
UNCOMMON
Not applicable
RARE
Amyotrophic lateral sclerosis (Lou Gehrig disease) 688
Parry-Romberg syndrome 703
Post-polio syndrome 704
Primary lateral sclerosis 705

Tu Tumor
COMMON
Not applicable
(continued)
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686 Chapter 33 Weakness

Tumor (continued)
WEAKNESS

UNCOMMON
Malignant Primary, such as:
• Brain primary tumors 710
Malignant Metastatic, such as:
Not applicable
Benign:
Not applicable
RARE
Malignant Primary, such as:
• Spinal primary tumors 711
Malignant Metastatic, such as:
• Brain metastases 710
• Spinal metastases 711
Benign, such as:
• Angiomatosis (Von Hippel-Lindau disease) 688

Co Congenital
COMMON
Not applicable
UNCOMMON
Muscular dystrophy 698
Tethered spinal cord syndrome 707
RARE
Arnold-Chiari malformation (Chiari malformation) 688
Bulbospinal muscular atrophy (Kennedy’s disease, X-linked bulbospinal neuronopathy) 691
Familial periodic paralysis 692
Friedreich’s ataxia 692
Hereditary spastic paraplegia (familial spastic paralysis) 693
Kearns-Sayre syndrome 695
Machado-Joseph disease 696
Monomelic amyotrophy (benign focal amyotrophy, Hirayama syndrome, O’Sullivan-McLeod
syndrome) 697
Neuropathies:
• Hereditary motor and sensory neuropathies 701
Pelizaeus-Merzbacher disease 704
Spinal muscular atrophy (Werdnig-Hoffmann disease, Kugelberg-Welander disease) 707

Ne Neurogenic/Psychogenic
COMMON
Not applicable
UNCOMMON
Hysterical paralysis 694
RARE
Todd’s paralysis 708

Note: These are estimates of relative incidence because few data are available for the less common conditions.
1528_Ch33_683-715 07/05/12 2:01 PM Page 687

Chapter 33 Acute Demyelinating Polyneuropathy (Guillain-Barré Syndrome) 687

Overview of Weakness metabolic demands such as cranial nerve or

WEAKNESS
postural muscles with normal to reduced mus-
Weakness, the inability to generate force, can cle tone and no sensory loss. Myopathic disor-
result from pathological disease conditions ders are diseases that affect muscle fibers;
that affect the nervous system or motor unit. therefore, weakness is most noticeable in prox-
In addition, factors such as age, disuse, immo- imal muscle groups with no sensory loss and
bilization, and musculoskeletal trauma can normal tendon reflexes until later in the disease
have a direct and immediate effect on muscle course when tendon reflexes may be depressed.
function that can result in weakness.1 Weak-
ness must be differentiated from generalized Description of Conditions That
fatigue. Individuals with conditions that cause May Lead to Weakness
fatigue may describe themselves as feeling
weak or lacking strength. Fatigue may be asso- ■ Acute Demyelinating
ciated with poor physical health, psychological Polyneuropathy (Guillain-Barré
health, or sleep disorders. In some cases, such Syndrome)
as multiple sclerosis, fatigue can be primarily
Chief Clinical Characteristics
associated with the disease state or may be a
This presentation typically involves progressive
secondary complication of functioning daily
paresthesias described as numbness, tingling, and
with a degenerative neurological disease that
prickling, and weakness over the course of sev-
results in progressive weakness.
eral days to a few weeks. Lower extremities in
Individuals who may have weakness that is
a distal to proximal pattern are usually affected
directly related to impairments in strength will
first, followed by upper extremities. Muscles of
often report changes in functional ability. In-
the trunk and face may be affected. If paralysis
ability to come to a standing position or clear
progresses to respiratory muscles, a ventilator
the foot during walking or an increased inci-
may be required. In the majority of cases, a mild
dence of falls may be related to changes in
gastrointestinal or respiratory infection pre-
lower extremity strength. Inability to manipu-
cedes symptoms.
late items with the hands, problems reaching
overhead, and changes in ability to lift and Background Information
carry loads may be related to changes in upper Symptoms are a result of immunologic reac-
extremity strength. tion causing demyelination of peripheral
Weakness of pathological origin can result nerves, and in severe cases, axonal degenera-
from involvement of the central nervous sys- tion as well. In addition to clinical presenta-
tem, peripheral nervous system, or the muscle tion, differential diagnosis is established by
itself. Therefore, it is common to determine if the presence of increased lymphocytes and an
the pattern of weakness and the associated increase in protein in cerebrospinal fluid as
neurological signs are consistent with an well as electromyographic findings of reduc-
upper motor neuron or lower motor neuron tion in amplitudes of muscle action poten-
syndrome, neuromuscular transmission disor- tials, slowed conduction velocity, conduction
der, or myopathic disease.2 An upper motor block in motor nerves, and prolonged distal
neuron syndrome indicates disease or injury of latencies. Standard treatment includes ad-
the central nervous system in which weakness ministration of intravenous immune globu-
is accompanied by hyperreflexia (increased lin and plasma exchange. The majority of in-
deep tendon reflexes). A lower motor neuron dividuals recover completely or almost
syndrome indicates disease or injury of the completely within a few weeks to a few
peripheral nervous system in which weakness months; however, the presence of axonal de-
is accompanied by hyporeflexia (decrease in generation increases the regeneration time
deep tendon reflexes). Neuromuscular trans- period to 6 to 18 months. Three percent to
mission disorders are characterized by fluctuat- 5% of individuals with this condition do not
ing weakness, particularly of muscles with high survive.3
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688 Chapter 33 Amyotrophic Lateral Sclerosis (Lou Gehrig Disease)

■ Amyotrophic Lateral Sclerosis listed above. Specific symptoms vary among


WEAKNESS

(Lou Gehrig Disease) individuals and depend on the size and loca-
Chief Clinical Characteristics tion of the tumors. Individuals with this con-
This presentation includes weakness that can dition are also at a higher risk than normal for
begin in the muscles of the lower or upper certain types of cancer, especially kidney can-
limbs, trunk, face or throat. People affected cer. Treatment varies according to the location
will complain of difficulty walking or running, and size of the tumor and its associated cyst. In
writing, speakiing clearly, or swallowing. The general, the objective is to treat the tumors
disease advances rapidly with death usually when they are causing symptoms but are still
occuring within 5 years due to respiratory small. Treatment of most cases usually involves
failure. surgical resection. Certain tumors can be
treated with focused high-dose irradiation.
Background Information Individuals with this condition need careful
A hallmark of this condition is the presence monitoring by a physician and/or medical
of weakness accompanied by fasciculations team familiar with the condition.
(muscle fiber twitching) and hyperreflexia of
one or more segmental reflexes. ALS is a ■ Arnold-Chiari Malformation
degenerative disease that affects the pyramidal (Chiari Malformation)
cells of the motor cortex and the motor Chief Clinical Characteristics
neurons in the brainstem and spinal cord. The This presentation may include visual or
presentation of hyperreflexia with weakness, swallowing disturbances in combination with
atrophy, and fasciculation is due to the pro- pain in the occipital or posterior cervical areas,
gressive and relentless death of upper and downbeating nystagmus, progressive ataxia,
lower motor neurons throughout the nervous progressive spastic quadriparesis, or cervical
system. Sensation, cognitive function, and syringomyelia.4,5
bowel and bladder control are not affected.
While there are some familial variants, the Background Information
majority of cases are idiopathic, thought to be This condition encompasses a number of con-
related to a possible toxic exposure. genital abnormalities at the base of the brain,
including extension of the cerebellar tissue or
■ Angiomatosis (Von displacement of the medulla and fourth ven-
Hippel-Lindau Disease) tricle into the cervical canal.4 This condition
Chief Clinical Characteristics has two main types. Individuals with the more
This presentation commonly includes headaches, common form of this condition, Type I, often
problems with balance and walking, dizziness, remain asymptomatic until adolescence or
weakness of the limbs, vision problems, and high adult life.4 Type II is primarily seen in infants
blood pressure. and young children. Please see the pediatric
section of this textbook for a more complete
Background Information description of this type. Diagnosis is made by
This condition is a rare, genetic multisystem magnetic resonance imaging, computed to-
condition characterized by the abnormal mography, myelography, or some combination
growth of tumors in certain parts of the body of these tests.5 Treatment varies depending on
(angiomatosis). Tumors of the central nervous clinical progression, and may include surgical
system are benign, are comprised of a nest of intervention such as an upper cervical
blood vessels, and are called hemangioblas- laminectomy or enlargement of the foramen
tomas (or angiomas in the eye). Heman- magnum. Even with surgery symptoms may
gioblastomas may develop in the brain, the persist or progress.4
retina of the eye, and other areas of the nerv-
ous system. Other types of tumors develop ■ Arteriovenous Malformation
in the adrenal glands, the kidneys, or the Chief Clinical Characteristics
pancreas. Cysts (fluid-filled sacs) and/or tumors This presentation may be characterized by
(benign or cancerous) may develop around the weakness, seizures and severe headache.
hemangioblastomas and cause the symptoms Hemorrhage may result in paresis, ataxia,
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Chapter 33 Botulism 689

dyspraxia, dizziness, tactile and proprioceptive ■ Bell’s Palsy

WEAKNESS
disturbances, visual disturbances, aphasia, Chief Clinical Characteristics
paresthesias, and cognitive deficits.3 This presentation typically involves unilateral
Background Information facial paralysis and is characterized by acute
This condition is caused by a tangle of arteries drooping of the eyelid and/or corner of the
and veins caused by a developmental abnormal- mouth, drooling, impairment of taste, and dry-
ity that likely arises during embryonic or fetal ness of the eye with or without excessive tear-
development. Approximately 12% of the 300,000 ing that progresses within 48 hours. Tactile
individuals in the United States with this condi- sensation is intact. Facial paralysis is commonly
tion are symptomatic. Neurological damage unilateral, though in rare cases may present
occurs due to reduction of oxygen delivery, bilaterally. All three quadrants of the face are
hemorrhage, or compression of nearby struc- affected. Long-term facial paralysis may lead to
tures of the brain or spinal cord due to the tan- synkinesis (imbalance of muscular activation),
gle of vessels. Computed tomography, magnetic resulting in significant facial distortion.7
resonance imaging, and arteriography confirm Background Information
the diagnosis. Ligation and embolization may be This condition is caused by an idiopathic in-
used to reduce the size of the lesion prior to flammation of the facial nerve, likely due to a
surgical excision, which is the preferred method viral infection, commonly herpes simplex.
of treatment. Stereotactic radiation and proton Diagnosis utilizes clinical examination to rule
beam therapy are alternative approaches to inva- out other causes of facial weakness; for exam-
sive methods of intervention. Up to 90% of in- ple, facial weakness due to cortical or subcorti-
dividuals who experience a hemorrhagic arteri- cal lesions is associated with impaired sensation
ovenous malformation survive.3 and the frontalis and levator palpebrae muscles
are weakened, but not paralyzed. Treatment
■ Behçet’s Disease involves antiviral and anti-inflammatory med-
Chief Clinical Characteristics ications and physical therapy to address paraly-
This presentation may include bilateral pyram- sis and symmetry of motion and to prevent
idal signs (signs related to lesions of upper synkineses.7-10 Natural recovery of facial motor
motor neurons or descending pyramidal control occurs within 3 to 6 months in 94% of
tracts, such as a positive Babinski sign or hyper- patients with incomplete paralysis, but residual
reflexia), headache, memory loss, hemiparesis, synkinesis and weakness often remains in those
cerebellar ataxia, balance deficits, sphincter with complete palsies.8
dysfunction, or cranial nerve palsies. In addi-
tion to these neurological signs individuals with ■ Botulism
this condition also may present with arthritis; Chief Clinical Characteristics
renal, gastrointestinal, vascular, and cardiac This presentation involves nausea and vomit-
diseases; and genital, oral, and cutaneous ing, followed by rapid onset and progression of
ulcerations.6 diplopia, blurred vision, ptosis, dysphagia,
flaccid descending paralysis, and possible pro-
Background Information
gression to respiratory failure. There are no
Mean age of onset is in the third decade of life.
central neurological manifestations or sensory
Diagnostic criteria according to an international
impairments.11,12
study group include presence of recurrent oral
ulceration, recurrent genital ulceration, eye Background Information
lesions, skin lesions, papulopustular lesions, Botulism toxin affects the function of cranial
and/or a positive pathergy test.3,6 Medical nerve musculature, skeletal muscle, and the
treatment typically consists of corticosteroids diaphragm by preventing the release of acetyl-
and immunosuppressants. Neurological symp- choline from synaptic terminals, thus blocking
toms tend to clear within weeks, but can some- neuromuscular transmission.12 The food-
times recur or result in permanent deficits.3 borne variety of this condition is the most
Onset before the age of 25 and male sex indicate common variant, followed by infant, wound,
a poorer prognosis. and intestinal. Differential diagnosis especially
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690 Chapter 33 Dejerine-Klumpke Palsy (Klumpke’s Palsy)

includes stroke, Guillain-Barré syndrome, and electrodiagnostic study.15 Many infants


WEAKNESS

myasthenia gravis.13 Rapid diagnosis via clini- improve or recover strength by 3 to


cal examination and administration of botu- 4 months of age with a 90% to 100% recov-
lism antitoxin result in decreased mortality and ery of function.14 If spontaneous recovery
shorter length of hospital stay.11 A longer inter- does not occur, surgical intervention is indi-
val between onset and treatment, ventilatory cated. Physical therapy is indicated to main-
failure, and complete paralysis are poor prog- tain range of motion and strength.16
nostic indicators.11,13 The majority of patients
who do not receive the antitoxin will die.13
■ Traumatic Brachial Plexus
Injury
BRACHIAL PLEXUS INJURIES Chief Clinical Characteristics
■ Dejerine-Klumpke Palsy This presentation includes upper extremity
(Klumpke’s Palsy) weakness and sensory loss related to damage
of the brachial plexus, a network of nerves
Chief Clinical Characteristics
that conducts signals from the spine to the
This presentation commonly includes weakness
shoulder, arm, and hand. Associated symptoms
in the ulnar nerve distribution with associ-
include hyporeflexia, and hypotonicity.
ated sensory loss in the C8–T1 dermatome.
Background Information
Background Information
Injuries to the brachial plexus are often due
This condition refers to paralysis of the
to trauma, the most common of which are
lower brachial plexus that results from max-
stretch injuries occurring during the birth
imal abduction of the shoulder.14 True forms
process. Brachial plexus injuries can be clas-
of this condition are rare.14 Diagnosis of
sified in terms of mechanism of injury,
injury and localization of the lesion require
closed (motor vehicle accident) versus open
clinical investigation and electrodiagnostic
(intraoperative injury and gunshot wounds);
study.15 There is a poorer prognosis for
location of injury (spinal nerve root, trunk,
recovery with Dejerine-Klumpke palsy com-
cord, peripheral nerve); or type of nerve
pared to Erb’s palsy or total brachial plexus
damage.15 There are four types of nerve
palsy resulting from the birth process, with
damage: avulsion is the most severe type, in
most individuals with this condition requir-
which the nerve is torn from the spinal root;
ing surgical intervention.16
rupture, in which the nerve is torn midsub-
■ Erb-Duchenne Palsy stance; neuroma, in which the injured nerve
(Erb’s Palsy) has scarred, causing a conduction block; and
neurapraxia or stretch, in which the nerve
Chief Clinical Characteristics
has been damaged but not torn. Diagnosis of
This presentation includes profound weak-
injury and localization of the lesion require
ness of the scapular muscles and deltoid with
clinical investigation, electrodiagnostic
loss of sensation in the dermatomal distribu-
study, and imaging.15,17 Management of
tion of the C5 and C6 dermatomes. The typ-
brachial plexus injuries differs depending on
ical resting posture is the arm held in shoul-
type and severity of injury. Open injuries
der adduction with fingers pointing backward
with vascular damage should be explored
(waiter’s tip position). Distal strength of the
operatively immediately. In the absence of
upper extremity remains intact.
clinical or electrophysiological recovery after
Background Information 2 to 4 months, gunshot wounds without vas-
This condition refers to paralysis of the cular compromise should undergo surgical
upper brachial plexus in response to an intervention. Spontaneous recovery occurs
excessive separation or stretch of the neck in many closed injures; therefore, surgical
and shoulder such as from a sliding injury intervention is delayed 4 to 5 months.15 Out-
or stretch during the birth process.14 Diag- comes are favorable in patients who have less
nosis of injury and localization of the severe nerve damage and those who undergo
lesion require clinical investigation and early operation when indicated.15 Despite
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Chapter 33 Cushing’s Syndrome (Hypercortisolism) 691

residual weakness and impaired functional successfully managed with conservative treat-

WEAKNESS
use of the extremity, the majority of patients ment including anti-inflammatory medication
report satisfaction with their quality of life and physical therapy.22 In severe disease, with
postinjury and/or postsurgical repair.18 significantly narrowed canal and severe neuro-
logical compromise, surgical decompression is
■ Bulbospinal Muscular Atrophy indicated and patients have the potential to
(Kennedy’s Disease, X-Linked recover strength and function and to decrease
Bulbospinal Neuronopathy) pain.22,23
Chief Clinical Characteristics
This presentation can be characterized by severe, ■ Critical Illness Polyneuropathy
diffuse muscle cramping and fasciculations, Chief Clinical Characteristics
muscle weakness in a limb-girdle distribution, This condition is characterized by symmetric
and postural hand tremor. Other symptoms limb muscle weakness, often sparing cranial
include variable bulbar muscle weakness, gyneco- nerve musculature, with hyporeflexia or are-
mastia, premature muscle exhaustion, and flexia, sensory loss, and involvement of the
hyporeflexia or areflexia.19,20 phrenic nerve with resultant diaphragm weak-
ness.24 Patients are often unable to wean from
Background Information mechanical ventilation after the acute present-
This condition is a rare, x-linked, progressive ing illness has resolved.25
neuromuscular disorder that is usually seen in
males between 30 and 50 years old. Diagnosis Background Information
is made by clinical features, electrophysiologi- This condition is estimated to be present in 25%
cal study, and genetic testing.19 There is cur- to 50% of patients in surgical and medical inten-
rently no proven treatment for this disease, sive care units. It generally occurs in the presence
but genetic counseling is recommended upon of sepsis, systemic inflammatory response syn-
diagnosis.21 This condition is usually associ- drome, and multiorgan failure within the first 2
ated with a normal life span, but individuals to 5 days.25 Electrodiagnostic investigation con-
may experience significant disability.19 firms the presence of denervation consistent
with sensorimotor axonal polyneuropathy.24
■ Cervical Spondylotic Myelopathy Critical medical care may include intubation,
Chief Clinical Characteristics physical therapy, prevention of pressure ulcers
This presentation can be characterized by weak- and compression neuropathies, and psychologi-
ness and sensory loss to light touch, vibration, cal support. There is no proven treatment for
and position sense occurring in a radicular this condition.24 Roughly half of patients with
pattern in the upper extremity. Presentation of mild forms of this condition who survive the
associated signs of upper and lower extremity initial causative illness completely return to the
paresis, hypertonia, and hyperreflexia indicates previous level of function.25 However, many
involvement of the corticospinal tract.4 continue to have persistent functional disabili-
ties, reduced quality of life, and restrictions in
Background Information participation 1 year after onset.26 Therefore, in-
This condition is caused by spondylitic changes tensive interdisciplinary rehabilitation is crucial
in the cervical spine that compress the cord. It to reduce disability and achieve autonomy and
occurs in middle to late age and is more com- social participation.25,26
mon in men than women. Upon clinical exam-
ination, patients will have cervical spine abnor- ■ Cushing’s Syndrome
malities, crepitus, restricted range of motion, (Hypercortisolism)
pain, and abnormal reflexes.3 Diagnosis in- Chief Clinical Characteristics
volves clinical history and examination, use of This presentation includes severe weakness of
imaging, electrophysiological testing, and use the proximal limb and girdle muscles often
of additional diagnostic tests to rule out com- manifested by difficulty climbing stairs or
peting diagnosis. Management is controversial. rising from a low chair. Additional signs and
Many individuals with this condition can be symptoms include fatigue, obesity of the upper
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692 Chapter 33 Deconditioning

body (including the hallmark feature of a moon- ■ Familial Periodic Paralysis


WEAKNESS

shaped face), easy bruising and bluish-red Chief Clinical Characteristics


stretch marks, high blood pressure, elevated This presentation involves a rapid onset of
blood glucose, memory loss, and depression.27,28 severe weakness and paralysis usually after a
Women with this condition may show an period of fasting, carbohydrate load, or rest
increased growth of facial and body hair and after a bout of strenuous exercise. Between
irregularity of menstruation. episodes, the affected muscles usually function
Background Information normally but myopathy may remain. Familial
This rare endocrine disorder is caused by periodic paralyses are channelopathies caus-
chronic exposure of the body’s tissues to excess ing underexcitability of the sarcolemma and
levels of cortisol due to medications or tumors resultant paralysis.30
of the pituitary or adrenal gland. Diagnosis is Background Information
confirmed by laboratory evidence of excessive Four types of familial periodic paralysis
cortisol in the urine and blood.27,28 Treatment can be clinically delineated: hyperkalemic,
is achieved through decreasing cortisol levels hypokalemic, paramyotonia congenita, and
via medication, surgery, or radiation. Most Anderson-Tawil syndrome.31 Clinical differen-
individuals with this condition show signifi- tiation is based on age of onset, duration and
cant improvement in symptom presentation severity of attack, precipitating factors, and as-
with treatment. However, in some individuals, sociated symptoms such as persisting proximal
residual symptoms may persist and some weakness in hypokalemia-induced forms of
kinds of tumors may recur. this condition, paramyotonia of the hands and
face in paramyotonia congenita, and ventricu-
■ Deconditioning lar arrhythmia and dysmorphic features in
Chief Clinical Characteristics Anderson-Tawil syndrome.32 Diagnosis is sup-
This presentation commonly involves a gradual ported with genetic testing, electromyography,
onset of muscle weakness and limited endurance serum creatine kinase, and muscle biopsy.
following immobilization, bed rest, or a period Prevention of attacks is achieved via dietary
of reduced physical activity. The etiology may or management, pharmaceutical prophylactics,
may not include another condition that caused and avoidance of precipitating factors. Patients
the initial reduction in physical activity level. with familial periodic paralysis benefit from
mild-to-moderate intensity regular exercise to
Background Information counter progressive weakness that persists
Deconditioning involves decreased functioning between attacks, maintain health, and prevent
related to impairments spanning multiple body future onset of attacks.
systems. The physiological maladaptations un-
derlying deconditioning include effects on the ■ Friedreich’s Ataxia
cardiovascular, skeletal muscle, bone, immune,
endocrine, and peripheral and central nervous Chief Clinical Characteristics
systems. This condition typically responds to a This presentation can be characterized by the
gradually progressive exercise program. Spe- onset of progressive gait and limb ataxia in
cific improvements noted at the body systems childhood to early adulthood. Associated
level will depend on the mode of training se- symptoms include loss of vibration sense and
lected. The physical therapist should be sensi- proprioception, absent deep tendon reflexes,
tive to the potential presence of comorbid con- weakness, dysarthria, and sensorineural
ditions that may affect the ability to initiate and hearing loss. Often, cardiac hypertrophy,
progress the exercise program. Additional con- diabetes mellitus, and optic atrophy are also
sultation with other health care providers may present.4,33,34
be necessary depending on the extent and eti- Background Information
ology of deconditioning, in order to optimize This autosomal recessive condition is caused by
the safety of the exercise program.29 a genetic mutation resulting in a progressive
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Chapter 33 Hydromyelia (Syringomyelia) 693

loss of large myelinated sensory axons, ■ Hereditary Spastic Paraplegia

WEAKNESS
followed by degeneration of the posterior (Familial Spastic Paralysis)
column and spinocerebellar and pyramidal Chief Clinical Characteristics
tracts.4,35 Magnetic resonance imaging may This presentation includes insidious, progres-
show atrophic changes characteristic of the sive difficulty with walking due to bilateral,
disorder, and should be performed along with symmetric lower extremity spastic weakness.
genetic testing and electrocardiography for Patients may report tripping, stumbling, and
definitive diagnosis. There is currently no falling, as well as urinary urgency.40
medical treatment to slow or stop disease pro-
gression, but individuals with this condition Background Information
may benefit from surgical intervention to cor- This condition can be divided into two types.
rect foot and spine deformities and allow for The complicated form of this condition is
improved mobility. Average lifetime survival is indicated by the presence of neurological
25 to 30 years of age.4,35 abnormalities such as dementia, mental retar-
dation, epilepsy, extrapyramidal disturbance,
■ Glycogen Storage Diseases ataxia, deafness, retinopathy, optic neuropathy,
(Glycogenosis, Pompe’s Disease, peripheral neuropathy, and skin lesions.
McArdle’s Disease, Cori’s Disease) The uncomplicated form is indicated by the
Chief Clinical Characteristics absence of these features.41 This condition is a
This presentation typically involves benign trun- group of inherited disorders with a primary
cal and proximal limb myopathy or exercise- pathological feature of axonal degeneration of
induced severe myalgia, cramping, and fatigue the distal ends of the longest ascending and
in adults.36 descending tracts, resulting in the characteris-
tic spasticity.40,41 Diagnosis is made by the
Background Information presence of symptoms of gait disturbance,
This condition refers to a group of 11 autoso- neurological findings of corticospinal tract
mal recessive diseases of glycogen storage deficits in the lower extremities, family history
affecting the liver, heart, and skeletal muscle.37 or genetic testing, and exclusion of any other
Pathophysiology is due to inherited deficien- disorders that could account for the symp-
cies of enzymes that regulate the synthesis or toms.40 Current treatment is limited to
degradation of glycogen.38 The many variants decreasing muscle spasticity through exercise
of this condition differ in the age of presenta- and medication. Physical therapy is beneficial
tion from infancy to adulthood and predomi- for the maintenance and improvement of
nance of presenting symptoms, such as muscle flexibility, muscle strength, gait, and
proximal weakness in Pompe’s disease, mixed cardiovascular fitness.40 Prognosis varies, and
proximal and distal weakness in Cori’s disease, those with an onset after adolescence are more
and cramping and fatigue after exercise in likely to have insidious worsening.40
McArdle’s disease.39 Diagnosis involves elec-
tromyography, blood studies, and muscle ■ Hydromyelia (Syringomyelia)
biopsy. Treatment consists of dietary therapy, Chief Clinical Characteristics
enzyme replacement, activity modification, This presentation involves insidious onset of
and symptom-based care. The prognosis for symptoms including upper and lower extrem-
individuals with glycogenoses varies according ity weakness and numbness, and less commonly,
to the variant, onset, and severity of symp- pain. Trauma usually precedes the onset on
toms. Serious long-term complications such as symptoms, but the time frame for subsequent
nephropathy, cirrhosis, cardiomyopathy, and development of weakness and sensory changes
progressive myopathy are a major concern.38 is variable.
This condition is characterized by slow pro-
gressive weakness over years that eventually Background Information
results in death secondary to failure of respira- This condition is caused by an abnormal
tory musculature. widening of the central canal of the spinal
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694 Chapter 33 Hysterical Paralysis

cord, leading to the accumulation of cere- proximal to distal pattern.3 Associated symp-
WEAKNESS

brospinal fluid and hydrocephalus. Differential toms include muscle ache and tenderness,
diagnosis must be made between hydromyelia fatigue, discomfort, weight loss, and low-grade
and other disorders such as syringomyelia, fever.
spinal cord tumor, and spinal arteriovenous
Background Information
malformation. Magnetic resonance imaging
This condition is an inflammatory myopathy
and electromyography are used to confirm the
with a severe onset, affecting children and
diagnosis of this condition. Surgery may be in-
adults, and females more often than males.
dicated to decrease or eliminate the symptoms.
Diagnostic criteria include typical changes
Prognosis is variable.
upon muscle biopsy, electrodiagnostic abnor-
malities characteristic of myopathy, and skin
■ Hysterical Paralysis
lesion/rash.3 Pharmacologic treatment with
Chief Clinical Characteristics steroids, immunosuppressants, and intra-
This presentation commonly includes a varied venous immunoglobulin is often combined
presentation of weakness associated with with physical therapy to preserve muscle
sensory loss, but no change in sphincter tone or function and prevent secondaryimpair-
reflexes.42 ments.44 Most cases respond to therapy.
Individuals with concomitant cardiac or pul-
Background Information monary involvement have a poorer prognosis
This condition is a type of conversion disorder.
The presentation of nonorganic loss of motor ■ Inclusion Body Myositis
function is often precipitated by a traumatic Chief Clinical Characteristics
event.43 All diagnostic testing, such as imaging, This presentation may include onset of prox-
laboratory investigation, and electrodiagnostic imal and distal muscle weakness that may
testing, fails to identify dysfunction. Inconsis- asymmetrically affect the extremities, with
tencies in history, physical examination, and common early involvement of the knee exten-
presentation following traumatic event should sor, ankle dorsiflexor, and wrist and finger
lead to suspicion of hysterical paralysis.42 The flexor greater than extensor muscle groups.45
DSM-IV-TR criteria must be met to fulfill the
diagnosis of conversion disorder. Intervention Background Information
includes treatment of any underlying depres- Isolated erector spinae weakness may cause
sion or anxiety disorders as well as behavior- “droopy neck syndrome.” Dysphagia is com-
oriented therapy and biofeedback.43 With mon in individuals with this condition. The
proper psychological intervention and discus- mean age of onset is 55 to 60 years, and
sion of physical findings and discrepancies with approximately 80% of individuals with
the patient, prognosis for full recovery of func- this condition begin to demonstrate symp-
tion is excellent, and 98% of patients/clients will toms and signs after age 50 years.46 The
demonstrate recovery within 1 year.43 histopathological hallmark of this condition
is protein aggregates consisting of inclusion
INFLAMMATORY MYOPATHIES bodies within affected muscle fibers. Patho-
physiology is unclear, although roles for
■ Dermatomyositis degeneration and humoral immunity have
Chief Clinical Characteristics been suggested. This condition typically is
This presentation typically involves patchy, unresponsive to immunosuppressive agents,
bluish-purple (heliotrope) discolorations of and optimal treatment remains unclear.
face, neck, shoulders, upper chest, elbows, Severe disease results in significant disability,
knees, knuckles, and back often preceding or but is rarely fatal.
coinciding with onset of symmetrical truncal
or proximal limb weakness. Individuals ■ Polymyositis
with this condition have difficulty arising Chief Clinical Characteristics
from a chair, climbing stairs, and lifting This presentation is characterized by onset
objects. Progression of weakness occurs in a of subacute or chronic and symmetrical
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Chapter 33 Lambert-Eaton Myasthenic Syndrome 695

weakness of proximal and trunk muscles, re- analysis and histological verification of

WEAKNESS
sulting in difficulty arising from a chair, the presence of ragged red fibers may be
climbing stairs, and carrying objects. helpful in determining diagnosis.48 Treat-
Polymyositis most commonly occurs in young ment is mostly symptomatic and supportive,
adulthood. Progression occurs in a proximal although cardiac symptoms may be managed
to distal pattern. Associated symptoms with medication. This condition is a slowly
include difficulty swallowing, muscle tender- progressive disorder and prognosis is often
ness, and an absence of dermatitis. determined by the degree of heart conduc-
tion impairment.50
Background Information
Diagnostic criteria for this condition include ■ Lambert-Eaton Myasthenic
weakness evolving over weeks to months Syndrome
sparing facial and eye muscles manifested in
patients 18 years and older; absence of Chief Clinical Characteristics
rash; no family history of neuromuscular, This presentation includes insidious onset of
endocrine, or neurogenic disease; no expo- symmetrical weakness in the trunk, pelvic
sure to myotoxic drugs; and exclusion of girdle, lower extremities, and shoulder girdle.
other myopathies via clinical examination, Report of a perception of increased strength
muscle biopsy, and exclusion of myopathies after a few muscle contractions, followed by
commonly associated with another autoim- weakness after prolonged exertion, is character-
mune disease or viral infection (ie, Crohn’s istic of this condition. Additional symptoms
disease, myasthenia gravis, or human im- include paresthesias, aching pain, dry mouth,
munodeficiency virus or acquired immun- constipation, difficult micturition, and impo-
odeficiency syndrome).47 Treatment involves tence.3 This presentation initially includes
steroidal, immunosuppressive, and/or intra- difficulty rising from a chair, climbing stairs, and
venous immunoglobulin medications. walking.
However, the optimal therapeutic regimen Background Information
remains unclear.44 Physical therapy is indi- The pathological process of this condition in-
cated to prevent muscle atrophy and con- volves a defect in the release of acetylcholine
tractures. Prognosis is variable. Severe dis- from presynaptic nerve terminals due to a loss
ease not responsive to therapy results in of voltage-sensitive calcium channels on the
significant disability, but is rarely fatal. presynaptic motor nerve channel. Approxi-
■ Kearns-Sayre Syndrome mately two-thirds of the known cases are asso-
ciated with cancer.3 Of those, small-cell lung
Chief Clinical Characteristics cancer is the most prevalent. This condition is
This presentation commonly includes a classic also associated with autoimmune diseases. The
triad of symptoms, involving progressive exter- syndrome of symmetrical weakness and easily
nal ophthalmoplegia, retinal pigmentary degen- fatigable proximal muscles combined with dry
eration, and heart block. Common additional mouth and aching pain suggests the diagnosis.
findings include cerebellar dysfunction, my- Differential diagnosis especially includes amy-
opathy, ataxia, sensorineural hearing loss, men- otrophic lateral sclerosis, multiple sclerosis,
tal retardation, growth hormone deficit with polymyositis, and myasthenia gravis. The Ten-
dwarfism, hypoparathyroidism, and diabetes silon test is the primary method used to distin-
mellitus.48,49 guish myasthenia gravis from this condition.
Background Information Treatment of the underlying pathological
Etiology has not been established, however process, usually a tumor or autoimmune dis-
this rare, sporadic mitochondrial disorder is ease, is the first goal, followed by administration
thought to occur via a mutation in either of cholinesterase inhibitors and immunosup-
the ovum or zygote.48 Diagnosis is established pressants.3 Prognosis varies depending on the
with a combination of clinical, radiologic, association with a tumor or autoimmune dis-
pathological, biochemical, and molecular ease, with the prognosis being worse in the
studies. Mitochondrial deoxyribonucleic acid presence of a tumor.
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696 Chapter 33 Lipid Storage Diseases of Muscle

■ Lipid Storage Diseases of Muscle of function. However, with rehabilitation,


WEAKNESS

Chief Clinical Characteristics many recover swallowing, improve motor and


This presentation involves progressive myopa- sphincter control, and/or are able to commu-
thy, with or without cardiomyopathy, appear- nicate verbally or through devices.53
ing late in infancy, childhood, or adult life.3
■ Lyme Disease (Tick Paralysis)
Background Information Chief Clinical Characteristics
Myopathy results from a deficit in the metabo- This presentation can include fluctuating signs
lism of fatty acids, secondary to carnitine or symptoms such as headache, neck stiffness,
deficiency. There are many variants of carni- nausea, vomiting, malaise, fever, pain,
tine deficiency. Primary systemic carnitine fatigue, and presence of a “bull’s-eye” rash.3
deficiency is autosomal recessive, presenting Over time, additional symptoms may include
with progressive myopathy and cardiomyopa- sensory changes, irritability, cognitive changes,
thy that is fatal if untreated.51 Carnitine depression, behavioral changes, seizures,
palmitoyltransferase deficiency is autosomal ataxia, chorea movements, pain, weakness,
recessive, presenting in teenage males, with balance deficits, arthritis, and cranial nerve
myalgia, cramps, weakness, and stiffness often involvement.3,54
precipitated by sustained exercise or fasting.52
Weakness may occur in any muscle group Background Information
without warning. Myopathy due to secondary Borrelia burgdorferi, an organism that infects
systemic carnitine deficiency results from ticks, is responsible for the transmission of this
dietary deprivation or impaired hepatic and condition to a human host.3 The diagnosis is
renal function.3 Diagnosis may include labora- confirmed with a thorough history, clinical
tory investigation, muscle biopsy, and electro- assessment, enzyme-linked immunosorbent
diagnostic testing used to rule out competing assay, and Western blot or immunoblot analysis.
diagnoses.51 Treatment involves dietary modi- In addition, magnetic resonance imaging or
fications, medication, and education. Progno- computed tomography may reveal multifocal or
sis is variable depending on type of deficiency periventricular cerebral lesions.3 Medical man-
and severity of myopathy. agement includes treatment with oral tetracy-
cline, penicillin, or intravenous ceftriaxone.3
■ Locked-In Syndrome Many individuals experience full recovery with
Chief Clinical Characteristics treatment; however, residual deficits may persist
This presentation includes complete paralysis for individuals with chronic Lyme disease.3
of all voluntary muscles characterized by
tetraplegia, paralysis of lower cranial nerves, ■ Machado-Joseph Disease
anarthria, and paralysis of horizontal gaze Chief Clinical Characteristics
sparing only vertical gaze and blinking.53 This presentation may involve visual impair-
Cognition and sensation are spared. ments such as nystagmus in combination with
slowly progressive ataxia, rigidity, dystonia,
Background Information weakness in the hands and feet, and difficulty
This condition is a rare neurological disorder with respiration and swallowing.
caused by a lesion to the ventral pons due to
obstruction of the basilar artery, traumatic Background Information
brain injury, tumor, central pontine myelinol- This condition is genetic, with an autosomal
ysis, or medication overdose.53 It is crucial to dominant pattern of inheritance and onset of
discern this condition from a minimally con- symptoms in adolescence or young adulthood.
scious state or vegetative state, because in the Differential diagnosis includes Parkinson’s dis-
latter condition the patient is not conscious ease and multiple system atrophy. The presence
and aware. The standard of care consists of of ataxia decreases the likelihood of Parkinson’s,
long-term multidisciplinary rehabilitation. and the early age of onset and visual symptoms
The prognosis for those with locked-in decrease the likelihood of multiple system atro-
syndrome is poor. The vast majority of phy. Diagnosis is established by clinical symp-
individuals do not return to premorbid levels toms and magnetic resonance imaging findings
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Chapter 33 Moyamoya Disease 697

of reduced width of superior and middle cere- encephalomyopathy with lactic acidosis and

WEAKNESS
bellar peduncles, atrophy of the frontal and stroke-like episodes, as well as other childhood-
temporal lobes, and decreased size of the pons onset disorders.4 A combination of clinical
and globus pallidus. There is no treatment for picture, histological findings of ragged red
this condition, and prognosis is poor.3 fibers, elevated serum lactate, and possible
family history contributes to the diagnosis of
■ Miller Fisher Syndrome this condition.4 There is no specific treatment,
Chief Clinical Characteristics but new research shows that patients may ben-
This presentation can be characterized by an efit from physical therapy for submaximal
acute onset of the classic triad of ophthalmople- exercise training.57 Most patients experience
gia, ataxia, and areflexia. Additional symp- lifelong progression of the disease, and prog-
toms include mydriasis, sensory loss, facial nosis varies according to the type of disease
palsy, bulbar palsy, dysesthesia, weakness, and and amount of involvement.4
urinary incontinence.55
■ Monomelic Amyotrophy (Benign
Background Information
Focal Amyotrophy, Hirayama
This condition is thought to be a variant
Syndrome, O’Sullivan-McLeod
form of acute demyelinating polyneuropathy
Syndrome)
(Guillain-Barré syndrome) and is usually
preceded by infectious gastrointestinal or Chief Clinical Characteristics
respiratory symptoms approximately 8 days This presentation typically involves slowly
before onset of symptoms.55 Diagnosis is progressive unilateral weakness and atrophy
based on clinical presentation. In addition, restricted to one limb (more commonly
elevated cerebrospinal fluid protein values arm/hand than leg/foot) followed by a period
and electrophysiological examination demon- of stabilization of symptoms. Sensation is intact.
strating conduction block or axonal damage Background Information
on limbs of normal strength can help rein- This condition usually occurs in males
force the diagnosis.56 Plasmapheresis and between the ages of 15 and 25 and is more
administration of intravenous immunoglob- frequently seen in Asia, particularly in Japan
ulins have both been found to be helpful in and India.58 Pathophysiology is unknown, but
decreasing recovery time.56 The natural this condition is thought to be due to a degen-
course of recovery in individuals with this eration of the lower motor neuron. Diagnosis
condition is good with minimal disability is made through history, clinical presentation,
seen 6 months after onset.55 electromyographic evidence of denervation
■ Mitochondrial Myopathies and polyphasics in the affected limb, and
possible imaging evidence of muscle atrophy.
Chief Clinical Characteristics Differential diagnosis particularly includes
This presentation typically involves a combina- spinal muscular atrophy, amyotrophic lateral
tion of exercise intolerance, ataxia, seizures, sclerosis, and post-polio syndrome. Clinical
myoclonus, headaches, small strokes, ophthal- presentation can delineate specific syndromes
moplegia, deafness, muscle cramps, and/or of this condition, such as O’Sullivan-McLeod
slowly progressive myopathy with proximal syndrome and Hirayama syndrome.59 Treat-
greater than distal involvement. Other less ment consists of muscle strengthening exer-
common symptoms that may be seen include cises and training in hand coordination. Weak-
dementia, lactic acidosis, ptosis, and cardiac ness continues to progress slowly over a period
conduction defects.4,57 of months to years before reaching a plateau.
Background Information Recovery of strength and function is possible,
This condition refers to a large group of but residual weakness remains.58
disorders that result from a mutation in the
mitochondrial genome, resulting in damage ■ Moyamoya Disease
to the mitochondria. These disorders include Chief Clinical Characteristics
Kearns-Sayre syndrome, myoclonus epilepsy This presentation may include weakness of
with ragged red fibers, mitochondrial an arm, a leg, or arm and leg unilaterally in
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698 Chapter 33 Multiple Sclerosis

combination with unsteady gait, involuntary include female sex, younger age of onset,
WEAKNESS

movement, speech and sensory impairments, relapsing-remitting type, complete recovery


headache, seizures, impaired mental develop- after the first relapse, and longer time interval
ment, visual disturbances, and nystagmus.4 between first and second exacerbation.64
Medical management may include the use of
Background Information
methylprednisolone, prednisone, cyclophos-
This rare condition results from progressive
phamide, immunosuppressant treatment, and
occlusion of the arteries of the circle of Willis.4
beta interferon.3 Physical, occupational, and
Diagnosis is based upon clinical findings and
speech therapy may be indicated to prevent
results of magnetic resonance imaging and
secondary sequelae and to optimize functional
magnetic resonance angiography. Images
activity and mobility. Some individuals may
will demonstrate the occlusion of the circle of
benefit from psychology/psychiatry and social
Willis as well as secondary cerebral infarction,
support as the disease progresses.
white matter lesions, atrophy, and hemor-
rhage.60 Treatment options include revascular- ■ Muscular Dystrophy
ization surgery and medical treatment to Chief Clinical Characteristics
prevent hypertension and further strokes.61 This presentation can be characterized by a
Rehabilitative therapies are used to treat func- symmetric distribution of muscular weakness
tional deficits that the patient may incur from and atrophy, intact sensation, preservation
a stroke secondary to the progression of the of cutaneous reflexes, and a strong familial
disease. Outcome depends on the severity of incidence.
secondary complications and presence of
subsequent occlusion. Background Information
This group of conditions involves purely
■ Multiple Sclerosis degenerative muscular diseases of the heredi-
Chief Clinical Characteristics tary type. The various types of muscular
This presentation may include weakness, pares- dystrophies are classified by distribution of
thesias, spasticity, hypertonicity, hyperreflexia, dominant weakness, varying in age of presen-
positive Babinski sign, incoordination, optic tation, severity of weakness and debility, and
neuritis, ataxia, vertigo, dysarthria, diplopia, prognosis for life expectancy. Duchenne
bladder incontinence, tremor, balance deficits, muscular dystrophy is an X-linked recessive
falls, and cognitive deficits.3 disorder, occurring predominantly in males.
This condition is characterized by proximal
Background Information weakness, pseudohypertrophy, and Gower’s
This condition may present as relapsing- sign (difficulty rising from the ground); this
remitting, primary progressive, or secondary condition is usually diagnosed in the first 3 to
progressive. The disease occurs most fre- 6 years of life. Becker-type muscular dystrophy
quently in women between the ages of 20 and has a similar presentation, however onset is
40 years. Only a small number of children or much later (average age 12 years), with indi-
individuals between 50 and 60 years of age are viduals remaining ambulatory well into adult-
diagnosed with this condition.3 This condition hood. Emery-Dreifuss muscular dystrophy
was originally thought to be secondary to presents in the second to third decade; this
environmental and genetic factors, but evi- phenotype includes proximal weakness, joint
dence suggests an autoimmune response to a contractures, and cardiac arrhythmias. Diffi-
viral infection, which subsequently targets culty raising arms above the head, muscular
myelin.3 The diagnosis may be confirmed by atrophy, and winging of the scapulae present-
a thorough history, physical examination, ing between 6 and 20 years of life are charac-
magnetic resonance imaging, analysis of cere- teristic of facioscapulohumeral muscular
brospinal fluid, and evoked potentials.3,62,63 dystrophy. Limb girdle muscular dystrophies
Life expectancy and cause of mortality are can present in the first to fifth decade of life,
similar for all types of this condition.3 Clinical with a characteristic presentation involving
characteristics that are associated with a longer primarily proximal weakness (scapulohumeral
time interval for progression of disability and pelvifemoral), but may initially present or
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Chapter 33 Neurological Complications of Acquired Immunodeficiency Syndrome 699

progress to include distal weakness, joint ■ Neurological Complications of

WEAKNESS
contractures, and cardiac involvement. Addi- Acquired Immunodeficiency
tional dystrophies include oculopharyngeal, dis- Syndrome
tal muscular dystrophies, congenital muscular Chief Clinical Characteristics
dystrophies, and myotonic dystrophy. Diagno- This presentation is variable and dependent on
sis requires laboratory studies, deoxyribonu- the affected neuroanatomical structures in an
cleic acid analysis, clinical examination, individual with acquired immunodeficiency
and family history when available. There is syndrome.67
no specific treatment. Medical management
consists of nutritional support, pharmacologic Background Information
management to retard progression or manage This condition may be categorized by:
symptoms, and physical therapy to protect
range of motion, strength, and function. 1. Meningitic symptoms including headache,
Supportive care and equipment recommenda- malaise, and fever (such as secondary to
tions are also provided. The prognosis varies meningitis, cryptococcal meningitis, tuber-
depending on the type of dystrophy; the life culous meningitis, and human immunode-
expectancy of individuals with Duchenne type ficiency virus headache)
is usually limited to late adolescence, whereas 2. Focal cerebral symptoms including hemi-
those with the scapulohumeral type live into paresis, aphasia, apraxia, sensory deficits,
the seventh or eighth decade. homonymous hemianopia, cranial nerve
involvement, balance deficits, incoordina-
■ Myasthenia Gravis tion, and/or ataxia (such as secondary to
cerebral toxoplasmosis, primary central nerv-
Chief Clinical Characteristics ous system lymphoma, and progressive
This presentation is characterized by weak- multifocal leukoencephalopathy)
ness that worsens during periods of activity and 3. Diffuse cerebral symptoms that involve
improves after periods of rest. Patients present cognitive deficits, altered level of conscious-
with variable degrees of weakness, ranging ness, hyperreflexia, Babinski sign, presence of
from ptosis or diplopia to critical respiratory primitive reflexes (such as secondary to
weakness. Muscles that control speech, facial postinfectious encephalomyelitis, acquired
expression, mastication, swallowing, breath- immunodeficiency dementia complex,
ing, and neck and limb movements may be cytomegalovirus encephalitis)
involved.65 4. Myelopathy associated with gait difficulties,
spasticity, ataxia, balance deficits, and hyper-
Background Information
reflexia (such as secondary to herpes zoster
This condition is caused by autoimmune
myelitis, vacuolar myelopathy that occurs
mediated acetylcholine receptor damage,
with acquired immunodeficiency syndrome
resulting in a deficit in neuromuscular trans-
dementia complex)
mission. Diagnosis is achieved through clinical
5. Peripheral involvement associated with
observation of skeletal muscle weakness
sensory changes, weakness, balance deficits,
increased by exercise and relieved by rest, im-
and pain (such as secondary to peripheral
provement with anticholinesterase medica-
neuropathy, acute and chronic inflamma-
tions, electrophysiological evidence of limited
tory demyelinating polyneuropathies).3,68
neuromuscular transmission, and the presence
of circulating acetylcholine receptor antibodies. Abnormal neurological findings are ob-
Medications are used to improve function of served during a clinical examination in approx-
the neuromuscular junction and alter the im- imately one-third of patients with acquired
mune response, but have significant side effects. immunodeficiency syndrome; however, on
Thymectomy and plasmapheresis are other autopsy most individuals with this condition
possible therapeutic interventions.66 With treat- have abnormalities within the nervous sys-
ment, most patients with myasthenia gravis tem.3 Diagnosis of the variable neurological
recover control of the affected musculature; complications associated with this condition
however, some weakness may remain or relapse. may be confirmed with laboratory tests,
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700 Chapter 33 Carpal Tunnel Syndrome

cerebrospinal fluid cultures, imaging, nerve con- impaired sensation in the legs and hands,
WEAKNESS

duction studies, and physical examination.3,54,67 progressing in a distal to proximal pattern.


Treatment appears to be limited primarily to Presentation of tingling, numbness, weak-
the use of antiviral medications.67 Physical and ness, areflexia, and abnormal sensation in a
occupational therapy may be indicated to young adult male or female raises the index
address equipment needs and caregiver/patient of clinical suspicion for this condition.71
training related to functional mobility.
Background Information
NEUROPATHIES This condition is caused by damage to the
myelin sheath of the peripheral nerves. Diag-
■ Carpal Tunnel Syndrome nosis requires acute onset of progressive
Chief Clinical Characteristics weakness of the limbs, sensory dysfunction
This presentation typically involves weakness with hyporeflexia or areflexia, elevated cere-
of grip strength and difficulty performing brospinal fluid protein, and demyelinative
tasks requiring grasp or manipulation of features on electromyography-nerve con-
objects, which is preceded by initial report of duction velocity studies. Pharmacologic
pain and numbness/tingling in the palmar management involves anti-inflammatories,
aspect of the thumb, index finger, middle immunosuppressive agents, intravenous
finger, and radial half of the ring finger with immunoglobulin therapy, and plasmaphere-
radiation up the forearm.68 sis.71 Physical therapy is indicated to im-
prove strength, mobility, and endurance and
Background Information
to prevent secondary impairments.72 Early
This condition results from compression of
diagnosis and treatment is essential to pre-
the median nerve as it passes through the
vent axonal loss. The course varies among
carpal tunnel. It is associated with repetitive
individuals, from spontaneous recovery to
stress, such as typing or performing assem-
bouts of recovery and relapse. Recovery is
bly line tasks. Differential diagnosis includes
seen in patients with a relapsing-remitting
cervical radiculopathy or compression of
course, young onset, and early treatment.
the median nerve proximal to the carpal
Individuals with axonal loss, chronic pro-
tunnel. Diagnosis is achieved through use of
gressive course with combined sensory and
special tests (eg, Phalen’s sign, monofilament
motor presentation, older age at onset,
testing, and provocative tests) and via elec-
and/or diabetes have a poor prognosis for
tromyography and nerve conduction veloc-
recovery of function.73,74
ity tests.69 Treatment consists of splinting,
pharmacologic management of inflamma- ■ Compression Neuropathy
tion and pain, modalities, stretching and
strengthening, ergonomic modifications, Chief Clinical Characteristics
and surgery if indicated.70 Nonsurgical care This presentation can be characterized by
including physical therapy is emphasized weakness that is often preceded by a lengthy
first, and it is most effective in those with history of pain, paresthesias, and numbness
mild impairment. If nonsurgical manage- in the distribution of a specific peripheral
ment fails, surgery is usually recommended. nerve. Associated lower motor neuron signs of
In those individuals with severe forms of hyporeflexia, atrophy, and fasciculations may
this condition who have been properly diag- occur, as well as trophic skin changes with
nosed, 70% report complete satisfaction severe compression neuropathies.
with pain relief. However, residual weakness Background Information
and reoccurrence may occur.68 This group of conditions is often called
mononeuropathy simplex, referring to a sin-
■ Chronic Inflammatory gle peripheral nerve. The distribution of sen-
Demyelinating Polyneuropathy sorimotor symptoms is specific to the com-
Chief Clinical Characteristics pressed nerve. The most common form of
This presentation is characterized by sym- this condition is carpal tunnel syndrome, in
metrical distal progressive weakness and which the medial nerve is compressed at the
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Chapter 33 Multifocal Motor Neuropathy 701

wrist. Other upper extremity neuropathies condition may be a result of vascular com-

WEAKNESS
include interdigital, ulnar, radial, and tho- plications disrupting the supply of nutrients
racic outlet syndrome. Lower extremity to the nerves.54,77 A thorough history, physi-
compression neuropathies include peroneal, cal examination (specifically including the
femoral, saphenous, lateral femoral cuta- assessment of deep tendon reflexes and sen-
neous, and obturator nerve lesions, as well as sory examination), electromyography/nerve
tarsal tunnel syndrome. Diagnosis involves conduction testing, and laboratory screen
clinical examination, detailed history, and helps to differentiate other causes of neu-
often electrodiagnostic testing. Prognosis for ropathy.75,76 Treatment consists of maintain-
recovery of sensation and strength is poor in ing a normal range of blood glucose lev-
the presence of lower motor neuron signs els.3,75,76 In addition, individuals with this
and trophic changes, indicating long-stand- condition may prevent complications by
ing compression. However, if diagnosed completing visual inspection of the skin and
early, prognosis for recovery of sensation routine podiatry care.76 Medications may
and strength is good. Treatment includes help to control symptoms such as paresthe-
accurate diagnosis, removal of compression sias or pain.3,54 Additional management may
via physical therapy intervention or surgery, include consultations with an orthotist to
and preventative interventions to preclude ensure proper fitting of foot wear and
recurrence of the neuropathy. physical therapy to minimize disability by
addressing impairments associated with lim-
■ Diabetes Mellitus-Induced itations in functional mobility.77
Neuropathies
Chief Clinical Characteristics ■ Hereditary Motor and Sensory
This presentation is variable, including man- Neuropathies
ifestations such as acute diabetic mononeu- Chief Clinical Characteristics
ropathies, which may include involvement This presentation includes distal sensory
of cranial nerves (eg, oculomotor or abducens abnormalities, such as numbness and tingling
nerve involvement) or peripheral nerves3; of the feet, and muscle weakness of distal mus-
multiple mononeuropathies and radicu- culature. Individuals affected with heredi-
lopathies, which may include unilateral or tary neuropathies may also report sweating
asymmetric pain, low back pain with or with- and dizziness upon standing.
out symptoms in leg, weakness, atrophy,
Background Information
diminished or absent deep tendon reflexes,
Hereditary neuropathies include hereditary
and sensory deficits3; distal polyneuropathy,
motor and sensory neuropathy, hereditary
the most common diabetic neuropathy, which
sensory neuropathy, hereditary motor neu-
consists of chronic, symmetric, distal sensory
ropathy, and hereditary sensory and auto-
deficits (eg, numbness and tingling), dimin-
nomic neuropathy. The majority of all heredi-
ished or absent deep tendon reflexes, balance
tary neuropathies are Charcot-Marie-Tooth
deficits, and weakness3; and autonomic
neuropathy. Inherited polyneuropathies are
neuropathy, which may involve resting
caused by genetic abnormalities. Diagnosis is
tachycardia, orthostatic hypotension, sexual
made by nerve conduction and electromyo-
impotence, exercise intolerance, abnormal
graphic studies. Prognosis for hereditary sen-
sweating, pupil abnormalities, weakness,
sory neuropathies is poor due to intractable
sensory deficits, and gastroparesis.3,54,75,76
pain.78 Prognosis for hereditary motor and
Background Information sensory neuropathies has also been found to
Approximately 15% to 20% of people with be unfavorable due to slowing of conduction
diabetes may present with the clinical velocity with age.79
signs and symptoms of this condition.3,75
However, approximately 50% will have neu- ■ Multifocal Motor Neuropathy
ropathic symptoms and may have abnormal- Chief Clinical Characteristics
ities in nerve conduction testing.3 Com- This presentation includes progressive, asym-
monly considered a metabolic disorder, this metrical weakness, muscle atrophy, cramps,
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702 Chapter 33 Peripheral Neuropathies

and fasciculations that develop slowly over neuropathy and temporal features. The diag-
WEAKNESS

several years. Other symptoms include wrist nosis may be confirmed after completing a
and foot drop, grip weakness, reduced ten- thorough history, physical examination, labo-
don reflexes in affected areas, and occasional ratory testing, and possibly electromyography/
cranial or phrenic nerve involvement. nerve conduction testing.3,85 Treatment and
prognosis will vary depending on the etiology
Background Information
and severity.
This condition is thought to be immunolog-
ically mediated, although the exact mecha- ■ Toxic Neuropathy
nism is unknown.80 Diagnosis is made by the
presence of definite motor conduction block Chief Clinical Characteristics
with normal sensory nerve conduction on This presentation can involve onset of weak-
electrophysiological study.81 Medical treat- ness, sensory deficits, and hyporeflexia in a
ment is intravenous immunoglobulin ther- distal to proximal polyneuropathy pattern
apy.81,82 This condition has a slow, progres- following exposure to a toxic agent.86 Presen-
sive course of deterioration of muscle tation of weakness is often preceded by abnor-
strength, but the disease generally does not mal changes in sensation, often hyperalgesia.
cause severe disability or death.81,82 Background Information
This condition may be caused by drugs,
■ Peripheral Neuropathies
organic compounds found in solvents and
Chief Clinical Characteristics glues, heavy metals (ie, lead, arsenic, thal-
This presentation includes weakness and sen- lium, gold), and plant or animal toxins. Most
sory changes along the distribution of a periph- forms of this condition are primarily sensory
eral nerve. Other potential signs and symptoms or sensorimotor in nature.2 Forms of this
may include impaired balance, diminished or condition that are associated with weakness
absent deep tendon reflexes, fasciculations, at onset can be due to lead (often presenting
syncope, abnormal sweating, orthostatic hy- as distal upper extremity weakness) or inges-
potension, resting tachycardia, and trophic tion of plant or shellfish toxins. Electrodiag-
changes.3,83 nostic testing and histopathological analysis
identify axonal damage. Effective medical
Background Information
management involves supportive care and
The patterns of this condition are variable and
removal or avoidance of the offending toxin.
may present as polyneuropathy, polyradicu-
The majority of cases of this condition are
lopathy, neuronopathy, mononeuropathy,
self-limited and improve after removal of the
mononeuropathy multiplex, and plexopa-
toxic agent.86
thy.3 Some of the etiologies associated with
peripheral neuropathies include trauma, in- ■ Neurosarcoidosis
flammation (eg, acute demyelinating polyneu-
Chief Clinical Characteristics
ropathy [Guillain-Barré syndrome], herpes
This presentation may be characterized by
zoster, Lyme’s disease, human immunodefi-
facial palsy, impaired taste, sight, smell or swal-
ciency virus), metabolic (eg, diabetes melli-
lowing; vertigo; loss of sensation in a stocking/
tus, uremia), nutritional (eg, vitamin B defi-
glove pattern; and weakness in a distal greater
ciencies commonly associated with alcohol
than proximal distribution.3
abuse, eating disorders, and individuals with
malabsorption syndromes), hereditary, idio- Background Information
pathic (eg, aging or unknown causes), and This condition is a manifestation of sarcoido-
toxic (eg, exposure to lead, arsenic, or thal- sis with central and/or peripheral nervous
lium or to chemotherapeutic drugs such as system involvement. It is characterized by
vincristine, cisplatin).83 Twenty percent of formation of granulomas in the central nerv-
individuals over the age of 60 are affected ous system. The lesion consists of lymphocytes
by a type of peripheral neuropathy.84 The and mononuclear phagocytes surrounding a
diagnosis of the specific disorder may be noncaseating epithelioid cell granuloma.
differentiated by the pattern of peripheral These granulomas represent an autoimmune
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Chapter 33 Parry-Romberg Syndrome 703

response to central nervous system tissues. the nervous system. This can eventually disrupt

WEAKNESS
This condition includes 5% of individuals or kill neurons. It results from exposure to sub-
with sarcoidosis. The diagnosis is established stances used in chemotherapy, radiation treat-
by the presence of clinical features, along with ment, drug therapies, and organ transplants, as
clinical and biopsy evidence of sarcoid granu- well as exposure to heavy metals, foods, or pes-
lomas in tissues outside the nervous system. ticides. Diagnosis is supported by clinical pres-
Approximately two-thirds of individuals expe- entation and lab tests for detection of the toxic
rience this illness only once, whereas the substance. Treatment is prioritized at removal
remainder experience chronic relapses. Primary of the offending toxin. Prognosis varies greatly
treatment for neurosarcoidosis is the adminis- depending on the level of exposure and individ-
tration of corticosteroids. ual’s comorbid medical conditions.

■ Neurosyphilis (Tabes Dorsalis, ■ Normal Pressure Hydrocephalus


Syphilitic Spinal Sclerosis, Chief Clinical Characteristics
Progressive Locomotor Ataxia) This presentation is characterized by progres-
Chief Clinical Characteristics sive difficulty in walking characterized by dimin-
This presentation can be characterized by hemi- ished cadence, widened base, short steps, and en
paresis, ataxia, aphasia, gait instability, falls, bloc turning (requiring more than 3 steps to
neuropathy, personality and cognitive changes, make a 90-degree turn). Movements involving
seizures, diplopia, visual impairments, hearing the axial musculature appear awkward and
loss, psychotic disorders, loss of bowel/bladder apraxic. Progression involves impairment in
function, pain, hyporeflexia, and hypotonia.87,88 mental function and sphincter incontinence.

Background Information Background Information


Treponema pallidum infects the human host by Symptoms are caused by increased pressure
way of contact with contaminated bodily flu- on the brain, specifically the frontal lobe, due
ids or lesions.87 This spirochete is responsible to an abnormal increase in cerebrospinal
for the diagnosis of syphilis; however, when fluid secondary to trauma, infection, space-
T. pallidum is present within the central nerv- occupying lesion, or unknown cause. It is
ous system the individual is diagnosed with most common in the elderly, but can occur
neurosyphilis.88 This condition occurs in ap- in people of any age. Symptoms must be
proximately 10% of individuals with untreated differentiated from disorders with similar
syphilis, and in 81% of these cases it presents presentation such as Alzheimer’s, Parkinson’s,
as meningovascular, meningeal, and general and Creutzfeldt-Jakob diseases.89 Diagnosis
paresis. Treatment includes use of various involves the clinical presentation combined
forms of penicillin or alternative choices for with imaging to identify ventricular enlarge-
those allergic to penicillin87 and may involve ment, intracranial pressure monitoring, and
rehabilitative therapies depending on the neuropsychological testing.89 Treatment en-
individual’s activity limitations or participa- tails shunt placement to drain cerebrospinal
tion restrictions. A better prognosis has been fluid and regular follow-up by a physician to
observed for individuals treated during early monitor shunt function. Without treatment,
neurosyphilis.88 symptoms continue to worsen. With shunt
placement, 60% of individuals with this condi-
■ Neurotoxicity tion improve significantly, and 30% will com-
pletely recover and return to premorbid levels
Chief Clinical Characteristics of function. Others have residual gait, sphinc-
This presentation includes limb weakness or teric, and cognitive deficits.90
numbness, loss of memory, vision, and/or intel-
lect, headache, behavioral problems, and sexual ■ Parry-Romberg Syndrome
dysfunction.
Chief Clinical Characteristics
Background Information This presentation involves atrophy of one
This condition occurs when exposure to natural side of the face, head, and associated structures.
or artificial toxins alters the normal activity of The left side of the face is usually affected.
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704 Chapter 33 Pelizaeus-Merzbacher Disease

Individuals may also report difficulties involv- accompanied by signs and symptoms of sys-
WEAKNESS

ing the tongue, lips, and salivary glands. temic infection including fever, nausea, vomit-
Headaches, facial pain, and seizures may occur. ing, pain, and respiratory distress.3
Background Information Background Information
This condition is a rare disease. Exact etiology The incidence of poliomyelitis has been
is unknown, although possible causes do nearly eradicated in the United States, but
include trophic malfunction of the cervical outbreaks continue in some areas of Africa,
sympathetic nervous system or viral or Borre- Southeast Asia, and India. This mainly affects
lia infection.91 Diagnosis is established by clin- children and immunocompromised individ-
ical presentation along with radiology reports uals. The virus that causes this condition
revealing deficient root development or root selectively attacks the anterior horn motor
resorption, and histological studies demon- neurons as well as cranial nerve motor
strating atrophy of the epidermis and infil- nuclei, resulting in denervation and further
trates of lymphocytes and monocytes. Treat- Wallerian degeneration.3 In individuals who
ment is symptomatic and may include facial survive the initial insult, recovery of strength
plastic surgery after the disease process has via neuronal sprouting may occur, resulting
stopped. This disease is a degenerative process in giant motor units. Paralysis may be
with a poor prognosis. reversed; however, the patient is at risk for
post-polio syndrome sequelae later in life.
■ Pelizaeus-Merzbacher Disease Physical therapy is indicated for supportive
Chief Clinical Characteristics care, prevention of secondary impairments,
This presentation commonly includes deterio- and education on prevention of exacerba-
ration of strength, coordination, motor abilities, tions of post-polio syndrome.3
and intellectual function.
■ Post-Polio Syndrome
Background Information
Chief Clinical Characteristics
Severity and onset of the disease range widely,
This presentation usually includes debilitating
depending on the type of genetic mutation,
and slowly progressive muscle weakness. As-
and extends from the mild, adult-onset spastic
sociated symptoms include atrophy, myalgia,
paraplegia to the severe form with onset at
fasciculations, cramps, respiratory insuffi-
infancy and death in early childhood.92 This
ciency, bulbar muscle dysfunction, and sleep
condition is an X-linked disease caused by a
apnea.95
mutation in the gene that controls the produc-
tion of a myelin protein called proteolipid Background Information
protein.92 Genetic diagnostic testing is the This condition affects an average of 50%
definitive method for diagnosing this condi- of survivors of poliomyelitis approximately
tion.93 There is no cure. Therefore, treatment 35 years after the initial onset.96 The syndrome
is based on symptoms and includes physical slowly progresses in a stepwise course. Severity
therapy, orthotics, and antispasticity agents of weakness is correlated to severity of acute
with goals to minimize the development of weakness and disability from original infec-
joint contractures, dislocations, and kyphosco- tion. Pathophysiology is unknown, but a com-
liosis.94 Individuals with severe forms of this mon hypothesis purports that over time, excess
condition experience progressive deterioration metabolic stress on remaining motor neurons
until death. Individuals with the adult-onset causes dropout of new nerve terminals and
form with spasticity may have nearly normal eventually death of the motor neurons.97
life span.4 Diagnosis is attained through exclusion of
other causes using laboratory studies, elec-
■ Poliomyelitis tromyography, imaging, and a history of acute
Chief Clinical Characteristics poliomyelitis infection.95 Treatment is achieved
This presentation typically involves acute, through lifestyle changes for energy conserva-
asymmetrical, flaccid paralysis with associated tion, weight loss, and use of assistive devices,
LMN signs of hyporeflexia and atrophy, pharmacologic agents to ameliorate fatigue, and
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Chapter 33 Brown-Sequard Syndrome 705

nonfatiguing exercise. Low-intensity, alternate- have been widely used in the treatment of

WEAKNESS
day, interval-strength, and cardiovascular exer- this condition.98
cise is indicated to combat fatigue, weakness,
and myalgias as long as care is taken to avoid ■ Spinal Cord Infarction (Vascular
overwork.95 Myelopathy)
Chief Clinical Characteristics
■ Primary Lateral Sclerosis This presentation depends on the location of
Chief Clinical Characteristics the infarction. The most common anterior
This presentation includes spastic parapare- spinal artery syndrome presents with bilat-
sis of voluntary muscles with associated eral, incomplete motor paralysis and loss of
upper motor neuron signs in the absence of pinprick and temperature sensation below the
lower motor neuron signs. Onset of difficulty level of the lesion, loss of deep tendon reflexes,
with gait, balance, and leg weakness appears sphincter paralysis, acute complaint of back
in the fifth to sixth decade of life and may pain, and spared proprioception. Onset of
progress to affect upper extremity and facial symptoms occurs over the course of minutes to
musculature. hours.
Background Information Background Information
Imaging, cerebrospinal fluid analysis, and elec- Most forms of this condition are insidious, or
tromyographic studies confirm the diagnosis. secondary to aortic surgery, aortic rupture,
In particular, this condition is differentiated atherosclerosis, aneurysm, or an acute deficit
from the more severe amyotrophic lateral scle- of perfusion. Diagnosis is often difficult at ini-
rosis only after a period of 3 years from onset tial presentation, because magnetic resonance
to ensure the absence of lower motor neuron imaging may be negative. Repeat magnetic
signs.3 Treatment for this condition addresses resonance imaging may show T2 hyperinten-
symptom management. Physical therapy is sity secondary to edema.99 This condition
indicated to prevent immobility. This condi- has a poor prognosis with often permanent
tion is not fatal. Progression of symptoms disability. Prognosis for recovery of ambula-
varies; some individuals maintain ambulatory tion is inversely correlated with the severity
status throughout life while others become of motor deficit at onset.100 Physical therapy
wheelchair bound. interventions address weakness, spasticity, and
mobility and ambulation deficits.
■ Rheumatoid Arthritis
Chief Clinical Characteristics SPINAL CORD INJURIES
This presentation may be characterized by mus- ■ Brown-Sequard Syndrome
cle weakness that is either generalized or more Chief Clinical Characteristics
focal in proximal muscles. Typically, this con- This presentation can be characterized by
dition presents with joint pains. Leg pain (18%) ipsilateral loss of strength and tactile discrim-
and leg numbness (14%) have been reported in ination, position, and vibration sense, with
people with this condition.98 contralateral hemianesthesia to pain and
temperature secondary to spinal cord injury,
Background Information disk herniation, tumor, ischemia, or inflam-
Women are twice as likely as men to be matory disorder.101,102
affected. Symptoms associated with this pro-
gressive inflammatory joint disease are Background Information
caused by synovial membrane thickening This condition occurs secondary to lateral
and cytokine production in synovial fluid. hemisection of the spinal cord, usually
Blood tests confirm the diagnosis if rheuma- below the cervical enlargement, disrupting
toid factor is detected. Typical radiographic ipsilateral corticospinal and dorsal column
findings include disk space narrowing, facet tracts and contralateral projections of the
erosion, and end-plate erosion.98 Steroidal, spinothalamic tract.4 Medical management
nonsteroidal, and biological medications consists of diagnosis via imaging, appropriate
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706 Chapter 33 Central Cord Syndrome

medical care in the acute care setting, following surgical intervention occurs in
WEAKNESS

surgical decompression of spinal cord if more than half of the patients following
indicated, and multidisciplinary rehabilita- surgical intervention.107
tion.103 Outcome is favorable in most
patients following surgical decompression of ■ Traumatic Spinal Cord Injury
disk herniation or tumor, demonstrating Chief Clinical Characteristics
vast improvement in motor function with This presentation is characterized by loss of
minimal residual sensory deficits.102 sensation in a dermatomal pattern, hyper-
tonicity, spasticity, hyperreflexia, and sphinc-
■ Central Cord Syndrome ter dysfunction in combination with muscle
Chief Clinical Characteristics weakness. Specific syndromes have charac-
This presentation commonly involves pres- teristic presentations secondary to the location
entation of profound weakness of the arms and of the lesion.
hands, and to a lesser extent the legs, commonly Background Information
due to traumatic spinal cord injury. Associ- Insult to the spinal cord results from frac-
ated sensory loss below the level of the lesion tured bone, displaced disk material, or a
and/or sphincter dysfunction may occur. foreign object transecting or injuring the
Background Information cord. Imaging is used to identify the cause of
Damage to the more centrally located as- injury and direct surgical stabilization or in-
cending and descending spinal tracts results tervention. Motor vehicle accidents and falls
in this characteristic presentation of motor are the most common etiology of this condi-
and sensory loss. Medical management con- tion.108 Physical examination of strength and
sists of diagnosis via imaging, appropriate sensation is used to assign a score from the
medical care in the acute care setting, reduc- American Spinal Injury Association.109 In-
tion of fracture and/or surgical decompres- juries are classified in terms of neurological
sion of spinal cord, and multidisciplinary level (ie, most rostral segment where my-
rehabilitation.104 Many individuals with otomal and dermatomal function is spared)
central cord syndrome recover the ability to and extent as either complete (total lack of
walk, but impairment of fine motor control sensory or motor function below level of
in the hands often remains.105 njury) or incomplete (some motor or sen-
sory function spared below the level of in-
■ Conus Medullaris Syndrome jury). Cervical locations of injury result in
tetraplegia and may cause paralysis or weak-
Chief Clinical Characteristics
ness of the respiratory musculature, requir-
This presentation typically involves weakness
ing mechanical ventilation and/or respira-
of the lower extremities in association with
tory strengthening.110 Thoracic or lumbar
hyperreflexia, bowel/bladder dysfunction,
locations of injury result in paraplegia.
sexual dysfunction, and sensory loss in a der-
Treatment consists of medical management,
matomal pattern of the sacral segments.4
multidisciplinary rehabilitation, equipment
Background Information prescription, and prevention of pressure
The presentation of weakness with upper ulcers, contractures, and further complica-
motor neuron symptoms is secondary to tions. Individuals with incomplete forms
injury to the conus medullaris, most com- of this condition may continue to recover
monly due to trauma (vertebral body strength and function, while individuals
fracture of acute disk herniations of the with complete forms of this condition have a
thoracolumbar junction).106 Diagnosis and poor prognosis for recovery and instead use
treatment involve clinical examination, im- compensatory techniques and equipment.
aging, and surgical investigation, decom- Individuals with this condition require ade-
pression, fusion, and fixation if indicated. quate follow-up medical care to prevent sec-
Improvement in spinal cord function, ondary impairments.111 Acute forms of this
bladder function, and nerve root recovery condition constitute a medical emergency.
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Chapter 33 Tethered Spinal Cord Syndrome 707

■ Spinal Muscular Atrophy 100,000 people.115 This condition is diagnosed

WEAKNESS
(Werdnig-Hoffmann Disease, using clinical presentation and positive find-
Kugelberg-Welander Disease) ings on computed tomography or magnetic
Chief Clinical Characteristics resonance imaging. Medication, surgery, and
This presentation is characterized by profound interdisciplinary therapy are the most com-
weakness and hypotonia in infancy, early child- mon treatments for this condition. The prog-
hood, or adolescence associated with an auto- nosis for recovery is predicted by the magni-
somal dominant genetic disorder. Deep tendon tude of initial deficit. Factors that are
reflexes are absent, but sphincter tone and associated with poor outcomes include coma,
sensation are intact. poor cognition, severe aphasia, severe hemi-
paresis with little return within 1 month,
Background Information visual perceptual disorders, depression, and
This condition is the most common neuromus- incontinence after 2 weeks.116,117
cular disease in children.112 Onset of spinal mus-
cular atrophy I (Werdnig-Hoffmann disease) ■ Systemic Lupus Erythematosus
occurs in infancy and is always fatal. Onset of Chief Clinical Characteristics
spinal muscular atrophy II is between 6 and This presentation can include abnormal vision,
18 months, as noted by the child’s ability to sit swallowing, taste, hearing, changes in mood or
but not stand or walk. Spinal muscular atrophy thinking, and seizures in combination with
III (Kugelberg-Welander disease) is the mildest fatigue, joint pain, and swelling affecting the
form with onset any time after age 18 months to hands, feet, knees, and shoulders.
early adulthood. Earlier onset is associated with
more severity and shorter life expectancy.113 In- Background Information
dividuals with spinal muscular atrophy will lose This condition affects mostly women of child-
function over time. There is no cure for spinal bearing age. It is a chronic autoimmune disor-
muscular atrophy. Therefore, treatment includes der that can affect any organ system, including
therapy to preserve mobility and reduce disabil- skin, joints, kidneys, brain, heart, lungs, and
ity as the disease progresses. The most common blood. Microinfarcts in the cerebral cortex and
cause of death is respiratory failure.114 brainstem, which lead to destructive and pro-
liferative changes in capillaries and arterioles,
■ Stroke (Cerebrovascular Accident) are primarily responsible for central nervous
system manifestations. Hypertension and
Chief Clinical Characteristics endocarditis can also predispose an affected
This presentation may include a wide range of individual to development of neurological
symptoms that correspond to specific areas of abnormalities. Multiple sclerosis is a disease
the brain that are affected, potentially includ- that may be mistaken for this condition, espe-
ing weakness or numbness, especially on one cially if the central nervous system manifesta-
side of the body or face, confusion, aphasia, bal- tions include visual dysfunction. The diagnosis
ance deficits or falls, visual disturbances, or is confirmed by the presence of skin lesions;
sudden severe headache with no known cause. heart, lung, or kidney involvement; and
Background Information laboratory abnormalities including low red or
This condition occurs when blood flow to the white cell counts, low platelet counts, or posi-
brain is interrupted either by blockage (is- tive ANA and anti-DNA antibody tests.118
chemia or infarction) or from hemorrhagic Treatment involves corticosteroid medication.
disruption. A thrombosis or embolic occlusion
of an artery causes an ischemic type of this ■ Tethered Spinal Cord Syndrome
condition. A hemorrhagic type of this condi- Chief Clinical Characteristics
tion can be caused by arteriovenous malfor- This presentation typically includes back pain,
mation, hypertension, aneurysm, neoplasm, associated with neurological deficits, and bowel
drug abuse or trauma. This condition is the and bladder dysfunction.119 The most common
most common and disabling neurological dis- manifestations of this condition are worsening
order in adults and occurs in 114 of every in motor function of the lower extremities (and
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708 Chapter 33 Thoracic Outlet Syndrome

less likely the upper extremities), changes in decreased pain and improved function is
WEAKNESS

muscle tone and deep tendon reflexes, progressive good for the majority of individuals with this
loss of articular dexterity, progressively worsen- condition.
ing scoliosis or kyphosis, and back or leg pain.120
■ Thyrotoxic Myopathy (Graves’
Background Information Disease)
This condition occurs commonly in children,
but also can present in undiagnosed adults. Chief Clinical Characteristics
Magnetic resonance imaging confirms the This presentation includes progressive weak-
diagnosis, with a low-lying (caudally posi- ness, wasting of the pelvic girdle and shoulder
tioned) conus medullaris present. Surgical muscles, fatigue, and heat intolerance. Physical
resection of a thickened filum terminale is a acts such as climbing stairs may be difficult.
common treatment. Individuals with this condition may develop
muscle damage to the eyes and eyelids, which
■ Thoracic Outlet Syndrome may affect mobility of the eye muscles, and
temporary, but severe, attacks of muscle weak-
Chief Clinical Characteristics
ness known as periodic paralysis.
This presentation can be characterized by
swelling or puffiness in the arm or hand; bluish Background Information
discoloration of the hand; a feeling of heaviness This condition is a rare neuromuscular disor-
in the arm or hand; deep, boring toothache-like der that may accompany hyperthyroidism
pain in the neck and shoulder region that seems (Graves’ disease) caused by overproduction of
to increase at night; easily fatigued arms and the thyroid hormone thyroxine. It is more
hands; superficial vein distention in the hand; common in middle-aged men and in individ-
paresthesias along the inside forearm and the uals of Asian descent.121 This condition is
palm; muscle weakness with difficulty gripping difficult to diagnose because reflexes, elec-
and performing fine motor tasks of the hand; at- tromyography, muscle enzymes, and muscle
rophy of the muscles of the palm; cramps of the biopsy are usually normal. Myopathy often
muscles on the inner forearm; pain in the arm improves as a result of restoring normal
and hand; and tingling and numbness in the thyroid function via medications, surgery, or
neck, shoulder region, arm, and hand. radioiodine therapy.122 Complete or partial
removal of the thyroid may be required in
Background Information severe cases. With treatment, muscle weakness
There are three types of this condition which often improves or may be reversed.
can coexist or occur independently: compres-
sion of the subclavian vein, compression of the ■ Todd’s Paralysis
subclavian artery, and a primary neurological
Chief Clinical Characteristics
syndrome. Multiple anatomical anomalies can
This presentation includes partial or complete
lead to thoracic outlet syndrome including an
paralysis on one side of the body following a
incomplete cervical rib, a taut fibrous band
seizure.
passing from the transverse process of C7 to
the first rib, a complete rib that articulates Background Information
with the first rib, or anomalies of the position This condition is rare and its etiology is
and insertion of the anterior and medial unknown. Diagnosis is made by clinical
scalene muscles. Diagnosis includes physical presentation and exclusion of competing
examination tests (ie, Adson’s test, extremity diagnoses, particularly stroke or a transient
abducted stress test, costoclavicular sign), ischemic attack via magnetic resonance imag-
radiology of the cervical spine, and nerve ing or computed tomography. Treatment is
conduction and electromyography studies. symptomatic, because recovery spontaneously
Nonsurgical approaches to treatment include occurs, usually within 48 hours. Prognosis
exercise, stretches, modalities, and analgesic is excellent for recovery of hemiparesis.
medication. Surgery is indicated if pain is However, specific etiology of the seizures will
persistent and severe neurogenic or vascular determine prognosis for resolution of seizures
features of the syndrome exist. Prognosis for and this condition.3
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Chapter 33 Transverse Myelitis 709

■ Toxic Myopathy from this condition and acute stroke, indi-

WEAKNESS
Chief Clinical Characteristics viduals should assume that all stroke-like
This presentation can be characterized by symptoms signal a medical emergency. A
generalized or local weakness, with or without prompt evaluation (within 60 minutes) is
muscle pain, associated neuropathy, or systemic necessary to identify the cause of this condi-
illness following exposure or ingestion of a toxic tion and determine appropriate therapy. De-
substance. pending on the individual’s medical history
and the results of a medical examination, the
Background Information doctor may recommend drug therapy or sur-
The presentation of this condition varies for gery to reduce the risk of stroke. Antiplatelet
each toxin or drug. Toxic substances produce medications, particularly aspirin, are a stan-
myopathy via various mechanisms. Primary dard treatment for individuals suspected of
etiology relates to directly damaging the mus- this condition and who also are at risk
cle cell. Secondary etiology occurs via elec- for stroke, including individuals with atrial
trolyte disturbances, excessive energy require- fibrillation.
ments, or inadequate delivery of oxygen and
nutrients due to muscle compression and ■ Transverse Myelitis
ischemia.123 This group of conditions is classi- Chief Clinical Characteristics
fied according to presentation, histopathologi- This presentation involves the gradual develop-
cal features, pathogenetic mechanism, or type ment of weakness and sensory changes below the
of toxin. Diagnosis relies on laboratory testing, level of the lesion, back or neck pain, and/
electrodiagnostic investigation, and often or bowel and bladder dysfunction over the
muscle biopsy.124 Treatment involves removal course of several hours to weeks.
of the toxic agent, supportive physical therapy
Background Information
to prevent secondary impairments and recover
This condition occurs when inflammation
strength, and pharmacologic intervention.
affects the spinal cord, but the brain can be
Prognosis is often good with early diagnosis
affected as well. Inflammation can result from
and removal of the toxic agent; however, when
viral infections, abnormal immune reactions,
myopathy progresses to involve respiratory
or ischemia or present as an idiopathic form.
and swallowing function or if the toxin affects
Diagnosis is established by exclusion through
other organs including the kidney, prognosis
imaging and blood tests. The first line of treat-
for full recovery is poor.124,125
ment requires accurate diagnosis of the under-
■ Transient Ischemic Attack lying pathology and decreasing inflammation
in the acute stage, usually by way of corticos-
Chief Clinical Characteristics
teroid medication. Physical therapy is indi-
This presentation can include weakness or
cated to address secondary impairments and
numbness in the face, arm, or leg, especially
provide supportive therapy. Recovery from
on one side of the body; confusion or diffi-
transverse myelitis usually begins within 2 to
culty in talking or understanding speech; trou-
12 weeks of the onset of symptoms and may
ble seeing in one or both eyes; and difficulty
continue for up to 2 years. The majority of
with walking, dizziness, or loss of balance and
recovery occurs within the first 3 to 6 months.
coordination.
About one-third of people affected with trans-
Background Information verse myelitis experience good or full recovery
This condition is a transient stroke that lasts from their symptoms, regaining the ability to
only a few minutes. It occurs when the blood ambulate. Another one-third is left with signif-
supply to part of the brain is briefly inter- icant deficits, while the remaining one-third
rupted. Symptoms of this condition, which demonstrates no recovery at all. Prognosis
usually occur suddenly, are similar to those varies between recovery without relapse to a
of stroke but do not last as long. Most symp- permanent presence of symptoms, with the
toms disappear within an hour, although primary poor prognostic factors being pain in
they may persist for up to 24 hours. Because the midthoracic region or an abrupt, severe
it is impossible to differentiate symptoms onset of symptoms.3
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710 Chapter 33 Traumatic Brain Injury

■ Traumatic Brain Injury TUMORS


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Chief Clinical Characteristics ■ Brain Metastases


This presentation typically includes weakness Chief Clinical Characteristics
in the presence of cognitive changes, altered This presentation may include weakness,
level of consciousness, seizures, nausea, vomit- headaches, seizures, dysphagia, cognitive
ing, coma, dizziness, headache, pupillary changes, behavioral changes, dizziness, vom-
changes, tinnitus, dysequilibrium, incoordina- iting, alterations in the level of consciousness,
tion, behavioral changes, spasticity, hypertonic- ataxia, aphasia, nystagmus, visual distur-
ity, cranial nerve lesions, and sensory and bances, dysarthria, balance deficits, falls,
motor deficits.3,126 lethargy, and incoordination.3,130
Background Information Background Information
This condition can be classified as mild, The majority of individuals with brain
moderate, or severe based on Glasgow Coma metastases have been previously diagnosed
Scale, length of coma, and duration of post- with a primary tumor; however, a small per-
traumatic amnesia.126 Magnetic resonance centage of individuals are diagnosed con-
imaging may be used to confirm the diagno- comitantly with brain metastases and the
sis.54 Treatment initiated at the scene of the ac- primary tumor. The most common cancers
cident and during the acute phase is focused resulting in subsequent brain metastases
on medical stabilization. It should be initiated include lung, breast, melanoma, colorectal,
during the acute phase in order to minimize and genitourinary tract. The new onset
complications.127 Low Glasgow Coma Scale, of neurological symptoms after a primary
longer length of coma, longer duration of tumor warrants the use of imaging such
post-traumatic amnesia, and older age tend as magnetic resonance imaging or computed
to be associated with poor outcomes.128 tomography to confirm the diagnosis.
Optimal rehabilitation is interdisciplinary and Treatment may include corticosteroids,
customized to address the specific individuals’ brain irradiation, surgery, chemotherapy,
disablement. radiotherapy, and rehabilitative therapies.
The prognosis is poor with death typically
■ Tropical Spastic Paralysis occurring within 6 months.
Chief Clinical Characteristics
This presentation commonly involves slowly ■ Brain Primary Tumors
progressive paresis of the lower extremities, Chief Clinical Characteristics
sphincter dysfunction early in the disease This presentation may include weakness,
course, paresthesias, and uncoordinated headaches, seizures, dysphagia, cognitive
movements.3 changes, behavioral changes, dizziness, vomit-
ing, alterations in the level of consciousness,
Background Information
ataxia, aphasia, nystagmus, visual disturbances,
The retrovirus human T-cell leukemia virus
dysarthria, balance deficits, falls, lethargy, and
type 1 causes a chronic infective-inflammatory
incoordination.3,54
disease of the spinal cord, which results in the
symptoms of this condition. Diagnosis is Background Information
confirmed by the presence of the serum of the Glioblastoma multiforme, astrocytoma, oligo-
antibodies to human T-cell leukemia virus dendroglioma, metastatic tumors, primary
type 1 in the cerebrospinal fluid. Magnetic central nervous system lymphomas, gangli-
resonance imaging also reveals thinness of the oglioma, neuroblastoma, meningioma, arach-
spinal cord. Treatment is primarily sympto- noid cysts, hemangioblastoma, medulloblas-
matic with focus on improved urinary func- toma, and acoustic neuroma/schwannoma
tion and decreased spasticity. Steroidal are some of the more common brain tumors.
medications and gamma globulin may be Specific diagnoses for brain tumors may be
used. The majority of individuals with this confirmed with imaging and biopsy. Treat-
disease survive.129 ment is variable depending on the type, size,
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Chapter 33 Vasculitis (Giant Cell Arteritis, Temporal Arteritis, Cranial Arteritis) 711

and location of the tumor and may include sensation are usually the first. The first test

WEAKNESS
surgical resection, chemotherapy, radiation, to diagnose brain and spinal column tumors
corticosteroids, and rehabilitative therapies. is a neurological examination. Special imag-
Prognosis is also variable and depends on ing techniques (computed tomography, mag-
the type and grade of tumor, severity of netic resonance imaging, positron emission
compression, and duration of compression. tomography) are also employed. Specific di-
agnoses may be confirmed with imaging and
■ Spinal Metastases biopsy. Treatment is variable depending on
Chief Clinical Characteristics the type, size, and location of the tumor and
This presentation can involve spasticity, may include surgical resection, chemother-
weakness, sensory alterations, bowel and apy, radiation, corticosteroids, and rehabilita-
bladder incontinence, neck pain, back pain, tive therapies. Prognosis is variable and
radicular pain, atrophy, cerebellar signs, depends on the type and grade of tumor,
balance deficits, falls, and cranial nerve severity of compression, and duration of
involvement.3,54,131 compression.
Background Information ■ Vasculitis (Giant Cell Arteritis,
This condition is the most frequent neoplasm Temporal Arteritis, Cranial
involving the spine.131 The most common Arteritis)
types and locations of primary tumors that Chief Clinical Characteristics
result in spinal metastases include breast, This presentation can be characterized by
lung, lymphoma, prostate, kidney, gastroin- headaches, psychiatric syndromes, dementia,
testinal tract, and thyroid.3,132 The diagnosis peripheral or cranial nerve involvement, pain,
is confirmed with gadolinium enhanced mag- seizures, hypertension, hemiparesis, balance
netic resonance imaging and computed to- deficits, neuropathies, myopathies, organ in-
mography.3,131 Treatment is variable depend- volvement, fever, and weight loss.3,54,133
ing on the tumor and may include surgical
resection, chemotherapy, radiation, corticos- Background Information
teroids, and rehabilitative therapies.3 Al- This condition is the result of an immune-
though the long-term prognosis is poor, indi- mediated response resulting in the inflamma-
viduals without paresis or pain and who are tion of vascular structures.3,133 It includes
still ambulatory have longer survival rates.132 a variety of disorders such as giant cell/
temporal arteritis (which is the most com-
■ Spinal Primary Tumors mon form), primary angiitis of the central
Chief Clinical Characteristics nervous system, Takayasu’s disease, periar-
This presentation may include spasticity, teritis nodosa, Kawasaki disease, Churg-
weakness, sensory alterations, bowel/bladder Strauss syndrome, Wegener’s granulomatosis,
incontinence, back pain, radicular pain, and secondary vasculitis associated with sys-
atrophy, cerebellar signs, balance deficits, falls, temic lupus erythematous, rheumatoid
and cranial nerve involvement.3 arthritis, and scleroderma.133 The diagnosis is
confirmed through history, physical exami-
Background Information nation, laboratory testing, angiography,
Types of this condition include ependymoma, biopsy, and imaging.3,54,133 Corticosteroids,
hemangioblastoma, myeloma, neurofibroma, cytotoxic agents, intravenous immunoglobu-
lymphoma, metastasis, meningioma, schwan- lin, and plasmapheresis may be used in the
noma, and astrocytoma. Extradural tumors, treatment of vasculitis.54,133 Prognosis is
such as meningiomas, produce a rapid onset variable and depends on the specific underly-
of symptoms, with weakness being predomi- ing disorder. For example, giant cell arteritis
nant. Intramedullary tumors, or ependymo- is typically self-limiting within 1 to 2 years;
mas, astrocytomas, and hemangioblastomas, however, death usually occurs within 1 year
present with slowly progressive symptoms, for individuals with primary angiitis of the
of which loss of pain and temperature central nervous system.133
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712 Chapter 33 References

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review of pathologic mechanisms. J Spinal Disord Tech. tions for the Physical Therapist. Philadelphia, PA: W. B.
Feb 2003;16(1):38–43. Saunders; 1998.
99. Masson C, Pruvo JP, Meder JF, et al. Spinal cord infarc- 116. Granger CV, Clark GS. Functional status and out-
tion: clinical and magnetic resonance imaging findings comes of stroke rehabilitation. Topics Geriatrics.
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WEAKNESS
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comment]. J Neurosurg. 1998;88(6):958–961. matic brain injury: an analysis of 5600 patients. J Neu-
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A systematic review of drug therapy for Graves’ hyper- 17(2):167–170.
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123. Sieb JP, Gillessen T. Iatrogenic and toxic myopathies. and treatment of brain metastases: a comprehensive
Muscle Nerve. Feb 2003;27(2):142–156. review. Crit Rev Oncol Hematol. 2004;52:199–215.
124. Walsh RJ, Amato AA. Toxic myopathies. Neurol Clin. 131. Perrin RG, Laxton AW. Metastatic spine disease: epi-
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17(6):877–907. Prognostic relevance of clinical symptoms in patients
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1528_Ch34_716-720 07/05/12 2:02 PM Page 716

CHAPTER34
Case Demonstration: Inability to Stand
■ Chris A. Sebelski, PT, DPT, OCS, CSCS

NOTE: This case demonstration was developed Though unsure of the exact timeline, it seems
using the diagnostic process described in that during the past 6 months, she has noted
Chapter 4 and demonstrated in Chapter 5. The increasing difficulty standing up after sitting
reader is encouraged to use this diagnostic on a low couch, getting around her house, and
process in order to ensure thorough clinical rea- pulling weeds in her garden nearby the house.
soning. If additional elaboration is required on She has a history of three falls in the past
the information presented in this chapter, please 6 months, none of which has been associated
consult Chapters 4 and 5. with serious injury. She presents to the session
with her son who drove her and is in the wait-
THE DIAGNOSTIC PROCESS ing room. Mrs. RS does drive short distances
from her home, maintains her own checking
Step 1 Identify the patient’s chief concern.
account, and attends two routine weekly func-
Step 2 Identify barriers to communication.
tions with her friends. She has refused any
Step 3 Identify special concerns.
assistive device and holds onto her son’s arm
Step 4 Create a symptom timeline and sketch
as she enters the clinic.
the anatomy (if needed).
When asked why she was falling and hav-
Step 5 Create a diagnostic hypothesis list
ing trouble getting around, Mrs. RS replied
considering all possible forms of remote and
that her “legs don’t work like they used to” and
local pathology that could cause the
she rubs her thighs as she says this. She states
patient’s chief concern.
she is slow to get up and get going in the
Step 6 Sort the diagnostic hypothesis list by
morning as she feels that her “legs need to
epidemiology and specific case
wake up,” and she reports nonspecific inter-
characteristics.
mittent “pains.” If her son walks too fast, then
Step 7 Ask specific questions to rule specific
she does sometimes get short of breath. She
conditions or pathological categories less
does wear glasses and saw an ophthalmologist
likely.
this past year for a “routine checkup.” She also
Step 8 Re-sort the diagnostic hypothesis list
wears one hearing aid while in “social settings.”
based on the patient’s responses to specific
Mrs. RS states she is in “good health” and de-
questioning.
nies a personal history of cardiac disease,
Step 9 Perform tests to differentiate among
respiratory pathology, or cancer. She denies
the remaining diagnostic hypotheses.
alcohol or recreational drug use and is a
Step 10 Re-sort the diagnostic hypothesis list
nonsmoker.
based on the patient’s responses to specific
Current daily medications include Lipitor,
tests.
a multivitamin, a fish oil dietary supplement,
Step 11 Decide on a diagnostic impression.
and 81 mg baby aspirin.
Step 12 Determine the appropriate patient
disposition. STEP #1: Identify the patient’s chief
concern.
● Difficulty standing, walking around her
Case Description house, and gardening
Mrs. RS is an 82-year-old retired teacher who STEP #2: Identify barriers to
lives alone in a single-story ranch-style home communication.
on a 3-acre property. She has been widowed ● Age of patient and environment. Although
for 10 years and has four grown children. this patient has communicated clearly her
716
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Chapter 34 Case Demonstration: Inability to Stand 717

needs and appears to be forthcoming with


Teaching Comments: A thorough exam-
information and challenges, she is a fall risk.
ination should include an exploration of
If needed, the therapist should be prepared
the relationship of the phrase “my legs don’t
to have clear conversations regarding the
work like they used to” and the onset of pain.
patient’s safety and her ability to continue
This information will appropriately adapt the
to live independently. These types of
breadth and depth of the examination.
conversations are challenging and require
heightened sensitivity by the therapist.
STEP #3: Identify special concerns. STEP #4: Create a symptom timeline and
● Insidious onset of weakness associated sketch the anatomy (if needed).
with pain.

Within 6 months
past year ago Today

Routine eye Increased 3 falls: Initial


exam for difficulty Unable to recall physical
glasses getting specific dates therapy
around evaluation

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.
T Trauma T Trauma
Compression neuropathy of femoral nerve Compression neuropathy of femoral nerve
Hydromyelia (syringomyelia) Hydromyelia (syringomyelia)
Spinal cord compression/radicular pain Spinal cord compression/radicular pain
Spinal cord injuries Spinal cord injuries (no mechanism of injury)
I Inflammation I Inflammation
Aseptic Aseptic
Chronic inflammatory demyelinating Chronic inflammatory demyelinating
polyneuropathy polyneuropathy (typically symmetrical
distal to proximal weakness)
Inflammatory myopathies: Inflammatory myopathies:
• Dermatomyositis • Dermatomyositis
• Inclusion body myositis • Inclusion body myositis
• Polymyositis • Polymyositis (age of patient)
Lambert-Eaton myasthenic syndrome Lambert-Eaton myasthenic syndrome
Lyme disease (tick paralysis) Lyme disease (tick paralysis)
Multifocal motor neuropathy Multifocal motor neuropathy (time course)
Multiple sclerosis Multiple sclerosis (age of patient)
Rheumatoid arthritis of lumbar spine, Rheumatoid arthritis of lumbar spine, hips,
hips, knees knees
Systemic lupus erythematosus Systemic lupus erythematosus (age of patient)
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718 Chapter 34 Case Demonstration: Inability to Stand

Septic Septic
Septic arthritis of lumbar spine, hips, knees Septic arthritis of lumbar spine, hips, knees
M Metabolic M Metabolic
Diabetic neuropathy Diabetic neuropathy (no history of diabetes
mellitus)
Medication toxicity/toxic myopathy Medication toxicity (per patient report, her
medications are managed by her internist
every 6 months)
Mitochondrial myopathies Mitochondrial myopathies (patient age not
common for age of onset)
Myasthenia gravis Myasthenia gravis (patient age not
common for age of onset)
Toxic myopathy Toxic myopathy (typically distal to proximal
presentation, no exposure to toxic agent)

Teaching Comments: Mrs. RS is on Lip-


itor, which has a known side effect of muscle
weakness and/or muscle pain. Mrs. RS is
being seen via direct access and therefore
part of the patient interview must be the fre-
quency of monitoring of her medications by
her internist. In response to this questioning,
she states she sees her MD every 6 months.

Va Vascular Va Vascular
Arteriovenous malformation Arteriovenous malformation
Normal pressure hydrocephalus Normal pressure hydrocephalus
Transient ischemic attack/stroke Transient ischemic attack/stroke (time
course of symptoms)
Vasculitis Vasculitis
De Degenerative De Degenerative
Deconditioning Deconditioning
Lumbar spine disk herniation with Lumbar spine disk herniation with
radiculopathy radiculopathy
Primary lateral sclerosis Primary lateral sclerosis (age of onset)
Progressive supranuclear palsy Progressive supranuclear palsy (time
course, age of onset, no visual
disturbance)
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Brain tumor • Brain tumor
Malignant Metastatic, such as: Malignant Metastatic, such as:
• Brain metastases • Brain metastases
• Spinal metastases • Spinal metastases
Benign: Benign:
Not applicable Not applicable
Co Congenital Co Congenital
Familial spastic paraplegia Familial spastic paraplegia
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Chapter 34 Case Demonstration: Inability to Stand 719

Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Depression Depression
Hysterical paralysis Hysterical paralysis
Hypochondriasis Hypochondriasis (may be less likely to
cause falling)

STEP #7: Ask specific questions to rule Septic


specific conditions or pathological Septic arthritis of lumbar-spine, hips, knees
categories less likely. (denies low back, hip, and knee pain)
● Do you have numbness or tingling in your M Metabolic
legs? No. With this answer, several patholo- Not applicable
gies that have neurological origins move to
less likely on the list. Va Vascular
● When you use your low back, hips, or
Arteriovenous malformation (no reports of
knees in an activity, do they hurt and feel recent illness, performs high cognitive
weak? No. Rules less likely primary pathol- functions independently including
ogy of the lumbar spine, hips, and knees. managing checking account and driving)
● Have you noticed feeling ill recently? No.
Normal pressure hydrocephalus (performs
Rules less likely conditions that are associ- high cognitive functions independently
ated with a prodromal illness. including managing checking account
and driving)
● Do you have problems with grasping or
Vasculitis (denies recent illness)
holding objects? No. Rules less likely condi-
tions associated with upper extremity De Degenerative
weakness. Deconditioning
Lumbar spine disk herniation with
STEP #8: Re-sort the diagnostic
radiculopathy (denies sensory symptoms,
hypothesis list based on the patient’s
low back pain)
responses to specific questioning.
Tu Tumor
T Trauma
Malignant Primary, such as:
Compression neuropathy of femoral nerve
• Brain tumor (no reports of recent illness,
(denies sensory symptoms)
performs high cognitive functions
Hydromyelia (syringomyelia) (denies
independently including managing
sensory symptoms)
checking account and driving)
Spinal cord compression/radicular pain
Malignant Metastatic, such as:
(denies sensory symptoms, low back pain)
• Brain metastases (no reports of recent
I Inflammation illness, performs high cognitive functions
Aseptic independently including managing
Inflammatory myopathies: checking account and driving)
• Dermatomyositis (no upper extremity • Spine metastases (denies low back pain)
weakness) Benign:
• Inclusion body myositis (no upper Not applicable
extremity weakness) Co Congenital
Lambert-Eaton myasthenic syndrome
Familial spastic paraplegia (patient age of
(denies sensory symptoms)
onset not typical)
Lyme disease (tick paralysis) (denies sensory
symptoms, denies recent illness, no Ne Neurogenic/Psychogenic
presence of rash) Depression
Rheumatoid arthritis of lumbar-spine, hips, Hysterical paralysis (denies sensory
knees (denies low back, hip, and knee pain) symptoms)
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720 Chapter 34 Case Demonstration: Inability to Stand

STEP #9: Perform tests to differentiate STEP #12: Determine the appropriate
among the remaining diagnostic patient disposition.
hypotheses. ● Initiate physical therapy treatment for

● Observation. The demeanor of the patient Mrs. RS to address diagnostic impression;


was observed to be calm and with clear inform primary internist of visit and plan
answers during questioning. Affect ap- of care.
peared appropriate, reducing the likelihood
of depression as a primary cause for the Case Outcome
patient’s self-reported functional deficits.
Mrs. RS gave several cues of functional
changes with inability to achieve a sitting or
standing position or ascend/descend stairs
Teaching Comments: Due to the preva- without upper extremity assistance. Only
lence of neurological disorders on the dif- partial data from the physical exam is given as
ferential diagnosis list that could have signs it directly relates to the differential diagnostic
via cognitive changes, the clinician may list. Further manual muscle testing of the
choose to utilize a clinical exam tool such proximal lower extremities found an asym-
as the Mini-Mental Status Examination. metrical nonmyotomal pattern of weakness
Although this tool is not diagnostic of a of the gluteal muscles, quadriceps, ham-
particular pathology, it does assist with strings, and calf musculature. Based on her
screening for cognitive loss.1 functional concerns and the American Col-
lege of Sports Medicine’s recommendations
for strength training in older adults, the plan
● Manual muscle tests of the muscles inner- of care for Mrs. RS consisted of a progressive
vated by the lumbar plexus and femoral resistance training program involving the
nerve: iliopsoas: 5/5 bilaterally; quadriceps: lower extremity major muscle groups and
4/5 bilaterally, suggesting a role for decondi- balance activities.3 The exercise prescription
tioning as a cause of the patient’s self- was for 8 to 12 repetitions per exercise. Mrs. RS
reported functional deficits. was seen initially 2 days a week at a clinic with
● Neurological exam. No asymmetries to a home exercise program on a third day. After
light touch within the lower extremities. 3 weeks, her sessions in the clinic were re-
Deep tendon reflexes: 2+ throughout the duced to one time a week due to transpor-
upper/lower extremities. tation challenges. At the 10th week of care
● Temperature. 97.8°F. (13 visits), she was discharged to her home
program. Significant improvement was noted
in her Timed Up and Go test, manual muscle
testing, and self-reported quality of life meas-
Teaching Comments: Several physiologi- ures including fall risk and perceived activity
cal factors play a role in the change of baseline level.
body temperature in older adults. Temperature
testing requires multiple tests in similar condi- References
tions, similar time frame and via the same 1. Crum RM, Anthony JC, Bassett SS, Folstein MF.
method in order to increase the accuracy.2 Population-based norms for the Mini-Mental State
Examination by age and educational level. JAMA. May
12, 1993;269(18):2386–2391.
2. Kenney WL, Munce TA. Invited review: aging and
STEP #10: Re-sort the diagnostic human temperature regulation. J Appl Physiol. Dec 2003;
hypothesis list based on the patient’s 95(6):2598–2603
3. Chodzko-Zajko WJ, Proctor DN, et al. American College
responses to specific tests. of Sports Medicine position stand. Exercise and physical
● Deconditioning activity for older adults. Med Sci Sports Exerc. 2009;41
(7):1510–1530.
STEP #11: Decide on a diagnostic
impression.
● Deconditioning
1528_Ch35_721-741 08/05/12 5:59 PM Page 721

CHAPTER 35
Palpitations
■ Jesus F. Dominguez, PT, PhD

Description of the Symptom ■ Marked hypertension (>220/110) or hy-


potension (<90/60 in an individual who is
This chapter describes pathology that may lead typically normotensive)
to palpitations. The term palpitations refers to ■ Occurring suddenly (paroxysmal) and last-
an uncomfortable awareness of the heartbeat, ing longer than 15 to 20 minutes with asso-
usually associated with a cardiac dysrhythmia. ciated symptoms
The individual may report noticing a forceful, Individuals who experience palpitations
rapid, irregular, or slow heartbeat and may and who also have a family history of sudden
experience associated symptoms that include death represent a high-risk population. These
light-headedness, shortness of breath, chest individuals merit special consideration and
discomfort, or frank syncope. the therapist should initiate a timely referral
to the appropriate health care provider for
Special Concerns definitive assessment.
The therapist should be prepared to adminis-
ter basic life support interventions or activate Overview of Palpitations
the emergency medical system should an
individual present with palpitations in associ- Palpitations are a conscious awareness of one’s
ation with any of the following: own heartbeat. The sensation may be de-
■ Significant shortness of breath, chest scribed as a pounding, racing, or irregular
discomfort, light-headedness, or presyn- heartbeat and can usually be felt in the
copal symptoms (faintness, dizziness, chest, throat, or neck. Often, the individual re-
weakness, etc) ports a fluttering in the chest or a skipped beat.

CHAPTER PREVIEW: Conditions That May Lead to Palpitations

T Trauma
COMMON
Exercise 731
UNCOMMON
Obstructive sleep apnea 734
RARE
Postsurgical repair of congenital heart disease 737

I Inflammation
COMMON
Aseptic
Not applicable
Septic
Fever 731
(continued)
721
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722 Chapter 35 Palpitations

Inflammation (continued)
PALPITATIONS

UNCOMMON
Aseptic
Cardiomyopathies:
• Arrhythmogenic right ventricular cardiomyopathy 727
• Dilated cardiomyopathy 727
• Hypertrophic cardiomyopathy (hypertrophic obstructive cardiomyopathy) 728
• Restrictive cardiomyopathy 728
Septic
Not applicable
RARE
Aseptic
Mastocytosis 733
Septic
Pericarditis 736

M Metabolic
COMMON
Central nervous system stimulants 728:
• Caffeine 729
• Ephedrine (herbal ephedra) 729
• Nicotine 729
Dehydration/hypovolemia 730
Menopause 733
Pregnancy 737
UNCOMMON
Anemia 725
Cocaine and other illicit stimulants 729
Electrolyte imbalance 731
Hyperthyroidism/thyrotoxicosis 732
Hypoglycemia 732
Side effect of medications 738:
• Alpha-1 blockers 738
• Beta blockers 738
• Beta-2 agonists 738
• Digoxin 739
• Lasix 739
• Nitrates and calcium channel blockers 739
• Potassium supplement 739
RARE
Not applicable

Va Vascular
COMMON
Congestive heart failure 729
Coronary artery/atherosclerotic heart disease 730
1528_Ch35_721-741 08/05/12 5:59 PM Page 723

Chapter 35 Palpitations 723

Vascular (continued)

PALPITATIONS
UNCOMMON
Orthostatic hypotension (postural orthostatic tachycardia syndrome) 735
Pulmonary hypertension 737
RARE
Pulmonary embolus 737

De Degenerative
COMMON
Not applicable
UNCOMMON
Pacemaker/automatic implantable cardioverter defibrillator failure 735
RARE
Sick sinus syndrome 738

Tu Tumor
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Malignant Primary:
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Myxoma 734
• Pheochromocytoma 736

Co Congenital
COMMON
Not applicable
UNCOMMON
Aortic insufficiency (aortic regurgitation) 727
Mitral valve prolapse 734
RARE
Wolff-Parkinson-White syndrome (pre-excitation syndrome) 739

Ne Neurogenic/Psychogenic
COMMON
Anxiety/panic disorder 726
Muscular fasciculations 734
(continued)
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724 Chapter 35 Palpitations

Neurogenic/Psychogenic (continued)
PALPITATIONS

UNCOMMON
Vasovagal syncope (neurocardiogenic syncope, common faint) 739
RARE
Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Palpitations may be precipitated by anxiety, patient’s pulse. However, the definitive


fever, lack of sleep, caffeine, certain medica- assessment tool for identifying a particular
tions, cocaine and other amphetamines, hy- dysrhythmia is the electrocardiographic
perthyroidism, anemia, and vigorous exercise. (ECG) monitor. In practice, most physical
More serious causes include abnormalities of therapy settings may not have immediate
the heart valves, electrical conduction system, access to an ECG monitor. For that reason,
and myocardial tissue, as well as coronary assessment of palpitations by physical thera-
artery disease and heart failure. These latter pists should be based on subjective data and
pathologies often lead to the development of pertinent physical findings (ie, the severity of
more complex cardiac dysrhythmias. Palpita- symptoms and the associated hemodynamic
tions may occur as isolated, transient events or consequences).
in association with more serious symptoms This chapter was written with the premise
such as dizziness, light-headedness, shortness that few, if any, patients who experience pal-
of breath, chest discomfort, and syncope. pitations will seek advice from a physical
Palpitations occur in most individuals, therapist prior to consultation with a physi-
even in the absence of underlying heart dis- cian. The more likely scenario is one in which
ease. In general, clinical experience suggests the patient is being seen by a therapist for
that they are most often due to cardiac dys- another health issue and happens to mention
rhythmias or anxiety.1,2 Although the major- these symptoms during the course of treat-
ity of these episodes represent little risk to ment. The patient should always be encour-
health, some cardiac dysrhythmias can lead to aged to report symptoms of palpitations to
sudden death. With experience, the therapist a physician for definitive evaluation and
can develop critical skills that allow a distinc- management, or more appropriately, the
tion to be made between benign palpitations therapist may inform the physician directly.
and more serious conditions that place the Furthermore, reporting of palpitations by an
patient’s life at risk. Normal sinus rhythm may individual with a family history of sudden
usually be inferred when the pulse is noted to death necessitates timely medical referral.
be regular and within the range of 60 to 100 Figure 35-1, which is a flowchart adapted
beats per minute (bpm), although atrial flut- from Bates,3 demonstrates examples of pulse
ter with a fixed ventricular response, 2º atri- rates and the most likely associated cardiac
oventricular block Type II, or accelerated rhythms. Box 35-1 presents key clinical find-
atrioventricular junctional rhythm cannot be ings noted by Abbott1 in patients with palpi-
ruled out by this method alone. A regular tations and their suggested diagnoses. Both
pulse that is >100 bpm typically indicates a references provide the physical therapist
tachycardia (either sinus, ventricular, or with general guidelines for dysrhythmia iden-
supraventricular), whereas one that is <60 bpm tification in the absence of ECG monitoring
can be associated with sinus bradycardia, atri- and likely precipitating diagnoses. However,
oventricular junctional rhythm, 2º atrioven- the therapist is reminded that the guidelines
tricular block, or complete heart block. Be- are not intended to supersede assessment
cause of its irregularly irregular pattern, atrial of symptom severity and hemodynamic
fibrillation is usually identifiable with a rea- status in guiding the appropriate course of
sonable degree of certainty by palpation of the action.
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Chapter 35 Anemia 725

PALPITATIONS
Is the rhythm regular?

Yes No

What is the rate? What is the pattern?

“Regularly “Irregularly
<60 60-100 >100
irregular” irregular”

Sinus NSR Sinus PVC Atrial fibrillation


bradycardia 2°AVB tachycardia PAC Atrial flutter with
2°AVB Atrial flutter SVT PNC variable block
3°AVB with regular Atrial flutter Sinus arrhythmia
AV nodal ventricular with rapid
rhythm response ventricular
Accelerated response
AV nodal Ventricular
rhythm tachycardia

Key:
NSR=normal sinus rhythm, PVC=premature ventricular contraction,
AVB=atrioventricular block, SVT=supraventricular tachycardia,
PAC=premature atrial contraction, PNC=premature nodal contraction, AV=atrioventricular

FIGURE 35-1 Flowchart depicting the most likely cardiac dysrhythmias associated with palpitations
distinguished on the basis of pulse rate and regularity. (Adapted from Bates.3)

Description of Conditions That 2. A diminished content of hemoglobin per


May Lead to Palpitations erythrocyte, or
3. A combination of both.5
■ Anemia It can arise from failed synthesis, premature
Chief Clinical Characteristics destruction, hemorrhage, or deficiencies in
This presentation includes a rapid and regular iron, vitamin B12, or folic acid. Other condi-
pulse, suggesting the presence of sinus tachycar- tions that render the patient susceptible to
dia. Associated symptoms include lethargy, cold anemia include recent major surgery (eg,
skin, depression, easy fatigability, shortness of orthopedic and cardiopulmonary surgeries),
breath, and cognitive impairment.4 pregnancy, lesions of the gastrointestinal tract,
sickle cell trait, and cancer.6 The resultant
Background Information
reduction in the blood’s oxygen-carrying ca-
This condition involves a decrease in the
pacity initiates a reflex sinus tachycardia in
oxygen-carrying capacity of blood secondary to:
attempts to maintain adequate tissue oxygena-
1. A decrease in the erythrocyte (red blood cell) tion while blood pressure is usually within
content of blood, the normal resting range.7 If this condition is
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726 Chapter 35 Anxiety/Panic Disorder


PALPITATIONS

BOX 35-1 Selected Key Clinical Findings and Their Suggested Diagnoses
in Patients With Palpitations (adapted from Abbott1)
Clinical Findings Suggested Diagnoses
● Single “skipped beat” ● Benign ectopy (most likely PACs or PVCs)
● Single forceful or pounding sensation ● PVC
● Rapid and regular pounding in the neck and ● Supraventricular tachycardia
chest
● Palpitations associated with stress or emotional ● Psychogenic or catecholamine-sensitive
upset dysrhythmia
● Palpitations associated with activity ● Sinus tachycardia, coronary artery disease, or
cardiomyopathy
● Generalized anxiety ● Panic attack or panic disorder

● Thyromegaly, heat intolerance, tremors, sweating, ● Hyperthyroidism


exophthalmos
● Medication or recreational drug use ● Drug-induced palpitations
● Pericardial friction rub ● Pericarditis
● Cardiac murmur or midsystolic click ● Valvular heart disease
● Palpitations since childhood ● Supraventricular tachycardia

● Inability to catch one’s breath ● PVCs or ventricular tachycardia

● Rapid and irregular pulse ● Atrial fibrillation

● Loud snoring, morning headaches, systemic ● Sleep apnea


hypertension
● Lethargy, cold skin, depression ● Anemia
● Sweating, headache, severe hypertension, feelings ● Pheochromocytoma
of doom
● Third heart sound, shortness of breath, peripheral ● Congestive heart failure
edema

suspected, the patient should be referred to his Background Information


or her primary care practitioner for further The body’s response to this condition is usu-
evaluation. The diagnosis is confirmed by ally short-lived and subsides when the individ-
routine blood testing. Treatment involves ual is reassured or the stressful stimulus is re-
addressing the underlying cause of anemia, moved. Although anxiety or stress is perceived
including iron supplementation. by many areas of the brain, the integrated re-
sponse ultimately leads to elevation of plasma
■ Anxiety/Panic Disorder cortisol and catecholamine levels. Both hor-
Chief Clinical Characteristics mones are responsible for raising blood pres-
This condition is characterized by an acute sure and cardiac output by increasing heart
increase in heartbeat that is both rapid and rate, cardiac contractile force, and vasocon-
forceful. The pulse is typically rapid and reg- striction. The response leads to a general state
ular and associated with a discrete period of arousal and prepares the individual for useful
of intense fear or discomfort during which defensive behavior (ie, the “fight-or-flight”
several predictable symptoms develop and response). In the absence of other adverse
peak in intensity within 10 minutes. These symptoms or underlying cardiovascular pathol-
symptoms can include palpitations, sweating ogy, palpitations due to stress pose no serious
or flushing, muscle tension, headaches, in- risk and the therapist should reinforce this
somnia, changes in appetite, nausea, vomit- in the mind of the patient. Disabling cases of
ing, diarrhea, edginess, and irritability.8 this condition should be referred to the
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Chapter 35 Dilated Cardiomyopathy 727

appropriate health care provider and may in- Reiter’s syndrome, ankylosing spondylitis,

PALPITATIONS
clude pharmacologic and psychological therapy. syphilis, or Ehlers-Danlos syndrome. Diagnosis
and assessment of severity are made on the
■ Aortic Insufficiency (Aortic basis of echocardiography and angiography.
Regurgitation) Patients should be referred to their medical
Chief Clinical Characteristics practitioner if this condition is suspected and
This presentation often involves weakness, sig- the therapist should be prepared to administer
nificant lethargy, and severe dyspnea. Isolated cardiopulmonary resuscitation or activate the
systolic hypertension is often noted on assessment emergency medical system if the palpitations
of blood pressure. An early symptom is an are associated with other signs or symptoms of
awareness of the heartbeat due to increased significant hemodynamic instability.
stroke volume as a result of elevated end-diastolic
volume. The heartbeat may be described as CARDIOMYOPATHIES
forceful or uncomfortable. Patients with this ■ Arrhythmogenic Right
condition may also present with anginal symp- Ventricular Cardiomyopathy
toms because of the reduced coronary perfu-
sion associated with decreased diastolic pressure. Chief Clinical Characteristics
Other symptoms include exertional dyspnea, This presentation includes dyspnea on exer-
orthopnea, paroxysmal nocturnal dyspnea, and tion, paroxysmal (of sudden onset) nocturnal
palpitations. In chronic forms of this condition, dyspnea, orthopnea, angina, palpitations,
there is a rapid runoff of blood back into the left and syncope. This variant’s presentation
ventricle during cardiac diastole that results in can also range from asymptomatic to signs
a low peripheral diastolic pressure. A wide pulse and symptoms of heart failure, fatigue, light-
pressure is also present secondary to the headedness, syncope, and palpitations that
elevated systolic pressure created by enhanced may be associated with sweating.
stroke volume and the low diastolic blood
Background Information
pressure created by the retrograde flow of blood.
The palpitations may be due to single pre-
Background Information mature ventricular contractions, atrial fibril-
A common clinical finding during cardiac aus- lation, or ventricular tachycardia. Diagnosis
cultation is a diastolic murmur typically heard is made by biopsy and typically reveals re-
best along the left sternal border, although it placement of myocardial tissue with fibrous
can be heard over the right second intercostal connective tissue and fat deposits.11 In some
space as well.9 The murmur of the acute form instances, the rhythm may deteriorate into
of this condition is short and low frequency in pulseless ventricular fibrillation and the
nature. The murmur of this condition in its patient will lose consciousness. Cardiopul-
chronic state is high in frequency, has a blowing monary resuscitation, use of an automated
quality, and tends to be of longer duration than external defibrillator if available, and activa-
in the acute form. There also appears to be an tion of the emergency medical system are
association between aortic insufficiency com- warranted.
bined with ventricular septal defect and an in-
creased incidence of dysrhythmias.10 The pal- ■ Dilated Cardiomyopathy
pitations are often the result of resting sinus Chief Clinical Characteristics
tachycardia in response to peripheral hypoten- This presentation involves dyspnea on exer-
sion. This condition is a disorder of the aortic tion, paroxysmal nocturnal dyspnea, or-
valve in which the annulus or leaflets weaken or thopnea, angina, palpitations, and syncope.
balloon, leading to retrograde flow of blood This variant is distinguished by the appre-
from the aorta into the left ventricle during di- ciation of pulmonary crackles (secondary
astole. It may be the result of any condition that to pulmonary edema), a weak or laterally
weakens the valve leaflets or annulus, including displaced cardiac point of maximal impulse,
congenital valvular defects (eg, bicuspid aortic sacral/pretibial/ankle edema, and sudden
valve), rheumatic fever, endocarditis, Marfan’s significant weight gain. Associated symp-
syndrome, hypertension, aortic dissection, toms may include general fatigue/tiredness,
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728 Chapter 35 Hypertrophic Cardiomyopathy (Hypertrophic Obstructive Cardiomyopathy)

shortness of breath, chest discomfort, and condition accounts for more than 50% of all
PALPITATIONS

palpitations.12 sudden death cases in young individuals be-


low the age of 25 years.14 Young patients with
Background Information
suspicious symptoms and in whom a previ-
Several predisposing conditions have been
ously undiagnosed harsh holosystolic mur-
linked to the development of this condition,
mur is heard along the right upper sternal
including atherosclerotic heart disease,
border and cardiac apex should be referred to
genetic/familial inheritance, viral infection,
their physician for further evaluation prior to
excessive alcohol consumption, pregnancy,
initiating vigorous physical exercise. The ther-
and autoimmune disease.13 This condition
apist is reminded that a preexisting primary
often progresses to congestive heart failure.
diagnosis of cardiomyopathy requires careful
The specific rhythm disturbance noted as
and direct patient monitoring.15 If palpita-
palpitations may be atrial fibrillation, ven-
tions are associated with hemodynamic com-
tricular ectopy/ventricular tachycardia, or
promise or collapse, the emergency medical
atrioventricular heart block. In rare cases, the
system should be activated immediately.
rhythm may deteriorate into ventricular fib-
rillation and the patient will lose conscious- ■ Restrictive Cardiomyopathy
ness. Immediate basic life support, including
Chief Clinical Characteristics
use of an automatic external defibrillator if
This presentation typically involves dyspnea on
available, should be administered while the
exertion, paroxysmal nocturnal dyspnea,
emergency medical system is being activated.
orthopnea, angina, palpitations, and syncope.
■ Hypertrophic Cardiomyopathy This variant can be characterized by shortness
(Hypertrophic Obstructive of breath, fatigue/lethargy, peripheral edema,
Cardiomyopathy) and the presence of abdominal ascites.
In some patients, Kussmaul’s sign (a rise in
Chief Clinical Characteristics jugular venous distension upon inspiration)
This presentation can include dyspnea on is also noted and suggests reduced right ventric-
exertion, paroxysmal nocturnal dyspnea, or- ular compliance.
thopnea, angina, palpitations, and syncope.
This variant also includes progressive short- Background Information
ness of breath, chest discomfort, chronic pal- Most often the source of this form of palpita-
pitations, and syncope typically manifesting tions is atrial fibrillation or atrioventricular
in early adulthood (mid-20s) and should be heart block secondary to infiltrative involve-
considered in young athletes who present with ment of the cardiac conduction system.13
these symptoms. A harsh systolic murmur With the latter rhythm disturbance, the ther-
heard best along the left sternal border may apist may note the patient’s pulse to be signi-
be appreciated upon auscultation. With dy- ficantly bradycardic (<60 bpm) and the
namic auscultation, the therapist will note patient may report light-headedness or pre-
that the murmur’s intensity increases when the syncopal aura. Diagnosis is made on the basis
patient is asked to perform and hold a Valsalva of clinical findings and diagnostic procedures
maneuver and decreases when the patient such as echocardiography, angiography, and
resumes normal breathing.13 endomyocardial biopsy. In the event of palpi-
Background Information tations with hemodynamic compromise, the
A decrease in the intensity of the murmur is emergency medical system should be acti-
also noted when the patient is asked to move vated immediately.
from a standing position to a squatting posi-
tion. In both cases, a larger left ventricular CENTRAL NERVOUS SYSTEM
end-diastolic volume is associated with a STIMULANTS
diminished murmur intensity. Dysrhythmias Chief Clinical Characteristics
associated with this condition can be of This presentation typically includes an
supraventricular or ventricular origin and increased heart rate, which is sensed by the
can lead to hemodynamic compromise. This individual as a rapid or bounding pulse.16 In
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Chapter 35 Congestive Heart Failure 729

most cases, the rhythm is a sinus tachycardia interview process will alert the therapist to

PALPITATIONS
and the pulse is noted to be rapid and regu- the habitual use of these stimulants as a pos-
lar, although some reentrant tachyarrhythmias sible cause of the palpitations. Subsequent
may be associated with an irregular pulse. recommendations to reduce or even elimi-
nate these substances from the diet and
Background Information
lifestyle are often beneficial in minimizing or
If the tachyarrhythmia is pronounced, dias-
eliminating future episodes.
tolic filling will be significantly impaired and
the pulse may be absent, causing the patient ■ Cocaine and Other Illicit
to rapidly become hemodynamically unsta- Stimulants
ble. Common substances that cause this con- Chief Clinical Characteristics
dition include: This presentation typically involves palpitations,
hypertension, shortness of breath, agitation,
■ Caffeine bizarre and/or erratic behavior, and chest discom-
This substance is a neuronal stimulant that fort. Often, the patient’s pupils become dilated
occurs naturally in various food products, in- and he or she may report a history of chronic nose
cluding coffee, tea, and cocoa. It is known to bleeding (this latter finding is the result of nasal
be a competitive antagonist of adenosine re- septum and vascular deterioration).
ceptors, potentiate increased intracellular cal-
cium release, and reduce AV nodal refractori- Background Information
ness, all of which can contribute to the genesis Careful questioning during the initial interview
of dysrhythmias.17 In low doses (<250 mg), may provide clues to the patient’s use of cocaine
caffeine is known to elicit elation, peaceful- or other sympathomimetic drugs. Classified as
ness, and pleasantness, whereas at high doses a sympathomimetic drug, cocaine simulates a
(>500 mg), it can lead to the emergence of state of heightened sympathetic activity by
unfavorable side effects, including anxiety, stimulating the release of epinephrine and
irritability, palpitations, and nausea.18 norepinephrine from the adrenal medulla
and blocking the reuptake of norepinephrine
■ Ephedrine (Herbal Ephedra) at preganglionic synaptic nerve endings.20 It is
known to induce dose-dependent increases in
This alkaloid substance possesses adrenergic
heart rate and systolic and diastolic blood
properties that include shortening of the car-
pressure.21 The palpitations are often driven by
diac refractory period, increased chronotropy,
cocaine-induced sinus tachycardia, but heart
increased inotropy, and increased peripheral
rhythm may deteriorate into ventricular tachy-
resistance as a result of vasoconstriction.19
cardia and ventricular fibrillation. Palpitations
Common side effects of ephedra include
may range from a self-limiting episode to sud-
hypertension associated with palpitations,
den death. In the event of the latter, the emer-
tachycardia, or both.
gency medical system should be activated and
■ Nicotine the therapist should begin to render basic life
support measures. Exercise is contraindicated
This substance acts in a manner similar to in patients who present with signs/symptoms
the endogenous neurotransmitter acetyl- suspicious for acute cocaine intoxication.
choline to stimulate postsynaptic neurons of
the autonomic nervous system. However, its ■ Congestive Heart Failure
effects are longer lasting than acetylcholine Chief Clinical Characteristics
because it is not degraded by cholinesterases. This presentation includes a wide variety of
In the event of palpitations associated with palpitation symptoms from slow and forceful
hemodynamic instability, administration of to rapid and weak. Associated symptoms may
basic life support measures should be pro- include shortness of breath, chest discomfort, or
vided immediately and activation of the light-headedness/syncope. Various physical
emergency medical system is warranted. findings, such as peripheral/pretibial edema,
Careful attention to the patient’s dietary pulmonary crackles, an auscultated S3 cardiac
habits and smoking history during the sound, cyanosis, and sudden and dramatic
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730 Chapter 35 Coronary Artery/Atherosclerotic Heart Disease

weight gain, provide clues to the presence of vulnerable myocardial cells become ischemic,
PALPITATIONS

this condition. hyperirritable, and discharge spontaneously,


Background Information driving abnormal cardiac rhythms. The
In patients with this condition who present ectopic rhythm can be supraventricular or
with palpitations, the dysrhythmias can origi- ventricular in origin, tachycardic or brady-
nate from either the ventricles or supraven- cardic (in the case of complete heart block),
tricular structures (eg, atrial fibrillation). and is difficult to differentiate on the basis of
However, ventricular dysrhythmias may be palpation alone. In the elderly population,
more likely to result in sudden death. In this heart block and sinus bradycardia are the most
condition’s chronic form, several factors con- common dysrhythmias, followed by prema-
tribute to the development of episodic dys- ture atrial and ventricular beats, with atrial
rhythmias. These include mechanical stretch fibrillation being the most common sustained
of cardiomyocytes leading to hyperirritability rhythm.24 In cases of tachyarrhythmias, the re-
and spontaneous discharge, abnormal intra- sulting increase in myocardial oxygen demand
cellular calcium handling, and repolarization may escalate the condition from one of tran-
abnormalities.22 Electrolyte balance is typically sient ischemia to one of frank infarction and
upset in these individuals, both as a result of the episode may become life threatening. Use
chronic fluid retention and the depletion of of sublingual nitroglycerin or supplemental
certain electrolytes by various medications oxygen prescribed by the patient’s physician
used to treat the condition (eg, potassium- may resolve minor episodes and the palpita-
depleting diuretics). The use of digoxin to im- tions usually subside. However, if the patient
prove cardiac contractility in these patients becomes unstable during episodes of palpita-
may in some cases precipitate bradycardia, sec- tions with associated angina pectoris, activa-
ondary to slowing of the electrical conduction tion of the emergency medical system is
through the atrioventricular node, or tachy- warranted. The therapist should be prepared
cardia, secondary to enhancement of auto- to administer basic life support measures
maticity in Purkinje fibers. By communicating (including the use of an automated external
with the patient’s physician, the therapist plays defibrillator if warranted) while awaiting
a crucial role during titration of the patient’s advanced cardiac life support assistance.
medical regimen. Definitive diagnosis is made ■ Dehydration/Hypovolemia
by echocardiography or angiography. As with
atherosclerotic heart disease, supplemental Chief Clinical Characteristics
oxygen prescribed by the patient’s physician This presentation involves increased heart rate
can improve symptoms and suppress the pal- and augmentation of cardiac contractile force
pitations in some cases. However, if the patient often described by the patient as a fast and
becomes unstable, the emergency medical sys- bounding pulse. Associated symptoms may in-
tem should be activated. clude lethargy, poor concentration, tremors,
light-headedness, constipation, and dry mouth.
■ Coronary Artery/Atherosclerotic
Heart Disease Background Information
In most cases, the rhythm is sinus tachycardia
Chief Clinical Characteristics and the therapist will note a rapid but regular
This presentation can include a pulse that is pulse that may be slightly diminished in
noted to range from bradycardic to tachycardic strength. If this condition is profound, the
during an ischemic event.23 Individuals with this therapist may note cyanosis of the lips, sunken
condition may also experience symptoms of eyes, cold extremities, failure of skin to bounce
angina, often described as substernal chest dis- back when it is lightly pinched and released,
comfort, pressure, heaviness, squeezing, or burn- confusion, and syncope. Dehydration due to
ing that radiates to the shoulders, arms (left sweat loss and/or inadequate fluid replace-
greater than right), neck, jaw, and epigastrium. ment during physical exertion can lead to a
Background Information significant decrease in central blood volume.
With decreased myocardial oxygen supply Reflexively, the sympathetic nervous system
(or increased demand in the case of exertion), increases heart rate and contractile force to
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Chapter 35 Fever 731

maintain mean arterial pressure. The magni- to the primary care physician for assessment if

PALPITATIONS
tude of the tachycardic response is highly the condition is suspected.
correlated with the severity of hypovolemia.
■ Exercise
Older patients tend to be at higher risk for
dehydration as age-related decreases in total Chief Clinical Characteristics
body water, sensitivity to aldosterone and an- This presentation typically involves a pulse
tidiuretic hormone, and thirst perception ren- that increases gradually and appropriately for
der them more vulnerable.25 This condition the intensity of the exercise bout. When the
can be avoided by reminding the patient to exercise bout is terminated, the pulse gradu-
drink fluids during exercise sessions in the ally returns to resting values. Assessment of
clinic, especially if the exercise is aerobic and the blood pressure will typically reveal elevated
will be maintained at high intensity for greater systolic values that are appropriate for the in-
than 20 to 30 minutes. Mild hypovolemia ne- tensity of physical exertion (eg, 10 to 12 mm Hg
cessitates fluid replacement, which may be increase for every metabolic equivalent increase
accomplished by having the patient lie in a in workload).
semirecumbent position and drink water as Background Information
the therapist monitors signs and symptoms. In response to the metabolic demands placed
More severe cases of hypovolemia associated on the cardiopulmonary system by activities
with unstable signs/symptoms usually require involving the contraction of large muscle
activation of the emergency medical system groups, cardiac output will increase. This is
and administration of intravenous fluids. primarily accomplished by enhanced cardiac
■ Electrolyte Imbalance automaticity and contractility, yielding a sub-
stantial increase in heart rate and a moderate
Chief Clinical Characteristics
increase in stroke volume. To the individual
This presentation may involve symptoms of
who is typically sedentary or otherwise unac-
palpitations often associated with lethargy,
customed to exercise, the resultant sinus
shortness of breath, chest discomfort, light-
tachycardia and vigorous force of cardiac con-
headedness, and syncope.
traction may be felt as a rapid or pounding
Background Information pulse. The response is mediated by enhanced
This condition (in particular, hypokalemia sympathetic outflow and circulating cate-
and hyperkalemia) may result from dehydra- cholamines involved in the “fight-or-flight”
tion, hemorrhage, hypovolemia, use of potas- response and does not typically lead to any
sium-depleting medications (eg, thiazide adverse consequences in the absence of un-
diuretics), and excessive potassium supple- derlying organic heart disease. This is a nor-
mentation. Deviation of electrolyte values mal response to exertion and will subside with
from normal ranges may predispose the patient cessation of the exercise bout. No emergency
to life-threatening dysrhythmias. For example, intervention is warranted.
hypokalemia (<4 mmol/L) is significantly ■ Fever
associated with serious ventricular dysrhyth-
mias, including ventricular tachycardia and Chief Clinical Characteristics
ventricular fibrillation.26 Hyperkalemia is also This presentation may include a rapid or
correlated with the generation of severe dys- bounding pulse that can often be felt in the area
rhythmias, purportedly by altering acid–base of the temples (eg, a pounding headache).
balance and cardiac myocyte function.27 Ven- These features can also be appreciated by pal-
tricular rhythm disturbances will significantly pating the patient’s pulse and noting a rapid,
impair cardiac output and may lead to hemo- regular rhythm that is most suggestive of sinus
dynamic collapse. The therapist will note tachycardia. This presentation may be associ-
absent pulses, and cardiopulmonary resuscita- ated with an acute pattern of fever and
tion should be initiated while the emergency malaise.
medical system is being activated. Electrolyte Background Information
imbalance can be confirmed by routine blood An assessment of temperature usually con-
tests and the therapist should refer the patient firms the presence of fever and identifies it as a
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732 Chapter 35 Hyperthyroidism/Thyrotoxicosis

possible contributor to the palpitations. Fever, (particularly atrial fibrillation) can occur and
PALPITATIONS

typically a core temperature >98.6ºF, is a sys- may pose a serious health risk for individuals
temic response to invading microorganisms or with known coronary artery disease or his-
other inflammatory processes. It is primarily tory of stroke.31 Individuals with this condi-
regulated by a cluster of neurons in the hypo- tion often fail to exhibit the expected decrease
thalamus that act as a physiological thermo- in heart rate during sleep, with little or no
stat. During febrile states, the autonomic difference between resting heart rate during
nervous system and inflammatory mediators the waking and sleeping hours. Monitoring
act to increase heart rate and route blood to heart rate during sleep is often helpful in con-
the body’s surface for heat exchange as well firming this finding. Palpitations in individu-
as to support the increased metabolic rate of als with this condition are typically chronic,
inflammatory cells.28 In most cases, this condi- felt during resting states, and exaggerated
tion precludes the patient from participating with activity. This condition is characterized
in exercise until the process resolves. Fever by overactivity of the thyroid gland and pri-
lasting longer than 3 to 4 days typically indi- marily results in elevated levels of thyroid
cates the need for referral to an appropriate hormones in the bloodstream, while thyro-
health care practitioner. toxicosis refers to the clinical syndrome re-
sulting from hyperthyroidism. Typical etiolo-
■ Hyperthyroidism/Thyrotoxicosis gies include Graves’ disease (often associated
Chief Clinical Characteristics with exophthalmos), excessive thyroid hor-
This presentation often includes hyperten- mone replacement therapy, toxic adenoma,
sion, dyspnea (ie, orthopnea, exertional dys- thyroiditis, goiter, and use of amiodarone or
pnea, and paroxysmal nocturnal dyspnea), iodine-containing radiographic contrast
and rapid, bounding palpitations that may agents. Thyroid hormones are known to
lead to feelings of dizziness or light-headedness. enhance myocardial contractility and elevate
Atrial fibrillation is not common before the the body’s metabolic rate, leading to arterial
age of 50 but is present in up to 20% of older vasodilation and possible hypotension. A
patients.29 Associated signs and symptoms reflex tachycardia may ensue to counteract
include nervousness, heat intolerance, fatigue, the hypotension. If this condition is sus-
weight loss despite increased appetite, sweat- pected, the individual should be referred to a
ing, tremors, and exophthalmos.30 In some medical practitioner for definitive assess-
individuals, a goiter may also be present ment. The diagnosis is made on the basis of
(Fig. 35-2). blood tests that indicate elevated thyroid hor-
mone levels. Management of this condition
Background Information
may include pharmacologic administration
The most common dysrhythmia associated
of iodine, antithyroid medications, and surgi-
with this condition is sinus tachycardia,
cal thyroidectomy.
although supraventricular dysrhythmias
■ Hypoglycemia
Chief Clinical Characteristics
This presentation can be characterized by a
pulse that is noted to be tachycardic but regu-
lar. The suspicion of hypoglycemia can be
quickly confirmed by asking the patient (if
diabetic) to utilize his or her personal glucose
monitor (or the facility’s monitor) if available.
Associated signs and symptoms often include
headache, slurred speech, dizziness, feelings of
“vagueness,” impaired motor function, anxi-
ety, sweating, shakiness, pallor, increased systolic
blood pressure, disorientation, weakness, and
FIGURE 35-2 Goiter. palpitations.32
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Chapter 35 Menopause 733

Background Information cutaneous mastocytosis (urticaria pigmentosa)

PALPITATIONS
The rhythm is usually a reflex tachycardia in typically present with dark brown lesions on the
response to the hypoglycemic state. Low skin that may become itchy if rubbed or
blood glucose (hypoglycemia) in patients scratched. Systemic mastocytosis is confirmed
with diabetes may result from excess inges- by taking a tissue biopsy (eg, bone marrow) and
tion of insulin/oral hypoglycemic agents examining it for the presence of increased mast
or insufficient food intake in relation to in- cell counts. This condition is a term for a group
sulin/oral hypoglycemic dose. In people of disorders characterized by an overabundance
who do not have diabetes, this condition of mast cells (especially plentiful in the skin and
may result from insufficient caloric intake/ digestive tract). In both forms, the mast cells are
starvation or an abnormal increase in physi- triggered to release chemotactic agents by the
cal activity or exercise in the absence of presence of an allergen.34 Antihistamine med-
proper nutrition. The onset of signs and ications and epinephrine are useful as a first-
symptoms typically occurs when blood sugar line treatment for an acute episode and the
falls below 50 mg/dL, and findings can be di- patient should be reminded to avoid the trig-
vided into two categories: those related to the gering source if known. The therapist should
activation of the autonomic nervous system monitor the patient for signs of hypotension
and those caused by altered cerebral function. and presyncope and be prepared to administer
The condition can be readily reversed by hav- supportive care. In the event of severe anaphy-
ing the patient immediately ingest a source lactic shock, the therapist should activate the
of concentrated carbohydrate such as sugar, emergency medical system.
honey, candy, or orange juice. Vital signs
■ Menopause
should be monitored until they return to nor-
mal values. The therapist should be prepared Chief Clinical Characteristics
to administer supportive care or activate the This presentation can be characterized by
emergency medical system should the patient a rapid pulse that may be associated with
lapse into a diabetic coma (typically preceded depression/moodiness/irritability, shortness of
by convulsions and unresponsiveness). Indi- breath, numbness or tingling of extremities, hot
viduals with this condition related to diabetes flashes, sleeplessness, loss of appetite, poor con-
mellitus should be reminded to check blood centration, and early morning awakenings.35
glucose levels periodically and to avoid exer- Background Information
cising during the peak insulin effect to pre- The palpitations associated with menopause
vent episodes of hypoglycemia. are generally benign and self-limiting. There
■ Mastocytosis is strong evidence to suggest that hormonal
changes accompanying menopause likely
Chief Clinical Characteristics
contribute to increased incidence of palpita-
This presentation may include rash, itchy
tions, likely mediated by the effects of estra-
skin, abdominal discomfort, diarrhea, nausea
diol on electrophysiological parameters.36 The
and vomiting, bone pain, ulcers, skin lesions,
palpitations may be secondary to altered
severe hypotension, fainting, and bronchiolar
autonomic control of cardiovascular func-
constriction with labored breathing during
tion in the presence of altered hormonal bal-
an anaphylactic reaction. Palpitations may also
ance. It has been reported that the female
be associated with the increased release of
sex hormones slow electrical conduction
histamine.33
within the right atrium, prolong the refrac-
Background Information tory period of atrial tissue, and slow the
Typically, the underlying rhythm is sinus tachy- propagation of electrical conduction through
cardia and the pulse is felt to be rapid and the atrioventricular node.37 Referral to a
regular. Symptoms typically stem from exces- physician for evaluation of palpitations is
sive release of histamine (a vasoactive chemical made primarily on the basis of the severity of
released by mast cells during the normal allergic symptoms and the presence of clinical find-
reaction to an antigen or allergen). Mastocyto- ings that suggest cardiovascular compromise
sis can occur in two forms. Individuals with during episodes.
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734 Chapter 35 Mitral Valve Prolapse

■ Mitral Valve Prolapse cause this condition. The spontaneous dis-


PALPITATIONS

Chief Clinical Characteristics charges may be secondary to vigorous sympa-


This presentation can include palpitations, chest thetic activity or in response to any neural irri-
pain, shortness of breath, fatigue, and light- tant.39 In the absence of neurological disorders,
headedness and may cause periodic syncopal this condition is typically benign. Although this
episodes in a small subset of patients.38 Most in- condition can be bothersome, the patient
dividuals with this condition are asymptomatic. should be assured that the twitching is not
Upon cardiac auscultation, a midsystolic click associated with any adverse physiological
is often heard best over the fifth intercostal space consequences and no treatment is required.
at the midclavicular line, often followed by a late ■ Myxoma
systolic murmur in the presence of mitral valve
regurgitation. Chief Clinical Characteristics
This presentation typically includes dyspnea,
Background Information orthopnea, and paroxysmal nocturnal dyspnea
The palpitations are usually supraventricular in in combination with dizziness/light-headedness,
origin and occur paroxysmally. The pulse is palpitations, and syncope.40 Cardiac ausculta-
typically rapid and regular, although very rapid tion may reveal a mid-diastolic murmur as the
rates may be difficult to palpate. The palpita- myxoma encroaches on the mitral opening dur-
tion episodes commonly are self-limiting and ing ventricular filling, as well as a tumor plop
last several minutes (less often, they may last heard in mid- to late diastole.
for hours) during which the patient may expe- Background Information
rience the other associated symptoms. The di- A myxoma is a primary, most often benign,
agnosis is confirmed by echocardiography, intracardiac tumor composed of connective
which usually reveals exaggerated systolic bow- tissue surrounded by a mucopolysaccharide
ing beyond the mitral annulus of one or both coat and anchored to the endocardium.
valve leaflets. Individuals with this condition Although rare, the vast majority (>75%) of
may be instructed to cough forcefully or per- myxomas form in the left atrium, and if a
form a Valsalva maneuver, bearing down murmur is present, it is heard best at the fifth
against a closed glottis, during episodes of pal- intercostal space, midclavicular line. This con-
pitations in an effort to convert the abnormal dition is more common in females than males
rhythm through parasympathetic vagal media- and involves a generally friable lesion with
tion. Quite often, individuals with this condi- small fragments that often break free from the
tion are also prescribed calcium channel block- main tumor. The site of embolization depends
ers or beta blockers to suppress the occurrence on the cardiac chamber location and the pres-
of palpitations. Occasionally, the tachycardia ence of an intracardiac shunt. Larger left atrial
may be prolonged and immediate medical in- myxomas usually interfere with normal car-
tervention is usually warranted, especially if the diac function and significantly obstruct blood
patient has underlying coronary artery disease flow through the mitral valve. Auscultatory
and becomes hemodynamically unstable. findings suggest, but do not confirm, the pres-
ence of an intracardiac tumor and should
■ Muscular Fasciculations
prompt the therapist to refer the patient to a
Chief Clinical Characteristics physician for definitive assessment. The diag-
This presentation commonly involves muscular nosis is usually confirmed by echocardiography.
twitching localized to the neck or chest muscula-
ture that may be misinterpreted as palpitations. ■ Obstructive Sleep Apnea
Background Information Chief Clinical Characteristics
This condition can be quickly identified by ob- This presentation typically involves snoring
servation of rapid muscular contractions, dur- loudly, morning headaches, systemic hyperten-
ing which examination of the pulse will reveal sion, and daytime somnolence. Individuals with
that the fasciculations and pulse are not coinci- this condition are aroused from sleep continu-
dent. Spontaneously discharging motor units ously throughout the night up to 40 to 60 times
that are visible as rapid muscular contractions per hour and often experience acute shortness
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Chapter 35 Pacemaker/Automatic Implantable Cardioverter Defibrillator Failure 735

of breath and palpitations. The palpitations can chest discomfort, urinary incontinence, and

PALPITATIONS
be slow or rapid, and the patient may often ex- syncope when assuming a more vertical position.
perience a slight sense of anxiety or restlessness.41
Background Information
Background Information Orthostatic hypotension is defined as a drop
Several conditions often associated with sleep in systolic and/or diastolic blood pressure
apnea include obesity, a short thick neck, en- when going from the supine position to sit-
larged adenoids, reduced tone of the soft palate, ting or standing. The accepted criteria is a
a deviated nasal septum, and nasal polyps. drop of ≥20 mm Hg in systolic pressure
Individuals with clinical findings suggestive and/or a drop of ≥10 mm Hg in diastolic pres-
of Pickwickian syndrome—characterized by sure within 3 minutes of standing.43 In some
obesity, alveolar hypoventilation, pulmonary patients, a reflex tachycardia (usually an in-
hypertension, cyanosis, daytime somnolence, crease in heart rate of ≥30 bpm) is noted as
secondary polycythemia, and right-sided the sympathetic nervous system attempts to
heart failure with peripheral edema—should compensate for the drop in arterial pressure,
also be evaluated for the occurrence of termed postural orthostatic tachycardia syn-
obstructive sleep apnea. An opportunity to drome (POTS).44 Absence of a heart rate in-
engage the patient’s spouse or significant crease in the presence of hypotension may im-
other during the interview process usually ply a more serious neurological component to
reveals a history of loud snoring and frequent the disorder. Other factors to consider when
apneic episodes throughout the night. Individ- evaluating a patient for orthostatic hypoten-
uals with this condition rarely attain the deep sion include the patient’s neurological status,
stages of sleep (rapid eye movement sleep) prescription of vasoactive medications (eg,
because of constant hypoxic arousal and, thus, calcium channel blocker and beta-blocker
they are sleep deprived. The origin of this con- medications), prolonged bed rest, and hemor-
dition may be central (cessation of respiratory rhagic/hypovolemic states. A tilt table test is
muscle effort leading to absence of airflow), most often the initial evaluative procedure for
obstructive (upper airway obstruction), or symptoms suggestive of this condition. Pa-
mixed (airflow and inspiratory efforts stop tients should be instructed to rise slowly from
early in the episode). There may be an asso- bed in the morning (eg, sitting at the edge of
ciated impairment of cardiac autonomic the bed and performing ankle/calf exercises)
function characterized by a rise in sympa- or when going from a sitting/squatting to
thetic tone accompanied by a withdrawal of standing position. Symptoms usually dissipate
parasympathetic activity.42 Chronic forms of when the patient is placed in a semirecumbent
this condition can eventually lead to significant or supine position, although some patients
pulmonary hypertension and cor pulmonale. may progress to frank syncope. In this case,
The use of a pulse oximeter during the activity the therapist should be prepared to activate
assessment is warranted for patients suspected the emergency medical system if the patient
of having sleep apnea, because it often reveals fails to regain consciousness spontaneously.
exercise-induced hemoglobin desaturation.
If pulmonary hypertension is suggested by ■ Pacemaker/Automatic
history and physical findings in individuals Implantable Cardioverter
suspected of having this condition, referral to Defibrillator Failure
a physician is necessary for further testing, Chief Clinical Characteristics
which often includes monitored sleep studies This presentation involves palpitations described
and echocardiography. as an irregular heart rhythm with a pulse that
is usually bradycardic and either regular or ir-
■ Orthostatic Hypotension regular, suggesting that the pacemaker is failing
(Postural Orthostatic Tachycardia to either sense or support the abnormal heart
Syndrome) rhythm. If failure of an automatic implantable
Chief Clinical Characteristics cardioverter defibrillator occurs in an individ-
This presentation can include dizziness, light- ual who reports palpitations, the pulse is
headedness, palpitations, shortness of breath, noted to be tachycardic and regular. Associated
1528_Ch35_721-741 08/05/12 5:59 PM Page 736

736 Chapter 35 Pericarditis

symptoms in either case may include lethargy, be reported.1,46 A pericardial friction rub that
PALPITATIONS

light-headedness, shortness of breath, and is likened to two pieces of leather rubbing


presyncope/syncope.45 against one another may be heard during
cardiac auscultation. Asking the patient to
Background Information
momentarily hold his or her breath while
Loss of capture, or the failure of a pacemaker
auscultating helps to distinguish a pericardial
to elicit a cardiac contraction (primarily
from a pleural friction rub (the latter would
ventricular), is an uncommon but potentially
disappear upon breath-holding).
life-threatening complication of cardiac pac-
ing. This is often caused by movement or Background Information
dislodgement of the lead wire or physical This condition can be either aseptic (eg, post-
damage to the lead wire itself. This results in myocardial infarction, radiation-induced,
failure of the pacemaker to stimulate ventric- drug-induced, or connective tissue disease) or
ular contraction after sensing a missed beat/ septic (eg, viral, pyogenic bacteria, or tubercu-
abnormal rhythm or failing to sense the ab- losis). Pericarditis is often self-limiting, but
normality altogether. Automatic implantable individuals with this condition should be
cardioverter defibrillator malfunction may referred to their primary care physician for
also involve displacement or damage of a lead supportive care that may include prescription
wire or failure of the device. When working of anti-inflammatory or antibiotic medication.
with an individual who has a permanent pace-
maker implanted, the therapist should be ■ Pheochromocytoma
aware of the pacemaker’s programmed pace Chief Clinical Characteristics
rate. This information is readily available This presentation typically includes sweating,
from patients’ pacemaker identification cards, headache, pallor, anxiety, palpitations, severe
which should be carried with them at all hypertension, and feelings of imminent death.47
times. Palpation of a carotid or radial pulse
rate that is slower than the documented paced Background Information
rate should alert the therapist to the possibil- This condition is caused by a rare tumor that
ity of pacemaker failure. Knowing the pro- arises from tissue in the chromaffin cells of the
grammed threshold rate of an individual’s au- adrenal glands. The tumor causes excessive
tomatic implantable cardioverter defibrillator production and release of epinephrine and
allows the therapist to prescribe exercise in- norepinephrine from the adrenal glands, lead-
tensities that maintain the heart rate below the ing to an enhanced sympathetic state. Among
threshold rate to avoid inadvertent electrical dis- their various effects, both epinephrine and
charge. In the event of inappropriate discharge norepinephrine act on alpha-1 adrenergic
or function, the automatic implantable car- receptors in the vasculature and beta-1 adren-
dioverter defibrillator can be disabled temporar- ergic receptors in myocardial tissue to elicit
ily by placing a large magnet over it while the vasoconstriction and enhance myocardial
emergency medical system is being activated. contractility and automaticity, respectively.
Likewise, if the individual becomes sympto- Pheochromocytoma may induce the release of
matically unstable, the therapist should be pre- large amounts of hormones after trauma or
pared to administer basic life support and acti- surgery and can lead to life-threatening com-
vate the emergency medical system. plications if unrecognized. Typically, bouts of
hypertension are cyclical in nature but can last
■ Pericarditis indefinitely. In cases of marked hypertension
Chief Clinical Characteristics or protracted periods of palpitations, the ther-
This presentation is characterized by chest pain apist should initiate referral to the primary
and fever as the hallmark symptoms. The chest medical practitioner. The diagnosis is suggested
pain almost always has a mechanical compo- by blood and urine tests that reveal elevated
nent in that it is aggravated by coughing, sneez- levels of epinephrine and norepinephrine in
ing, deep inspiration, and lying supine. This the circulation. Computed tomography or
characteristic distinguishes the condition from magnetic resonance imaging usually detects
angina. The sensation of palpitations may also the adrenal tumor and confirms the diagnosis.
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Chapter 35 Pulmonary Hypertension 737

■ Postsurgical Repair of Congenital ventricular tachycardia the source of the dys-

PALPITATIONS
Heart Disease rhythmia.51 Patients who are pregnant and re-
Chief Clinical Characteristics port palpitations associated with symptoms of
This presentation can be characterized by pal- cardiovascular instability should be referred to
pitations, light-headedness, and dizziness their primary care physician for evaluation. In
months to years after surgical intervention to the event of cardiovascular compromise, sup-
repair congenital heart disease. In symptomatic portive interventions and activation of the
patients, the presence of a surgical scar over the emergency medical system may be necessary.
precordium or report of heart surgery during the
■ Pulmonary Embolus
intake interview often suggests the possibility of
this syndrome. Chief Clinical Characteristics
This condition may involve an acute onset of
Background Information
nonspecific dyspnea, hemoptysis, and chest dis-
The development of atrial tachyarrhythmias
comfort, as well as tachypnea, wheezing,
(most commonly, atrial fibrillation) is a recog-
cyanosis, syncope, and tachycardia manifested
nized phenomenon that occurs in some
as palpitations.52 Individuals with this condi-
patients with this condition, including atrial
tion quite frequently feel great anxiety and may
septal defect, ventricular septal defect, and
have a sense of impending doom. With large pul-
tetralogy of Fallot.48 The mechanism for tach-
monary emboli, jugular venous distension may
yarrhythmias often involves direct trauma to
be observed and an S3 sound may be heard at
cardiac tissue during the surgical procedure,
the cardiac apex.
rendering some cells susceptible to hyperirri-
tability and spontaneous depolarization. The Background Information
symptoms may appear soon after the proce- This condition is usually the result of dis-
dure or remain latent for prolonged periods. lodgement of a portion of a venous thrombus
Further evaluation by a primary care physician that ultimately lodges in small branches
is usually indicated. of the pulmonary arterial tree. Risk factors
include surgery, trauma, immobilization,
■ Pregnancy obesity, stroke, cancer, spinal cord injury,
Chief Clinical Characteristics pregnancy/oral contraceptives, increasing
This presentation includes a rapid and bound- age, and prolonged placement of indwelling
ing pulse often associated with shortness of central venous catheters. An individual in
breath, and there may be an increase in the whom this condition is suspected must receive
occurrence of symptoms to term.49 At times, the immediate medical attention. The therapist
palpitations may precipitate dizziness, presyn- should activate the emergency medical system
cope, or frank syncope.50 and be prepared to administer basic life sup-
port should the individual become unstable.
Background Information
Episodes are usually benign and self-limiting. ■ Pulmonary Hypertension
Significant changes occur in hormonal and
Chief Clinical Characteristics
hemodynamic function during pregnancy that
This presentation commonly includes shortness
predispose women to the development of dys-
of breath, fatigue, chest discomfort, palpitations,
rhythmias. Changes in hormone levels during
and syncope.53 Physical findings mimic those of
pregnancy (particularly progesterone) have
right-sided heart failure, such as jugular venous
been associated with enhanced sympathetic
distension, peripheral edema, abdominal
activity and the precipitation of dysrhyth-
ascites, and an S3 sound that is heard on auscul-
mias.36 The enhanced maternal blood volume
tation. These latter signs are associated with the
and associated increase in stroke volume may
development of cor pulmonale.
lead to the sensation of a forceful, bounding
pulse in some patients. The type of dysrhyth- Background Information
mia associated with palpitations is often sinus This condition is associated with thickening
tachycardia or supraventricular tachycardia. and hypertrophy of the medial layer of the
Only very rarely is atrial fibrillation or pulmonary arteries that ultimately involves
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738 Chapter 35 Sick Sinus Syndrome

intimal layer proliferation. Eventually, mean SIDE EFFECT OF MEDICATIONS


PALPITATIONS

pulmonary arterial pressure exceeds 25 mm Hg, Chief Clinical Characteristics


while pulmonary capillary wedge pressure This presentation may be characterized by pal-
remains below 15 mm Hg.54 Use of a pulse pitations ranging from a slow, bounding to a
oximeter during the initial activity assessment rapid, weak pulse and is associated with various
typically reveals hemoglobin desaturation sug- prescribed and over-the-counter medications.
gestive of significant pulmonary hypertension
with impaired oxygenation, although it is usu- Background Information
ally diagnosed by echocardiography and right- The following medications in particular may
sided cardiac catheterization. If this condition cause this presentation:
is suspected, the individual should be referred
to his or her primary care physician for further ■ Alpha-1 Blockers
evaluation. Alpha-1 blockers lower blood pressure by
blocking alpha-1 receptors located in vascular
■ Sick Sinus Syndrome smooth muscle to cause vasodilation, while
Chief Clinical Characteristics angiotensin-converting enzyme inhibitors block
This presentation may vary from significant the conversion of angiotensin I to angiotensin
vagotonia to severe tachycardia-bradycardia II (an extremely potent vasoconstrictor). Both
syndrome during which the patient may classes of medications may result in signifi-
feel very rapid palpitations and become cantly lowered blood pressure that can precip-
symptomatic during the subsequent and itate a reflex tachycardic response from the
prolonged bradycardic episodes. Associated heart that is felt as palpitations by the patient.
symptoms may include light-headedness, One of the clinical signs associated with this
anxiety, shortness of breath, and syncope. phenomenon is a very rapid pulse in the
Palpation of the pulse usually reveals a parox- presence of marked hypotension.
ysmal episode of rapid pulse followed by a
period of slow pulse that may be cyclical ■ Beta Blockers
or erratic in nature. These act to block beta-1 (selective) or both
Background Information beta-1 and beta-2 (non-selective) receptors
This condition refers to a wide range of dis- (beta-1 found primarily in cardiac tissue and
orders of the conduction system of the heart beta-2 primarily in vascular and bronchial
including failed sinoatrial node impulse gen- tissue). The important cardiac effects result
eration and impaired internodal conduc- in a slowing of the heart rate and a reduction
tion.55 In some cases, there is histological ev- in the force of contraction. One of the first
idence of sclero-degenerative changes within signs of excessive beta blockade is a marked
the cardiac conduction pathways. The source sinus bradycardia with associated symp-
of the tachyarrhythmia is usually supraven- toms of unusual fatigue, shortness of breath
tricular, but ventricular dysrhythmias may (especially with non-selective beta blockers),
result in the event of sinus arrest. Medical light-headedness, or syncope.56
treatment usually consists of antiarrhythmic
therapy and often a cardiac pacemaker is ■ Beta-2 Agonists
necessary to prevent untoward consequences Beta-2 agonists (medications that act on bron-
in the event of complete sinus arrest. In a chiolar smooth muscle to elicit bronchodila-
previously undiagnosed patient, noting a tion) are often prescribed to individuals who
cyclic or erratic acceleration and deceleration have asthma or other reversible airway dis-
of the pulse associated with symptoms war- eases. Minor adverse side effects of beta-2 ago-
rants timely referral to the physician. In ex- nist administration include headache, tremor,
treme cases of symptomatic forms of this and palpitations and the severity of symptoms
condition, the therapist should be prepared appears to be dose dependent.57 At higher
to administer supportive therapy if needed doses, there is a “spillover” effect that leads to
and activate the emergency medical system inadvertent beta-1 stimulation and enhanced
as appropriate. cardiac contractility.
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Chapter 35 Wolff-Parkinson-White Syndrome (Pre-Excitation Syndrome) 739

■ Digoxin ■ Potassium Supplement

PALPITATIONS
Digoxin is a cardiac glycoside that acts on the An overdose of a potassium supplement
heart to slow electrical impulse transmission can result in dangerously high levels of
through the atrioventricular node and to serum potassium and also precipitate life-
increase ventricular contractile force by threatening ventricular dysrhythmias. Patients
impairing sodium-potassium pump activity, taking a combination of Lasix and potas-
enhancing reverse sodium-calcium ex- sium supplement should be questioned for
changer activity, and increasing the sensitiv- the presence of frequent or prolonged
ity of ryanodine receptors on the sarcoplas- episodes of palpitations that may indicate
mic reticulum to calcium-induced calcium electrolyte imbalance. The therapist should
release.58 Individuals prescribed this medica- remind the patient to consult with their
tion often experience a sense of a “bound- physician in cases where potassium supple-
ing” heartbeat, especially at night prior to mentation is self-initiated.
falling asleep. In addition to its side effects,
overdose of this medication can result in a ■ Vasovagal Syncope
significant slowing of the heart rate and the (Neurocardiogenic Syncope,
individual may experience associated symp- Common Faint)
toms of nausea, dizziness, light-headedness, Chief Clinical Characteristics
general malaise, or syncope, suggesting This presentation involves prodromal symptoms,
digoxin toxicity. which include nausea, abdominal discomfort,
light-headedness, dizziness, palpitations, short-
■ Lasix
ness of breath, diaphoresis, and chest pain.60 The
Lasix is a loop diuretic that can significantly pulse is typically slow and regular, and blood
lower plasma volume and serum potassium pressure measurements may reveal hypotension.
levels. This may lead to destabilization of
the myocardial resting membrane potential, Background Information
resulting in the development of serious The precise mechanism responsible for this
ventricular dysrhythmias. Symptoms of ex- condition is not well understood. Individuals
cessive plasma volume loss and potassium with this condition usually have difficulty
depletion also include confusion, dizziness, standing for prolonged periods of time and
and unusual fatigue.59 exhibit delayed or diminished neurocardiovas-
cular responses when assuming an upright pos-
■ Nitrates and Calcium Channel ture. Predisposing factors include hypovolemia,
Blockers anemia, sympathetic blocking medications, and
Nitrates (eg, nitroglycerin) are metabolized antihypertensive medications. Similar to ortho-
and converted to nitric oxide (a vascular static hypotension and POTS, having the indi-
smooth muscle relaxer) and calcium channel vidual assume a more recumbent position and
blockers act to prevent calcium entry into administering fluids will often cause the symp-
vascular smooth muscle. Both medications toms to abate. A tilt table test is the diagnostic
elicit vascular smooth muscle relaxation and procedure of choice for confirming this condi-
can reduce arterial blood pressure. In some tion, and the individual should be referred to
cases, a reflex sinus tachycardia may ensue his or her primary care physician for definitive
in response to systemic hypotension and assessment.
patients may experience palpitations. Indi-
viduals who present with symptoms while ■ Wolff-Parkinson-White
taking any cardioinhibitory or vasoactive Syndrome (Pre-Excitation
medications should be referred to their pri- Syndrome)
mary physician for evaluation of appropriate Chief Clinical Characteristics
medication dosage and should be monitored This presentation ranges from asymptomatic and
closely during treatment sessions to assess undiagnosed to palpitations associated with
heart rate and blood pressure responses to chest discomfort, shortness of breath, light-
exercise. headedness, and syncope.
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740 Chapter 35 References

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PALPITATIONS

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Auton Res. 2008;18(Suppl 1):8-13. Med. 2003;2(4):287–297.
44. Johnson JN, Mack KJ, Kuntz NL, et al. Postural ortho- 57. Armstrong DJ, Mottram DR. Beta-2 agonists. In: Mot-
static tachycardia syndrome: a clinical review. Pediatr tram DR, ed. Drugs in Sport. 5th ed. New York, NY:
Neurol. Feb 2010;42(2):77-85. Routledge; 2011.
45. Nanthakumar K, Dorian P, Ham M, et al. When pace- 58. Wasserstrom A. Are we ready for a new mechanism of
makers fail: an analysis of clinical presentation and action underlying digitalis toxicity? J Physiol. Nov
risk in 120 patients with failed devices. Pacing Clin 2011;589:5015.
Electrophysiol. Jan 1998;21(1 pt 1):87–93. 59. Ciccone CD. Medications. In: DeTurk WE, Cahalin LP,
46. Sparano DM, Parker Ward R. Pericarditis and pericar- eds. Cardiovascular and Pulmonary Physical Therapy: An
dial effusion: management update. Curr Treat Options Evidence-Based Approach. New York, NY: McGraw-Hill;
Cardiovasc Med. 2011;13:543-555. 2011.
47. Goldman L, Ausiello D. Cecil Textbook of Medicine. 60. Tan MP, Parry SW. Vasovagal syncope in the older
22nd ed. Philadelphia, PA: Saunders; 2004. patient. J Am Coll Cardiol. 2008;51(6):599-606.
48. Nakagawa H, Shah N, Matsudaira K, et al. Characteriza- 61. Kulig J, Koplan BA. Wolff-Parkinson-White syndrome
tion of reentrant circuit in macroreentrant right atrial and accessory pathways. Circ. 2010;122:e480-e483.
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CHAPTER 36
Persistent Cough
■ Jeffrey S. Rodrigues, PT, DPT, CCS

Description of the Symptom prepared to administer basic life support


measures should an individual present with
This chapter describes pathology that may lead any of the following:
to a persistent cough. A persistent cough is de- ■ Tussive syncope, which is an episode of per-
scribed as one that the person is unable to stop sistent coughing that becomes so severe in in-
despite basic therapeutic intervention, as in a tensity that the person has a syncopal event
common cold remedy. A cough is considered ■ Productive cough that is:
persistent, or chronic, after 3 weeks in duration.1 ■ Pink, frothy (suggestive of pulmonary
embolism or pulmonary edema)
Special Concerns ■ Bright red (suggestive of an active bleed)
The physical therapist should consider referral ■ Dark red (suggestive of an old bleed)
to an appropriate health care provider or be ■ Green (suggestive of an active infection)

CHAPTER PREVIEW: Conditions That May Lead to Persistent Cough

T Trauma
COMMON
Aspiration of a foreign object 745
Aspiration pneumonia 746
Exercise-induced bronchospasm (exercise-induced asthma) 751
Inhaled irritant or foreign object 752
UNCOMMON
Chest injury 748
Pneumothorax 754
RARE
Not applicable

I Inflammation
COMMON
Aseptic
Asthma 746
Septic
Bronchiectasis 746
Bronchitis 748
Common cold 749
Pertussis (whooping cough) 752
Pneumonia:
• Bacterial pneumonia (community-acquired pneumonia, nursing home–acquired pneumonia) 753
• Viral pneumonia 753
Tuberculosis 757

742
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Chapter 36 Persistent Cough 743

Inflammation (continued)

PERSISTENT COUGH
UNCOMMON
Aseptic
Pleural effusion 753
Septic
Not applicable
RARE
Not applicable

M Metabolic
COMMON
Emphysema 750
Tobacco smoke 757
UNCOMMON
Sarcoidosis 756
Side effect of medications 756
Upper airway cough syndrome 758
Volume overload 758
RARE
Gastroesophageal reflux disease 751

Va Vascular
COMMON
Cor pulmonale 749
UNCOMMON
Congestive heart failure 749
Mitral valve stenosis/regurgitation 752
Pulmonary hypertension 755
RARE
Pulmonary embolism 755

De Degenerative
COMMON
Idiopathic pulmonary fibrosis 751
UNCOMMON
Not applicable
RARE
Not applicable
(continued)
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744 Chapter 36 Persistent Cough


PERSISTENT COUGH

Tu Tumor
COMMON
Not applicable
UNCOMMON
Malignant Primary, such as:
• Pulmonary carcinomas 754
Malignant Metastatic, such as:
• Metastases, including from primary kidney, breast, pancreas, and colon disease 752
Benign:
Not applicable
RARE
Not applicable

Co Congenital
COMMON
Alpha-1-antitrypsin emphysema 745
Cystic fibrosis 750
UNCOMMON
Not applicable
RARE
Not applicable

Ne Neurogenic/Psychogenic
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Psychogenic cough 754
Swallowing difficulties associated with a stroke 756

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Overview of Persistent Cough mechanism caused by mechanical stimulation


of the larynx and causes expiratory effects,
A cough is the body’s defense mechanism for and the enhancement of mucociliary stimula-
removing irritating substances from the tion for airway clearance.2,3 A cough is only
bronchial airways, thus providing airway effective as a defense mechanism down to the
hygiene. A persistent cough can disrupt the sixth or seventh generation of the bronchi
individual’s lifestyle by causing sleep distur- (Fig. 36-1).4 Some of the more common
bances and throat and voice soreness and causes of a persistent cough include pro-
diminish the ability to clear secretions in the longed exposure to tobacco smoke, complica-
future. tions due to chest trauma, inhaled irritants,
Two mechanisms can trigger a cough: the infections, inflammation of bronchial airway
expiratory reflex, which serves as a defensive tissues, bronchial airway tumors, postnasal
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Chapter 36 Aspiration of a Foreign Object 745

susceptible to developing emphysema, chronic

PERSISTENT COUGH
bronchitis, and other respiratory symptoms.
Occupational exposure to dust and chemical
irritants/fumes and exposure to first- or
second-hand smoke5 will lead to early devel-
opment of emphysema in the person with A1A
deficiency. The persistent cough is triggered
by the emphysematous changes that occur in
the lungs. Medical treatment may consist of
administration of A1A concentrate to keep
A1A serum levels therapeutic.6 The physical
Lung therapist’s intervention should include aspects
of a pulmonary rehabilitation program in-
cluding airway hygiene techniques and aerobic
exercises at a submaximal level.
■ Aspiration of a Foreign Object
Chief Clinical Characteristics
This presentation includes persistent cough with
a slight wheeze in its mild form. In more severe
cases, the presentation may include stridor, dys-
FIGURE 36-1 Generational structure of the airways pnea, rapid and shallow respirations, decreased
in the human lung. oxygen saturation, and cyanosis and may even-
tually lead to a syncopal event.7 This presenta-
tion will likely involve the universal choking
drip, or an underlying pulmonary or cardiac sign (hands at the throat).
disease.
Background Information
Description of Conditions That Aspiration of a foreign object is more com-
May Lead to Persistent Cough mon in infants and children than adults. The
objects are usually accidentally aspirated. Fre-
■ Alpha-1-Antitrypsin Emphysema quently, children are misdiagnosed initially in
this situation.8 Objects aspirated in children
Chief Clinical Characteristics
tend to be centrally lodged, whereas objects
This presentation includes persistent cough
will likely be lodged in the right main stem
combined with dyspnea on exertion, decreased
bronchus in adults. In a child, the main stem
exercise tolerance, prolonged expiration,
bronchi are more horizontally oriented and
wheezes and/or crackles with distant breath
as a person becomes an adult the bronchi be-
sounds on pulmonary auscultation, cough pro-
come more vertically orientated (the right
ductive of sputum, and excessive use of acces-
more so than the left side). The body uses a
sory muscles for respiration.
cough as a defense mechanism to try to re-
Background Information move the object. In mild cases the presentation
The primary pulmonary complication of can commonly be mistaken for an asthmatic
alpha-1-antitrypsin (A1A) deficiency is em- episode. Breath sounds on auscultation will
physema and the most common pulmonary be diminished or absent distal to the lodged
feature is chronic obstructive pulmonary object.7 Aspirated objects have included items
disease.5 This condition is a hereditary disor- such as chewed meat, peanuts, popcorn ker-
der characterized by low serum levels of nels, candy, pins, pen caps, and razor blades.9,10
A1A. Individuals with this deficiency are Individuals with alcoholism tend to be prone
at increased risk for developing premature to aspiration of objects and fluids when intox-
emphysema, often in the third and fourth icated. If the symptoms are mild, referring
decade of life. 6 Individuals with severe the individual to urgent care or an emergency
A1A deficiency are known to be genetically department would be appropriate. If the
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746 Chapter 36 Aspiration Pneumonia

symptoms are severe and acute, the physical Background Information


PERSISTENT COUGH

therapist should activate the emergency med- The stimulating factor irritates the lining of
ical system and be prepared to perform the the bronchial airway and a hyperadaptive
Heimlich maneuver if the individual’s status response is provoked, resulting in a narrowing
warrants. If this does not produce the object, of the airway. This leads to an irritation of
the individual will likely need to have a bron- the cough receptors, which provokes a cough
choscopy or surgery to remove it. response. If the individual is in an area that
exposes him or her to such triggers as pollen,
■ Aspiration Pneumonia dust, or animal dander, a prudent course of
Chief Clinical Characteristics action would be to have the individual exercise
This presentation is characterized by cough and in a different area away from the trigger, if pos-
associated with dyspnea, chest pain, wheezing, sible. For example, if outdoors, move the exer-
fever, nausea, or vomiting. The individual will cise program indoors. Cold, dry air is another
present with diminished breath sounds in the cause of exacerbation for individuals with
areas in which the aspirated contents exist. this condition. Exercising in a warm, moist
Background Information climate or performing activities in a pool would
Aspiration is identified as the inhalation of help to reduce triggering an asthma-induced
oropharyngeal or gastric contents into the coughing episode. Individuals who are prone
larynx and lower respiratory tract. Aspiration to “asthma attacks,” especially during exercise,
pneumonia will arise after the aspiration of should try to exercise in an area free from any
colonized oropharyngeal contents.11 Com- airborne irritants and in a warm, moist envi-
monly aspirated items include chewed food or ronment. Asthma is the most common cause
liquids. In the elderly aspiration is common in of persistent cough in children. If an asthma
those with dysphagia,12 swallowing difficulties exacerbation occurs, the individual should be
following stroke,13 or with a decreased cough instructed to stop any activity, rest, and use any
reflex. A decreased cough reflex will diminish medically prescribed medications such as in-
the defensive properties of the cough and the haled bronchodilators or corticosteroids until
individual will be prone to aspiration. The the symptoms subside. The physical therapist
individual will then develop a persistent should try to alter the environment to avoid
cough as the body tries to remove the aspi- further episodes. If the individual is unable to
rated contents that are directly irritating the control the situation within a reasonable
cough receptors. The individual’s oxygen amount of time, the physical therapist should
saturation may be lower due to the decreased be prepared to activate the emergency medical
gas exchange. If the above signs and symp- system so as to avoid progression to tussive
toms appear, the physical therapist should syncope.
ascertain a recent history from the individual ■ Bronchiectasis
or family member to rule in the possibility
of an aspiration pneumonia that has devel- Chief Clinical Characteristics
oped or is in the process of developing. The This presentation involves persistent cough
individual should be referred to his or her pri- that is productive of purulent, foul-smelling
mary physician if stable. If the individual a sputum with or without hemoptysis, dyspnea,
ppears to be in respiratory distress, the physi- fever, or pleurisy and can have digital club-
cal therapist should activate the emergency bing in severe cases. Daily sputum production
medical system. can range from 10 mL/day in mild cases to
more than 150 mL/day in severe cases.15 Indi-
■ Asthma viduals with this condition frequently have a
Chief Clinical Characteristics persistent cough throughout the year and com-
This presentation typically involves a chronic monly have hemoptysis.
or intermittent persistent cough, especially in Background Information
response to factors or stimuli such as pollen, Bronchiectasis is defined as an abnormal dila-
dust, animal dander, environmental conditions, tion of a bronchus that is irreversible (Fig. 36-2).
certain foods, and exercise.14 The dilation can be due to an infection such as
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Chapter 36 Bronchiectasis 747

commonly includes use of oral, intravenous,

PERSISTENT COUGH
or aerosolized antibiotics.15 The individual
may also use mechanical devices such as the
Flutter valve (VarioRaw Percutive SARL,
Aubonne, Switzerland) (Fig. 36-3) or a high-
frequency chest compression vest to help clear
secretions. In severe cases resection of a seg-
ment, lobe, or entire lung may be necessary.16
The physical therapist’s intervention should
consist of airway hygiene by percussion and
A
vibration, postural drainage, assisted cough
techniques such as the active cycle of breath-
ing, and pulmonary rehabilitation.17 The
physical therapist should be aware of the indi-
vidual’s baseline signs and symptoms and note
any increase in the frequency or production of
sputum with the cough. The individual should
be referred back to his or her primary care

Inha
led a
ir

C Inhaled
air
FIGURE 36-2 Computed tomography scan of a
patient with bronchiectasis: (A) upper airways;
(B) middle airways; (C) lower airways.

tuberculosis, inflammation due to aspiration,


the presence of a tumor or foreign bodies in
the airways, and genetic disorders such as cys-
tic fibrosis. This condition is technically not a
disease, but the outcome of various insults and
trauma to the lungs. Regardless of the cause,
an inadequate host defense mechanism and se-
vere inflammation are the two most common
factors that culminate in this condition. Chest
B
radiography will likely demonstrate increased
bronchial markings in the lower lobes and in- FIGURE 36-3 (A) Example of Flutter valve;
creased airway dilation. Medical treatment (B) patient using a Flutter valve.
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748 Chapter 36 Bronchitis

physician if an increase in sputum production ■ Chest Injury


PERSISTENT COUGH

is reported. Chief Clinical Characteristics


This presentation typically involves guarding over
■ Bronchitis the affected area of an injury to the thorax in
Chief Clinical Characteristics combination with diminished chest wall move-
This presentation can be characterized by symp- ment and pain in and around the affected area.
toms mimicking the upper respiratory infec-
tion of a common cold. The symptoms progress Background Information
to a dry, irritating cough, fever and chest pain Direct trauma to the upper body including the
due to increased frequency of coughing. Its chest wall, lungs, and throat are apparent
chronic form is a condition in which a persist- causes of cough following a chest injury. The
ent productive cough lasts for at least 3 months trauma can include a physical injury resulting
for two consecutive years.17,18 The sputum is from a motor vehicle accident, fall, physical
commonly yellow-green in color and produced assault, or penetrating wound. The trauma can
in small amounts. Lung auscultation reveals also occur from surgery to the regions
expiratory wheezes and crackles. mentioned above. In the case of a flail chest
secondary to trauma, the breathing mechanics
Background Information will be altered. During inspiration the chest
This condition is defined as an inflammation wall will expand, but the injured area will draw
of the trachea and bronchial tree that results in inward. During expiration the chest wall con-
a persistent cough. In its acute form, the cause tracts, while the area of injury protrudes.17
of the cough can be irritants such as smoke, The reaction that causes the cough is similar in
fumes/gases, or viral infections. As the condi- nature regardless of the origin of trauma. If
tion worsens the cough becomes more produc- the trauma is directly affecting the bronchial
tive of purulent sputum.17 Cigarette smokers3 airway, the cough mechanism is protective in
and those persons exposed to significant nature and the purpose is to remove the cause
amounts of second-hand smoke18 are predis- of the irritation. When the trauma is not
posed to developing chronic bronchitis. The directly affecting the bronchial airway (as in
individual with chronic bronchitis presents the case of a crush or penetrating chest wall
with persistent cough and sputum production injury), the surrounding areas will become
that is typically worse in the morning and inflamed and swollen. In this case the excess
evening than during midday. These individuals fluids can accumulate in the lungs. These
can possibly develop airflow obstructions due excess fluids will become an irritation to the
to the constant sputum production.3 The air- bronchial airway. The cough reaction will be to
flow obstruction over time will progress to re- remove the fluids so that normal gas exchange
duced chest expansion, air trapping, wheezing, can take place. When the trauma is surgical in
cyanosis, and decreased exercise tolerance.17 nature, fluids will arise due to the inflamma-
The acute form of this condition can develop tory response of the surrounding tissues. The
into its chronic form if not treated early. The cough response is similar in quality to the
physical therapist should refer an individual to physical trauma mentioned above. The physi-
his or her primary physician if the individual is cal therapist should be able to recognize the
exhibiting the signs and symptoms of chronic source of the trauma and respond accordingly.
bronchitis. The physical therapist’s treatment If the cause of the cough is due to direct
should focus on removing secretions, increas- trauma to the airways (physical or surgical),
ing ventilation, decreasing the work of breath- the physical therapist should suggest splinting
ing, and increasing exercise tolerance. Percus- techniques to relieve pain and give external
sion and vibration, postural drainage, and support. If the cause of the cough is indirect
assisted cough techniques are useful in clearing trauma, the physical therapist should incorpo-
the lungs of sputum. Progressive low-level rate postural drainage, percussion, vibration,
aerobic exercises can be a practical approach to deep-breathing exercises, and assisted cough
increasing exercise tolerance. techniques in an attempt to facilitate removal
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Chapter 36 Cor Pulmonale 749

of the secretions. The physical therapist should wheezing, and persistent coughing are com-

PERSISTENT COUGH
be aware of the underlying causes and physical mon signs in individuals with bronchial
structures present prior to administering any hyperreactivity.21 Medical treatment usually
of the above pulmonary physical therapy tech- consists of a combination of vasodilators,
niques. Percussion and vibration directly over diuretics, digoxin, beta blockers, antiarrhyth-
fractured ribs are contraindicated but encour- mic agents, and anticoagulants.22 Implement-
aged around the site.17 ing a cardiac rehabilitation program would be
beneficial.23 The individual who is newly diag-
■ Common Cold nosed or having an exacerbation of symptoms
Chief Clinical Characteristics would begin in Phase I of a cardiac rehabilita-
This presentation commonly involves runny tion program as an inpatient and progress to
nose, sneezing, fever, general body aches, and Phase II and Phase III as an outpatient over a
cough. An inflamed throat that has become period of several months.
irritated can induce this condition.
■ Cor Pulmonale
Background Information
The chronic cough can also be triggered by an Chief Clinical Characteristics
upper airway cough syndrome, more com- This presentation involves chronic productive
monly known as postnasal drip, from a runny cough, dyspnea on exertion, wheezing, lower
nose. The direct irritation of a substance on extremity edema, jugular venous distention,
the cough receptors in the upper airway struc- and in chronic cases digital clubbing.17
tures can generate a cough.19,20 It would be
Background Information
wise to inform the individual to return home
This condition is characterized as pulmonary
until the symptoms dissipate and he or she is
heart disease. The right side of the heart may
no longer potentially infectious to the physical
become enlarged due to pulmonary hyperten-
therapist or other individuals. If the symptoms
sion, pulmonary embolism, chronic obstruc-
persist, or are severe, the individual should be
tive pulmonary disease, idiopathic pulmonary
referred to his or her primary care physician
fibrosis, and Pickwickian syndrome.24 The
for further evaluation and treatment.
additional fluid irritates the bronchial airways,
■ Congestive Heart Failure resulting in a reactive chronic cough. Medical
management includes diuretics, supplemental
Chief Clinical Characteristics
oxygen use, and dietary salt restrictions.17 The
This presentation can be characterized by dys-
physical therapist should take to the role of
pnea on exertion, orthopnea, persistent cough,
preventing the onset of cor pulmonale by ad-
peripheral edema, and fatigue. On examination,
dressing the underlying cause. Aerobic exer-
individuals with this condition may exhibit
cises, mobility training, and breathing exer-
systolic murmur, crackles/rales on pulmonary
cises could have an important effect on the
auscultation, a third (S3) and/or fourth (S4)
above-mentioned causes. Individual education
heart sound on cardiac auscultation, jugular
would also be beneficial, including how to
venous distention, or peripheral cyanosis.
monitor lower extremity edema and signs of
Background Information respiratory failure. The physical therapist
This condition is characterized by left ventric- should be aware of any acute signs and symp-
ular contractile dysfunction resulting in toms of a severe form of this condition to the
ventricular dilation and poor contraction. point that the individual experiences pallor,
Contractile dysfunction can be caused by sweating, severe chest pain, weak and rapid
myocardial ischemia or infarction, coronary pulse, anxiety, and diminished level of con-
artery disease, or bacterial/viral infection. As sciousness. In this situation, the physical ther-
the heart pump fails, the blood returning to apist should activate the emergency medical
the heart from the lungs is backed up, causing system immediately, because these signs and
overload in the pulmonary circulation, which symptoms suggest the condition has pro-
leads to bronchial hyperreactivity. Dyspnea, gressed to the point of being life threatening.
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750 Chapter 36 Cystic Fibrosis

■ Cystic Fibrosis attention, or the physical therapist should be


PERSISTENT COUGH

Chief Clinical Characteristics ready to activate the emergency medical


This presentation classically involves a chronic system. If the hemoptysis is a dark red, it re-
cough that is usually productive of sputum veals blood that had yet to be expectorated.
throughout the course of the day.
■ Emphysema
Background Information Chief Clinical Characteristics
This condition is a genetic disease in which This presentation typically includes reports
exocrine gland function is altered. Excess of shortness of breath, persistent cough with
mucus can be produced in the linings of the sputum production, wheezing, and decreased ex-
lungs, pancreatic duct, gastrointestinal tract, ercise tolerance.29 Physical examination reveals
or other areas where exocrine glands are a prolonged expiratory phase during respiration,
located.25 An exacerbation may be due to an increased anterior-posterior diameter of the
infectious process that commonly has the chest (barrel chest appearance), flattened di-
symptoms of increased cough frequency with aphragm, and distant breath sounds on pul-
an increase of greenish sputum, fever, malaise, monary auscultation with wheezes and/or
and decreased appetite. The contact and irrita- crackles.
tion from increased amounts of sputum in the
airways during an exacerbation will increase Background Information
the frequency of the productive cough. Sur- This condition is the most common obstruc-
vival rates continue to improve in individuals tive pulmonary disease. It is characterized as
with this condition, and the impact of enlarged air spaces due to the disruption of
exercise plays a significant role.26 Aerobic27 the elastic fibers in the alveolar wall leading to
and anaerobic28 exercise programs are show- destruction that is irreversible.30 The emphy-
ing significant promise for improving exercise sematous changes produce airway obstruc-
capacity and quality of life. Physical therapists tion with impaired ventilation and gas ex-
will likely be involved with more individuals change in the lungs, resulting in decreased
with this condition as the need for exercise diffusion of oxygen. A persistent cough is
programs for this population becomes more noted as a method of enhancing gas exchange
common. Due to the type of exercise being by removing any irritating stimuli. The elastic
performed, the frequency of coughing may in- recoil of the lungs is lost, resulting in the re-
crease from the loosening of the sputum in the tention of air. A history of tobacco smoking
lungs. In this instance the individual should be or exposure to second-hand smoke or occu-
advised to perform a pulmonary hygiene tech- pational toxins can make a person prone to
nique of his or her choice. This may consist of developing emphysema. Medical intervention
postural drainage with or without percussion commonly consists of smoking cessation,
and vibration, assisted coughing techniques, use of bronchodilators, and limited use of
or the use of mechanical devices such as a supplemental oxygen. In severe cases lung
high-frequency chest compression vest or volume reduction surgery may be performed
Flutter valve (see Fig. 36-3). If the coughing to remove hyperinflated blebs or bullae. The
episode increases in frequency and duration physical therapist’s treatment should include
without some relief, it is possible for the aspects of a pulmonary rehabilitation pro-
individual to progress to tussive syncope gram including airway hygiene techniques,
and/or hemoptysis (blood in the sputum). The such as percussion and vibration, and pos-
individual and physical therapist should be tural drainage and aerobic exercises at a sub-
advised to continue to monitor the color of the maximal level. Individual education should
sputum for any changes to a bright red. Bright also be included for pursed-lip breathing
red hemoptysis is indicative of an active techniques. If the individual has undergone
bleed in the lung tissue. At this point, the indi- surgery for removal of emphysematous blebs
vidual should be advised to limit pulmonary or bullae, the physical therapist should also
hygiene activities and try to minimize cough- address any postsurgical movement dysfunc-
ing and physical activity and seek medical tion that may be affecting the individual. See
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Chapter 36 Idiopathic Pulmonary Fibrosis 751

the entry for Chest Injury earlier in this chap- in the morning and after meals. This condition

PERSISTENT COUGH
ter for reference as needed. is common among individuals who are non-
smokers with a normal chest radiograph and
■ Exercise-Induced Bronchospasm not taking angiotensin-converting enzyme
(Exercise-Induced Asthma) inhibitors.35
Chief Clinical Characteristics Background Information
This presentation can include a persistent cough This condition involves retrograde movement
that will usually occur 10 to 15 minutes into an of gastric contents into the esophagus. In indi-
exercise session and will then continue for up viduals with this condition, persistent cough
to 15 minutes after the conclusion of the exer- is one of the most common complaints.36
cise session.31 The mechanism of cough in an individual
Background Information with gastroesophageal reflux disease is from
This condition is a reaction of the bronchial microaspiration of gastric contents or a vagally
airways during bouts of exercise, usually in mediated esophageal-tracheobronchial reflex.
response to a change in environmental condi- Medical treatment includes vigorous acid sup-
tions. The airway narrowing consists of in- pression, elevating the head of the individual’s
creased mucus production in the airway along bed 10 cm, weight loss, diet modification to
with spasm of the smooth muscle in the airway high-protein and low-fat foods, omitting
lining. The response has a clinical appearance high-acid foods, antacid use prior to bedtime,
similar to that of an asthmatic episode, yet the and limiting food intake 2 hours prior to
mast cells in the bronchial airway do not re- bedtime.36 Referral should be made to the in-
spond as they do during an asthma attack.32 It dividual’s primary physician for a 24-hour
occurs during prolonged exercise, and is more esophageal pH test. Physical therapists treating
likely to occur when the ambient air is cold individuals with this condition in its acute
and dry.33 The easiest way to avoid cough form should avoid having the individual flex
secondary to this condition is to remove or forward at the trunk or hips and also avoid
minimize precipitating factors. Conducting vigorous activities in the early morning or
the exercise session in an area with warm, after meals, because these situations raise the
moist air will help to eliminate a primary likelihood of symptom aggravation.
precursor to this condition. A mild warm-up
■ Idiopathic Pulmonary Fibrosis
session prior to the start of vigorous exercise
will help to prepare the airways for the exercise Chief Clinical Characteristics
session and likely reduce the hyperreactivity of This presentation involves an irritating, non-
the bronchial passages. Medical treatment in- productive cough and exertional dyspnea.37
volves bronchodilator medications that can Lung auscultation will reveal a “Velcro”-like
help reduce the bronchial reaction. These quality crackle most noticeable at the bases.
medications can include long-acting beta- Pulmonary function tests will demonstrate a re-
adrenergic agonists such as cromone, for- strictive disease pattern with decreased vital
moterol, and salmeterol.34 The individual who capacity, residual volume, and total lung
is prone to exercise-induced bronchospasm capacity.
should be instructed to take the medications Background Information
prior to the exercise session to prevent cough This condition is a specific form of chronic
from this condition. fibrosing interstitial pneumonia that is limited
to the lung. The “stiffness” of the lungs causes
■ Gastroesophageal Reflux Disease a rapid, shallow breathing pattern. This is due
Chief Clinical Characteristics to a ventilation/perfusion mismatch and the
This presentation commonly includes dyspho- individual can become hypoxic during exer-
nia, hoarseness, sore throat, gum inflammation, cise. This will lead to a worsening of the above
dental erosion, chest pain, heart burn, dysp- cough and dyspnea symptoms. Pulmonary re-
nea, sputum production with coughing, and habilitation may help improve the quality of
wheezing.35 The cough will be more prevalent life and prepare the individual for possible
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752 Chapter 36 Inhaled Irritant or Foreign Object

lung transplantation. The physical therapist ■ Mitral Valve Stenosis/


PERSISTENT COUGH

should progress through the program slowly Regurgitation


so as not to exacerbate symptoms. If the symp- Chief Clinical Characteristics
toms worsen to tussive syncope or increased This presentation includes persistent cough, de-
hypoxemia, the physical therapist should acti- creased exercise tolerance, orthopnea, dyspnea
vate the emergency medical system as war- on exertion, and occasionally hemoptysis in
ranted. The prognosis is poor and most indi- combination with a systolic murmur best heard
viduals die within 3 to 8 years from onset of at the apex of the heart, rales on pulmonary aus-
symptoms.38 cultation, a third (S3) and/or fourth (S4) heart
■ Inhaled Irritant or Foreign Object sound on cardiac auscultation and jugular
venous distention.
Chief Clinical Characteristics
This presentation typically involves a chronic or Background Information
intermittent persistent cough in the presence This condition is characterized by the leaflets of
of an irritant. the mitral valve becoming stiff and closing in-
completely, usually due to the annulus (rim) of
Background Information the valve being stretched. Left arterial and/or
The cough reaction in the airway is similar to ventricular enlargement can pull and stretch on
that of an asthmatic reaction in which the ir- the mitral valve annulus. With this condition,
ritant causes an inflammatory response. One the blood coming from the lungs into the left
of the most common causes of a persistent side of the heart will be slowed and can back up
cough is inhalation of an irritant or foreign into the lungs, leading to volume overload in the
body. Some examples of inhaled irritants are lungs.39 The chronic overload in the pulmonary
smoke, odors from heavy perfume or clean- circulation is accompanied by bronchial hyper-
ing products, and dust. Along with direct reactivity. Symptoms of dyspnea, wheezing, and
contact, the inhaled irritant can trigger a persistent coughing are common in individuals
cough episode by irritating the cough recep- with bronchial hyperreactivity.21 This condition
tors. The best plan of action is to remove the usually occurs secondary to prior rheumatic
individual from the area where the irritant is fever occurring in childhood, yet the individual
located. does not become symptomatic until the adult
years. Other etiologies include coronary artery
■ Metastases, Including from disease, bacterial endocarditis, and left ventricu-
Primary Kidney, Breast, Pancreas, lar dilation. Medical treatment consists of
and Colon Disease antibiotic, digitalis diuretic, and vasodilator
Chief Clinical Characteristics medications. In cases of significant forms of this
This presentation commonly includes persist- condition, repair or replacement of the mitral
ent cough, hemoptysis, dyspnea, chest discom- valve will be warranted. A cardiac rehabilitation
fort, weight loss, and voice hoarseness, in asso- program is optimal in the surgical and postsur-
ciation with possible symptoms related to the gical management of this condition.23 The phys-
primary disease. ical therapist should be aware that the individual
will be self-limiting in his or her exercise toler-
Background Information
ance if the condition worsens.
The lungs are the most common site of metas-
tases from other types of cancer. When tumor ■ Pertussis (Whooping Cough)
cells migrate from their primary site they can
Chief Clinical Characteristics
work their way into the circulation or lym-
This presentation is characterized by a persist-
phatic system. The lungs filter these systems.
ent violent cough with a loud inspiratory
The most common cancers to metastasize to
“whoop,” post-tussive vomiting, and wheezing.
the lungs are kidney, breast, pancreas, colon,
and uterus. Treatment of this condition de- Background Information
pends on the type and extent of metastases, in- Pertussis is most common in children who have
cluding surgical resection, chemotherapy, and not been vaccinated against this condition,40
radiation therapy. but it can also affect adolescents and adults.
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Chapter 36 Viral Pneumonia 753

Adults tend to have a persistent cough for physical therapist should activate the emer-

PERSISTENT COUGH
longer duration, are less likely to have a gency medical system. Except for pleural effu-
“whoop,” and are more likely to wake at night sion caused by congestive heart failure, the
due to their cough in combination with sweat- medical treatment for pleural effusion is to re-
ing attacks.41 This condition is a respiratory move the fluid by thoracocentesis or to place a
illness caused by the bacterium Bordetella chest tube.44 If the individual has recently had a
pertussis. In the United States, children are reg- thoracocentesis performed to drain a pleural
ularly vaccinated with DTaP (diphtheria and effusion, the physical therapist should be aware
tetanus toxoids and acellular pertussis) in a of the signs and symptoms of a possible pneu-
five-dose series starting from 2 months of age mothorax that may have developed due to the
until 4 to 6 years of age. Medical treatment procedure.
for this condition includes use of antibiotic
medications, such as macrolides (eg, erythro- PNEUMONIA
mycin, azithromycin, or clarithromycin) or ■ Bacterial Pneumonia
trimethoprim-sulfamethoxazole.42 Individuals (Community-Acquired
suspected of having this condition should be Pneumonia, Nursing
referred to their primary care physician imme- Home–Acquired Pneumonia)
diately for evaluation and management. This Chief Clinical Characteristics
action becomes even more pressing if there is a This presentation usually includes persistent
known outbreak of pertussis locally. The phys- cough, sputum production with occasional
ical therapist also should self-monitor for hemoptysis, dyspnea, fatigue, and pleuritic
symptoms and seek testing for B. pertussis if chest pain.45 Pulmonary auscultation will
they develop. likely reveal decreased or absent breath sounds
■ Pleural Effusion and crackles.46
Chief Clinical Characteristics Background Information
This presentation commonly includes dyspnea, The onset of illness is usually abrupt. The
persistent cough, and pleuritic chest pain. Phys- persistent cough is produced by the in-
ical examination may reveal decreased chest creased sputum production irritating the
expansion, diminished or absent breath sounds, cough receptors. Medical treatment consists
dullness to percussion, and possible friction rub. of some form of penicillin or amoxicillin.45
A physical therapist’s intervention should
Background Information include airway hygiene techniques such as
This condition is a collection of fluid between percussion and vibration, postural drainage,
the visceral pleura and parietal pleura that assisted cough techniques, and deep-
occurs as fluid production exceeds fluid absorp- breathing exercises. Mobilizing the individ-
tion in the pleural space. The etiology of this ual would also be beneficial in assisting to
condition involves congestive heart failure, clear the lungs of sputum.
atelectasis, pneumonia, impaired lymphatic
drainage, and kidney or liver failure.43 The ■ Viral Pneumonia
cough is due to the excess fluid and dyspnea,
Chief Clinical Characteristics
which irritate the cough receptors in the
This presentation commonly involves persist-
bronchial airways and diminish gas exchange.
ent, dry, nonproductive cough and increasing
The cough reflex is triggered in a response
dyspnea. Chest auscultation reveals crackles
designed to remove the excess fluids. In severe
or wheezes. The individual may also exhibit
cases, the individual may have a tracheal shift
fever, decreased appetite, headache, and fa-
away from the affected side44 and may develop
tigue. This condition most commonly occurs
mild hypoxemia. In this case the physical thera- after the onset of influenza or inhalation of
pist should administer supplemental oxygen, as some other airborne virus.
prescribed by a physician, to maintain oxygen
saturation at a level where the individual is Background Information
comfortable and maintains a calm respiratory This condition can be community acquired
rate. If the symptoms persist or worsen, the or nosocomial. The cough reflex is triggered
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754 Chapter 36 Pneumothorax

by stimulation of the cough receptors from the ages of 10 and 30 years who have a
PERSISTENT COUGH

the infectious material in the airways. A tall, thin body habitus are more prone to de-
chest radiograph will show infiltrates in the veloping a spontaneous pneumothorax.47
areas affected. The aim of treatment in an Tension pneumothorax is characterized by
individual with a viral pneumonia is to air entering the pleural space but not exiting.
increase oxygen transportation. This in- This condition is caused by a one-way or
cludes pulmonary percussion and vibration, ball valve fistula. The ipsilateral lung becomes
postural drainage, deep-breathing exercises, compressed and the mediastinum shifts
assisted cough techniques, and mobilizing contralaterally. A tension pneumothorax is a
the individual. The physical therapist should medical emergency because the individual
be aware of the potential for contamination will develop decreased cardiac output, refrac-
or infection from one individual to another tory hypoxemia, and hypotension. The physi-
or to the physical therapist. The affected in- cal therapist will need to activate the emer-
dividual should be treated away from other gency medical system. Medical treatment
susceptible individuals. Physical therapists consists of chest tube placement to remove
should protect themselves by wearing a mask the trapped air. The use of high concentra-
that covers their nose and mouth. The tions of supplemental oxygen will reabsorb
affected individual may also consider wear- the lesion four times faster than if no supple-
ing a mask to protect others. Performing mental oxygen was used.47 This situation
hand hygiene before and after treatment of may resolve without the use of a chest tube in
an affected individual will help reduce the individuals with small pneumothoraces
spread of infection. (<15% of the hemithorax). The physical ther-
apist should have supplemental oxygen avail-
■ Pneumothorax able for use, as prescribed by the physician,
Chief Clinical Characteristics during therapy sessions. If the individual
This presentation typically involves pleuritic appears to be in respiratory distress during a
chest pain, dyspnea, and dry cough and the in- session, the physical therapist should activate
dividual may have absent breath sounds with the emergency medical system.
hyperresonant percussion over the affected area.
■ Psychogenic Cough
Background Information
This condition is free air that leaks into the Chief Clinical Characteristics
pleural space between the visceral and pari- This presentation includes dry, chronic cough-
etal pleura. The cough seen in an individual ing with no known somatic etiology. It is most
with this condition results from irritation commonly seen in children and adolescents but
of the cough receptors from the increased it also appears in adults.48
intrathoracic pressure. There are three main Background Information
classifications of this condition. Traumatic This condition should be considered when
pneumothorax is caused by a trauma or in- medical therapies for postnasal drip, asthma,
jury, direct or indirect, to the thorax. Com- and gastroesophageal reflux disease fail.48 In
mon causes include gunshot wound, knife children and adolescents, school phobia, fear
wound, and motor vehicle accidents. Sponta- of rejection, and need for attention are com-
neous pneumothorax occurs as the structural mon causes of psychogenic cough.49 The phys-
integrity of the pleural tissue is weakened. ical therapist should refer the individual back
The tissue becomes so weakened that a bleb to his or her primary physician for further
or bullae can rupture, allowing air into the evaluation and management if this condition
pleural space. Individuals with asthma, cystic is suspected. Treatment may consist of psy-
fibrosis, emphysema, pulmonary infections, chosocial intervention.
and empyema are likely candidates for devel-
oping a spontaneous pneumothorax. This ■ Pulmonary Carcinomas
condition can also occur in healthy individu- Chief Clinical Characteristics
als. Healthy individuals who smoke, have a This presentation commonly includes persistent
positive family history, and males between cough, hemoptysis, dyspnea, chest discomfort,
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Chapter 36 Pulmonary Hypertension 755

weight loss, and voice hoarseness. If the individ- ■ Pulmonary Embolism

PERSISTENT COUGH
ual has bronchoalveolar cell carcinoma, he Chief Clinical Characteristics
or she can produce copious amounts of thin This presentation includes dyspnea, pleuritic
secretions.50 chest pain, persistent cough, hemoptysis, and
Background Information syncope.52 Individuals with a history of deep
Cigarette smoking is the primary cause of venous thrombosis are susceptible. Other
carcinoma in the lung, and exposure to factors that put an individual at risk include
asbestos is the most common occupational immobility, resulting in blood stasis, endothe-
exposure cause for a person developing lung lial injuries due to lower extremity trauma or
carcinoma.50 There are many types of carci- surgery, and hypercoagulable states arising
noma of the bronchial airways including from such situations as oral contraceptive use
small-cell lung cancer (ie, oat cell carcinoma) or cancer.
and non–small-cell lung cancers (ie, squa-
Background Information
mous cell, adenocarcinoma, and large-cell
This condition is defined as a blood clot that
carcinomas).17 This condition has a strong
has become lodged in a pulmonary artery and
association with rheumatoid arthritis, and
significantly inhibits or ceases blood flow to
may even precede joint involvement.51 In an
the lung tissue. The obstruction is commonly a
individual who is in the early stages of local-
blood clot, but this condition can also be
ized lung cancer, the symptoms do not vary
caused by air, fat, bone marrow, foreign intra-
much from the symptoms related to cigarette
venous material, or tumor cells.17 The persist-
smoking; therefore, the individual may not
ent cough results from the irritation caused by
seek medical attention promptly.17 Individu-
the pulmonary congestion due to the em-
als with this condition may have tumors
bolism and the ensuing respiratory distress.
located in the main stem or segmental bronchi,
Medical treatment will depend on the severity
causing a cough. Pulmonary auscultation
of the blocked pulmonary artery. In an acute
will usually reveal normal findings. Individu-
case of severe disease, sudden death may occur.
als suspected of having this condition should
In the case of severe disease in which signs and
be referred to their primary care physician.
symptoms appear significant and hemody-
Complete blood count, chest radiography,
namic instability or respiratory compromise is
computed tomography, positron emission
evident, the physical therapist should activate
tomography, pulmonary function tests,
the emergency medical system. In other cases
ventilation/perfusion scan, and biopsy of the
where the signs and symptoms appear not as
suspected tumor are procedures that can be
severe, the individual should be directed to an
performed to rule in the diagnosis. Medical
emergent care facility. Medical treatment in-
treatment for the individual who is diag-
cludes thrombolytic medications to dissolve
nosed with carcinoma of the lungs can in-
the clot.52
clude radiation and chemotherapy. In certain
cases, surgical resection of the tumor and ■ Pulmonary Hypertension
possibly some of the surrounding tissues
Chief Clinical Characteristics
may be necessary. Physical therapists should
This presentation commonly involves dyspnea
construct a therapeutic program to improve
on exertion, fatigue, cough, wheezing, lower
the individual’s pulmonary function along
extremity edema, coarse breath sounds, and
with any possible complications due to the
angina.
radiation or chemotherapies. This may in-
clude decreased endurance and muscle weak- Background Information
ness due to the side effects of loss of appetite These symptoms arise from the fluid over-
and weight loss resulting from the above load in the lungs and the resulting pulmonary
treatments. Frequents rest breaks and inter- congestion irritating the cough receptors.
val training may be beneficial for the individ- Pulmonary hypertension is classified as an
ual. Following surgery physical therapists elevation of the pulmonary artery pressure
should also address movement dysfunction above normal.53 This condition is classified as
related to chest trauma. primary or secondary. The primary form of
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756 Chapter 36 Sarcoidosis

this condition is due to an unknown etiol- failure who takes an angiotensin-converting


PERSISTENT COUGH

ogy.54 Secondary disease has a known etiology enzyme inhibitor.


and can include pulmonary vasoconstriction
Background Information
due to scleroderma, Eisenmenger’s syndrome
The action of angiotensin-converting en-
(a right-to-left shunt in the cardiac septum),
zyme (ACE) inhibitors is arterial and venous
left-sided heart failure, or other causes. Pul-
vasodilation. The metabolism of bradykinin
monary hypertension can only be confirmed
is affected by the ACE inhibitor and accumu-
via cardiac catheterization. Medical treatment
lates in the airways. The reactivity of the
includes prostacyclins, diuretics, cardiac gly-
smooth muscles of the airway and the sensi-
cosides, supplemental oxygen,53 and vasodila-
tivity of the cough reflex are enhanced. Some
tors such as sildenafil.55 The physical therapist
examples of common ACE inhibitors appear
should be familiar with the individual’s med-
in Table 36-1. A thorough history will indi-
ical regime. Coordinating therapy during the
cate whether this condition is the potential
peak of medical intervention will help reduce
source of the persistent cough. If the cough
the cough symptoms and help to prevent the
persists to the point of adversely affecting the
individual from developing an exacerbation
individual’s exercise routine, then the indi-
of symptoms.
vidual may be referred to his or her physician
■ Sarcoidosis in regards to changing to a different medica-
tion that is safe, effective, and achieves the
Chief Clinical Characteristics
same desired results as the ACE inhibitor.
This presentation includes persistent cough,
Once the ACE inhibitor is stopped, the
exertional dyspnea, and wheezing.
persistent cough usually subsides within a
Background Information couple of weeks.
This condition is an idiopathic systemic gran-
ulomatous disorder. The well-formed granulo- ■ Swallowing Difficulties
mas become inflamed and are usually near the Associated with a Stroke
connective tissue sheath of the pulmonary Chief Clinical Characteristics
vessels and bronchioles and usually irritate This presentation involves symptoms ranging
cough receptors.56 This condition is the most from a persistent cough with a slight wheeze
common cause of bilateral hilar adenopathy, to stridor, dyspnea, rapid and shallow respi-
or enlargement of the lymph nodes at the rations, decreased oxygen saturation, and
hilum. The lungs are the most frequently cyanosis. An individual with this condition
affected organ system. Pulmonary function who has a history of dysphagia related to
tests normally demonstrate a restrictive dis- stroke7 may be prone to having a syncopal
ease pattern with lower total lung capacity, event.
functional residual capacity, residual volume,
and diffusing capacity for carbon monoxide.
This condition is diagnosed by bronchoscopy TABLE 36-1 ■ Common Angiotensin-
and transbronchial biopsies. The individual Converting Enzyme (ACE)
with pulmonary sarcoidosis has a good chance Inhibitor Medications
of remission within 2 to 5 years from diagno- GENERIC NAME
sis.56 Medical treatment usually consists of (ALL END IN –PRIL) TRADE NAME
inhaled corticosteroids or glucocorticoids57 in
combination with supplemental oxygen. No Benazepril hydrochloride Lotensin
emergency intervention is warranted. Captopril Capoten

■ Side Effect of Medications Enalapril maleate Vasotec

Chief Clinical Characteristics Lisinopril Zestril, Prinivil


This presentation involves a chronic, dry, non- Quinapril hydrochloride Accupril
productive cough,58 especially in an individual Ramipril Altace
with known hypertension or congestive heart
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Chapter 36 Tuberculosis 757

Background Information Similar findings occur in those who are

PERSISTENT COUGH
Dysphagia, or difficulty swallowing, is a com- exposed to second-hand tobacco smoke. The
mon occurrence following a stroke.11 Individ- cough reflex for those exposed to first-hand
uals with dysphagia tend to take longer to tobacco smoke becomes diminished.60 In
chew and swallow their food and also have a individuals who begin to demonstrate em-
tendency to “pocket” chewed food in the physematous changes in the lungs from first-
mouth after swallowing.59 This puts the indi- hand tobacco smoke, cessation of smoke
vidual recovering from a stroke at risk for as- exposure does not undo but stabilizes the
pirating food and liquids. In turn, individuals changes that had occurred.61 The persistent
with this condition are at risk for developing cough is enhanced by the continued mucus
aspiration pneumonia. The individual will re- production and therefore will not signifi-
spond with a cough as a defensive mechanism cantly dissipate. The physical therapists’ treat-
to remove the aspirated contents. The physical ment aim would be to improve pulmonary
therapist treating an individual recovering function through pulmonary clearance tech-
from a stroke should refer the individual for niques, aerobic exercise, and individual edu-
evaluation of his or her swallowing.59 The cation. For the individual exposed to second-
physical therapist should visualize the stroke hand tobacco smoke, encouragement to avoid
individual’s oral cavity if a persistent cough the source of the tobacco smoke, especially
is noted, especially if it is after mealtime or during exercise treatments, would be practi-
after the individual has taken medications. cal. For the individual exposed to first-hand
If the symptoms become acutely severe, the smoke, the physical therapist should alert the
physical therapist should activate the emer- individual to smoking cessation programs.
gency medical system.
■ Tuberculosis
■ Tobacco Smoke Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically includes fever,
This presentation includes a persistent, likely night sweats, anorexia, weight loss, weakness,
productive cough, dyspnea, wheezing, and persistent cough, pleuritic chest pain, and
decreased oxygen saturation. Individuals hemoptysis.
with this condition may have a barrel-chest
Background Information
appearance.
This condition is an infectious disease of
Background Information the lungs caused by inhalation of airborne tu-
The airway inflammation generally present bercle bacilli, triggering an inflammation re-
in smokers causes a persistent cough, sputum sponse that, in turn, produces fluid leukocytes
production, and reactive airway disease due and macrophages. The infection can spread
to the inhaled noxious stimulant. For some into extrapulmonary sites such as lymph
time now tobacco smoke has been linked to nodes, pleura, bones, joints, kidneys, or brain.
many symptoms and signs, particularly per- A chest radiograph will reveal infiltrates in
sistent cough. When airways are exposed to the middle and lower zones that can irritate
smoke, they become inflamed. The short- the cough receptors.62 Individuals suspected of
term response (cough) to tobacco smoke is a having this condition should be referred to
protective action. The cilia in the airway are their primary care physician immediately.
part of the defense mechanism of the lungs. Care should be taken to avoid close contact
These cilia get destroyed with prolonged ex- with the individual because this condition is
posure to tobacco smoke. The long-term commonly transmitted by inhalation of in-
pathological consequences include swelling fected airborne particles. Medical treatment
of the bronchial airways, increased mucus includes antituberculosis medications such
production, and increased airway reactivity as isoniazid, rifampin, pyrazinamide, ethamb-
characteristic of chronic bronchitis and utol, and streptomycin. This course of treat-
chronic obstructive lung disease and the tis- ment can last for 6 to 9 months.62 Individuals
sue destruction characteristic of emphysema. who have this infectious disease are placed in
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758 Chapter 36 Upper Airway Cough Syndrome

an isolated room in the hospital. Due to the ■ Volume Overload


PERSISTENT COUGH

limited area in which to exercise and the neces- Chief Clinical Characteristics
sity for isolation, the individual is prone to This presentation may involve dyspnea, per-
cardiopulmonary and physical decondition- sistent cough, wheezes, and fatigue. Pulmonary
ing, disuse atrophy, and progressive dyspnea.17 auscultation will commonly reveal crackles.
The physical therapist is often challenged with
providing the individual with functional and Background Information
effective exercise activities within the confines This is a condition in which the extracellular
of the individual’s room. Therapeutic activities fluid in the body increases. Common causes of
and exercises using free weights or resistive volume overload include congestive heart fail-
bands would be practical. Bringing (and leav- ure and kidney failure but can also include
ing) a stationary bicycle in the individual’s chronic obstructive pulmonary disease.66 The
room would address the aerobic component of lungs, heart, abdomen, and extremities are
an exercise program. The physical therapist common places for excess fluid to accumulate.
should be aware that any objects used by an The excess fluid that collects in the lungs is
individual with this condition must be thor- similar in characteristics to a pleural effusion,
oughly cleaned with a germicidal solution cor pulmonale, congestive heart failure, or
prior to use by another individual because tu- mitral valve stenosis/regurgitation, all of
bercle bacilli can live in sputum not exposed to which lead to a persistent cough. Medical
sunlight for many months. If the objects are treatment generally consists of loop diuretics
improperly cleaned, they may become vectors such as furosemide (Lasix) or Aldactone to
for disease transmission. help remove the excess fluid. The cause or lo-
cation of the volume overload should dictate
■ Upper Airway Cough Syndrome the physical therapist’s treatment. If the cause
Chief Clinical Characteristics of the volume overload is due to congestive
This presentation involves the sensation of heart failure or congestive obstructive pul-
“something dripping” down the back of the in- monary disease, the physical therapist should
dividual’s throat or the need to clear the throat implement items from cardiac or pulmonary
often, a condition commonly referred to as rehabilitation programs, respectfully. Aerobic
postnasal drip. This is usually due to rhinitis con- exercises and pulmonary hygiene therapy
sisting of nasal decongestion, sneezing, or techniques would be beneficial to the individ-
rhinorrhea, or sinusitis consisting of nasal ual depending on the location of the excess
discharge or sneezing, or a combination of fluids. The use of compression stockings or
both.63 wrapping of the lower extremities, in conjunc-
tion with diuretic usage, assists in moving
Background Information fluids away from the lower extremities. If the
This condition is considered one of the most symptoms progress to limiting the individual’s
common causes of persistent cough.64,65 The participation with rehabilitation, he or she
direct stimulation of the cough receptors in should be referred to a physician for further
the upper respiratory tract triggers the cough follow-up.
reflex. The individual may be in an infectious
state if an infection is the cause. The individ- References
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16. Balkanli K, Genc O, Dakak M, et al. Surgical manage- reflux in chronic cough and asthma. Chest. May 1997;
ment of bronchiectasis: analysis and short-term results 111(5):1389–1402.
in 238 patients. Eur J Cardiothorac Surg. Nov 2003; 37. Hope-Gill BD, Hilldrup S, Davies C, Newton RP,
24(5):699–702. Harrison NK. A study of the cough reflex in idiopathic
17. Goodman CC. The respiratory system. In: Goodman pulmonary fibrosis. Am J Respir Crit Care Med. Oct 15,
CC, Boissonnault WG, eds. Pathology: Implications for 2003;168(8):995–1002.
the Physical Therapist. Philadelphia, PA: W. B. Saunders; 38. Selman M, Thannickal VJ, Pardo A, Zisman DA,
1998. Martinez FJ, Lynch JP 3rd. Idiopathic pulmonary
18. Radon K, Busching K, Heinrich J, et al. Passive smoking fibrosis: pathogenesis and therapeutic approaches.
exposure: a risk factor for chronic bronchitis and Drugs. 2004;64(4):405–430.
asthma in adults? Chest. Sep 2002;122(3):1086–1090. 39. Romano MA, Bolling SF. Update on mitral repair in
19. Pratter MR. Cough and the common cold: ACCP dilated cardiomyopathy. J Card Surg. Sep–Oct 2004;
evidence-based clinical practice guidelines. Chest. 19(5):396–400.
Jan 2006;129(1 suppl):72S–74S. 40. Rivest P, Richer F, Bedard L. Difficulties associated
20. Pratter MR. Chronic upper airway cough syndrome with pertussis surveillance. Can Commun Dis Rep.
secondary to rhinosinus diseases (previously referred to Feb 15 2004;30(4):29–33, 36.
as postnasal drip syndrome): ACCP evidence-based 41. Goldrick BA. Pertussis on the rise. Am J Nurs. Jan 2005;
clinical practice guidelines. Chest. Jan 2006;129 105(1):69–71.
(1 suppl):63S–71S. 42. Outbreaks of pertussis associated with hospitals—
21. Brunnee T, Graf K, Kastens B, Fleck E, Kunkel G. Kentucky, Pennsylvania, and Oregon, 2003. MMWR.
Bronchial hyperreactivity in patients with moderate Jan 28 2005;54(3):67–71.
pulmonary circulation overload. Chest. May 1993; 43. Sahn SA. Pleural effusions. In: Polly PE, Heffner JE, eds.
103(5):1477–1481. Pulmonary/Respiratory Therapy Secrets. 2nd ed. Philadel-
22. Guyatt GH, Devereaux PJ. A review of heart failure phia, PA: Hanley and Belfus; 2002:459–462.
treatment. Mt Sinai J Med. Jan 2004;71(1):47–54. 44. Wells CL. Pulmonary pathology. In: DeTurk WE,
23. Cahalin LP, Ice RG, Irwin S. Program planning and Cahalin LP, eds. Cardiovascular and Pulmonary Physical
implementation. In: Irwin S, ed. Cardiopulmonary Physi- Therapy. An Evidence-Based Approach. New York, NY:
cal Therapy. 3rd ed. St. Louis, MO: Mosby; 1995:142–184. McGraw-Hill Medical; 2004:151–188.
24. Weitzenblum E. Chronic cor pulmonale. Heart. Feb 45. Brandenburg JA, Marrie TJ, Coley CM, et al. Clinical
2003;89(2):225–230. presentation, processes and outcomes of care for
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patients with pneumococcal pneumonia. J Gen Intern crossover study. J Am Coll Cardiol. Apr 7 2004;
PERSISTENT COUGH

Med. Sep 2000;15(9):638–646. 43(7):1149–1153.


46. Meehan TP, Chua-Reyes JM, Tate J, et al. Process of care 56. Fontenot AP. Sarcoidosis. In: Polly PE, Heffner JE,
performance, patient characteristics, and outcomes in eds. Pulmonary/Respiratory Therapy Secrets. 2nd ed.
elderly patients hospitalized with community-acquired Philadelphia, PA: Hanley and Belfus; 2002:270–273.
or nursing home-acquired pneumonia. Chest. May 2000; 57. Mixides G, Guy E. Sarcoidosis confined to the airway
117(5):1378–1385. masquerading as asthma. Can Respir J. Mar 2003;
47. Saavedra MT, Hanley ME. Pneumothorax. In: Polly PE, 10(2):114–116.
Heffner JE, eds. Pulmonary/Respiratory Therapy Secrets. 58. Franova S, Nosal’ova G, Antosova M, Nosal S. Enalapril
2nd ed. Philadelphia, PA: Hanley and Belfus; 2002: and diltiazem co-administration and respiratory side
463–466. effects of enalapril. Physiol Res. 2005;54(5):515–520.
48. Mastrovich JD, Greenberger PA. Psychogenic cough in 59. Leder SB, Espinosa JF. Aspiration risk after acute stroke:
adults: a report of two cases and review of the literature. comparison of clinical examination and fiberoptic
Allergy Asthma Proc. Jan–Feb 2002;23(1):27–33. endoscopic evaluation of swallowing. Dysphagia.
49. Bhatia MS, Chandra R, Vaid L. Psychogenic cough: a Summer 2002;17(3):214–218.
profile of 32 cases. Int J Psychiatry Med. 2002; 60. Dicpinigaitis PV. Cough reflex sensitivity in cigarette
32(4):353–360. smokers. Chest. Mar 2003;123(3):685–688.
50. Lee-Chiong TL, Matthay RA. Lung cancer. In: Polly PE, 61. Groneberg DA, Chung KF. Models of chronic obstruc-
Heffner JE, eds. Pulmonary/Respiratory Therapy Secrets. tive pulmonary disease. Respir Res. 2004;5:18.
2nd ed. Philadelphia, PA: Hanley and Belfus; 2002: 62. Small PM, Fujiwara PI. Management of tuberculosis in
370–379. the United States. N Engl J Med. Jul 19 2001;345(3):
51. Baruch AC, Steinbronn K, Sobonya R. Pulmonary 189–200.
adenocarcinomas associated with rheumatoid nodules: 63. McGarvey LP, Heaney LG, Lawson JT, et al. Evaluation
a case report and review of the literature. Arch Pathol and outcome of patients with chronic non-productive
Lab Med. Jan 2005;129(1):104–106. cough using a comprehensive diagnostic protocol.
52. Lipson DA, Palevsky HI. Thromboembolic disease. In: Thorax. Sep 1998;53(9):738–743.
Polly PE, Heffner JE, eds. Pulmonary/Respiratory 64. Jaspersen D. Extra-esophageal disorders in gastroe-
Therapy Secrets. 2nd ed. Philadelphia, PA: Hanley and sophageal reflux disease. Dig Dis. 2004;22(2):115–119.
Belfus; 2002:237–247. 65. Philp EB. Chronic cough. Am Fam Physician. Oct 1,
53. McLaughlin VV, Rich S. Pulmonary hypertension. 1997;56(5):1395–1404.
Curr Probl Cardiol. Oct 2004;29(10):575–634. 66. de Leeuw PW, Dees A. Fluid homeostasis in chronic
54. Rubin LJ. Primary pulmonary hypertension. N Engl J obstructive lung disease. Eur Respir J Suppl. Nov 2003;
Med. Jan 9, 1997;336(2):111–117. 46:33s–40s.
55. Sastry BK, Narasimhan C, Reddy NK, Raju BS. Clinical
efficacy of sildenafil in primary pulmonary hyperten-
sion: a randomized, placebo-controlled, double-blind,
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CHAPTER37
Dyspnea
■ Ragen L. Agler, PT, DPT, ATC

Description of the Symptom the emergency medical system should an


individual present with dyspnea in association
This chapter describes pathology that may lead with any of the following:
to dyspnea. Dyspnea or shortness of breath ■ Loss of consciousness
refers to the inability to feel adequately oxy- ■ Chest discomfort, presyncopal episodes, or
genated. The individual may report a smothering palpitations
feeling, difficulty taking a breath, chest tightness ■ An oxygen saturation of <88%
or constriction, and/or an increased effort to ■ Cyanosis of the lips or fingernail beds
breathe. The term dyspnea is derived from the ■ Marked hypertension (ie, >220/110) or
Latin dys-pnoea, meaning “difficult breath.” hypotension (ie, <90/60) in an individual
who is typically normotensive
Special Concerns
The therapist should be prepared to adminis-
ter basic life support interventions or activate

CHAPTER PREVIEW: Conditions That May Lead to Dyspnea

T Trauma
COMMON
Chest injury 766
Exercise 769
Pneumothorax 773
UNCOMMON
Not applicable
RARE
Not applicable

I Inflammation
COMMON
Aseptic
Asthma 765
Bronchitis 766
Cardiomyopathy 766
Pericarditis 772
Septic
Bronchitis 766
Cardiomyopathy 766
Gastroesophageal reflux disease 770
Pericarditis 772
(continued)

761
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762 Chapter 37 Dyspnea

Inflammation (continued)
DYSPNEA

COMMON
Pneumonia:
• Bacterial pneumonia (community-acquired pneumonia, nursing home–acquired pneumonia) 772
• Viral pneumonia 773
UNCOMMON
Aseptic
Allergic reaction 764
Idiopathic pulmonary fibrosis 770
Pleural effusion 772
Septic
Not applicable
RARE
Not applicable

M Metabolic
COMMON
Emphysema 769
Mountain/altitude sickness 771
Renal failure 775
Tobacco smoke 776
Transfusion reaction 776
UNCOMMON
Anemia 764
Decompression sickness 768
Diabetic ketoacidosis 769
Pregnancy 774
Sarcoidosis 775
RARE
Cocaine toxicity 767

Va Vascular
COMMON
Aortic aneurysm 765
Arrhythmias 765
Congestive heart failure 767
Cor pulmonale 767
Coronary artery disease 768
Mitral valve stenosis/regurgitation 771
Myocardial infarction (heart attack) 771
Pulmonary embolus 774
Pulmonary hypertension 775
Sickle cell disease/crisis 776
UNCOMMON
Not applicable
RARE
Not applicable
1528_Ch37_761-778 07/05/12 2:08 PM Page 763

Chapter 37 Dyspnea 763

DYSPNEA
De Degenerative
COMMON
Deconditioning 768
UNCOMMON
Not applicable
RARE
Not applicable

Tu Tumor
COMMON
Malignant Primary:
Not applicable
Malignant Metastatic:
Not applicable
Benign, such as:
• Benign lung nodules 766
UNCOMMON
Malignant Primary, such as:
• Pulmonary carcinoma 774
Malignant Metastatic, such as:
• Metastases, including from primary kidney, breast, pancreas, and colon disease 770
Benign:
Not applicable
RARE
Not applicable

Co Congenital
COMMON
Mitral valve prolapse 770
UNCOMMON
Not applicable
RARE
Not applicable

Ne Neurogenic/Psychogenic
COMMON
Anxiety/panic disorder 764
UNCOMMON
Not applicable
RARE
Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.
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764 Chapter 37 Allergic Reaction

Overview of Dyspnea potentially fatal. Individuals who present with


DYSPNEA

shortness of breath interfering with the ability


Dyspnea is a conscious awareness of one’s own to carry on a conversation with the therapist
breathing (which is normally an unconscious or oxygen saturation of less than 88% should
event). The sensation may be described as a receive emergency medical attention.
smothering feeling and the inability to get
enough air. The individual often presents with ■ Anemia
labored breathing, a distressed anxious expres- Chief Clinical Characteristics
sion, dilated nostrils, a protrusion of the This presentation includes lethargy, cold skin,
abdomen, and an expanded chest. Dyspnea is a depression, easy fatigability, shortness of breath,
common event that occurs in most individu- and cognitive impairment.2
als, even in the absence of pathology. It may Background Information
be precipitated by vigorous exercise, anxiety, This condition involves a decrease in the
deconditioning, certain medications, seasonal oxygen-carrying capacity of blood secondary
allergies, and anemia. However, because dysp- to (1) a decrease in the erythrocyte (red blood
nea can be the symptom of a very serious med- cell) content of blood, (2) a diminished
ical condition, the therapist must be able to content of hemoglobin per erythrocyte, or
critically evaluate the cause of dyspnea and (3) a combination of both.3 It can arise from
take the appropriate actions to ensure the failed synthesis, premature destruction, hem-
patient’s safety. In most cases, including those orrhage, or deficiencies in iron, B12, or folic
where the patient’s initial referring condition acid. Other conditions that render the patient
is not that of dyspnea, the patient should be susceptible to anemia include recent major
strongly encouraged to report any dyspnea to surgery (eg, orthopedic and cardiopulmonary
his or her primary physician to ensure that the surgeries), pregnancy, lesions of the gastroin-
proper medical workup is completed. testinal tract, sickle cell trait, and cancer.4 The
resultant reduction in the blood’s oxygen-
Description of Conditions That carrying capacity initiates a reflex sinus tachy-
May Lead to Dyspnea cardia in attempts to maintain adequate tissue
oxygenation while blood pressure is usually
■ Allergic Reaction within the normal resting range.5 If this condi-
Chief Clinical Characteristics tion is suspected, the patient should be
This presentation includes short quick breaths, referred to his or her primary care practitioner
tingling in the lips, tightness in the throat, and for further evaluation. The diagnosis is con-
a rash or welt on the skin after exposure to an firmed by routine blood testing. Treatment
allergen. Patient can also present with wheezing, involves addressing the underlying cause of
cough, and nausea.1 anemia, including iron supplementation.

Background Information ■ Anxiety/Panic Disorder


When an individual is exposed to an allergen Chief Clinical Characteristics
(ie, pollen, food, medication, and insect bites), This condition is characterized by dyspnea with
the B cells produce immunoglobulin E (IgE) and without exertion and the inability to catch
antibodies. These antibodies bind to the one’s breath. Patients may also present with
receptors of basophils and tissue mast cells. dizziness, palpitations, and unexplained weak-
On subsequent exposures, the allergen reacts ness.6 Pulse is typically rapid and regular and
to the IgE antibody, which causes degranula- associated with a discrete period of intense fear
tion of the basophils and tissue mast cells.1 or discomfort during which several predictable
This results in histamine release, which causes symptoms develop and peak in intensity within
an increase in vascular permeability and 10 minutes. These symptoms can include pal-
smooth muscle contraction. The smooth pitations, chest pain/discomfort, feeling faint or
muscle contraction causes bronchospasm, dizzy, sweating, shaking/trembling, paresthe-
resulting in dyspnea. If the allergic reaction is sias, chills/hot flashes, fear of losing control,
serious, anaphylactic shock occurs, which is and fear of dying.7
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Chapter 37 Asthma 765

Background Information also report changes in their heartbeat or the

DYSPNEA
The body’s response to this condition is usually feeling of their heart fluttering or skipping a
short lived and subsides when the individual is beat, fatigue, or syncope.10
reassured or the stressful stimulus is removed.
Although anxiety or stress is perceived by many Background Information
areas of the brain, the integrated response This group of conditions is caused by a mal-
ultimately leads to elevation of plasma cortisol function in the heart’s electrical system.10 A
and catecholamine levels. Both hormones are large variety of arrhythmias can occur. Many
responsible for raising blood pressure and of them can result in a decrease in cardiac
cardiac output, the former directly leading to output. The primary concern with decreased
increased heart rate and force of contraction. cardiac output is dyspnea.10 The diagnosis is
The response leads to a general state of arousal confirmed with cardiac auscultation and
and prepares the individual for useful defensive electrocardiography. If an individual has a
behavior (ie, the “fight-or-flight” response). In history of this condition and has a new onset
the absence of other adverse symptoms or of dyspnea, it is of critical importance for
underlying cardiovascular pathology, palpita- the individual to receive emergency medical
tions due to stress pose no serious risk and the attention.
therapist should reinforce this in the mind of
■ Asthma
the patient. Disabling cases of this condition
should be referred to the appropriate health Chief Clinical Characteristics
care provider and may include pharmacologic This presentation typically involves dyspnea in
and psychological therapy. combination with a chronic or intermittent
persistent cough, especially in response to
■ Aortic Aneurysm factors or stimuli such as pollen, dust, animal
Chief Clinical Characteristics dander, environmental conditions, certain
This presentation can include dyspnea upon foods, and exercise.11
exertion. Individuals with an abdominal aor-
Background Information
tic aneurysm can also present with a pulsatile
The stimulating factor irritates the lining of
abdominal mass, abdominal pain, and abdom-
the bronchial airway and a hyperadaptive
inal rigidity.8 Individuals with a thoracic aor-
response is provoked, resulting in a narrowing
tic aneurysm can present with hoarseness,
of the airway. If the individual is in an area that
wheezing, coughing, hemoptysis, chest pain,
exposes him or her to such triggers as pollen,
back pain, or abdominal pain.9
dust, or animal dander, a prudent course of
Background Information action would be to have the individual exercise
Many individuals with this condition are in a different area away from the trigger, if pos-
asymptomatic. This condition is defined as sible. For example, if outdoors, move the exer-
the focal dilation of the aorta with at least a cise program indoors. Cold, dry air is another
50% increase over normal arterial diameter. It is cause of exacerbation for individuals with this
caused primarily by a degenerative process of condition. Exercising in a warm, moist climate
the aortic medial layer secondary to atheroscle- or performing activities in a pool would help
rosis. Increased concentration of proteolytic to reduce triggering an asthma-induced dysp-
enzymes in the aorta appears to be the cause of neic episode. Individuals who are prone to
the aortic media degradation. X-ray, ultrasound, “asthma attacks,” especially during exercise,
computed tomography, and angiography con- should try to exercise in an area free from any
firm the diagnosis. Individuals suspected of airborne irritants and in a warm, moist envi-
having this condition must be referred to their ronment. If an asthma exacerbation occurs,
primary care physician immediately. the individual should be instructed to stop the
activity, rest, and use any medically prescribed
■ Arrhythmias medications such as inhaled bronchodilators
Chief Clinical Characteristics or corticosteroids until the symptoms subside.
This presentation may include shortness of The physical therapist should try to alter
breath with and without exertion. Patients can the environment to avoid further episodes of
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766 Chapter 37 Benign Lung Nodules

dyspnea. If the individual is unable to control physical energy level and, therefore, their abil-
DYSPNEA

the situation within a reasonable amount of ity to fully participate in physical therapy
time, the physical therapist should be prepared treatment may be impaired.
to activate the emergency medical system.
■ Cardiomyopathy
■ Benign Lung Nodules Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can involve dyspnea with and
This presentation includes a new onset of short- without exertion, in addition to swelling of the
ness of breath with exertion, as well as possible extremities, abdominal distention, fatigue,
blood in the sputum; chest, shoulder, or back dizziness, and fainting during physical activities.17
pain; and wheezing.12,13
Background Information
Background Information There are three primary types of this condi-
Up to 70% of solitary pulmonary nodules are tion, including restrictive, dilated, and hyper-
benign lung tumors. Although not considered trophic.17,18 This condition can be caused
a significant health problem, these tumors uncontrolled diabetes, drug abuse, radiation
can increase the patient’s risk of pneumonia, therapy, fibrosis, malignancies or autosomal
atelectasis, and hemoptysis.14 Chest x-ray and dominant genetic factor. A viral, bacterial, rick-
computed tomography confirm the diagnosis, ettsial, metazoal, or protozoal infection also
and biopsy of neoplasia may be necessary may account for this presentation. Regardless
to determine the tumor cell type. Treatment of the type of cardiomyopathy, the cardiac
typically involves observation, although indi- output is severely altered secondary to myocar-
viduals with this condition who demonstrate dial contractile disruption and arrythmias.17,18
bloody sputum, new onset of dyspnea, or The diagnosis is confirmed by murmur or an
instability of hemodynamic/oxygen transport S3 heart sound on cardiac auscultation in com-
should be referred for emergency evaluation bination with echocardiography. It is of critical
and treatment by a physician. importance that the emergency medical system
be activated immediately if this condition is
■ Bronchitis suspected. In addition, if an individual with
Chief Clinical Characteristics known cardiomyopathy reports dyspnea with
This presentation includes shortness of breath exertion that does not subside with rest, the
upon exertion, frequently combined with fever, emergency medical system must be activated.
cough with sputum production, muscle aches,
and fatigue. Individuals with this condition ■ Chest Injury
may also report a sore throat, runny or stuffy Chief Clinical Characteristics
nose, and/or a headache.15 This presentation typically involves guarding over
the affected area of an injury to the thorax, in
Background Information
combination with diminished chest wall move-
Acute bronchitis—inflammation of the trachea,
ment and pain in and around the affected area.
bronchi, or bronchioles16—can be associated
with smoking, inhalation of dust or chemical Background Information
pollutants, or infectious disease process. Dur- Direct trauma to the upper body including the
ing an acute episode, there is a decrease in chest wall, lungs, and throat is an apparent
bronchial mucociliary function due to the cause of cough following a chest injury. The
mucous membranes being hyperemic and trauma can include a physical injury resulting
edematous. This leads to increased mucus from a motor vehicle accident, fall, physical
production.15 Individuals who present to the assault, or penetrating wound. The trauma can
clinic with this clinical picture should be also occur from surgery to the regions men-
referred to their primary care physician for tioned above. In the case of a flail chest second-
medical follow-up. Physical therapy interven- ary to trauma, the breathing mechanics will be
tion can continue as long as the oxygen satu- altered. During inspiration the chest wall will
ration does not fall below 88%. Individuals expand, but the injured area will draw inward.
with this condition will not have a typical During expiration the chest wall contracts,
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Chapter 37 Cor Pulmonale 767

while the area of injury protrudes.19 The reac- nervous system can result in abnormal heart

DYSPNEA
tion that causes the cough is similar in nature rhythms. These arrhythmias can lead to dysp-
regardless of the origin of trauma. If the nea.20 This diagnosis can be confirmed with a
trauma is directly affecting the bronchial airway, history positive for cocaine use and abuse.
the cough mechanism is protective in nature
and the purpose is to remove the cause of the ■ Congestive Heart Failure
irritation. When the trauma is not directly Chief Clinical Characteristics
affecting the bronchial airway (as in the case of This presentation can be characterized by
a crush or penetrating chest wall injury), the dyspnea on exertion, orthopnea, persistent
surrounding areas will become inflamed and cough, peripheral edema, and fatigue. On exam-
swollen. In this case the excess fluids can accu- ination, individuals with this condition may
mulate in the lungs. These excess fluids will demonstrate systolic murmur, crackles/rales on
become an irritation to the bronchial airway. pulmonary auscultation, a third (S3) and/or
The cough reaction will be to remove the fluids fourth (S4) heart sound on cardiac auscultation,
so that normal gas exchange can take place. jugular vein distention, or peripheral cyanosis.
When the trauma is surgical in nature, fluids
Background Information
will arise due to the inflammatory response
This condition is characterized by left ventricu-
of the surrounding tissues. The cough response
lar contractile dysfunction resulting in ventric-
is similar in quality to the physical trauma
ular dilation and poor contraction. Contractile
mentioned above. The physical therapist
dysfunction can be caused by myocardial
should be able to recognize the source of the
ischemia or infarction, coronary artery disease,
trauma and respond accordingly. If the cause of
or bacterial/viral infection. As the heart pump
the cough is due to direct trauma to the airways
fails, the blood returning to the heart from the
(physical or surgical), the physical therapist
lungs is backed up, causing overload in the pul-
should suggest splinting techniques to relieve
monary circulation and leading to bronchial
pain and give external support. If the cause
hyperreactivity. Dyspnea, wheezing, and per-
of the cough is indirect trauma, the physical
sistent coughing are common signs in individu-
therapist should incorporate postural drainage,
als with bronchial hyperreactivity.21 Medical
percussion, vibration, deep-breathing exercises,
treatment usually consists of a combination of
and assisted cough techniques in an attempt to
vasodilators, diuretics, digoxin, beta blockers,
facilitate removal of the secretions. The physi-
antiarrhythmic agents, and anticoagulants.22
cal therapist should be aware of the underlying
Implementing a cardiac rehabilitation program
causes and physical structures present prior to
would be beneficial.23 The individual who is
administering any of the above pulmonary
newly diagnosed or having an exacerbation of
physical therapy techniques. Percussion and
symptoms would begin in Phase I of a cardiac
vibration directly over fractured ribs are con-
rehabilitation program and progress to Phase
traindicated but encouraged around the site.19
III over a period of several months.
■ Cocaine Toxicity
■ Cor Pulmonale
Chief Clinical Characteristics
This presentation commonly involves shortness Chief Clinical Characteristics
of breath with and without exertion in associ- This presentation involves dyspnea on exertion,
ation with a high blood pressure and heart rate, chronic productive cough, wheezing, lower
tremors, and sweaty skin. Psychosis can also be extremity edema, jugular venous distention,
a common feature.20 and in chronic cases digital clubbing.19
Background Information Background Information
Cocaine is presently the second most abused This condition is characterized as pulmonary
illicit stimulant in the United States. The major heart disease. The right side of the heart may
effect of cocaine is the stimulation of the become enlarged due to pulmonary hyperten-
sympathetic nervous system. This results in an sion, pulmonary embolism, chronic obstruc-
increased heart rate and increased blood pres- tive pulmonary disease, idiopathic pulmonary
sure. The overstimulation of the sympathetic fibrosis, and Pickwickian syndrome.24 Medical
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768 Chapter 37 Coronary Artery Disease

management includes diuretics, supplemen- therapist should be prepared to administer


DYSPNEA

tal oxygen use, and dietary salt restrictions.19 basic life support measures (including the
The physical therapist should take to the role use of an automated external defibrillator if
of preventing the onset of cor pulmonale by warranted) while awaiting advanced cardiac
addressing the underlying cause. Aerobic life support assistance.
exercises, mobility training, and breathing
exercises could have an important effect on ■ Decompression Sickness
the above-mentioned causes. Individual edu- Chief Clinical Characteristics
cation would also be beneficial, including This presentation may involve short, shallow
how to monitor lower extremity edema and breathing after an episode of scuba diving in
signs of respiratory failure. The physical ther- combination with joint pain in the ankles,
apist should be aware of any acute signs and knees, wrists, hips, shoulders, and elbows;
symptoms of severe forms of this condition headaches; confusion; pain with breathing; and
to the point that the individual experiences itching of the face, arms, and upper torso.25,26
pallor, sweating, severe chest pain, weak and Background Information
rapid pulse, anxiety, and diminished level of The gas nitrogen leads to the pathology of
consciousness. In this situation, the physical decompression sickness. As a scuba diver
therapist should activate the emergency med- descends, more nitrogen enters the tissues due
ical system immediately, because these signs to the increase in pressure. If a diver ascends
and symptoms suggest the condition has slowly, the excess nitrogen can be expelled
progressed to become life threatening. through the lungs. However, if the diver
■ Coronary Artery Disease ascends too quickly, the nitrogen will come out
of solution in the body fluids and tissues and
Chief Clinical Characteristics convert to the gas phase, forming bubbles in
This presentation can include dyspnea in the blood and tissues of the body.26,27 These
combination with symptoms of angina, often bubbles can involve the skin, muscles, and
described as substernal chest discomfort, pressure, lymphatic system (Type I); the lungs, brain,
heaviness, squeezing, or burning that radiates and ears (Type II); or they can lead to arterial
to the shoulders, arms (left greater than right), gas embolism (Type III).25,26 It is of critical
neck, jaw, and epigastrium. importance that the emergency medical system
Background Information be activated secondary to potentially fatal con-
With decreased myocardial oxygen supply (or sequences of this condition. Typical treatment
increased demand in the case of exertion), involves supportive intervention combined
vulnerable myocardial cells become ischemic with a hyperbaric chamber protocol.
and hyperirritable and discharge sponta- ■ Deconditioning
neously, driving abnormal cardiac rhythms.
The ectopic rhythm can be supraventricular or Chief Clinical Characteristics
ventricular in origin, tachycardic or bradycardic This presentation may be characterized by
(in the case of complete heart block), and is shortness of breath with low-level exertion and
difficult to differentiate on the basis of palpa- a history of being sedentary.28
tion alone. Arrhythmia leads to dyspnea. In Background Information
cases of tachyarrhythmias, the resulting increase Deconditioning is the result of a person being
in myocardial oxygen demand may escalate the inactive. This can be caused by an illness, an
condition from one of transient ischemia to injury, or a sedentary lifestyle. With exercise,
one of frank infarction and the episode may the body responds with an increased heart
become life threatening. Use of sublingual ni- rate and an increased respiratory rate.28 The
troglycerin or supplemental oxygen prescribed increased respiratory rate can lead to dyspnea.28
by the patient’s physician may resolve minor Due to the fact that deconditioned individuals
episodes. However, if the patient becomes can present with symptoms while they exercise
unstable during episodes of dyspnea with that could indicate a pathology, a thorough
associated angina pectoris, activation of the pre-exercise screening examination completed
emergency medical system is warranted. The by the physical therapist will allow the therapist
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Chapter 37 Exercise 769

to differentiate dyspnea caused by this condition lungs, resulting in decreased diffusion of

DYSPNEA
from dyspnea caused by a serious pathology. oxygen. The elastic recoil of the lungs is
In the absence of serious pathology, individu- lost, resulting in retention of air. A history of
als with this condition should be assured that smoking tobacco is common; exposure to
this is a normal response to increased physical second-hand smoke or occupational toxins
activity and counseled in how to control symp- also can make a person prone to developing
toms by modifying the intensity of exercise and emphysema. Medical intervention commonly
other behavioral methods as appropriate. consists of smoking cessation, use of bron-
chodilators, and limited use of supplemental
■ Diabetic Ketoacidosis oxygen. In severe cases lung volume reduc-
Chief Clinical Characteristics tion surgery may be performed to remove
This presentation typically involves confusion, hyperinflated blebs or bullae. The physical
agitation, dyspnea, tachycardia, and fruity therapist’s treatment should include aspects
breath odor.29 of a pulmonary rehabilitation program
Background Information including airway hygiene techniques such as
In a fasting state in a healthy individual, the percussion and vibration, postural drainage,
body changes from carbohydrate metabolism and aerobic exercises at a submaximal level.
to fat oxidation. During this process the free Individual education about pursed-lip
fatty acids are converted to acetate, which is breathing techniques should also be included.
then turned into ketoacids.29 This is exported If the individual has undergone surgery for
from the liver to the peripheral tissues. Despite removal of emphysematous blebs or bullae,
the amount of circulating glucose in an indi- the physical therapist should also address any
vidual with insulin-dependent diabetes, the postsurgical movement dysfunction that may
carbohydrate cannot be used secondary to be affecting the individual.
a lack of insulin. This results in a maximal ■ Exercise
production of ketones, which leads to this
condition.29 Primary intervention involves Chief Clinical Characteristics
prevention by timing physical activity with the This presentation typically involves a breathing
insulin schedule and by maintaining a regular rate that increases gradually and appropriately
insulin dosing regimen. However, when pres- for the intensity of the exercise bout. When the
ent, this condition is considered a medical exercise bout is terminated, the breathing rate
emergency. gradually returns to resting values. Assessment
of the blood pressure will typically reveal elevated
■ Emphysema systolic values that are appropriate for the
Chief Clinical Characteristics intensity of physical exertion (eg, 10 to 12 mm Hg
This presentation includes complaints of short- increase for every metabolic equivalent increase
ness of breath, persistent cough with sputum pro- in workload).
duction, wheezing, and decreased exercise Background Information
tolerance.30 Physical examination reveals a In response to the metabolic demands placed
prolonged expiratory phase during respiration, on the cardiopulmonary system by activities
increased anteroposterior diameter of the chest involving the contraction of large muscle
(barrel chest appearance), flattened diaphragm, groups, cardiac output will increase. This is
and distant breath sounds on pulmonary aus- primarily accomplished by enhanced cardiac
cultation with wheezes and/or crackles. automaticity and contractility, yielding a sub-
Background Information stantial increase in heart rate that parallels
This condition is the most common obstruc- this increase in respiratory rate. To the indi-
tive pulmonary disease. It is characterized as vidual who is typically sedentary or otherwise
enlarged air spaces due to disruption of the unaccustomed to exercise, the resultant short-
destruction of elastic fibers in the alveolar wall, ness of breath may be considered unusual and
which is irreversible.31 The emphysematous pathological. The response is mediated by
changes produce airway obstruction with enhanced sympathetic outflow and circulating
impaired ventilation and gas exchange in the catecholamines involved in the “fight-or-flight”
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770 Chapter 37 Gastroesophageal Reflux Disease

response and does not typically lead to any Background Information


DYSPNEA

adverse consequences in the absence of under- This condition is a specific form of chronic
lying organic heart and lung disease. This fibrosing interstitial pneumonia that is limited
condition is a normal response to exertion to the lung. The “stiffness” of the lungs causes a
and will subside with cessation of the exercise rapid, shallow breathing pattern. This is due to
bout. No emergency intervention is warranted. a ventilation/perfusion mismatch and the indi-
vidual can become hypoxic during exercise.
■ Gastroesophageal Reflux Disease This will lead to a worsening of the above cough
Chief Clinical Characteristics and dyspnea symptoms. Pulmonary rehabilita-
This presentation commonly includes dyspnea, tion may help improve the quality of life and
dysphonia, hoarseness, sore throat, gum inflam- prepare the individual for possible lung trans-
mation, dental erosion, chest pain, heart burn, plantation. The physical therapist should
sputum production with coughing, and wheez- progress through the program slowly so as not
ing.32 The cough will be more prevalent in the to exacerbate symptoms. If the symptoms
morning and after meals. This condition is com- worsen to tussive syncope or increased hypox-
mon among individuals who are nonsmokers emia, the physical therapist should activate the
with a normal chest radiograph and not taking emergency medical system as warranted. The
angiotensin-converting enzyme inhibitors.32 prognosis is poor and most individuals die
within 3 to 8 years from onset of symptoms.35
Background Information
This condition involves retrograde movement ■ Metastases, Including from
of gastric contents into the esophagus. In indi- Primary Kidney, Breast, Pancreas,
viduals with this condition, persistent cough is and Colon Disease
one of the most common complaints.33 The
Chief Clinical Characteristics
mechanism of cough in an individual with gas-
This presentation commonly includes dyspnea,
troesophageal reflux disease is from microaspi-
hemoptysis, persistent cough, chest discomfort,
ration of gastric contents or a vagally mediated
weight loss, and voice hoarseness, in association
esophageal-tracheobronchial reflex. Medical
with possible symptoms related to the primary
treatment includes vigorous acid suppression,
disease.
elevating the head of the individual’s bed 10 cm,
weight loss, diet modification to high-protein Background Information
and low-fat foods, omitting high-acid foods, The lungs are the most common site of metas-
antacid use prior to bedtime, and limiting food tases from other types of cancer. When tumor
intake 2 hours prior to bedtime.33 Referral cells migrate from their primary site they can
should be made to the individual’s primary work their way into the circulation or lymphatic
physician for a 24-hour esophageal pH test. system. The lungs filter these systems. The most
Physical therapists treating individuals with common cancers to metastasize to the lungs
this condition in its acute form should avoid are kidney, breast, pancreas, colon and uterus.
having the individual flex forward at the trunk Treatment of this condition depends on the type
or hips and also avoid vigorous activities in and extent of metastases, including surgical
the early morning or after meals, because these resection, chemotherapy, and radiation therapy.
situations raise the likelihood of symptom
aggravation. ■ Mitral Valve Prolapse
Chief Clinical Characteristics
■ Idiopathic Pulmonary Fibrosis This presentation can include shortness of
Chief Clinical Characteristics breath, chest pain, palpitations, fatigue, and
This presentation involves exertional dyspnea light-headedness and may cause periodic syn-
and an irritating, nonproductive cough.34 Lung copal episodes in a small subset of patients.36
auscultation will reveal a “Velcro”-like quality Most individuals with this condition are asymp-
crackle most noticeable at the bases. Pulmonary tomatic. Upon cardiac auscultation, a midsys-
function tests will demonstrate a restrictive tolic click is often heard best over the fifth
disease pattern with decreased vital capacity, intercostal space, midclavicular line, often
residual volume, and total lung capacity. followed by a late systolic murmur.
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Chapter 37 Myocardial Infarction (Heart Attack) 771

Background Information digitalis diuretic, and vasodilator medications.

DYSPNEA
The diagnosis is confirmed by echocardiogra- In cases of significant forms of this condition,
phy or angiography, which usually reveals repair or replacement of the mitral valve will
exaggerated systolic bowing beyond the mitral be warranted. Cardiac rehabilitation program
annulus of one or both valve leaflets. Individu- is optimal in the surgical and postsurgical
als with this condition may be instructed to management of this condition.23 The physical
cough forcefully or perform a Valsalva maneu- therapist should be aware that the individual
ver, bearing down against a closed glottis, dur- will be self-limiting in his or her exercise toler-
ing episodes of palpitations in an effort to break ance if the condition worsens.
the abnormal rhythm through vagal mediation.
Quite often, individuals with this condition are ■ Mountain/Altitude Sickness
also prescribed calcium channel blockers or Chief Clinical Characteristics
beta blockers to suppress the occurrence of This presentation typically involves dyspnea
palpitations. Occasionally, the tachycardia may with exertion after a hike, ski trip, or mountain
be prolonged and immediate medical interven- climb in association with headache, difficulty
tion is usually warranted, especially if the patient sleeping, nausea, and the sensation of a bound-
has underlying coronary artery disease and ing pulse.38
becomes hemodynamically unstable.
Background Information
■ Mitral Valve Stenosis/ As altitude increases, the atmospheric concen-
Regurgitation tration of oxygen decreases.38 For example at
Chief Clinical Characteristics 8,000 and 18,000 feet there is 25% less and
This presentation includes dyspnea on exer- 50% less available oxygen compared to sea level,
tion, persistent cough, decreased exercise toler- respectively.39 Typical physiological response to
ance, orthopnea, and occasionally hemoptysis a decrease in oxygen is an increase in respiratory
in combination with a systolic murmur best rate and heart rate. As CO2 is eliminated with
heard at the apex of the heart, rales on pul- each exhalation, the kidneys increase excretion
monary auscultation, a third (S3) and/or fourth of bicarbonate to maintain pH balance. How-
(S4) heart sound on cardiac auscultation and ever, if the respiratory rate remains high for an
jugular venous distention. extended period of time (24 to 44 hours), the
kidneys are unable to excrete enough bicarbon-
Background Information ate to maintain the balance and, thus, the blood
This condition is characterized by the leaflets becomes alkalotic. This pH change leads to the
of the mitral valve becoming stiff and closing dilation and leaking of the microvascular
incompletely, usually due to the annulus (rim) beds,39 resulting in an accumulation of fluids in
of the valve being stretched. Left arterial all body tissues. Respiratory distress may result
and/or ventricular enlargement can pull and from pulmonary edema. Physical therapists
stretch on the mitral valve annulus. With this should be vigilant for this condition, since it
condition, the blood coming from the lungs may occur in individuals who participate in
into the left side of the heart will be slowed and activities at lower elevations, as well. This
can back up into the lungs, leading to volume condition is a medical emergency.
overload in the lungs.37 The chronic overload
in the pulmonary circulation is accompanied ■ Myocardial Infarction (Heart
by bronchial hyperreactivity. Symptoms of Attack)
dyspnea, wheezing and persistent coughing Chief Clinical Characteristics
are common in individuals with bronchial This presentation often includes chest pain, left
hyperreactivity.21 This condition usually occurs arm or shoulder pain, jaw pain, upper back
secondary to prior rheumatic fever occurring pain, palpitations, and dyspnea.40–42
in childhood, yet the individual does not
become symptomatic until the adult years. Background Information
Other etiologies include coronary artery disease, This condition involves irreversible necrosis
bacterial endocarditis, and left ventricular dila- of the heart muscle secondary to prolonged
tion. Medical treatment consists of antibiotic, ischemia.40,41 Atherosclerosis is a disease
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772 Chapter 37 Pericarditis

process that is primarily responsible for this to their primary care physician for supportive
DYSPNEA

condition. Plaque fissures and ruptures result care that may include prescription of anti-
from stress at the atherosclerotic lesion. These inflammatory or antibiotic medication.
plaque ruptures are considered the major
■ Pleural Effusion
trigger for coronary thrombosis.40–42 Strenu-
ous physical activity or emotional stress leads Chief Clinical Characteristics
to an increased sympathetic nervous system This presentation commonly includes dysp-
response, increased blood pressure, increased nea, persistent cough, and pleuritic chest pain.
heart rate, and increased force production Physical examination may reveal decreased
during ventricular contraction. In turn, this chest expansion, diminished or absent breath
sequence of events increases the stress at the sounds, dullness to percussion, and possible
atherosclerotic lesion and causes the plaque to friction rub.
rupture.40–42 During acute forms of this condi- Background Information
tion, ST-segment elevations, dynamic T-wave This condition is a collection of fluid between
changes, or ST depressions may be present the visceral pleura and parietal pleura, which
upon electrocardiographic analysis. Upon occurs as fluid production exceeds fluid
auscultation an S4 gallop may be detected. If absorption in the pleural space. The etiology
this condition is suspected, the emergency of this condition involves congestive heart fail-
medical system (EMS) must be activated imme- ure, atelectasis, pneumonia, impaired lym-
diately to decrease the risk of sudden death. phatic drainage, and kidney or liver failure.43
Basic life support (BLS) should be initiated The cough is due to the excess fluid and dysp-
immediately after the EMS system is activated. nea, which irritate the cough receptors in the
If after completing the BLS assessment of the bronchial airways and diminish gas exchange.
patient’s condition, the patient continues to The cough reflex is triggered in a response to
present with an acute myocardial infarction, it remove the excess fluids. In severe cases, the
is recommended that the therapist administer individual may have a tracheal shift away from
a baby aspirin to the patient. the affected side44 and may develop mild
hypoxemia. In this case the physical therapist
■ Pericarditis
should administer supplemental oxygen to
Chief Clinical Characteristics maintain oxygen saturation at a level where
This presentation is characterized by chest pain the individual is comfortable and maintains a
and fever as the hallmark symptoms. Chest pain calm respiratory rate. Except for pleural effu-
almost always has a mechanical component in sion caused by congestive heart failure, the
which it is aggravated by coughing, deep inspi- medical treatment for pleural effusion is to
ration, or lying supine. This characteristic remove the fluid by thoracocentesis or place-
distinguishes this condition from angina. Dys- ment of a chest tube.44 If the individual has
pnea may be reported. Pericardial friction rub recently had a thoracocentesis performed to
that is likened to two pieces of leather rubbing drain a pleural effusion, the physical therapist
against one another may be heard during should be aware of the signs and symptoms
cardiac auscultation. Asking the patient to mo- of a possible pneumothorax that may have
mentarily hold his or her breath while auscul- developed due to the procedure.
tating helps to distinguish friction rub from
this condition and a pleural friction rub (the lat- PNEUMONIA
ter would disappear upon breath-holding). ■ Bacterial Pneumonia
Background Information (Community-Acquired
This condition can be either aseptic (eg, post- Pneumonia, Nursing
myocardial infarction, radiation induced, drug Home–Acquired Pneumonia)
induced, or connective tissue disease) or septic Chief Clinical Characteristics
(eg, viral, pyogenic bacteria, and tuberculosis). This presentation usually includes dyspnea,
This condition is often self-limiting, but indi- persistent cough, sputum production with oc-
viduals with this condition should be referred casional hemoptysis, fatigue, and pleuritic
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Chapter 37 Pneumothorax 773

chest pain.45 Pulmonary auscultation will ■ Pneumothorax

DYSPNEA
likely reveal decreased or absent breath sounds Chief Clinical Characteristics
and wheezes/crackles.46 This presentation typically involves dyspnea,
Background Information pleuritic chest pain, and dry cough and the
The onset of illness is usually abrupt. Med- individual may have absent breath sounds with
ical treatment consists of some form of hyperresonant percussion over the affected
penicillin or amoxicillin.45 Physical thera- area.
pists’ intervention should include airway Background Information
hygiene techniques such as percussion and This condition is free air that leaks into the
vibration, postural drainage, assisted cough pleural space between the visceral and pari-
techniques, and deep-breathing exercises. etal pleura. The cough seen in an individual
Mobilizing the individual would also be with this condition results from the irritation
beneficial in assisting to clear the lungs of the cough receptors from the increased
of sputum. intrathoracic pressure. There are three main
classifications of this condition. Traumatic
■ Viral Pneumonia pneumothorax is caused by a trauma or
Chief Clinical Characteristics injury, direct or indirect, to the thorax. Com-
This presentation commonly involves increas- mon causes include gunshot wound, knife
ing dyspnea and persistent, dry, nonproduc- wound and motor vehicle accidents. Sponta-
tive cough. Chest auscultation reveals rhonchi, neous pneumothorax occurs as the structural
rales, or wheezes. The individual may also integrity of the pleural tissue is weakened.
exhibit fever, decreased appetite, headache The tissue becomes so weakened that a bleb
and fatigue. This condition most commonly or bullae can rupture, allowing air into the
occurs after the onset of influenza or inhala- pleural space. Individuals with asthma, cystic
tion of some other airborne virus. fibrosis, emphysema, pulmonary infections
and empyema are likely candidates for devel-
Background Information oping a spontaneous pneumothorax. This
This condition can be community acquired condition can also occur in healthy individu-
or nosocomial. The cough reflex is triggered als. Healthy individuals who smoke, have a
by stimulation of the cough receptors from positive family history, and males between
the infectious material in the airways. A chest the ages of 10 and 30 years who have a tall,
radiograph will show infiltrates in the areas thin body habitus are more prone to develop-
affected. The aim of treatment in an individ- ing a spontaneous pneumothorax.47 Tension
ual with a viral pneumonia is to increase pneumothorax is characterized by air entering
oxygen transportation. This includes pul- the pleural space but not exiting. This condi-
monary percussion and vibration, postural tion is caused by a one-way or ball valve
drainage, deep-breathing exercises, assisted fistula. The ipsilateral lung becomes com-
cough techniques, and mobilizing the indi- pressed and the mediastinum shifts contralat-
vidual. The physical therapist should be erally. A tension pneumothorax is a medical
aware of the potential for contamination or emergency because the individual will develop
infection from one individual to another or decreased cardiac output, refractory hypox-
to the physical therapist. The affected indi- emia and hypotension. The physical therapist
vidual should be treated away from other will need to activate the emergency medical
susceptible individuals. Physical therapists system. Medical treatment consists of chest
should protect themselves by wearing a mask tube placement to remove the trapped air.
that covers their nose and mouth. The The use of high concentrations of supple-
affected individual may also consider wearing mental oxygen will reabsorb the lesion four
a mask to protect others. Performing hand times faster than if no supplemental oxygen
hygiene before and after treatment of an was used.47 This situation may resolve without
affected individual will help reduce the the use of a chest tube in individuals with small
spread of infection. pneumothoraces (<15% of the hemithorax).
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774 Chapter 37 Pregnancy

The physical therapist should have supple- cause for a person developing lung carcinoma.52
DYSPNEA

mental oxygen available for use during ther- There are many types of carcinoma of the
apy sessions. If the individual appears to be bronchial airways including small-cell lung can-
in respiratory distress during a session, the cer (ie, oat cell carcinoma) and non–small-cell
physical therapist should activate the emergency lung cancers (ie, squamous cell, adenocarci-
medical system. noma, and large-cell carcinomas).19 This condi-
tion has a strong association with rheumatoid
■ Pregnancy arthritis, and may even precede joint involve-
Chief Clinical Characteristics ment.53 In an individual who is in the early
This presentation includes shortness of breath stages of localized lung cancer, symptoms do not
often associated with a rapid and bounding vary much from the symptoms related to ciga-
pulse, and there may be an increased occur- rette smoking; therefore, the individual may not
rence of symptoms to term.48 At times, the seek medical attention promptly.19 Individuals
palpitations may precipitate dizziness, presyn- with this condition may have tumors located in
cope, or frank syncope.49 the main stem or segmental bronchi, causing
dyspnea and cough. Pulmonary auscultation
Background Information will usually reveal normal findings. Individuals
Episodes of dyspnea are usually benign and suspected of having this condition should be
self-limiting. Significant changes occur in hor- referred to their primary care physician. Com-
monal and hemodynamic function during plete blood count, chest radiography, computed
pregnancy that predispose women to the tomography, positron emission tomography,
development of dysrhythmias and correspon- pulmonary function tests, ventilation/perfusion
ding dyspnea. Changes in hormone levels scan, and biopsy of the suspected tumor are
during pregnancy (particularly progesterone) diagnostic. Medical treatment for the individual
have been associated with enhanced sympa- who is diagnosed with carcinoma of the lungs
thetic activity and the precipitation of dys- can include radiation and chemotherapy. In
rhythmias.50 Only very rarely is atrial fibrillation certain cases, surgical resection of the tumor and
or ventricular tachycardia the source of the possibly some of the surrounding tissues may be
dysrhythmia.51 Mechanical compression of the necessary. Physical therapists should construct
diaphragm by way of increasing fetal volume a therapeutic program to improve the individ-
compressing against the abdominal contents ual’s pulmonary function along with any possi-
and volume overload are other factors that ble complications due to the radiation or
may cause dyspnea in individuals with this chemotherapies. This may include decreased
condition. Patients who are pregnant and endurance and muscle weakness due to the side
report palpitations associated with symptoms effects of loss of appetite and weight loss
of cardiovascular instability should be referred resulting from the above treatments. Frequents
to their primary care physician for evaluation. rest breaks and interval training may be benefi-
In the event of cardiovascular compromise, cial for the individual. Following surgery, physi-
supportive interventions and activation of the cal therapists also should address movement
emergency medical system may necessary. dysfunction related to chest trauma.
■ Pulmonary Carcinoma
■ Pulmonary Embolus
Chief Clinical Characteristics
This presentation commonly includes persistent Chief Clinical Characteristics
dyspnea, cough, hemoptysis, chest discomfort, This presentation includes dyspnea, pleuritic
weight loss, and voice hoarseness. Individuals who chest pain, persistent cough, hemoptysis, and syn-
have bronchoalveolar cell carcinoma can produce cope.54 Individuals with a history of deep venous
copious amounts of thin secretions.52 thrombosis are susceptible. Other factors that
put an individual at risk include immobility
Background Information resulting in blood stasis, endothelial injuries
Cigarette smoking is the primary cause of due to lower extremity trauma or surgery,
carcinoma in the lung, and exposure to asbestos and hypercoagulable states arising from such
is the most common occupational exposure situations as oral contraceptive use or cancer.
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Chapter 37 Sarcoidosis 775

Background Information ■ Renal Failure

DYSPNEA
This condition is defined as a blood clot that has Chief Clinical Characteristics
become lodged in a pulmonary artery and This presentation can involve shortness of
significantly inhibits or ceases blood flow to the breath with exertion. Patients can also present
lung tissue. The obstruction is commonly a with abdominal distention and nausea.
blood clot, but this condition can also be caused
by air, fat, bone marrow, foreign intravenous Background Information
material, or tumor cells.19 Medical treatment This condition, which can be either acute
will depend on the severity of the blocked or chronic, is the result of a glomerular or
pulmonary artery. In an acute case of severe tubulointerstitial lesion.58 It is associated
disease, sudden death may occur. In the case of with increased serum creatinine and or urea
severe disease in which signs and symptoms levels. The seven primary characteristics of
appear significant and hemodynamic instability renal failure are azotemia, acidosis, hyper-
or respiratory compromise is evident, the physi- kalemia, abnormal fluid volume control,
cal therapist should activate the emergency hypocalcemia, anemia, and hypertension.58–60
medical system. In other cases where the The abnormal control of fluid volume results
signs and symptoms appear not as severe, the in a decreased ability to concentrate the
individual should be directed to an emergent urine. This progresses to the inability to
care facility. Medical treatment includes throm- dilute urine, resulting in retention of sodium
bolytic medications to dissolve the clot.54 and water. This retention can lead to conges-
tive heart failure, which is the primary cause
■ Pulmonary Hypertension of dyspnea in this patient population.58 Med-
Chief Clinical Characteristics ical treatment typically involves hemodialy-
This presentation commonly involves dyspnea sis. When treating these patients, a therapist
on exertion, fatigue, cough, wheezing, lower must monitor symptoms. If dyspnea worsens
extremity edema, coarse breath sounds, and with activities, the primary care physician
angina. must be contacted to rule out worsening
congestive heart failure.
Background Information
The symptoms of this condition arise from the ■ Sarcoidosis
fluid overload in the lungs and the resulting
Chief Clinical Characteristics
pulmonary congestion. Pulmonary hyperten-
This presentation includes persistent cough,
sion is classified as an elevation of the pul-
exertional dyspnea, and wheezing.
monary artery pressure above normal.55 The
two classifications of this condition are pri- Background Information
mary and secondary. The primary form of this This condition is an idiopathic systemic
condition is due to an unknown etiology.56 granulomatous disorder. The well-formed
Secondary disease has a known etiology and granulomas become inflamed and are usually
can include pulmonary vasoconstriction due near the connective tissue sheath of the
to scleroderma, Eisenmenger’s syndrome (a pulmonary vessels and bronchioles.61 This
right-to-left shunt in the cardiac septum), left- condition is the most common cause of
sided heart failure, or other causes. Pulmonary bilateral hilar adenopathy, or enlargement of
hypertension can only be confirmed via car- the lymph nodes at the hilum. The lungs are
diac catheterization. Medical treatment in- the most frequently affected organ system.
cludes prostacyclins, diuretics, cardiac glyco- Pulmonary function tests normally demon-
sides, supplemental oxygen,55 and vasodilators strate a restrictive disease pattern with lower
such as sildenafil.57 The physical therapist total lung capacity, functional residual
should be familiar with the individual’s med- capacity, residual volume, and diffusing
ical regime. Coordinating therapy during the capacity for carbon monoxide. This condi-
peak of medical intervention will help reduce tion is diagnosed by bronchoscopy and
the cough symptoms and help to prevent the transbronchial biopsies. The individual
individual from developing an exacerbation of with pulmonary sarcoidosis has a good
symptoms. chance of remission within 2 to 5 years from
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776 Chapter 37 Sickle Cell Disease/Crisis

diagnosis.61 Medical intervention usually swelling of the bronchial airways, increased


DYSPNEA

consists of inhaled corticosteroids or gluco- mucus production, and increased airway


corticoids62 in combination with supple- reactivity characteristic of chronic bronchitis
mental oxygen. No emergency intervention and chronic obstructive lung disease and the
is warranted. tissue destruction characteristic of emphy-
sema. Similar findings occur in those who
■ Sickle Cell Disease/Crisis are exposed to second-hand tobacco smoke.
Chief Clinical Characteristics The cough reflex for those exposed to first-
This presentation includes sudden, acute chest hand tobacco smoke becomes diminished.65
pain and shortness of breath in combination with In individuals who begin to demonstrate
acute abdominal and joint pain. emphysematous changes in the lungs from
first-hand tobacco smoke, cessation of
Background Information smoke exposure does not undo but stabilizes
This condition is the most common heredi- the changes that had occurred.66 The persist-
tary blood disorder.63 It most commonly ent cough therefore will not significantly
affects individuals of African indigenous dissipate. The physical therapist’s treatment
descent.63,64 It is also called hemolytic ane- aim would be to improve pulmonary function
mia due to the shortening of the life span through pulmonary clearance techniques,
of the red blood cell.63,64 Patients with this aerobic exercise, and individual education.
disorder experience what is called sickle cell For the individual exposed to second-hand
crisis. During such a crisis, the patient may tobacco smoke, encouragement to avoid
develop bone crisis that results in acute the source of the tobacco smoke, especially
sudden bone pain. Development of acute during exercise treatment, would be practical.
chest syndrome is possible. At the first For the individual exposed to first-hand
sign of this condition, physical therapy treat- smoke, the physical therapist should alert the
ment should discontinue and the patient individual to smoking cessation programs.
should be referred back to his or her pri-
mary physician for follow-up and additional ■ Transfusion Reaction
management. Chief Clinical Characteristics
This presentation involves dyspnea, skin rash,
■ Tobacco Smoke fever, back pain, or dizziness within 24 hours
Chief Clinical Characteristics after a transfusion.67
This presentation includes dyspnea, a persistent
and likely productive cough, wheezing, and Background Information
decreased oxygen saturation and the individual The three types of transfusion reactions are
may have a barrel-chest appearance. acute hemolytic reactions, immune-mediated
hemolytic reactions, and nonimmune hemolytic
Background Information reactions.67 Dyspnea related to this condition
The airway inflammation generally present usually resolves promptly without specific
in smokers causes the persistent cough, spu- treatment or complications. However, in the
tum production, and reactive airway disease presence of a serious anaphylactic response,
that result from inhalation of a noxious stim- this condition can become more serious,
ulant. For some time now tobacco smoke has leading to hemolysis, sepsis, and death.67 If
been linked to many symptoms and signs, an individual has received a transfusion in
particularly persistent cough. When airways the past 24 hours and presents with shortness
are exposed to smoke they become inflamed. of breath, wheezing, or hypotension, the
The short-term response (cough) to tobacco therapist must assume that a serious transfu-
smoke is a protective action. The cilia in the sion reaction is occurring. The therapist
airway are part of the defense mechanism should contact the primary care physician
of the lungs. These cilia get destroyed with as well as be prepared to activate the emer-
prolonged exposure to tobacco smoke. The gency medical system if the individual’s
long-term pathological consequences include symptoms worsen.
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Chapter 37 References 777

References 23. Cahalin LP, Ice RG, Irwin S. Program planning and

DYSPNEA
1. Atkinson TP, Kaliner MA. Anaphylaxis. Med Clin North implementation. In: Irwin S, ed. Cardiopulmonary Physical
Am. Jul 1992;76(4):841–855. Therapy. 3rd ed. St. Louis, MO: Mosby; 1995:142–184.
2. Ludwig H, Strasser K. Symptomatology of anemia. 24. Weitzenblum E. Chronic cor pulmonale. Heart. Feb
Semin Oncol. Apr 2001;28(2 suppl 8):7–14. 2003;89(2):225–230.
3. Widmaier EP, Raff H, Strang KT. Vander, Sherman, and 25. Hardy KR. Diving-related emergencies. Emerg Med Clin
Luciano’s Human Physiology: The Mechanisms of Body North Am. Feb 1997;15(1):223–240.
Function. 9th ed. New York, NY: McGraw-Hill; 2003. 26. Jerrard DA. Diving medicine. Emerg Med Clin North
4. Pujade-Lauraine E, Gascon P. The burden of anaemia in Am. May 1992;10(2):329–338.
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presentation, processes and outcomes of care for Clinical efficacy of sildenafil in primary pulmonary
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2002:370–379. New York, NY: W.B. Saunders; 1994.
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54. Lipson DA, Palevsky HI. Thromboembolic disease. In: 67. Capon SM, Goldfinger D. Acute hemolytic transfusion
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Belfus; 2002:237–247. ment. Transfusion. Jun 1995;35(6):513–520.
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CHAPTER 38
Edema
■ Marisa Perdomo, PT, DPT

Description of the Symptom of joint shape and definition, loss of muscle


strength, pain, and changes in skin color,
This chapter describes pathology that may texture, and temperature.2
lead to edema. Edema is enlargement of the
soft tissues secondary to excess water accu- Special Concerns
mulation. It can be classified as intracellular ■ Bilateral edema of the hands or feet; may or
or extracellular. Intracellular edema develops may not be progressive
when there is direct injury to tissues, causing ■ Sudden onset of edema without traumatic
inflammatory insult to the cell or alterations event
in cellular metabolism and resulting in an ■ Edema that occurs simultaneously with
increase in intracellular sodium and water. fever, sweats, and chills
Extracellular edema is an abnormal accumu- ■ Distal edema with reports of shortness of
lation of fluid and protein molecules in the breath either with exertion or at rest
intercellular spaces of tissues.1 Edema that ■ Calf pain and edema after trauma
develops in the arms or legs, which is the ■ Edematous body part with red streaks
focus of this chapter, is called peripheral ■ Edema involving the face or arm that is
edema. Peripheral edema may be due to present with discoloration of the chest,
either a systemic dysfunction such as cardiac arm, or face; loss of carotid pulses; dyspha-
or renal insufficiency or an obstruction such gia; wheezing; chest pain; headaches; dizzi-
as a blood clot or tumor. The development of ness; or orthopnea
edema may be associated with loss of skin ■ Total body edema or total quadrant
mobility, loss of joint range of motion, loss edema

CHAPTER PREVIEW: Conditions That May Lead to Edema

T Trauma
COMMON
Not applicable
UNCOMMON
Lymphedemas:
• Secondary lymphedema 790
Thoracic outlet syndrome 794
RARE
Not applicable
(continued)

779
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780 Chapter 38 Edema


EDEMA

I Inflammation
COMMON
Aseptic
Phlebitis 793
Septic
Cellulitis 784
Erysipelas (St. Elmo’s fire) 787
UNCOMMON
Not applicable
RARE
Aseptic
Angioedema 783
Lymphangiitis 789
Septic
Necrotizing fasciitis 792

M Metabolic
COMMON
Hypothyroidism 788
Medication-induced edemas:
• Adrenergic blockers 791
• Calcium channel blockers 791
• Corticosteroids 791
• Nonsteroidal anti-inflammatory drugs 791
• Oncologic agents 791
UNCOMMON
Ascites/cirrhosis of the liver 783
Hyperthyroidism/thyrotoxicosis (myxedema) 788
Lipedema 788
Nephrotic syndrome 792
Protein energy malnutrition (kwashiorkor) 793
RARE
Not applicable

Va Vascular
COMMON
Chronic venous insufficiency (varicose veins) 785
Congestive heart failure 787
Deep venous thrombosis 787
Post-thrombotic syndrome 793
UNCOMMON
Not applicable
RARE
Capillary leak syndrome 784
Superior vena cava syndrome 794
1528_Ch38_779-797 07/05/12 2:08 PM Page 781

Chapter 38 Edema 781

EDEMA
De Degenerative
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Not applicable

Tu Tumor
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Malignant Primary, such as:
• Lymphoma 795
Malignant Metastatic, such as:
• Metastases, including from primary breast, kidney, lung, prostate, and thyroid disease 795
Benign:
Not applicable

Co Congenital
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Lymphedemas:
• Primary lymphedema (Milroy’s disease, Meige disease) 789

Ne Neurogenic/Psychogenic
COMMON
Post-stroke hand edema 793
UNCOMMON
Complex regional pain syndrome (reflex sympathetic dystrophy, causalgia) 785
RARE
Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.
1528_Ch38_779-797 07/05/12 2:08 PM Page 782

782 Chapter 38 Edema

Overview of Edema cold depending on vascular involvement or the


EDEMA

presence of an infection. The individual may


Peripheral edema may be the first clinical sign report feeling pressure, tightness, fullness,
of systemic and peripheral disease states. Be- pain, tingling, numbness, heaviness, achiness
cause edema itself can cause body structure and hypersensitivity of the affected limb. The
and function deficits, this sign is common edema may be either painful or painless.
among individuals who present to physical Although peripheral edema has various
therapy with a broad variety of conditions. causes, the physiological mechanisms for the
The first task in providing treatment to an in- formation of edema are the result of alterations
dividual with edema is to determine the un- in Starling’s law of equilibrium.3–5 In general,
derlying cause of the swelling. Physical therapy the formation of peripheral edema is due to:
interventions may be contraindicated because ● an increase in capillary filtration rate that
treatment of the edema could potentially be exceeds the maximum lymphatic transport
life threatening. capacity;
The presentation of edema has some char-
● a decrease in venous capillary and lym-
acteristics that are uniform across all causes,
phatic resorption rate and a decrease in
and other features that differ across causes. In
lymphatic transport capacity; or
the early stages of edema formation, palpation
of the edematous body part causes pitting and ● a combination of both.6
the edema is soft. Over time, the edema may Accurate determination of the cause of
become harder to pit and may become fibrotic edema depends on an understanding of the
and brawny in nature. Skin color can vary from physiology governing fluid movement across
normal to pink (suggesting an inflammatory capillary beds. Filtration and reabsorption of
cause), red (suggesting an infectious cause), fluid depend on the difference between capil-
blue (suggesting a venous cause), or white lary blood pressure and extravascular hydro-
(suggesting an arterial cause). Individuals with static pressure, the difference between the
venous edema may develop hemosiderin capillary and extravascular colloid osmotic
stains, lipodermatosclerosis, eczema, and pressures, and the permeability of the blood
venous ulcers (Fig. 38-1). If infection is the capillary wall. The sum of these pressure
cause of edema, fever and flu-like symptoms gradients allows for fluid movement out of
may be present. Individuals with secondary the arterial capillary vessel (filtration) and
lymphedema may develop hyperkeratosis of reabsorption along the venous capillary vessel
the skin and papillomatosis (Fig. 38-2). If the and the initial lymphatic vessels. Protein
etiology of edema involves the cardiovascular molecules are impermeable to the venous
system, the individual also may report symp- capillary wall and must be reabsorbed by the
toms of fatigue, shortness of breath, orthop- lymphatic vessels, thereby preventing edema
nea, or a sensation of abdominal fullness. Skin formation. In a healthy lymphatic system, any
temperature can vary from normal to warm to increase in extracellular fluid will be removed
by the lymph and venous systems. However, if
the volume of interstitial fluid exceeds the
maximum lymphatic transport capacity, then
edema will develop. Over time, with a normal
lymphatic system, the lymphatic transport
capacity will “catch up” to the fluid overload
and the edema will dissipate.
One key to treatment of individuals with
edema is to determine whether the edema is a
result of overloading the lymphatic and venous
system with fluid or an impairment of venous
and lymphatic resorption and transportation.
For individuals with lymphatic and venous
FIGURE 38-1 Venous edema (lipodermatosclerosis). overloading, intervention is directed toward
1528_Ch38_779-797 07/05/12 2:08 PM Page 783

Chapter 38 Ascites/Cirrhosis of the Liver 783

EDEMA
Affected
upper
extremity

A B
FIGURE 38-2 Secondary lymphedema. Potential associated findings: (A) hyperkeratosis; (B) papillomatosis.
(Insets A and B from Goldsmith, LA, Lazarus, GS, Tharp, MD. Adult and Pediatric Dermatology: A Color Guide
to Diagnosis and Treatment. 1997. Philadelphia, FA Davis Company, with permission.)

reducing the quantity of interstitial fluid; for trauma; and reactions to food.11 There is also
individuals with limited resorption and trans- a hereditary form of this condition that
portation, intervention is based on methods to occurs in childhood and requires lifelong
improve pressure gradients in order to facilitate attention for possible reactions. This condi-
the resorption and transportation processes. tion is an immune-mediated disorder that is
present in up to 10% of the U.S. population.
Description of Conditions That It is caused by a temporary increase in capil-
May Lead to Edema lary permeability of small blood vessels.
Diagnosis is confirmed with a complete med-
■ Angioedema ical history and the clinical presentation of
Chief Clinical Characteristics the patient. Treatment includes medications
This presentation may include nonpitting such as antihistamines, steroids, and in severe
edema that most commonly affects the face, cases adrenaline.7 Physical therapy is not in-
hands, and neck; less commonly, the buttocks, dicated in patients with this condition. This
genitals, and abdominal organs may be affected. condition is a medical emergency when it
Symptoms may also include tingling, paresthe- affects the larynx, and it requires immediate
sias, or pruritus.7–10 medical intervention.
Background Information ■ Ascites/Cirrhosis of the Liver
Causes of angioedema include drug reac- Chief Clinical Characteristics
tions, most commonly to nonsteroidal anti- This presentation typically includes an ab-
inflammatory medications and angiotensin- domen that becomes distended or swollen. In se-
converting enzyme inhibitor medications; vere cases, the edema progresses and may include
1528_Ch38_779-797 07/05/12 2:08 PM Page 784

784 Chapter 38 Capillary Leak Syndrome

bilateral lower extremities. Individuals may can be acute or chronic in nature. In severe
EDEMA

report increased abdominal girth, respiratory cases, renal damage may occur and result in
distress, and satiety. hypovolemic shock. This condition is a diag-
nosis of exclusion. Also, a blood test should
Background Information
reveal the presence of serum IgG-K parapro-
This condition involves excess fluid accumula-
tein. While there is no standard successful
tion within the peritoneal cavity, and symp-
treatment approach, various pharmacologic
toms depend directly on the amount of fluid
treatments have demonstrated positive results,
in the cavity. This condition is a clinical find-
such as prednisone, furosemide, theophylline,
ing with a variety of causes. Asymptomatic
loop diuretics, and terbutaine.14 Medical
liver disease is one of the most common
management for the cause of capillary leak
causes. Liver disease develops as a result of
syndrome is necessary. Once the condition is
portal hypertension and hypoalbuminemia,
controlled, if there is any residual peripheral
which are often associated with patients with
edema, physical therapy may have a role in its
cirrhosis of the liver. Individuals with a history
treatment.
of alcohol abuse and advanced cancer are at
high risk for developing this condition. Ultra- ■ Cellulitis
sonography confirms the presence of fluid in
Chief Clinical Characteristics
the peritoneal cavity. If fluid presence is con-
This presentation may include erythema, pain
firmed, analysis of the fluid is necessary to dif-
and tenderness over the infected area with
ferentiate from other causes such as cancer,
associated edema, in association with possible
congestive heart failure, and tuberculosis.12
serous drainage, fever, chills, headaches, and
The ascetic fluid should be analyzed for
malaise. The skin becomes swollen and hot and
serum-ascetic albumin gradient, amylase con-
may develop an “orange peel” texture as this con-
centration, white cell count and red cell count,
dition progresses (Fig. 38-3). The individual
triglyceride concentration, Gram stain and
may present with an increased resting heart
culture, pH < 7, and cytology. Treatment de-
rate and may, in extreme or severe cases, even
pends on this condition’s cause. In the major-
become disoriented. The regional lymph nodes
ity of patients, cirrhosis leading to portal
of the involved body part may become enlarged,
hypertension is the major cause and is man-
tender or painful to palpation.15,16
aged with diuretic and dietary salt restric-
tion.12,13 Medications such as spironolactone Background Information
and a low-dose loop diuretic are commonly This condition refers to an acute inflammation
prescribed.13 However, when this condition of the dermis and subcutaneous tissue. It is one
is associated with cancer, the condition will
not respond to similar treatments. Physical
therapy is not indicated in patients with this
condition.
■ Capillary Leak Syndrome
Chief Clinical Characteristics
This presentation typically includes recurrent
episodes of peripheral edema and hypopro-
teinemia. Edema often includes both lower
extremities and may include the face, lungs,
and pericardium.14
Background Information
In this rare and complex condition, microvas-
cular damage causes an increase in capillary FIGURE 38-3 Cellulitis. (From Goldsmith, LA, Lazarus,
permeability and a rapid and sudden increase GS, Tharp, MD. Adult and Pediatric Dermatology:
in the capillary filtration rate. This condition A Color Guide to Diagnosis and Treatment. 1997.
was first noted in the literature in 1960 and Philadelphia, FA Davis Company, with permission.)
1528_Ch38_779-797 07/05/12 2:08 PM Page 785

Chapter 38 Complex Regional Pain Syndrome 785

of the most common bacterial skin infections perforating veins; obstruction; or both. As the

EDEMA
typically caused by Staphylococcus and Strepto- calf contracts, the increased pressure will be
coccus species.16,17 The infective agent may enter transferred to the superficial veins, resulting in
the body through any break or opening in the venous hypertension. Varicose veins are classi-
skin and spread via the lymphatic system. In fied into “grades” based on severity. Varicose
lower extremity cellulitis, pedal edema is an veins are described as “dilated palpable subcu-
early sign. Individuals who have peripheral taneous veins usually larger than 4 mm.” Cur-
edema from other etiologies (eg, chronic ve- rently two classification systems are used to
nous insufficiency and lymphedema) are at risk assess the clinical presentation of chronic
for developing cellulitis. A complete blood venous insufficiency: the CEAP classification
count that indicates an elevated white blood cell system and the Venous Clinical Severity Score
count confirms the diagnosis. Tissue culture is system (Table 38-1). These classification sys-
required to identify the exact infective agents tems attempt to incorporate the clinical signs
that are involved. This condition must particu- and symptoms with the results from duplex
larly be differentiated from other forms of in- ultrasonography to diagnose and classify the
fection, such as necrotizing fasciitis, folliculitis, severity of this condition. Interventions for
erysipelas, impetigo, and gas gangrene.18,19 both conditions include the recommendation
Treatment includes intravenous antibiotics. If of compression garments, exercise (eg, walking
the etiology of this condition involves systemic while wearing the garments and pool exer-
infection, this condition may become life cises), and elevation of the legs.20,21
threatening without proper treatment.18
■ Complex Regional Pain Syndrome
■ Chronic Venous Insufficiency (Reflex Sympathetic Dystrophy,
(Varicose Veins) Causalgia)
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes dilated subcuta- This presentation includes pain that is out of
neous veins, hyperpigmentation, edema, pain, proportion to the injury, burning pain, allody-
lipodermatosclerosis, and ulceration. This nia, hyperesthesias, vasomotor changes, swelling,
condition is sometimes equated with post- loss of joint range of motion, loss of skin mobil-
thrombotic syndrome.20 ity, and contractures. Edema that is associated
Background Information with this condition may be pitting or nonpitting.
This condition may be caused by incompe- It is usually present in the early stages and may
tent valves (reflux) in the superficial, deep, or decrease in the later stages.22

TABLE 38-1 ■ Criteria for the CEAP Classification and Venous Clinical Severity Score
CEAPa Classification
CATEGORY RECOMMENDED COURSE OF ACTION
CEAP I Superficial spider (reticular) No need to refer to a vascular specialist for this
veins only cosmetic problem
CEAP II Varicose veins Refer to vascular specialist for evaluation and
management, with level of urgency increasing
CEAP III Varicose veins and leg
from routine at level II to more urgent at level V
swelling
CEAP IV Varicose veins and evidence
of venous stasis skin changes
CEAP V Varicose veins with healed
cutaneous ulcer
CEAP VI Varicose veins with open Refer very urgently to a vascular specialist for
cutaneous ulcer evaluation and treatment
(continued)
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786 Chapter 38 Complex Regional Pain Syndrome


EDEMA

TABLE 38-1 ■ Criteria for the CEAP Classification and Venous Clinical Severity Score—cont’d
Venous Clinical Severity Score63
ABSENT (0) MILD (1) MODERATE (2) SEVERE (3)
Pain None Occasional, Daily, moderately Daily, severely limiting,
nonlimiting, limiting, requires requires regular use of
does not require occasional use of analgesic medication
analgesic analgesic medication
medication
Varicose None Few Multiple Extensive
veins
Venous None Evening ankle Afternoon edema, Morning edema above
edema edema only edema occurring the ankle requiring
above the ankle activity change and
elevation
Skin None Diffuse but Diffuse over the lower Wide distribution
pigmentation limited in area one-third of the calf (above the lower
and old (brown) (gaiter area) or recent one-third of the calf)
(purple) and recent
Inflammation None Mild cellulitis Moderate cellulitis of Severe cellulitis
limited to region the lower one-third affecting the region
around an ulcer of the calf above the lower
one-third of the calf,
venous eczema present
Induration None Focal, around Medial or lateral lower Entire lower one-third
the malleoli leg, but less than the of the lower leg or
(<5 mm) lower one-third of more
the leg
Number of 0 1 2 >2
active ulcers
Active ulcer None <3 months Greater than 3 months Not healed >1 year
duration but less than 1 year
Active ulcer None <2-cm diameter 2- to 6-cm diameter >6-cm diameter
size
Compression None or not Intermittent use Wears elastic stockings Full compliance
therapy compliant most days with stockings and
elevation
aCEAP is a mnemonic that stands for clinical, etiologic, anatomical, and pathophysiological. The CEAP classification has
been used to stratify subjects from large populations to ensure comparison of like individuals in clinical and research
applications. Since its inception for this purpose, the CEAP classification has been simplified to guide management
and referral decisions by clinicians.

Background Information symptoms. Diagnostic tests such as x-ray, bone


This condition is subdivided into two types: scans, and bone density tests are helpful to
complex regional pain syndrome 1 (previously confirm abnormalities and can support the
known as reflex sympathetic dystrophy), diagnosis. Physical therapy focuses on early
which develops after an initiating noxious mobilization of the affected joints and preven-
event without nerve damage, and complex tion of the sequelae of disuse, atrophy, and loss
regional pain syndrome 2 (previously known of function. However, a multidisciplinary treat-
as causalgia), which develops after injury to a ment approach is critical to minimize the de-
nerve. Diagnosis is based on clinical signs and bilitating consequences of this condition.22,23
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Chapter 38 Erysipelas (St. Elmo’s Fire) 787

■ Congestive Heart Failure Background Information

EDEMA
Chief Clinical Characteristics The clinical presentation of this condition
This presentation includes peripheral/pretibial in the upper extremity may include sudden
edema in association with shortness of breath, onset of upper extremity edema, pain, ten-
chest discomfort, or light-headedness/syncope. derness, venous distention, and skin color
Various physical findings, such as palpitations, changes of the arm, face, supraclavicular
pulmonary crackles, an auscultated S3 cardiac fossa, and shoulder.24 Risk factors for this
sound, cyanosis, and sudden and dramatic condition in the upper extremity include
weight gain, provide clues to the presence of use of central venous catheters such as in the
this condition. delivery of chemotherapy agents, congestive
heart failure, pregnancy, rigorous and repeti-
Background Information tive activity of the upper extremities, and
Electrolyte balance is typically upset in individ- venous compression as in thoracic outlet
uals with this condition, as a result of both syndrome (Paget-Schroetter syndrome).24
chronic fluid retention and the depletion of This condition should be suspected in the
certain electrolytes by various medications lower extremity in any individual who pres-
(eg, potassium-depleting diuretics). The use of ents with sudden onset of unilateral calf pain
digoxin to improve cardiac contractility in these and swelling. The diagnosis is confirmed
patients may in some cases precipitate brady- with Doppler ultrasound, magnetic reso-
cardia secondary to slowing of the electrical nance imaging, or computed tomography.
conduction through the atrioventricular node, D-dimer blood testing can be used as a
or tachycardia secondary to enhancement of method to rule out a DVT, thus negating the
automaticity in Purkinje fibers episodes. By need for further testing. However, because
communicating with the patient’s physician, other pathologies such as cancer, late preg-
the therapist plays a crucial role during titration nancy and sepsis produce elevated D-dimer
of the patient’s medical regimen. Definitive levels, it does not discriminate specifically
diagnosis is made by echocardiography or an- for a diagnosis of a DVT.25 Early diagnosis
giography. As with coronary artery disease, is important because of the potential risk
supplemental oxygen prescribed by the patient’s of a thrombus resulting in a pulmonary em-
physician can improve symptoms and suppress bolism or stroke. Treatment typically in-
the palpitations in some cases. However, if volves anticoagulants including heparin or
the patient becomes unstable, the emergency low-molecular-weight heparin to prevent
medical system should be activated. extension of the thrombus. Once sufficiently
anticoagulated, the patient is transitioned
■ Deep Venous Thrombosis to Coumadin. In addition, a compression
Chief Clinical Characteristics garment, usually a knee-high garment or
This presentation typically includes pain and arm sleeve, is recommended to assist in
swelling, warmth, skin color changes, a palpa- venous return and prevent recurrence. For
ble cord, and dilation of the superficial veins. this condition in the lower extremity, calf ex-
If the collateral veins are sufficient to allow for ercises and a walking program are important
venous drainage, there will be no edema. to assist in venous return and help prevent
General risk factors include a history of trauma, venous stasis.
history of a previous deep venous thrombosis
(DVT), a period of immobilization, recent ■ Erysipelas (St. Elmo’s Fire)
surgery, cancer, intravenous chemotherapy Chief Clinical Characteristics
treatment, indwelling central line, present oral This presentation can be characterized by ery-
contraceptive use, and medical comorbidities thema, pain and tenderness over the infected
such as congestive heart failure, stroke, pul- area with associated edema, in association
monary obstructive disease, obesity, or an inher- with possible serous drainage, fever, chills,
ited coagulopathy. With repeated thromboses or headaches, vomiting, and malaise. The skin
the presence of cardiovascular comorbidities, lesion associated with this condition involves
edema distal to the clot will be present. clear bright red patches of skin with definite
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788 Chapter 38 Hyperthyroidism/Thyrotoxicosis (Myxedema)

borders and raised margins as a distinct form disease (often associated with exophthal-
EDEMA

of cellulitis (see Fig. 38-3). mos), excessive thyroid hormone replacement


therapy, toxic adenoma, thyroiditis, goiter,
Background Information
amiodarone use, or use of iodine-containing
This commonly occurs in populations such as
radiographic contrast agents. Thyroid hor-
the elderly, neonates, immunocompromised
mones are known to enhance myocardial
individuals, and young children. Risk factors
contractility and elevate the body’s metabolic
include individuals with diabetes mellitus,
rate, leading to arterial vasodilation and
those with nephrotic syndrome, and those
possible hypotension. If this condition is
with a compromised immune system.26 Red
suspected, the individual should be referred
streaks (lymphatic streaking) may be present,
to a medical practitioner for definitive
indicating that the infection is spreading via
assessment. The diagnosis is made on the
the lymphatic vessels. Localized edema is pres-
basis of blood tests that indicate elevated
ent and the individual may report pain or local
thyroid hormone levels. Management of
tenderness.16,18,19 The most common location
this condition may include pharmacologic
is the legs and face. Erysipelas is a distinct type
administration of iodine, antithyroid med-
of superficial cellulitis and therefore involves
ications, and surgical thyroidectomy.
the epidermis. It can rapidly lead to a systemic
toxicity and therefore requires immediate re- ■ Hypothyroidism
ferral to a physician for individuals suspected
of having this condition.18,19 The medical di- Chief Clinical Characteristics
agnosis of erysipelas is usually based on clini- This presentation may be characterized by
cal presentation; however, diagnostic tests such severe fatigue in combination with edema of
as a complete blood count with differential, a the eyelids, face, and dorsum of the hand.
needle aspiration with culture, and a swab cul- Myxedema also may be present.
ture of the nose/throat may assist in differenti- Background Information
ating this pathology from other causes. Antibi- This condition develops as a result of de-
otics are required. If left untreated, this creased production or levels of T4 and T3
condition can lead to nephritis, abscesses, and hormones.3,29 This condition may be sus-
a life-threatening systemic infection called pected if an individual presents with pretibial
septicemia.16,18,19 edema and unusual fatigue that does not
■ Hyperthyroidism/Thyrotoxicosis improve with rest.29,30 Lab values for thyroid-
(Myxedema) stimulating hormone are elevated and can be
detected before abnormal plasma levels of T3
Chief Clinical Characteristics and T4 hormones are observed. Blood values
This presentation often includes pretibial edema for serum T3 and free T4 are elevated, while
and dorsal foot edema (pretibial myxedema), serum TSH is decreased and radioactive iodine
hypertension, dyspnea (ie, orthopnea, exer- uptake is increased. Thyroid hormone replace-
tional dyspnea, and paroxysmal nocturnal ment therapy is given for individuals with this
dyspnea), and palpitations that may lead to condition. In many cases, the thyroid gland is
feelings of dizziness or light-headedness.27 surgically removed and hormone replacement
Associated signs and symptoms include nervous- therapy is required.
ness, heat intolerance, fatigue, weight loss
despite increased appetite, goiter, sweating, ■ Lipedema
tremors, and exophthalmos.28
Chief Clinical Characteristics
Background Information This presentation involves gradual and
This condition is characterized by overactivity progressive increase in the size of bilateral
of the thyroid gland and primarily results lower extremities often mistaken for obesity,
in elevated levels of thyroid hormones in lymphedema, or venous dysfunction.31 Typi-
the bloodstream; thyrotoxicosis refers to the cally this condition begins with the leg edema
clinical syndrome resulting from hyperthy- beginning just above the malleoli. There is no
roidism. Typical etiologies include Graves’ involvement of the feet (negative Stemmer
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Chapter 38 Primary Lymphedema (Milroy’s Disease, Meige Disease) 789

sign). Palpation is tender and painful with swelling, and red streaks along the vessels that

EDEMA
a soft end feel, and no pitting is produced. drain to the lymph nodes.
Neither leg elevation nor diet and weight loss
decrease the diameter of the legs. Typically, Background Information
the individual’s history is unremarkable for This condition is an inflammation of lym-
cellulitis and leg ulcers. phatic vessels draining the body region that
has an infection or is inflamed.35 If this condi-
Background Information tion is suspected, referral to a physician is
This condition occurs in women and in some warranted. Treatment includes antibiotic
cases there are familiar tendencies. This condi- medications for the infection. Septicemia can
tion involves abnormal fat deposits in the develop if the underlying infection spreads
subcutaneous tissue, which cause abnormal into the venous system. Physical therapy is not
enlargement of the lower extremities, pelvis, indicated for this condition.
and buttock. The size and shape of the lower
extremities are disproportionately larger when LYMPHEDEMAS
compared to the upper extremities. There are ■ Primary Lymphedema (Milroy’s
cases of all four extremities being involved; Disease, Meige Disease)
however, the more common presentation in-
volves just the lower extremities. The majority Chief Clinical Characteristics
of individuals with this condition are often This presentation can involve a distal to
misdiagnosed with lymphedema, venous insuf- proximal progression of unilateral or bilat-
ficiency, or obesity or not diagnosed at all.3,32–34 eral extremity edema in an individual with
possible hypoparathyroidism, yellow nails,
Diagnostic tests such as magnetic resonance
ptosis, and webbing at the neck. 36 Most
imaging and computed tomography scans
demonstrate the presence of subcutaneous fat commonly, the lower extremities are in-
and can add in ruling out competing diagnosis volved; however, all four extremities may
such as obesity and lymphedema. Manual lym- be affected. Initially the edema is soft and pit-
phatic mobilizations, compression bandaging ting, but over time the edema will progress
with short-stretch bandages, and exercise can to become hard, brawny, and indurated, and
reduce the size of the limb significantly. pitting can become extremely difficult. The
Strength training is highly recommended. Indi- limb can become quite large with abnormal
viduals with this condition must wear com- deposition of fat cells, which leads to loss of
pression garments after the initial physical joint spaces and a columnar-like appear-
therapy treatment has ended. Individuals with ance. Skin changes such as papillomatosis
this condition can be instructed in a home pro- and hyperkeratosis and development of
gram of self-lymph drainage massage tech- lymphocytes can occur.
niques, self-bandaging, and a progressive Background Information
resistance training program. Maximal reduc- This condition develops as a result of a
tion of the extremities can take more than malformation of the lymph vessels or
1 year to achieve in some cases. In some indi- lymph nodes. This malformation occurred
viduals, lymphatic insufficiency develops over as the embryo was developing in utero. The
time and therefore the individual would be di- limb is at risk for developing cellulitis,
agnosed with lipedema and secondary lym- erysipelas, and fungal infections. The infec-
phedema. In this case, the key clinical symptom tions increase the lymphatic load and fur-
is edema of the feet and a positive Stemmer ther increase the edema. In severe cases,
sign. Physical therapy may be indicated to help elephantiasis may develop.36,37 Hypoplasia
address secondary lymphedema. is the most common cause of this condi-
tion. Several ages of onset are possible
■ Lymphangiitis including at or around the time of birth
Chief Clinical Characteristics (congenital lymphedema; Milroy’s disease);
This presentation typically includes high fever, at puberty, when it is called lymphedema
chills, swollen lymph nodes, and pain, warmth, praecox (Meige disease); and during
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790 Chapter 38 Secondary Lymphedema

adulthood (lymphedema tarde). This con- develops, the edema becomes harder to pit
EDEMA

dition remains a diagnosis of exclusion and palpation of the limb becomes hard
from all other causes of peripheral edema. and firm.
Lymphoscintigraphy, bioelectrical imped-
ance, magnetic resonance imaging, and Background Information
computed tomography can assist in deter- As the edema becomes indurated, the skin
mining the appropriate diagnosis. The can develop a “peau d’orange” appearance.
individual with lymphedema will require Over time hyperkeratosis and papillomato-
lifelong self-treatment and physical therapy sis may develop. The skin may begin to
intervention if the individual experiences break down and infections such as cellulitis
repeated infections or has difficulty in con- and erysipelas may occur. In lower extrem-
trolling acute exacerbations.5,33,38–42 The ity lymphedema, inability to pinch the skin
most commonly accepted treatment for at the base of the metatarsal heads (positive
lymphedema includes manual lymphatic Stemmer sign) is present.5,40,42 If the condi-
drainage techniques, use of short-stretch tion is untreated, repeated infections may
compression bandages or compression occur and the combination of obstruction
braces, exercise, and skin care to prevent and infection can lead to elephantiasis. The
infection. Once the limb size has been limb becomes grossly enlarged; hyperker-
maximally reduced, the individual is fitted atosis, papillomatosis, and the development
with an appropriate compression garment. of a lymphocele may occur and result in
Lymphedema requires lifelong care with severe disfigurement and disability. This
a home program and replacement of com- condition occurs as a result of abnormal
pression garments every 3 to 6 months. accumulation of protein molecules and
Referral to physical therapy is necessary if fluid in the interstitial spaces of the body
the individual experiences an exacerbation from an acquired impairment of the lym-
of the lymphedema. phatic system that affects reabsorption of
the interstitial fluid and transportation of
■ Secondary Lymphedema lymph (lymphatic drainage). Cancer sur-
Chief Clinical Characteristics gery and radiation therapy are considered
This presentation typically includes heavi- the most frequent cause of secondary lym-
ness, achiness, tightness, tingling and numb- phedema in the United States, while para-
ness of the involved extremity, and painless sitic infection is the most common primary
swelling of a limb that initially fluctuates cause worldwide. Diagnosis of lymphedema
with rest or elevation but with time does is primarily a diagnosis of exclusion. All
not dissipate. The clinical presentation of other causes of edema are ruled out before
secondary lymphedema is the same as pri- this diagnosis is reached. Lymphoscintigra-
mary lymphedema with typically only one phy, bioelectrical impedance, magnetic
limb affected. If the hand or foot is involved, resonance imaging, and computed tomog-
the individual will have difficulty making raphy can be used to assist in the diagnosis.
a fist or curling the toes. These concerns may Treatment of this condition begins first
worsen with activity and increase in heat and with treating any infections that might be
humidity or during changes in elevation. present. Treatment may include manual
The distal aspect of the limb (foot or hand) lymphatic drainage mobilizations followed
may be affected initially; however, in some by compression bandages with short-
cases the proximal part of the limb may swell stretch materials, joint range of motion,
first. The edema usually spreads distally proper skin care, and exercise. Patient edu-
to proximally if left untreated. Initially, cation regarding skin care, independent
pitting edema is present and palpation of the bandaging techniques, and lymph drainage
limb is soft. Chronic edema causes fibrosis massage techniques are critical in order for
to develop within the subcutaneous tissues, the individual to control the lymphedema.
which may lead to a loss of skin mobility The individual with this condition will
and decrease range of motion. As fibrosis require lifelong self-treatment and physical
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Chapter 38 Secondary Lymphedema 791

therapy intervention if the individual expe- their cardiologist before initiating treatment

EDEMA
riences repeated infections or has difficulty for peripheral edema.
in controlling acute exacerbations.5,33,38–42
CORTICOSTEROIDS
MEDICATION-INDUCED
These agents—including prednisone, dex-
EDEMAS
amethasone, and Decadron—are used for a
Chief Clinical Characteristics variety of pathologies including cancer and
Many medications cause varying degrees of autoimmune conditions and following
peripheral edema. transplant surgeries. These medications can
Background Information cause sodium and fluid retention and potas-
In these cases, the peripheral edema is not sium depletion.44 The individual will most
appropriate for physical therapy interven- likely present with systemic peripheral
tion; however, this condition may be mis- edema. Depending on the dose, the individ-
taken for forms of edema, such as secondary ual may present with “Cushing-like” features
lymphedema, that is appropriate for physi- of the face. Physical therapy is not indicated
cal therapy intervention. Any individual and referral to a physician is necessary.
who is suspected of having this condition NONSTEROIDAL
should be referred to a physician for addi- ANTI-INFLAMMATORY DRUGS
tional evaluation. Changing the dose and
type of medication, or providing additional The presentation of edema with this class of
supportive treatment as appropriate, com- medications involves peripheral edema that
monly address this condition. The following especially affects the bilateral feet and an-
list provides an example of some of the kles. These medications are very commonly
medications that have the potential to cause encountered in physical therapy practice
peripheral edema. because they are commonly prescribed for
individuals with pain. This class of medica-
ADRENERGIC BLOCKERS tions includes ibuprofen, naproxen, and
These medications may promote the adverse iodine.
effects of orthostatic hypotension, dizziness,
ONCOLOGIC AGENTS
syncope, and peripheral edema of bilateral
feet, ankles, and legs. Typically, these agents Tamoxifen (Nolvadex; hormone therapy) is
are used in patients with hypertension or a selective estrogen receptor that inhibits
those experiencing cardiac arrhythmias. the effects of estrogen by binding on the es-
Medications in this class include bretylium trogen receptor protein in cancer cells. It is
and guanadrel. most often used for patients with breast
cancer whose tumors are estrogen receptor
CALCIUM CHANNEL BLOCKERS positive. Generalized peripheral edema is
These medications may cause pitting edema occasionally reported.44 The clinical pres-
in bilateral feet and ankles. In some cases the entation is a generalized edema that forms
edema may progress proximally. Other ad- fairly uniformly throughout the upper and
verse effects such as orthostatic hypotension, lower extremities. Docetaxel (Taxotere) is a
dizziness, headache, and nausea may also be chemotherapy agent most commonly used
present. These classes of medications are to treat breast cancer and non–small-cell
used for individuals experiencing hyperten- lung cancer. Systemic edema is also referred
sion, angina pectoris, cardiac arrhythmias, to as fluid retention syndrome and is a
atrial flutter, and fibrillation. They include common occurrence.44 Individuals with
dihydropyridine, benzodiazepine, verapamil, edema associated with this medication are
and Calan. The severity of the edema varies given steroids before infusion of docetaxel
between each type of this class of medication in order to minimize the systemic edema.
taken.43 Any individual who presents with This condition should be carefully differ-
lower extremity swelling and is taking this entiated from secondary lymphedema.
class of medication should be evaluated by Doxorubicin is a chemotherapy agent
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792 Chapter 38 Necrotizing Fasciitis

most commonly used in the treatment of Background Information


EDEMA

breast, bladder, liver, stomach, and lung This condition is a life-threatening infection
cancer, Hodgkin’s and non-Hodgkin’s lym- of the fascia (plus the underlying skin
phomas, leukemia, and bone and soft- and subcutaneous tissue) characterized by
tissue sarcomas.44 Congestive heart failure acute onset and rapid progression. A biopsy
is a potential serious adverse effect; there- or examination during surgery is required in
fore, individuals receiving doxorubicin order to accurately diagnose necrotizing
who develop any symptoms of bilateral fasciitis. Magnetic resonance imaging may
peripheral edema with or without short- be used to assist the physician in differentiat-
ness of breath should be referred to their ing necrotizing fasciitis from cellulitis.17
oncologist for cardiac evaluation. With Immediate medical attention is critical;
greater heart damage the lower extremity treatment includes surgical debridement,
edema becomes more proximal and may intravenous antibiotics, and meticulous
progress to involve the trunk and upper wound care.
extremities. Individuals treated for a child-
hood cancer are at significantly higher risk
■ Nephrotic Syndrome
for developing cardiac complications such Chief Clinical Characteristics
as congestive heart failure. This risk may be This presentation may involve bilateral lower
further increased if radiation therapy was extremity pitting edema, specifically the
applied to the thorax. Therefore, if an indi- ankles, lower extremities, and sometimes the
vidual with a history of a childhood cancer abdomen, eyes, and eyelids, in association
(such as leukemia or lymphoma) presents with ascites, pleural effusions, and pericardi-
to physical therapy with a diagnosis of tis. Individuals with this condition also may
bilateral lower extremity edema, the thera- present with shortness of breath (pulmonary
pist must consult with the physician to rule edema), and males may also present with
out any cardiac involvement.45,46 Individu- edema of the scrotum and penis. In children
als being treated for cancer are also at risk for the clinical presentation can also include
developing lymphedema secondary to lymph facial edema and periorbital edema.47
node dissection and radiation therapy. The Background Information
physical therapist should be aware of the Individuals with nephrotic syndrome de-
differences in clinical presentation of some- velop peripheral edema as a result of signifi-
one with peripheral edema caused by med- cant loss of plasma protein molecules from
ications versus someone who is at risk for the glomerular capillaries that are excreted
developing lymphedema (systemic edema via urine (proteinuria). The result is a
with increase in fluid retention in all four decrease in plasma protein concentration
extremities versus a single limb swelling). (hypoalbuminemia) and an increase in pro-
tein concentration in the urine. In addition,
■ Necrotizing Fasciitis sodium accumulates in the extracellular
Chief Clinical Characteristics tissue spaces as a result of an imbalance
This presentation commonly involves ery- between sodium intake and sodium output.
thema and edema without demarcated borders; There are several causes of nephrotic syn-
severe pain (a diagnostic symptom different drome, including cancer, drugs such as
from cellulitis); tense shiny skin, progressing heroin, gold and captopril, systemic lupus
to ischemic skin as tissue necrosis advances; erythematosus, diabetes mellitus, and a vari-
clear or hemorrhagic blisters, progressing ety of glomerular diseases. Tests include a
to bullae filled with gray odiferous fluid urinalysis, complete blood count, imaging,
(termed dishwater pus); dry black eschar (in and a kidney biopsy, although there is no
advanced disease); separation of necrotic gold standard test to confirm the diagno-
tissue along fascial planes with myonecrosis; sis.12,48,49 Diuretics are usually the first med-
high fever and chills; decreased urinary ication provided to prevent renal sodium
output; change in mental status; and weakness retention, and amiloride and furosemide aid
and fatigue. in decreasing the peripheral edema.50
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Chapter 38 Protein Energy Malnutrition (Kwashiorkor) 793

■ Phlebitis ■ Post-Thrombotic Syndrome

EDEMA
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation can be characterized by This presentation typically includes pain, heav-
edema (inflammation) distal to the injured iness, and cramping, which improve with leg
vein, pain, and tenderness and redness of the elevation in combination with clinical signs
tissue surrounding the vein. of edema, skin changes, hyperpigmentation,
induration, redness, venous ectasia, lipoder-
Background Information
matosclerosis, and a healed or active ulcer in
This condition refers to inflammation of a
an individual with history of deep venous
vein. There are many causes, but the most
thrombosis.20
common include thrombosis, chemical irri-
tants, malignancy, indwelling catheters, and Background Information
infection. Duplex ultrasonography is neces- Risk factors include age, family history, pro-
sary if a larger and deeper vein is involved, longed standing, history of a blood clot,
whereas venography typically suffices for phlebitis, smoking, and obesity. This condition
smaller more distal venules. If this condition is is a chronic venous insufficiency caused by a
suspected, the affected body part (more com- deep venous thrombosis. This syndrome is
monly the lower leg) should be elevated. The caused by a combination of events involving
underlying cause of this condition must be the thrombus, the inflammatory response, and
addressed. Treatment depends on the underly- the recanalization of the vein. These events can
ing cause, and may involve anticoagulant, an- damage the venous valves and result in valvu-
tibiotic, or thrombolytic agents.35 lar incompetence leading to venous hyperten-
sion, edema, tissue hypoxia, and in severe cases
■ Post-Stroke Hand Edema venous ulcers.53,54 Doppler ultrasound may
Chief Clinical Characteristics confirm the presence of deep venous throm-
This presentation involves edema of the hand bosis and identify incompetent valves. How-
and wrist area that can cause pain, joint stiff- ever, clinical symptoms must also be present to
ness, loss of range of motion, shortening of con- confirm the diagnosis of this condition.53,54
nective tissue, and adhesions51 in association Although studies are limited, evidence sup-
with paralysis and dependency. ports a reduction in edema related to this con-
dition in individuals who wore a compression
Background Information
garment.20
Approximately one-half of cases of this condi-
tion are associated with shoulder-hand syn- ■ Protein Energy Malnutrition
drome.52 Hand edema in this condition that is (Kwashiorkor)
also associated with shoulder-hand syndrome
Chief Clinical Characteristics
overlies the metacarpals and is accompanied
This presentation is characterized by edema,
by relatively little pain or tenderness. Assess-
anorexia, general loss of interest in surround-
ment of edema is difficult and may be missed
ings, and irritability. It most typically affects
if based solely on observation51; for hand
children between the ages of 1 and 3 who have
edema, volumetric measurements appear to be
sustained a prolonged dietary protein deficit.
most accurate. Swelling and hand edema are
Edema is typically present about the abdom-
common among individuals following stroke
inal region and face.
in the early stages of clinical rehabilitation,
even in individuals with good hand function. Background Information
Hand edema is most common among patients This condition is thought to be caused by
with severe paresis of the hand, hypertonic fin- limitation in protein, micronutrient, and
gers, and hyposensibility. Treatment involves a antioxidant content in the diet. However,
variety of modalities, such as compression predictors for the development of kwashior-
therapy, elevation, continuous passive motion, kor versus related conditions that do not
massage, and splinting. However, present involve edema (ie, marasmus and marasmus
research has yet to demonstrate an optimal kwashiorkor) remain unclear. Although
intervention for this condition.51,52 this condition is classically associated with
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794 Chapter 38 Superior Vena Cava Syndrome

children in developing countries during pain in the neck and shoulder region that
EDEMA

times of famine, it is also present in devel- seems to increase at night, easily fatigued arms
oped nations. In developed nations, children and hands, superficial vein distention in the
at particular risk include those living in large hand, paresthesias along the inside forearm and
urban areas within low socioeconomic groups the palm, muscle weakness with difficulty grip-
that are without the means to ensure proper ping and performing fine motor tasks of the
nutrition or dietary education. Treatment hand, atrophy of the muscles of the palm,
involves correction of dietary insufficiency. cramps of the muscles on the inner forearm,
Prognosis for recovery is good, although pain in the arm and hand, and tingling and
delayed treatment may result in persistent numbness in the neck, shoulder region, arm,
disruption of growth and development.55 and hand.
■ Superior Vena Cava Syndrome Background Information
Diagnosing this condition can be controver-
Chief Clinical Characteristics sial because disagreement exists about the
This presentation involves facial, neck, and arm “types” of thoracic outlet syndrome (TOS).
swelling, distention of the veins of the neck and In general, three common types of this condi-
arms, cyanosis of the chest, arms, and face, loss tion are presented in the literature, which can
of venous neck pulses, dyspnea, dysphasia, coexist or occur independently: compression
wheezing, coughing, chest pain, headaches, of the subclavian vein, compression of the
dizziness, orthopnea, and syncope. Upon aus- subclavian artery, and a primary neurological
cultation, diminished breath sounds may be syndrome. There is also a subset of neurolog-
present. ical TOS referred to as “disputed” neurogenic
Background Information type of TOS. This type presents clinically
This condition occurs as a result of obstruc- with primarily sensory symptoms and does
tion of the venous drainage of the upper body not typically present with definitive objective
and an increase in central venous pressure. findings.61 Multiple anatomical anomalies
Malignant tumors are responsible for about can lead to thoracic outlet syndrome, includ-
90% of the syndrome and the rest are caused ing an incomplete cervical rib, a taut fibrous
by aortic aneurysms, fibrotic mediastinitis, band passing from the transverse process of
and tuberculosis.56,57 This condition is most C7 to the first rib, and a complete rib that
commonly caused by lung cancer, followed articulates with the first rib, or anomalies of
by non-Hodgkin’s lymphoma and is rare in the position insertion of the anterior and
other cancers such as Hodgkin’s lymphoma, medial scalene muscles and the pectoralis
acute lymphoblastic leukemia, thyroid can- minor tendon.61,62 Traditionally diagnosis in-
cer, neuroblastoma, rhabdomyosarcoma, cludes physical examination tests (ie, Adson’s
Ewing’s sarcoma, breast cancer, non–small-cell test, extremity abducted stress test, costoclav-
lung cancer, and germ cell tumors. The in- icular sign, Roos test, Eden maneuver, and the
creased use of indwelling central venous Halstead maneuver), radiology of the cervical
catheters to administer chemotherapy agents spine, magnetic resonance imaging, and
may cause a thrombosis to develop and cause nerve conduction and electromyography
this condition.56–60 Diagnosis includes a studies. Unfortunately, Adson’s test is not a
detailed physical examination, blood work, reliable test to rule in or rule out a diagnosis
biopsy, and chest radiograph. The best treat- of TOS.62 Clinical neural tissue provocation
ment is to remove the cause or decrease the testing may provide support for a diagnosis of
size of the obstruction. neurogenic TOS.62 Nonsurgical approaches
to treatment include manual therapy such as
■ Thoracic Outlet Syndrome joint mobilizations, first rib mobilizations,
Chief Clinical Characteristics exercise, stretches, modalities, and analgesic
This presentation can be characterized by medication. The hand or finger edema can
swelling or puffiness in the arm or hand, bluish dissipate with the preceding physical therapy
discoloration of the hand, a feeling of heaviness interventions. Surgery is indicated if pain is
in the arm or hand, deep, boring toothache-like persistent and severe neurogenic or vascular
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Chapter 38 Metastases, Including From Primary Breast, Kidney, Lung, Prostate 795

features of the syndrome exist. Prognosis for joint range of motion, and venous distention.

EDEMA
decreased pain and improved function is Rarely is peripheral edema the only symp-
good for the majority of individuals with this tom of this condition. However, it may be the
condition. symptom that brings the individual to the
physician.
TUMORS
Background Information
■ Lymphoma This condition is caused by obstruction of
Chief Clinical Characteristics lymph nodes due to a malignancy. As this
This presentation commonly includes lymph condition progresses and lymph nodes be-
node tenderness and swelling, weight loss, come more obstructed, swelling increases
night sweats, fever, and fatigue in associa- and the tissue becomes fibrotic, indurated,
tion with possible edema and pruritus. Bone and difficult to induce pitting. Upon further
pain, shortness of breath, cough, and abdom- questioning, the individual may report fa-
inal pain are possible depending on the dom- tigue that is usually not improved with rest
inant sites of involvement. Clinical features and progressively worsens over time. Diag-
may wax and wane. nosis includes magnetic resonance imaging,
computed tomography, bone scan, ultra-
Background Information sound, complete blood count, and surgical
This condition refers to a whole host of ma- biopsy as needed to identify the source of
lignancies affecting B and T cells. Broadly, primary disease. Treatment of the underly-
this condition is divided among Hodgkin’s ing tumor is the primary intervention for
and non-Hodgkin’s lymphomas. Hodgkin’s this condition. If the peripheral edema per-
lymphomas are a group of five separate sists even though the tumor has shrunk or
conditions that arise from a specific B-cell has disappeared, then the individual may be
abnormality, whereas non-Hodgkin’s lym- diagnosed with secondary lymphedema and
phomas number approximately 30. A com- physical therapy may provide the appropri-
bination of genetic and environmental fac- ate intervention.
tors has been implicated in the development
of this condition. Environmental factors in- References
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EDEMA

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Hemost. Feb 2002;28(1):89–99. J Clin Oncol. Apr 20 2005;23(12):2629–2636.
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16(3):123–127. 1997;59:S21–27.
27. Aronow WS. The heart and thyroid disease. Clin Geriatr 48. Andreoli TE. Edematous states: an overview. Kidney Int
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28. Wilkins LW. Professional Guide to Signs and Symptoms. 49. Vande Walle JG, Donckerwolcke RA. Pathogenesis of
4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; edema formation in the nephrotic syndrome. Pediatr
2003. Nephrol. Mar 2001;16(3):283–293.
29. Hierholzer K, Finke R. Myxedema. Kidney Int Suppl. Jun 50. Deschenes G, Feraille E, Doucet A. Mechanisms of
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30. Lindsay RS, Toft AD. Hypothyroidism. Lancet. Feb 8 ideas. Nephrol Dial Transplant. Mar 2003;18(3):454–456.
1997;349(9049):413–417. 51. Post MW, Visser-Meily JM, Boomkamp-Koppen HG,
31. Harwood CA, Bull RH, Evans J, Mortimer PS. Lym- Prevo AJ. Assessment of oedema in stroke patients:
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32. Koss T, Lanatra N, Stiller MJ, Grossman ME. An unusual 1265–1270.
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33. Tiwari A, Cheng KS, Button M, Myint F, Hamilton G. stroke hand oedema and shoulder-hand syndrome.
Differential diagnosis, investigation, and current treatment Scand J Rehabil Med. Mar 2000;32(1):4–10.
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EDEMA
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Arch Intern Med. Jan 12 2004;164(1):17–26. 2002;69(3):209–210, 213–214, 216–217 passim.
54. Sieggreen M. Venous disorders: overview of current 60. Thirlwell C, Brock CS. Emergencies in oncology. Clin
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55. Ahmed T, Rahman S, Cravioto A. Oedematous malnu- 61. Campbell WW, Landau ME. Controversial entrapment
trition. Indian J Med Res. Nov 2009;130(5):651–654. neuropathies. Neurosurg Clin N Am. Oct 2008;19(4):
56. Van Putten JW, Schlosser NJ, Vujaskovic Z, Leest AH, 597–608, vi–vii.
Groen HJ. Superior vena cava obstruction caused by 62. Sanders RJ, Hammond SL, Rao NM. Diagnosis of tho-
radiation induced venous fibrosis. Thorax. Mar 2000; racic outlet syndrome. J Vasc Surg. Sep 2007;46(3):
55(3):245–246. 601–604.
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Med Clin North Am. May 2009;27(2):243–255. Meissner MH, Moneta GL. Venous severity scoring: An
58. Brigden ML. Hematologic and oncologic emergencies. adjunct to venous outcome assessment. J Vasc Surg. Jun
Doing the most good in the least time. Postgrad Med. 2000;31(6):1307–1312.
Mar 2001;109(3):143–146, 151–154, 157–158 passim.
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CHAPTER39
Case Demonstration:
Edema and Shoulder Pain
■ Marisa Perdomo, PT, DPT ■ Chris A. Sebelski, PT, DPT, OCS, CSCS

NOTE: This case demonstration was developed specializes in pain management with a referral
using the diagnostic process described in diagnosis of “left shoulder pain, decreased
Chapter 4 and demonstrated in Chapter 5. The range of motion, and lymphedema.” Her chief
reader is encouraged to use this diagnostic concern is further loss of motion and increasing
process in order to ensure thorough clinical rea- pain, which she describes as “dull, achy upper
soning. If additional elaboration is required on arm pain” that radiates down to the left elbow
the information presented in this chapter, please and occasionally radiates up to the left side of
consult Chapters 4 and 5. her neck. She reports skin and muscle tightness
of the anterior chest region, which prevents her
THE DIAGNOSTIC PROCESS from moving her arm above her head. Three
cortisone injections within the past 3 weeks did
Step 1 Identify the patient’s chief concern.
not change her pain concerns or limitations.
Step 2 Identify barriers to communication.
Her physician advised her to rest, but this did
Step 3 Identify special concerns.
not seem to help the pain. Additionally, she re-
Step 4 Create a symptom timeline and sketch
ports occasional lower back pain and left hip
the anatomy (if needed).
pain with symptoms of general fatigue. She
Step 5 Create a diagnostic hypothesis list
denies feeling more ill than usual lately.
considering all possible forms of remote and
SR was diagnosed with stage IIIC left invasive
local pathology that could cause the
lobular carcinoma approximately 10 months
patient’s chief concern.
prior to this physical therapy consult. Medical
Step 6 Sort the diagnostic hypothesis list by
treatment included a left modified radical
epidemiology and specific case characteristics.
mastectomy with axillary lymph node dissec-
Step 7 Ask specific questions to rule specific
tion followed by Arimidex (hormonal ther-
conditions or pathological categories less
apy) and 5 to 6 weeks of radiation therapy. The
likely.
radiated field included the left chest wall,
Step 8 Re-sort the diagnostic hypothesis list
axilla, and supraclavicular fossa. The onset of
based on the patient’s responses to specific
left shoulder pain began during the fourth
questioning.
week of radiation therapy. Present cancer
Step 9 Perform tests to differentiate among
treatment includes only Arimidex therapy, and
the remaining diagnostic hypotheses.
she has taken an antihypertensive medication
Step 10 Re-sort the diagnostic hypothesis list
“for years.” SR began physical therapy and
based on the patient’s responses to specific
occupational therapy toward the end of her
tests.
radiation treatments.
Step 11 Decide on a diagnostic impression.
Interventions to date included lymphedema
Step 12 Determine the appropriate patient
management such as manual lymphatic
disposition.
drainage, compression bandaging, light exer-
cises, and issuance of a compression garment.
Case Description Physical therapy interventions included soft
tissue mobilizations and general stretching
Mrs. SR is a 79-year-old Asian female referred and range-of-motion exercise for the left shoul-
to physical therapy by an anesthesiologist who der. Concurrently, she received acupuncture

798
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Chapter 39 Case Demonstration: Edema and Shoulder Pain 799

treatments. Unfortunately, she reported only


sternum (internal mammary lymph nodes) or
slight improvement in her left shoulder symp-
inferior clavicular lymph node involvement.
toms though she did notice a decrease in the
This information further increases the possi-
cording of her axilla.
bility of metastatic disease. A phone call to
Her husband of 50 years died of metasta-
her oncologist and referring physician is indi-
tic cancer 1 year prior to her diagnosis. Prior
cated to obtain additional detailed back-
to her cancer diagnosis, SR volunteered one
ground information, such as:
afternoon per week at a local cancer center.
● Was chemotherapy offered? Was chemother-
However, since completing radiation therapy apy refused? (options: doxorubicin, cyclophos-
she reports that she is very tired and has diffi- phamide, paclitaxel, with or without hormone
culty volunteering. SR reports that she is “out therapy/Herceptin, and Arimidex)
of shape” because she is now unable to per- ● How many lymph nodes were positive?
form a daily 30-minute walking routine. She ● Any present metastases?
lives in a large two-story home and her grown ● What imaging tests were performed and what
children are urging her to sell the home be- were the results?
cause it is too large for her to independently
manage.

STEP #1: Identify the patient’s chief


concern.
Teaching Comments:
● Left shoulder loss of motion, increasing
T: size and location of the tumor
pain in the left shoulder with occasional
N: number of regional lymph nodes involved lower back pain, left hip pain, and fatigue
M: presence of metastases
STEP #2: Identify barriers to
In operable stage IIIC, the cancer (1) is
communication.
found in 10 or more axillary lymph nodes, or
● Patient hesitant to discuss cancer history.
(2) is found in the lymph nodes below the col-
larbone, or (3) is found in axillary lymph nodes Her nonverbal behavior when answering
and in lymph nodes near the breastbone. In in- questions regarding her breast cancer diag-
operable stage IIIC, the cancer has spread to nosis demonstrated that the patient was not
the lymph nodes above the collarbone. comfortable discussing the medical treat-
Staging a malignant tumor is part of the ment and prognosis.
medical diagnostic process and assists in STEP #3: Identify special concerns.
determining the appropriate medical treat- ● Prior diagnosis of stage IIIC carcinoma.
ment as well as the long-term prognosis. In Increases the likelihood that metastatic
this case, the knowledge of a “stage IIIC” disease is responsible for the patient’s
breast cancer will guide the therapist’s differ- symptoms.
ential diagnosis list. According to the National ● Presentation of pain. Lack of effect of rest
Cancer Institute, stage IIIC breast cancer is de- on the patient’s symptoms should increase
fined as the following: “there may be no the index of clinical suspicion for metastatic
tumor found in the breast or the tumor may be disease.
any size and may have spread to the chest
● Report of concurrent fatigue and pain.
wall and/or the skin of the breast. Cancer has
Fatigue combined with daily reports of left
spread to lymph nodes above or below the
shoulder pain (joint pain) and the new on-
collarbone and may have spread to axillary
set of occasional hip pain is significant due
lymph nodes or to lymph nodes near the
to her cancer history.1 Contextually, this
breastbone. Therefore, the interpretation of
combination of symptoms can be inter-
the information thus far by a physical therapist
preted several ways:
may be that SR has operable stage IIIC with
1. The fatigue is expected due to the age of
significant axillary node involvement and
the patient and the traditional recovery
probable lymph node involvement near the
from cancer therapy.
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800 Chapter 39 Case Demonstration: Edema and Shoulder Pain

2. The combination of fatigue + joint pain


Teaching Comments: Range-of-motion
raises the possibility of metastatic disease
loss and chest wall flexibility/elasticity issues
as a potential cause of this patient’s
following the termination of radiation therapy
symptoms.
are expected. Due to the inflammatory process
3. Consideration of the patient’s recent
initiated by radiation, the stimulation of
social history makes it seem completely
fibroblasts to produce collagen can last for up
plausible that “these life events” resulted
to 1 year after radiation therapy.2 Therefore, it
in depression-related fatigue.
is expected that if an individual does not ac-
tively maintain joint range of motion and soft
tissue pliability following radiation, the tissues
may continue to contract and result in greater
loss of range of motion 6 months to 1 year
after treatment than previously experienced
immediately at the end of radiation therapy.3,4

STEP #4: Create a symptom timeline and


sketch the anatomy (if needed).
1 1
10 9 /2 9 8 /2 8 3 1
months months months months months months month
ago ago ago ago ago ago ago Today

Diagnosed Arimidex Rehabilitation New referral


with breast and radiation and acupuncture to physical
cancer begin begin therapy

Mastectomy Upper extremity Upper extremity Initial


symptoms begin pain worsens physical
Hip pain begins therapy
Referral to pain evaluation
management

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.
Causes of Edema Causes of Edema
T Trauma T Trauma
Secondary lymphedemas: Secondary lymphedemas:
• Radiation-induced • Radiation-induced
• Surgical lymph node dissection • Surgical lymph node dissection
Thoracic outlet syndrome Thoracic outlet syndrome (no symptoms
distal to elbow)
I Inflammation I Inflammation
Aseptic Aseptic
Angioedema Angioedema (region of symptoms, patient age)
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Chapter 39 Case Demonstration: Edema and Shoulder Pain 801

Lymphangiitis Lymphangiitis (denies recent illness)


Phlebitis Phlebitis (no history of recent trauma, less
typical in the upper extremity)
Septic Septic
Cellulitis Cellulitis
Erysipelas Erysipelas
Necrotizing fasciitis Necrotizing fasciitis (no recent illness)
M Metabolic M Metabolic
Ascites/cirrhosis of the liver Ascites/cirrhosis of the liver (less typical of
the upper extremity)
Hyperthyroidism Hyperthyroidism
Hypothyroidism Hypothyroidism (not typical of the upper
extremities)
Lipedema Lipedema (not typical of the upper
extremities)
Medication-induced edemas: Medication-induced edemas:
• Angiotensin-converting enzyme inhibitors • Angiotensin-converting enzyme inhibitors
(patient not taking this medication)
• Antihypertensives • Antihypertensives (has been taking
medications for many years without
symptoms)
• Calcium channel blockers • Calcium channel blockers (patient not
taking this medication)
• Chemotherapy agents • Chemotherapy agents
• Corticosteroids • Corticosteroids (patient not taking this
medication)
• Nonsteroidal anti-inflammatories • Nonsteroidal anti-inflammatories (patient
not taking this medication)
• Selective and nonselective • Selective and nonselective
cyclooxygenase-2 inhibitors cyclooxygenase-2 inhibitors (patient not
taking this medication)
Myxedema Myxedema (not typical of the upper
extremities)
Nephrotic syndrome Nephrotic syndrome (not typical of the
upper extremities)
Va Vascular Va Vascular
Capillary leak syndrome Capillary leak syndrome (not typical of the
upper extremity)
Cardiomyopathy Cardiomyopathy (involvement of one
extremity uncommon)
Chronic venous insufficiency Chronic venous insufficiency (not typical of
the upper extremities)
Congestive heart failure Congestive heart failure (involvement of
one extremity uncommon)
Deep venous thrombosis Deep venous thrombosis
Peripheral vascular disease Peripheral vascular disease (unilateral
involvement of the upper extremity less
common)
Post-thrombotic syndrome Post-thrombotic syndrome (not typical of
the upper extremities)
Superior vena cava syndrome Superior vena cava syndrome
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802 Chapter 39 Case Demonstration: Edema and Shoulder Pain

De Degenerative De Degenerative
Not applicable Not applicable
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Lymphoma • Lymphoma
Malignant Metastatic, such as: Malignant Metastatic, such as:
• Metastases, including from primary • Metastases, including from primary
breast, kidney, lung, prostate, and thyroid breast, kidney, lung, prostate, and thyroid
disease disease
Benign: Benign:
Not applicable Not applicable
Co Congenital Co Congenital
Primary lymphatic malformation: Primary lymphatic malformation:
• Milroy’s disease • Milroy’s disease (patient age, less typical
of the upper extremities in isolation)
• Meige disease • Meige disease (patient age, less typical of
the upper extremities in isolation)
Non-Milroy’s disease Non-Milroy’s disease (patient age)
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Complex regional pain syndrome Complex regional pain syndrome
Post-stroke hand edema Post-stroke hand edema (no recent history
of stroke)
Remote Remote
T Trauma T Trauma
Cervical disk herniation Cervical disk herniation
Internal organ injuries: Internal organ injuries:
• Diaphragm • Diaphragm (no history of sufficient
trauma)
• Liver • Liver (no history of sufficient trauma)
• Lung • Lung (no history of sufficient trauma)
• Spleen • Spleen (no history of sufficient trauma)
Status postlaparoscopic procedure Status postlaparoscopic procedure (no
recent history of laparoscopy)
Thoracic outlet syndrome Thoracic outlet syndrome
I Inflammation I Inflammation
Aseptic Aseptic
Acute cholecystitis Acute cholecystitis (limited shoulder range
of motion)
Costochondritis (Tietze’s syndrome) Costochondritis (Tietze’s syndrome) (no
chest pain)
Gaseous distention of the stomach Gaseous distention of the stomach (limited
shoulder range of motion)
Inflammatory bowel diseases: Inflammatory bowel diseases:
• Crohn’s disease • Crohn’s disease (no abdominal pain,
limited shoulder range of motion)
• Ulcerative colitis • Ulcerative colitis (no abdominal pain,
limited shoulder range of motion)
Rheumatoid arthritis–like diseases of the Rheumatoid arthritis–like diseases of the
cervical spine: cervical spine:
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Chapter 39 Case Demonstration: Edema and Shoulder Pain 803

• Inflammatory muscle diseases • Inflammatory muscle diseases (not


associated with upper extremity edema)
• Psoriatic arthritis • Psoriatic arthritis (not associated with
upper extremity edema)
• Scleroderma • Scleroderma (not associated with upper
extremity edema)
• Systemic lupus erythematosus • Systemic lupus erythematosus (not
associated with upper extremity edema)
Septic Septic
Acute viral/idiopathic pericarditis Acute viral/idiopathic pericarditis
Cat-scratch disease Cat-scratch disease (not typically associated
with pain)
Cervical epidural abscess Cervical epidural abscess
Cervical lymphadenitis Cervical lymphadenitis
Hepatitis Hepatitis (no abdominal pain, no recent
illness)
Perihepatitis (Fitz-Hugh-Curtis syndrome) Perihepatitis (Fitz-Hugh-Curtis syndrome)
(time course)
Pleuritis Pleuritis (no chest pain)
Pneumonia Pneumonia (no recent upper respiratory
illness)
Subphrenic abscess Subphrenic abscess (time course)
Ulcers: Ulcers:
• Duodenal • Duodenal (no abdominal pain)
• Gastric • Gastric (no abdominal pain)
M Metabolic M Metabolic
Ectopic pregnancy Ectopic pregnancy (patient age)
Osteomalacia Osteomalacia (few risk factors, primary
shoulder pain not a characteristic feature)
Va Vascular Va Vascular
Acute myocardial infarction Acute myocardial infarction (time course)
Aneurysm (such as involving the aortic or Aneurysm (such as involving the aortic or
subclavian arteries) subclavian arteries)
Coronary artery insufficiency Coronary artery insufficiency
Pulmonary embolus Pulmonary embolus (time course)
Upper extremity deep venous thrombosis Upper extremity deep venous thrombosis
(Paget-Schroetter syndrome) (Paget-Schroetter syndrome) (time
course)
De Degenerative De Degenerative
Osteoarthritis/osteoarthrosis of the cervical Osteoarthritis/osteoarthrosis of the cervical
spine spine
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Breast tumor • Breast tumor
• Pancoast tumor • Pancoast tumor (no symptoms distal to
the elbow)
Malignant Metastatic: Malignant Metastatic:
Not applicable Not applicable
Benign: Benign:
Not applicable Not applicable
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804 Chapter 39 Case Demonstration: Edema and Shoulder Pain

Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Anxiety Anxiety
Depression Depression
Radiation-induced brachial plexopathy Radiation-induced brachial plexopathy
(time course)
Local Local
T Trauma T Trauma
Axillary web syndrome Axillary web syndrome
Dislocations: Dislocations:
• Acromioclavicular joint • Acromioclavicular joint (no history of
sufficient trauma)
• Glenohumeral joint • Glenohumeral joint (no history of
sufficient trauma)
• Sternoclavicular joint • Sternoclavicular joint (no history of
sufficient trauma)
Fractures: Fractures:
• Bankart lesion • Bankart lesion (no history of sufficient
trauma)
• Bennett lesion • Bennett lesion (no history of sufficient
trauma)
• Clavicle • Clavicle (no history of sufficient trauma)
• Hills Sachs lesion • Hills Sachs lesion (no history of sufficient
trauma)
• Proximal humerus (such as insufficiency • Proximal humerus (such as insufficiency
fracture) fracture)
• Scapula • Scapula (no history of sufficient trauma)
Glenohumeral joint sprain/subluxation Glenohumeral joint sprain/subluxation (no
history of sufficient trauma)
Glenoid labrum tear Glenoid labrum tear (no history of sufficient
trauma)
Muscle strains: Muscle strains:
• Levator scapula • Levator scapula
• Pectoralis muscle group • Pectoralis muscle group (location of
symptoms)
• Rotator cuff • Rotator cuff
• Upper trapezius • Upper trapezius
Myofascial pain secondary to radiation Myofascial pain secondary to radiation
fibrosis or mastectomy fibrosis or mastectomy
Nerve entrapments: Nerve entrapments:
• Median • Median
• Musculocutaneous • Musculocutaneous
• Radial • Radial
• Ulnar • Ulnar
Subacromial impingement syndrome Subacromial impingement syndrome
Thoracic outlet syndrome Thoracic outlet syndrome (no symptoms
distal to elbow)
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Chapter 39 Case Demonstration: Edema and Shoulder Pain 805

I Inflammation I Inflammation
Aseptic Aseptic
Adhesive capsulitis Adhesive capsulitis
Bursitis Bursitis (symptoms began during radiation
treatment)
Chronic fatigue syndrome Chronic fatigue syndrome (this is a
diagnosis of exclusion)
Complex regional pain syndrome Complex regional pain syndrome
Fibromyalgia Fibromyalgia (this is a diagnosis of
exclusion)
Myofascial pain syndrome Myofascial pain syndrome
Neuralgic amyotrophy (Parsonage Turner Neuralgic amyotrophy (Parsonage Turner
syndrome) syndrome) (time course, age)
Polymyalgia rheumatica Polymyalgia rheumatica (unilateral
symptoms uncommon)
Reiter’s syndrome Reiter’s syndrome (no recent illness)
Rheumatoid arthritis Rheumatoid arthritis (first involvement of
the shoulder is uncommon)
Rheumatoid arthritis–like diseases of the Rheumatoid arthritis–like diseases of the
shoulder: shoulder:
• Ankylosing spondylitis • Ankylosing spondylitis (patient age, first
involvement of the shoulder is
uncommon)
• Inflammatory muscle diseases • Inflammatory muscle diseases (unilateral
symptoms uncommon)
• Psoriatic arthritis • Psoriatic arthritis (first involvement of the
shoulder is uncommon)
• Scleroderma • Scleroderma (first involvement of the
shoulder is uncommon)
• Systemic lupus erythematosus • Systemic lupus erythematosus (first
involvement of the shoulder is
uncommon)
Rotator cuff tendinitis Rotator cuff tendinitis (symptoms began
during radiation treatment)
Septic Septic
Osteomyelitis Osteomyelitis
Septic arthritis Septic arthritis
Skeletal tuberculosis (Pott’s disease) Skeletal tuberculosis (Pott’s disease) (no
recent illness)
M Metabolic M Metabolic
Amyloid arthropathy Amyloid arthropathy
Cancer-related fatigue syndrome Cancer-related fatigue syndrome
Gout Gout (uncommon presentation at the
shoulder)
Hereditary neuralgic amyotrophy Hereditary neuralgic amyotrophy (patient
age at first presentation)
Heterotopic ossification (myositis ossificans) Heterotopic ossification (myositis ossificans)
Medication-induced joint pain Medication-induced joint pain
Pseudogout Pseudogout (time course)
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806 Chapter 39 Case Demonstration: Edema and Shoulder Pain

Va Vascular Va Vascular
Aneurysm (such as involving the subclavian Aneurysm (such as involving the subclavian
or axillary arteries) or axillary arteries)
Avascular necrosis of the humeral head Avascular necrosis of the humeral head
Compartment syndrome Compartment syndrome (time course)
Deep venous thrombosis Deep venous thrombosis (time course)
Quadrilateral space syndrome Quadrilateral space syndrome (time course)
De Degenerative De Degenerative
Osteoarthritis/osteoarthrosis: Osteoarthritis/osteoarthrosis:
• Acromioclavicular joint • Acromioclavicular joint
• Glenohumeral joint • Glenohumeral joint
• Sternoclavicular joint • Sternoclavicular joint
Rotator cuff tear Rotator cuff tear
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
• Breast cancer in remaining lymph nodes • Breast cancer in remaining lymph nodes
• Chondrosarcoma • Chondrosarcoma (patient age)
• Lymphoma • Lymphoma
• Osteosarcoma • Osteosarcoma
Malignant Metastatic, such as: Malignant Metastatic, such as:
• Metastases, including from primary breast, • Metastases, including from primary breast,
kidney, lung, prostate, and thyroid kidney, lung, prostate, and thyroid
disease disease
Benign, such as: Benign, such as:
• Enchondroma • Enchondroma (condition is painless)
• Lipoma • Lipoma
• Osteoblastoma • Osteoblastoma
• Osteochondroma • Osteochondroma (patient age)
• Osteoid osteoma • Osteoid osteoma (patient age)
• Unicameral bone cyst • Unicameral bone cyst (patient age)
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Erb’s palsy Erb’s palsy (patient age at first symptom
presentation)
Neuropathic arthropathy (Charcot- Neuropathic arthropathy (Charcot-Marie-
Marie-Tooth disease) Tooth disease) (time course)

STEP #7: Ask specific questions to rule ● Did your shoulder pain start during radia-
specific conditions or pathological tion treatment? Yes, making less likely that
categories less likely. axillary cording or any acute inflammatory
condition is the source of her pathology.
Teaching Comments: The focus of the ● Do you have chest pain with physical
first session was for the clarification of arm exertion? No, ruling less likely primary
pain and lymphedema because they were the cardiac pathology.
chief concerns of SR. However, due to the bar- ● Do you have numbness or tingling? No,
riers to communication and the cautions of decreasing index of clinical suspicion for
the case, the interview and the examination cervical spine or neurogenic pathology.
process may take several sessions within this ● Have you had x-ray or magnetic resonance
patient population. imaging of your shoulder in the past
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Chapter 39 Case Demonstration: Edema and Shoulder Pain 807

3 months? No, so possibility of metastases I Inflammation


or primary bone tumor unable to be excluded. Aseptic
STEP #8: Re-sort the diagnostic Not applicable
hypothesis list based on the patient’s Septic
responses to specific questioning. Acute viral/idiopathic pericarditis (no
Causes change in symptoms with physical
exertion)
T Trauma
Cervical epidural abscess (no numbness
Secondary lymphedemas: and tingling)
• Radiation-induced Cervical lymphadenitis
• Surgical lymph node dissection
M Metabolic
I Inflammation
Not applicable
Aseptic
Va Vascular
Not applicable
Aneurysm (such as involving the aortic or
Septic
subclavian arteries)
Cellulitis
Coronary artery insufficiency (no change in
Erysipelas
symptoms with physical exertion)
M Metabolic
De Degenerative
Medication-induced edemas:
Osteoarthritis/osteoarthrosis of the cervical
• Chemotherapy agents
spine
Hyperthyroidism
Tu Tumor
Va Vascular
Malignant Primary, such as:
Deep venous thrombosis
• Breast tumor
Superior vena cava syndrome (no pain with
Malignant Metastatic:
exertion)
Not applicable
De Degenerative Benign:
Not applicable Not applicable
Tu Tumor Co Congenital
Malignant Primary, such as: Not applicable
• Lymphoma Ne Neurogenic/Psychogenic
Malignant Metastatic, such as:
Anxiety
• Metastases, including from primary
Depression
breast, kidney, lung, prostate, and thyroid
disease Local
Benign: T Trauma
Not applicable Axillary web syndrome (onset not
Co Congenital coincident with radiation treatment)
Not applicable Fractures:
• Proximal humerus (such as insufficiency
Ne Neurogenic/Psychogenic
fracture)
Complex regional pain syndrome Muscle strains:
Remote • Levator scapula
T Trauma • Rotator cuff
• Upper trapezius
Cervical disk herniation (no numbness and
Nerve entrapments:
tingling)
• Median
Thoracic outlet syndrome (no numbness
• Musculocutaneous
and tingling)
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808 Chapter 39 Case Demonstration: Edema and Shoulder Pain

• Radial noted with minimal cording, decreasing the


• Ulnar index of suspicion for axillary web syn-
Subacromial impingement syndrome drome. Coloration and temperature of the
I Inflammation skin was grossly normal throughout the
neck and shoulders, reducing the likelihood
Aseptic
of infection. Mild lymphedema was noted
Adhesive capsulitis
in the left shoulder axilla and left lateral
Complex regional pain syndrome
chest wall, increasing the likelihood of sec-
Myofascial pain syndrome (secondary to
ondary lymphedema.
radiation; onset not coincident with
radiation therapy) ● Auscultation. Cardiac auscultation re-
vealed normal heart sounds, ruling less
Septic likely primary cardiac pathology. Rate and
Osteomyelitis rhythm also were grossly normal, ruling
Septic arthritis less likely hyperthyroidism. Pulmonary
M Metabolic auscultation was unremarkable for crack-
Amyloid arthropathy les, rales, or absent breath sounds, decreas-
Cancer-related fatigue syndrome ing likelihood of primary pulmonary
Heterotopic ossification (myositis ossificans) pathology.
Medication-induced joint pain ● Neurological tests. Reflexes and sensation
Va Vascular were normal throughout the upper extrem-
ities, reducing the likelihood of primary
Aneurysm (such as involving the subclavian
cervical spine pathology.
or axillary arteries)
Avascular necrosis of the humeral head ● Range of motion of the cervical spine,
shoulder, lumbar spine, and hip. Cervical
De Degenerative spine range of motion was normal without
Osteoarthritis/osteoarthrosis: reproduction of symptoms in the right
• Acromioclavicular joint upper extremity, ruling even less likely pri-
• Glenohumeral joint mary cervical spine pathology. Left shoulder
• Sternoclavicular joint active and passive range of motion revealed
Rotator cuff tear moderately limited movement in all planes
Tu Tumor secondary to reproduction of symptoms,
Malignant Primary, such as: increasing suspicion of pathology specific to
• Breast cancer in remaining lymph nodes the shoulder region. Lumbar and hip ranges
• Lymphoma of motion were normal and did not change
• Osteosarcoma the patient’s pain.
Malignant Metastatic, such as: ● Palpation. Rotator cuff tendons, acromio-
• Metastases, including from primary breast, clavicular joint, and sternoclavicular joint
kidney, lung, prostate, and thyroid disease were nontender.
Benign, such as:
• Lipoma
• Osteoblastoma
Co Congenital Teaching Comments: It is probable that
Not applicable the soft tissue fibrosis resulting in decreased
muscle length of the pectoralis major/minor
Ne Neurogenic/Psychogenic and loss of skin mobility are due to radiation
Not applicable therapy. These soft tissue alterations can lead
STEP #9: Perform tests to differentiate to altered positioning of the glenohumeral
among the remaining diagnostic joint within the fossa. At this point in the
hypotheses. exam, it will be difficult to determine the
structural cause of the shoulder symptoms if
● Observation of the face, neck, and shoul-
they are musculoskeletal.
der. Well-healed left axillary incision was
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Chapter 39 Case Demonstration: Edema and Shoulder Pain 809

STEP #10: Re-sort the diagnostic Septic


hypothesis list based on the patient’s Cervical lymphadenitis (no swelling)
responses to specific tests. M Metabolic
Causes Not applicable
T Trauma Va Vascular
Secondary lymphedemas: Aneurysm (such as involving the aortic or
• Radiation-induced subclavian arteries)
• Surgical lymph node dissection
De Degenerative
I Inflammation
Osteoarthritis/osteoarthrosis of the cervical
Aseptic spine (normal cervical range of motion
Not applicable without reproduction of left shoulder
Septic pain)
Cellulitis (no swelling, increase in skin Tu Tumor
temperature)
Malignant Primary, such as:
Erysipelas (no change in skin color,
• Breast tumor
swelling, skin temperature)
Malignant Metastatic:
M Metabolic Not applicable
Medication-induced edemas: Benign
• Chemotherapy agents Not applicable
Hyperthyroidism (normal cardiac rate and Co Congenital
rhythm)
Not applicable
Va Vascular
Ne Neurogenic/Psychogenic
Deep venous thrombosis (no swelling,
Anxiety
change in skin temperature)
Depression
De Degenerative
Local
Not applicable
T Trauma
Tu Tumor
Fractures:
Malignant Primary, such as: • Proximal humerus (such as insufficiency
• Lymphoma fracture) (shoulder range of motion)
Malignant Metastatic, such as: Muscle strains:
• Metastases, including from primary • Levator scapula
breast, kidney, lung, prostate, and thyroid • Rotator cuff
disease • Upper trapezius
Benign Nerve entrapments:
Not applicable • Median
Co Congenital • Musculocutaneous
Not applicable • Radial
• Ulnar
Ne Neurogenic/Psychogenic
Subacromial impingement syndrome
Complex regional pain syndrome
I Inflammation
Remote
Aseptic
T Trauma Adhesive capsulitis (pattern of shoulder
Not applicable range-of-motion limitation)
I Inflammation Complex regional pain syndrome
Septic
Aseptic
Osteomyelitis
Not applicable
Septic arthritis
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810 Chapter 39 Case Demonstration: Edema and Shoulder Pain

M Metabolic in the literature include weight loss or gain


Amyloid arthropathy (no swelling) greater than 10% of body weight in 1
Cancer-related fatigue syndrome month, night pain, or constant pain. It is
Heterotopic ossification (myositis ossificans) this author’s experience that more common
Medication-induced joint pain complaints include fatigue (low intensity
with slow onset that steadily and gradually
Va Vascular
increases over time) that is not relieved by
Aneurysm (such as involving the subclavian rest and reports of a “dull ache” that initially
or axillary arteries) appears to be intermittent and then pro-
Avascular necrosis of the humeral head gresses to constant. Additionally, the patient
De Degenerative currently reports occasional low back pain
Osteoarthritis/osteoarthrosis: and hip pain. The mere presence of associ-
• Acromioclavicular joint (joint is ated pains should also alert the clinician to
nontender) the possibility of metastasis. Although the
• Glenohumeral joint complaints of low back pain and hip pain
• Sternoclavicular joint (joint is nontender) were not the patient’s chief concern as of
Rotator cuff tear (negative palpation) this visit, during subsequent visits these
comments will have to be examined and
Tu Tumor
provocative tests completed to deduce if
Malignant Primary, such as: there is a nonmechanical versus muscu-
• Breast cancer in remaining lymph nodes loskeletal cause.
• Lymphoma An established diagnosis of metastatic
• Osteosarcoma breast cancer would not preclude physical
Malignant Metastatic, such as: therapy; physical therapy is an acceptable
• Metastases disease, including from intervention even in the presence of
primary breast, kidney, lung, prostate, metastatic disease. The therapist must con-
and thyroid disease sult with the medical team to determine the
Benign, such as: structures involved that must be considered
• Lipoma in determining ongoing physical therapy in-
• Osteoblastoma terventions. It is the remaining possibility of
Co Congenital undetected metastatic disease of the hip and
Not applicable lumbar spine, and the serious consequences
Ne Neurogenic/Psychogenic that accompany that diagnosis, that necessi-
tates a formal return visit by the patient to
Not applicable the oncologist.
STEP #11: Decide on a diagnostic ● Cancer-related fatigue syndrome with de-
impression. pression-related fatigue. Cancer-related fa-
The preliminary diagnostic impression is a tigue syndrome is well documented and is a
mix of permanent structural changes, tran- result of physical deconditioning and psy-
sient orthopedic impairments, and the poten- chosocial factors that occur as a result of the
tial serious consequences of progression of cancer and its treatment modalities.5,6
metastatic disease. The most likely conditions ● Medication-induced arthralgia. Arimidex
of those that remain include (in alphabetical is an aromatase inhibitor, which prevents es-
order): trogen from being produced in post-
● Breast cancer metastasis affecting the menopausal women. It is effective in con-
brachial plexus, supraclavicular lymph trolling the growth of metastatic breast
nodes, or remaining axillary lymph nodes. cancer. Presumably it has fewer side effects
Based on the stage of the patient’s breast than Tamoxifen; however, side effects may
cancer this diagnosis should be considered include joint pain, stiffness, and arthritic-
present until proven otherwise. Classic clin- type complaints. Given that this patient has
ical signs and symptoms of cancer reported left shoulder pain with occasional low back
1528_Ch39_798-811 14/05/12 12:24 PM Page 811

Chapter 39 Case Demonstration: Edema and Shoulder Pain 811

and left hip pain, Arimidex could be a po- Case Outcome


tential contributor to this individual’s
source of pain. If the joint pain is due to A lumbar and hip evaluation was performed
Arimidex or is medication induced, the pa- and completed by the patient’s third visit to
tient’s pain would be bilateral and involve physical therapy. Physical therapy could not al-
many joints. It is therefore unlikely that this leviate or reproduce the patient’s hip pain.
adverse effect is the primary cause of her Based on these findings, the physical therapist
musculoskeletal complaints. The specific reconnected with the patient’s oncologist im-
complaint of left hip pain is suspicious and, mediately and relayed these concerns. Mag-
based on the author’s clinical experience, netic resonance imaging and a bone scan were
raises the concern for metastatic disease. ordered and findings did indicate a new
metastatic lesion to the hip. The finding of a
● Radiation fibrosis resulting in myofascial new metastatic lesion did not alter the original
restrictions of the left upper extremity physical therapy plan for the upper quadrant.
muscles. The underlying joints within the ra-
diated field as identified by the radiation field References
tattoos will also have restriction. Finally, 1. Deyo RA, Diehl AK. Cancer as a cause of back pain:
observation of the skin discoloration will cue frequency, clinical presentation, and diagnostic strate-
the clinician to the integumentary system gies. J Gen Intern Med. May–Jun 1988;3(3):230–238.
2. O’Sullivan B, Levin W. Late radiation-related fibrosis:
and its altered skin texture and mobility. pathogenesis, manifestations, and current management.
The consequence of this altered mobility is Semin Radiat Oncol. 2003;13(3):274–289.
scapular dyskinesia. It is frequently related 3. Davis AM, Dische S, Gerber L, Saunders M, Leuing SF,
to several of the remaining musculoskeletal O’Sullivan B. Measuring postirradiation subcutaneous
soft-tissue fibrosis: state-of-the-art and future directions.
diagnoses on the hypothesis list such as Semin Radiat Oncol. 2003;13(3):203–213.
subacromial impingement, rotator cuff ten- 4. Bentzen SM. Preventing or reducing late side effects of
dinitis/tendinosis, glenohumeral joint re- radiation therapy: radiobiology meets molecular pathol-
striction/capsulitis, and bursitis. ogy. Nature Rev Cancer. 2006;6(9):702–713.
5. Ahlberg K, Ekman T, Gaston-Lohansson F, Mock V.
STEP #12: Determine the appropriate Assessment and management of cancer-related fatigue in
patient disposition. adults. Lancet. 2003;362:640–650.
6. Curt G. The impact of fatigue on patients with cancer:
● Initiate physical therapy treatment with overview of FATIGUE 1 and 2. Oncologist. 2000;5(suppl
consultation of the oncologist. 2):9–12.
1528_Ch40_812-832 07/05/12 2:10 PM Page 812

CHAPTER 40
Failure of Wounds to Heal
■ Rose Hamm, PT, DPT, CWS, FACCWS

Description of the Symptom in determining if it is chronic than are the


characteristics of the wound tissue.
This chapter describes pathology that may lead
to wounds that fail to heal. Chronic wounds Special Concerns
are defined as wounds that “fail to progress ■ Sinus, undermining, or fistula formation
through a normal, orderly, and timely sequence ■ Bone involvement that may lead to
of repair or wounds that pass through the osteomyelitis
repair process without restoring anatomic and ■ Friable granulation tissue
functional results.”1 Without adequate treat- ■ Contractures
ment of the wound etiology and comorbidi- ■ Deformities of adjacent joints
ties, the composition of the wound tissue and ■ Malignant changes
fluid (called chronic wound fluid) will develop ■ Systemic amyloidosis
certain properties that inhibit healing. The ■ Calcification2
length of time a wound exists is less important ■ Failure to respond to standard care

CHAPTER PREVIEW: Conditions That May Lead to Failure of Wounds to Heal

T Trauma
COMMON
Burns 818
Neuropathic wounds 825
Traumatic injury 830
UNCOMMON
Not applicable
RARE
Not applicable

I Inflammation
COMMON
Aseptic
Bullous pemphigoid 818
Foreign body reaction 822
Pemphigus 826
Pyoderma gangrenosum 828
Septic
Cellulitis 819
Dermal viral infections 821
Fungal infection 822
Necrotizing fasciitis 825
Osteomyelitis 826

812
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Chapter 40 Failure of Wounds to Heal 813

Inflammation (continued)

FAILURE OF WOUNDS TO HEAL


UNCOMMON
Not applicable
RARE
Not applicable

M Metabolic
COMMON
Allergic responses:
• Contact dermatitis 815
• Drug hypersensitivity syndromes 815
• Spider bites 816
UNCOMMON
Not applicable
RARE
Not applicable

Va Vascular
COMMON
Arterial insufficiency 816
Calciphylaxis 818
Chronic venous insufficiency 820
Coumadin-induced skin necrosis (warfarin-induced skin necrosis) 820
Cryoglobulinemia 821
Lymphedemas:
• Primary lymphedema (Milroy’s disease, Meige disease) 822
• Secondary lymphedema 823
Martorell’s ulcer 824
Raynaud’s disease 829
Sickle cell disease 829
Vasculitis 830
UNCOMMON
Buerger disease (thromboangiitis obliterans) 817
RARE
Not applicable

De Degenerative
COMMON
Pressure ulcers 827
UNCOMMON
Not applicable
RARE
Not applicable
(continued)
1528_Ch40_812-832 07/05/12 2:10 PM Page 814

814 Chapter 40 Failure of Wounds to Heal


FAILURE OF WOUNDS TO HEAL

Tu Tumor
COMMON
Not applicable
UNCOMMON
Malignant Primary, such as:
• Basal cell carcinoma 817
• Squamous cell carcinoma 829
Malignant Metastatic, such as:
• Kaposi’s sarcoma 822
Benign:
Not applicable
RARE
Malignant Primary, such as:
• Cutaneous lymphoma 821
• Melanoma 824
Malignant Metastatic, such as:
• Marjolin’s ulcer 824
Benign:
Not applicable

Co Congenital
COMMON
Not applicable
UNCOMMON
Not applicable
RARE
Not applicable

Ne Neurogenic/Psychogenic
COMMON
Not applicable
UNCOMMON
Factitious disorder 822
RARE
Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.
1528_Ch40_812-832 07/05/12 2:10 PM Page 815

Chapter 40 Drug Hypersensitivity Syndromes 815

Overview of Wounds That Fail

FAILURE OF WOUNDS TO HEAL


to Heal
A chronic wound can have any of the following
characteristics:
● Necrotic and unhealthy tissue
● Impaired hemodynamics (for example,
hypoxia, ischemia, or edema)
● Collagen degradation resulting in unhealthy
extracellular matrix FIGURE 40-1 Contact dermatitis causes skin changes
such as erythema, edema, pruritus, and vesicles.
● Rolled edges without epithelial migration
as a result of senescent fibroblasts and ker-
atinocytes that are unresponsive to normal a carrier protein and transported by Langer-
facilitating factors hans cells to T cells that become sensitive to
● Overgrowth of epithelium due to the lack of the antigen. Upon reexposure, the T cells ini-
underlying connective tissue tiate an inflammatory response that is visible
● Recurrent wound deterioration as a result of as this condition.3 After identification and
superficial epithelial bridging removal of the irritating substance, contact
● Chronic inflammation resulting in lack of dermatitis is usually adequately managed
healthy granulation tissue with local treatment; however, in severe cases
● Increased bacterial loads with or without referral to a medical specialist is indicated.
clinical infection (pain, warmth, erythema, In addition to eliminating exposure to the
edema, drainage, odor) antigen or irritant, local care involves imme-
● Presence of biofilm on the wound surface, diate thorough washing of the exposed area
sometimes visible as a thin opaque layer. with warm water and mild soap, use of
topical creams (eg, steroids or antibiotics),
The four most common causes of chronic and avoidance of tape, adhesive dressings, or
wounds are pressure, arterial insufficiency, nonprescription topical ointments that may
chronic venous insufficiency, and neuropathy exacerbate this condition. Diffuse cellulitis
(sensory, motor, and/or autonomic). If these may develop if this condition is improperly
four etiologies are ruled out, atypical etiologies treated.
need to be identified so the underlying cause
of the wound can be treated in addition to ■ Drug Hypersensitivity
local wound care. Syndromes
Chief Clinical Characteristics
Description of Conditions That May This presentation usually includes fever,
Lead to Failure of Wounds to Heal malaise, pharyngitis, small joint polyarthritis,
and cervical lymphadenopathy. Skin erup-
ALLERGIC RESPONSES tions, which occur in approximately 85% of
■ Contact Dermatitis
individuals with this condition, may be
eczematous eruptions with erythema, vesicles,
Chief Clinical Characteristics and scales with itching; red rash or measle-like
This presentation involves erythema, edema, eruptions that usually begin in pressured or
pruritus, and vesicular lesions in the contact dependent areas and rapidly become general-
area (Fig. 40-1). ized; vesicular or bullous eruptions; erythema
Background Information and edema followed by hyperpigmentation at
This condition occurs from direct contact of a specific site of drug injection; generalized
the skin with an antigen (eg, latex, metals, pustules usually on the face or between skin-
poisonous plants, drugs, tape) or with pro- folds; subcutaneous nodules that are tender
longed exposure to an irritant (eg, detergent, and erythematous, usually on the anterior legs;
perfume, chemical). The antigen is bound to or wheals, welts, or hives (Fig. 40-2).
1528_Ch40_812-832 07/05/12 2:10 PM Page 816

816 Chapter 40 Spider Bites

surrounding erythema; bluish and cyanotic


FAILURE OF WOUNDS TO HEAL

color in a blanched ring surrounded by ery-


thema (“bull’s-eye” lesion) within a few hours;
a hemorrhagic bleb that becomes several cen-
timeters with generalized fine punctate rash;
systemic symptoms of nausea, vomiting, and
malaise; and subcutaneous fat necrosis that
expands to 3 to 10 cm, becomes gangrenous,
desiccates, and becomes eschar that requires
6 weeks to 4 months to heal.
FIGURE 40-2 Drug hypersensitivity syndrome. Background Information
Initial signs of drug hypersensitivity include rash or While many arachnids, which include spiders
measle-like symptoms in dependent extremities. and scorpions, may cause reactions to bites,
most necrotic skin wounds are caused by the
genus Loxosceles.4 Of the many species of
Background Information Loxosceles, the brown recluse spider is the
Symptoms usually occur 2 to 6 weeks after most prevalent and has the most potent
the first drug exposure, and the allergen is venom.5 This condition involves an inflam-
usually the drug most recently included in matory response to the venomous toxin. The
the individual’s medications. This condition resulting platelet aggregation and endothe-
is a systemic allergic reaction to ingested lial edema occlude the capillaries, causing
drugs, which can vary in the number of ischemia and necrosis. Standard wound care
involved organs, severity of symptoms, type is sufficient in mild cases; however, if symp-
of lesions, and number of skin eruptions. toms of severe involvement occur, emergent
Cutaneous pathology involves immunoglob- medical care is indicated. In addition to
ulin hypersensitivity reactions that result in the soft tissue and skin loss, disseminated
destruction of the basal membrane binding intravascular coagulation is a complication
the epidermis and dermis, leading to the that requires immediate medical attention.6
variety of skin eruptions listed above. Medical
intervention is dependent on the severity of ■ Arterial Insufficiency
the reaction and symptoms, including Chief Clinical Characteristics
meticulous local wound care for milder cases This presentation usually involves wounds that
and emergent medical care for severe cases, are located on the peripheral extremities (toes,
especially the life-threatening syndromes of fingers, dorsum of the forefoot or hand, inter-
toxic epidermal necrolysis and Stevens- digital spaces, or lateral malleolus where
Johnson syndrome. Individuals who exhibit pressure areas are unable to heal) and appear
signs of this condition should be referred to as “punched-out” wounds with even, sharp
the physician or emergency department for demarcation of the edges. The wound is typically
immediate care. dry and necrotic with black sloughing peri-
■ Spider Bites wound skin. The digits may progress from a
dusky color (typical if the occlusion is an acute
Chief Clinical Characteristics embolism or thrombosis) to a dark, dry, black
This presentation commonly involves slight mummified appearance if the anoxia is chronic
erythema, localized urticaria, and discomfort (Fig. 40-3).
for 3 to 5 days for cases of minor envenoma-
tion. For cases of moderate envenomation, Background Information
discomfort begins 4 to 6 hours after the bite This condition is caused by ischemia as a result
and involves pale skin with a small blister in of interruption of the blood flow in either
the center, and resolution of symptoms with the macrovascular (large, named arteries) or
topical care after 1 to 2 weeks. Cases of severe microvascular (arterioles and capillaries) circu-
envenomation involve pain 6 to 12 hours lation. The individual also may report claudi-
after the bite; two tiny puncture marks with cation, defined as exercise-induced leg pain, or
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Chapter 40 Buerger Disease (Thromboangiitis Obliterans) 817

FAILURE OF WOUNDS TO HEAL


FIGURE 40-3 Arterial wound caused by arterial
insufficiency. FIGURE 40-4 Nonhealing wound on the forearm was
diagnosed by biopsy as a basal cell carcinoma.

resting or night pain, which occurs during the epidermis.7 Caused almost exclusively by
night when the blood pressure tends to be chronic exposure to sunlight, this condition
lower. Associated conditions include periph- usually occurs on the face, neck, shoulders, back,
eral arterial disease, diabetes mellitus, hyper- and scalp. Furthermore, people who tend to
tension, and acute arterial embolism after burn rather than tan, and have fair skin, light
trauma or vascular/cardiovascular surgery. hair, or blue, gray, or green eyes are more suscep-
Screening tests that can be performed by the tible to this condition.8 Two specific types of
therapist include pulse examination, capillary this condition—ulcerating basal cell carcinoma
refill, rubor of dependency, and ankle-brachial and basalioma terebrans—can become open
index. Individuals suspected of this condition wounds with the risk of becoming infected.
should be referred to a vascular surgeon for a Ulcerating basal cell carcinoma is identified by
thorough vascular examination to determine hemorrhagic crusting and hard pearly edges;
the location and severity of the occlusion; basalioma terebrans, by deep tisue necrosis with
debridement is contraindicated prior to revas- red granulated surfaces.9 Individuals suspected
cularization unless infected necrotic tissue of having this condition should be referred to
is present. Complications include infection a primary care physician or dermatologist for
(including osteomyelitis), failed or occluded additional evaluation. Physical therapy interven-
bypass grafts with further tissue necrosis or tion for wound management is not indicated.
failure to heal, and dehisced surgical incisions.
■ Buerger Disease
■ Basal Cell Carcinoma (Thromboangiitis Obliterans)
Chief Clinical Characteristics Chief Clinical Characteristics
This condition is characterized by two or more This presentation includes pain (including rest-
of the following characteristics: persistent, non- ing pain), tenderness, red skin, cyanosis, thin
healing wound of more than 3 weeks’ duration; shiny skin, and thickened malformed nails
red, irritated patch that may or may not cause (Fig. 40-5). In addition, the hands and feet are
itching and pain; pearly or translucent bump or usually cool and mildly edematous. Ulcerations
nodule that can be any of a variety of colors are common on the toes, feet, or fingers.
(for example, red, pink, white, tan, brown,
Background Information
black); pink lesion with rolled edges, crusted
The pathology of Buerger disease is nonathero-
center, and superficial blood vessels; and white,
sclerotic inflammation of the small- and
yellow, or waxy scar-like lesion with poorly
medium-sized peripheral arteries and veins in
defined edges (Fig. 40-4).
combination with thrombi and vasospasm of
Background Information arterial segments of the feet and/or hands. This
This condition is the most common form of condition is most common in men who
skin cancer, and it originates in the cells of are heavy smokers. It can be differentiated from
the basal membrane between the dermis and peripheral vascular disease by the presence of
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818 Chapter 40 Bullous Pemphigoid

■ Burns
FAILURE OF WOUNDS TO HEAL

Chief Clinical Characteristics


This presentation can be characterized by dif-
ferent features depending on the severity of the
burn. Burns are categorized as superficial
(previously first degree) if they are character-
ized by immediate but not long-lasting pain, ery-
thema, localized edema, and blisters after
24 hours. Partial-thickness burns (previously sec-
ond degree) involve skin loss, blisters within
minutes of injury, pain throughout the healing
process, and scarring (Fig. 40-6A); they are clas-
sified as either superficial partial-thickness skin
loss (involving the epidermis and upper dermis)
or deep partial-thickness skin loss (involving
FIGURE 40-5 Gangrenous wound on the toe of a the epidermis and deep dermis). Full-thickness
patient with Buerger disease. The wound has the burns (previously third degree) involve a waxy
appearance of an arterial wound; however, the white appearance, surrounding partial-thickness
patient is a young male in his 20s. involvement, diffuse edema, epithelialization
from the hair follicles after 7 to 10 days, loss of
epidermis and dermis, involvement of under-
normal proximal pulses.10 Individuals suspected lying subcutaneous tissue, muscle, or bone, dry
of having this condition should be referred to leathery appearance, black or white eschar,
a physician. Intervention includes cessation of visible thrombosed veins, and loss of pain and
smoking, vasodilators to prevent vasospasms, sensation (Fig. 40-6B).
and exercise to increase blood flow to the
affected extremity. Complications include gan- Background Information
grene, ulceration, and resulting loss of function Burns may be caused by exposure to thermal
and impairments if the disease exacerbates. heat, chemicals, electric current, or radiation.
Activation of the emergency medical services
■ Bullous Pemphigoid at the time of onset is indicated if the burn is
Chief Clinical Characteristics due to chemicals, if the burns are full thick-
This presentation involves pruritic, urticarial, ness or extensive partial thickness, or if they
papular lesions that become large flaccid bul- are on the face, hands, or perineum. Subse-
lae when serous or hemorrhagic fluid fills the quent management of these burns is best pro-
space between the dermal/epidermal layers. vided at a burn center.13 Because superficial
Bullae occur most frequently on the axillae, and partial-thickness burns usually heal in a
abdomen, thighs, groin, lower legs, and flexor timely sequence with standard wound care,
surfaces of the arm. individuals with nonhealing burn wounds
being treated in an outpatient setting should
Background Information be referred to an emergency department or
This condition is an autoimmune disease that primary care physician to determine the reason
causes binding of immunoglobulin autoanti- for poor healing. The most frequent compli-
bodies to the epidermal basement membrane, cations of burn injuries are infection, hypov-
resulting in subepidermal blistering; it may be olemic shock, hypoproteinemia, and loss of
idiopathic, drug induced, or related to ultraviolet range of motion due to adhesions.
exposure.11 Individuals suspected of having this
condition should be referred to a physician for ■ Calciphylaxis
medical care, including anti-inflammatory Chief Clinical Characteristics
medications, antibacterials, and immunosup- This presentation commonly involves painful,
pressants.12 Local wound management is indi- indurated, purplish skin lesions that progress
cated after a definitive diagnosis is made and rapidly to skin necrosis and gangrene (Fig. 40-7).
medical management has been initiated. The lesions usually occur on the trunk and lower
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Chapter 40 Cellulitis 819

FAILURE OF WOUNDS TO HEAL


A B
FIGURE 40-6 (A) Partial-thickness burns involve the epidermis and part of the dermis. (B) Full-thickness burns
involve all of the dermis and extend into the subcutaneous tissue.

Usual wound management involves aggressive


sharp or surgical debridement of all necrotic
tissue, topical antibiotics, and moist wound
dressings using sterile technique. Complications
are secondary skin infection, sepsis, and death,
with mortality rates as high as 60%. Individuals
with lesions on the distal extremities and who
receive early diagnosis and treatment have
better survival rates.15
■ Cellulitis
Chief Clinical Characteristics
FIGURE 40-7 Wounds caused by calciphylaxis This presentation commonly includes erythema,
usually occur in individuals receiving long-term edema, pain, drainage, warm to touch, indura-
hemodialysis. tion, skin discoloration, and flaky or scaling
skin. Periwound skin of a preexisting wound
extremities and are frequently bilateral and may be unresponsive to or exacerbated by stan-
symmetrical. Individuals with this condition dard wound care. In addition, there may or
will have palpable pulses distal to the lesions, may not be systemic signs of infection, including
unlike patients with peripheral arterial disease. fever, elevated white blood count, or erythema-
tous streaking.
Background Information
The pathology involves microvascular calcifi- Background Information
cation with superimposed intimal fibroblastic This condition is caused by a bacterial infec-
hyperplasia. This condition, often associated tion of the dermis and subcutaneous tissue;
with hypercalcemia, hyperphosphatemia, and most commonly the infective agents are
hyperparathyroidism, occurs in patients with Staphylococcus aureus and Streptococcus. Indi-
end-stage renal disease. Individuals suspected viduals with skin lesions, venous insuffi-
of having this condition should be referred ciency, lymphedema, diabetes mellitus, and
immediately to a renal specialist or endocri- immunosuppression are at higher risk for
nologist. Interventions include parathyroidec- developing cellulitis. Medical interventions
tomy, treatment to normalize calcium and include oral or intravenous antibiotics,
phosphorus levels, and pain management.14 depending on the severity and the cultured
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820 Chapter 40 Chronic Venous Insufficiency

bacteria, and prophylactic antibiotics for valves are usually the cause of fluid reflux. The
FAILURE OF WOUNDS TO HEAL

patients with frequent episodes.16 Immediate buildup of chronic interstitial fluid in the dis-
referral to a primary care physician is advised. tal leg causes venous hypertension and triggers
Standard wound management, including cellular and chemical changes in the tissue,
edema management with elevation and com- typical of a systemic inflammatory response,
pression, is recommended for both acute and which result in skin breakdown. Comorbid
chronic conditions. Prophylactic compression pathologies that increase the risk for develop-
hosiery is advised for patients with venous ing this condition include varicose veins, deep
insufficiency or lymphedema. If not already venous thrombosis, previous vein surgery,
present, cellulitis may cause open lesions in multiple pregnancies, obesity, congestive heart
the affected area. failure, coronary artery bypass surgery with
saphenous vein harvesting, hip trauma, ankle
■ Chronic Venous Insufficiency immobility, and prolonged standing. Screen-
Chief Clinical Characteristics ing tests to determine the cause of venous in-
This presentation typically involves wounds that sufficiency include approximation of central
occur proximal to the malleoli on the lower one- venous pressure, augmented venous flow, valve
third of the leg (gaiter area), and are character- competency, percussion test, and ankle-
ized by uneven edges, copious serous drainage, brachial index. Medical intervention for
and shallow depth with a red granulated base antibiotic therapy is indicated if there are signs
(Fig. 40-8). In addition, the surrounding skin of clinical infection or cellulitis, the most com-
will have hyperpigmentation caused by the dep- mon complication of venous ulcers. Standard
osition of hemosiderin in the skin when red blood wound care, compression therapy, and exercise
cells trapped in the interstitial tissue are lysed; lipo- to activate the venous pump can be initiated if
dermatosclerosis, defined as the scaly or bark there are no signs of infection. If infection is
appearance of the skin; a dilated long saphenous suspected, referral to a primary care physician
vein; atrophie blanche, defined as ivory white or vascular surgeon is indicated. Individuals
atrophic plaques in the skin; unilateral or bilat- with this condition also should be advised to
eral edema; dermatitis or eczema with or without avoid prolonged sitting or standing. The most
exudate; or thickened skin that is warm to the common complication is cellulitis.
touch due to the inflammatory process. The
venous wound is usually less painful than the ar- ■ Coumadin-Induced Skin Necrosis
terial one, and the pain is alleviated by elevation. (Warfarin-Induced Skin Necrosis)
Background Information Chief Clinical Characteristics
This condition occurs when there is obstruc- This presentation is characterized by severely
tion, reflux, or both that inhibits the return of painful full-thickness skin necrosis on the trunk
fluid through the veins to the heart. Incompetent or lower extremities where significant subcuta-
neous fat is present. Initial complaints include
paresthesias, sensations of pressure, and
extreme pain. Progression of the disease, which
is fairly rapid, is characterized by edema and
erythema, followed by petechiae, hemorrhagic
bullae, and finally necrotic eschar.17

Background Information
The exact etiology of this condition is unknown;
however, toxic vasculitis, acquired coagulopa-
thy, and hypersensitivity are suggested causes.
Symptoms usually occur within the first
10 days after initiating Coumadin therapy, but
FIGURE 40-8 Chronic venous insufficiency wound. may occur later. This condition is associated
Note the dark hemosiderin staining in the skin around with either serious medical diagnoses or chronic
the wound. illnesses such as deep venous thrombosis,
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Chapter 40 Dermal Viral Infections 821

stroke, and coronary artery disease. Immediate non-Hodgkin’s lymphomas. Hodgkin’s lym-

FAILURE OF WOUNDS TO HEAL


referral to a primary care physician is indicated phomas are a group of five separate conditions
to achieve reversal of the disease process and that arise from a specific B-cell abnormality,
prevent progression of the necrosis. Aggressive whereas non-Hodgkin’s lymphomas number
wound care is indicated to prevent infection approximately 30. A combination of genetic
and facilitate closure. and environmental factors has been implicated
in the development of this condition. Environ-
■ Cryoglobulinemia mental factors include exposure to solvents and
Chief Clinical Characteristics organic chemicals, pesticides and herbicides,
This presentation typically involves palpable wood preservatives, and radiation. In addition,
purpura, Raynaud’s phenomenon, skin rash, states of immunocompromise, including
supramalleolar ulcers, distal ischemia, gan- autoimmune conditions and acquired immun-
grene, livedo reticularis (purplish mottling of the odeficiency virus, have been implicated in this
skin), and acrocyanosis. Ulcerations are severely condition’s development. Interventions range
painful. Systemic manifestations may include from phototherapy, topical chemotherapy, local
renal involvement, peripheral neuropathy electron beam or x-ray therapy, and/or inter-
(mixed or purely sensory), and central nervous feron alpha for local lesions to systemic
system involvement.18 chemotherapy, corticosteroids, antibiotics, and
various combinations of treatment for extracu-
Background Information
taneous involvement. Individuals suspected of
Cryoglobulins are circulating immunoglobulins
having this condition should be referred to an
or immunoglobulin complexes that, under cer-
oncologist; no physical therapy interventions
tain pathological conditions, precipitate from
are indicated.
serum at cold temperatures (<37°C) causing
thrombi within the microvascular system, which ■ Dermal Viral Infections
results in ulceration.19 This condition is associ- Chief Clinical Characteristics
ated with autoimmune, chronic inflammatory, This presentation commonly includes a rash or
immunoproliferative, and infectious diseases. a cluster of inflamed vesicles that usually erupt
The most common types of this condition are and become crusty. Pain, paresthesias, burning,
associated with hepatitis C and chronic liver or itching may precede the appearance of skin
failure. Ulcerations occur as a result of either lesions.
small vessel obstruction or inflammation of the
vessel walls induced by the deposition of the Background Information
cryoglobulins. Individuals suspected of having This condition is caused by minute intracellular
this condition should be referred to a physician parasites that depend on the enzymes of the
or emergency department for treatment of the host cells for survival, causing the host to
underlying disease in conjunction with receiving produce neutralizing antibodies that result in
standard wound care and compression therapy. the viral activity.20 The most common dermal
Most common complications include infection viral infections are:
(including osteomyelitis), arthralgia, loss of 1. Herpes simplex virus, most commonly man-
joint range of motion, and gait impairments. ifested as oral, perioral, or nasal cold sores or
fever blisters
■ Cutaneous Lymphoma 2. Herpes varicella or chickenpox manifested by
Chief Clinical Characteristics diffuse macules, papules, and vesicles during
This presentation involves firm, erythematous, childhood
violaceous, and/or brown lesions that usually 3. Herpes zoster or shingles manifested by vesic-
occur on the trunk, face, or extremities accom- ular eruptions that follow a specific dermatome
panied by pruritus. 4. Warts, a benign round rough gray lesion that
can occur anywhere on the skin as a result of
Background Information
the human papillomavirus.
This condition refers to a whole host of malig-
nancies affecting B and T cells. Broadly, this If detected early, individuals suspected of
condition is divided among Hodgkin’s and having this condition should be referred to the
1528_Ch40_812-832 07/05/12 2:10 PM Page 822

822 Chapter 40 Factitious Disorder

primary physician or dermatologist for medical Background Information


FAILURE OF WOUNDS TO HEAL

management. No topical treatment is indicated This condition is caused by fungi, called tinea,
for herpes simplex or herpes varicella. that thrive on keratin in the stratum corneum,
hair, and nails. These fungi are classified by the
■ Factitious Disorder affected location. For example, tinea pedis is on
Chief Clinical Characteristics the foot, tinea capitis on the scalp, tinea cruris
This presentation is characterized by wounds on the groin, and tinea manus on the hand.
with a consistent appearance of healing, Onychomycosis is a fungal infection of the
including a good granulation base. nails, seen commonly in patients with diabetes
mellitus or peripheral vascular disease. Fungal
Background Information
infections commonly occur in individuals
The wounds are caused by self-inflicted injuries
with systemic administration of antibiotics, dia-
such as compulsive scratching; individuals
betes mellitus, pregnancy, immunosuppression,
with this condition usually have associated
Cushing’s disease, certain blood neoplasms,
psychiatric disorders. Associated signs to
debilitated states, or infants under 6 months of
observe are prematurely removed bandages,
age with decreased immune reactivity.3 Individ-
scratching of the periwound skin, and other
uals with tinea capitis, onychomycosis, or severe
suspicious behaviors such as self-mutilation.
fungal infections should be referred to a primary
Both medical and psychiatric referrals are
care physician; no physical therapy treatment is
indicated and standard wound care should be
indicated.
provided in conjunction with medical and
psychiatric intervention. The most common ■ Kaposi’s Sarcoma
complication is infection. Chief Clinical Characteristics
This presentation usually includes multifocal
■ Foreign Body Reaction purplish brown macules that develop into
Chief Clinical Characteristics plaques and nodules, usually on the lower
This presentation typically involves drainage, extremities or upper body. The lesions can be
edema, erythema, skin necrosis, and pain that painful and pruritic.
increases with weight-bearing activities (when Background Information
the foot or hand are involved). This condition is classified as a vascular malig-
Background Information nancy.21 It most frequently occurs in individu-
Common examples of foreign bodies include als who are immune deficient (such as second-
splinters, needles, or other sharp objects in the ary to human immunodeficiency virus and
plantar foot; suture remnants or staples in or acquired immunodeficiency syndrome) or in
around surgical incisions; bone chips or debris individuals who take immunosuppressive med-
in traumatic injuries; or metal slivers in high-risk ications (such as following organ transplanta-
occupations. A foreign body embedded in tion). Lesions are usually treated medically or
subcutaneous or deep wound tissue may be surgically.22 Local treatment of the sarcoma is
undetectable until an inflammatory response is not indicated; however, wounds may occur in
initiated and superficial signs are noted. If the tissue irradiated for treatment of this condition.
foreign body cannot be easily located and re- These wounds may or may not respond to stan-
moved with sterile instruments, individuals with dard wound care and are often recalcitrant due
this condition should be immediately referred to to the inhibitory effects of the radiation on the
an emergency room or primary care physician cellular mitotic activity.
for surgical removal. Complications of foreign
bodies are infection and further soft tissue dam- LYMPHEDEMAS
age with delayed or prolonged healing times. ■ Primary Lymphedema (Milroy’s
Disease, Meige Disease)
■ Fungal Infection Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can involve a distal to prox-
This presentation involves dry, scaly, erythe- imal progression of unilateral or bilateral ex-
matous, pruritic patches on the skin. tremity edema in an individual with possible
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Chapter 40 Secondary Lymphedema 823

hypoparathyroidism, yellow nails, ptosis, and with weeping of clear lymphatic drainage and

FAILURE OF WOUNDS TO HEAL


webbing at the neck.23 There may or may not surrounding low-grade inflammation. If the
be open chronic wounds with weeping of clear hand or foot is involved, the individual will
lymphatic drainage and surrounding low- have difficulty making a fist or curling the
grade inflammation. Most commonly, the toes. These concerns may worsen with activ-
lower extremities are involved; however, all four ity, increased heat and humidity, or during
extremities may be affected. Initially the changes in elevation. The distal aspect of the
edema is soft and pitting, but over time the limb (foot or hand) may be affected initially;
edema will progress to become hard, indurated, however, in some cases the proximal part of
and fibrotic with little or no pitting. The limb the limb may swell first. The edema usually
can become quite large with abnormal dep- spreads distally to proximally if left untreated.
osition of fat cells, which leads to loss of joint Initially, pitting edema is present and palpa-
spaces and a columnar-like appearance. Skin tion of the limb is soft. Chronic edema causes
changes (such as papillomatosis and hyperk- fibrosis to develop within the subcutaneous
eratosis) and development of lymphocytes tissues, which may lead to a loss of skin
can occur. mobility and decrease range of motion. As
fibrosis develops, the edema becomes harder
Background Information
to pit and palpation of the limb becomes hard
This condition develops as a result of a
and firm.
malformation of the lymph vessels or lymph
nodes that occurred as the embryo was Background Information
developing in utero. The limb is at risk for As the edema becomes indurated, the skin
developing cellulitis, erysipelas, and fungal can develop a “peau d’orange” appearance.
infections. The infections increase the lym- Over time hyperkeratosis and papillomatosis
phatic load and further increase the edema. may develop. The skin may begin to break
In severe cases, elephantiasis may develop. down and infections such as cellulitis and
Hypoplasia is the most common cause of erysipelas may occur. In lower extremity
this condition. Several ages of onset are pos- lymphedema, inability to pinch the skin at
sible including at or around the time of birth the base of the metatarsal heads (positive
(congenital lymphedema; Milroy’s disease); Stemmer sign) is present.25 If the condition
at puberty, when it is called lymphedema is untreated, repeated infections may occur
praecox (Meige disease); and during adult- and the combination of obstruction and
hood (lymphedema tarde). This condition infection can lead to elephantiasis. The limb
remains a diagnosis of exclusion from all becomes grossly enlarged; hyperkeratosis,
other causes of edema. Lymphoscintigraphy, papillomatosis, and the development of a
bioelectrical impedance, magnetic resonance lymphocele may occur and result in severe
imaging, and computed tomography can disfigurement and disability. This condition
assist in determining the appropriate diag- occurs as a result of abnormal accumulation
nosis. The individual with lymphedema will of protein molecules and fluid in the inter-
require lifelong self-treatment, compression stitial spaces of the body from an acquired
therapy, and physical therapy intervention if impairment of the lymphatic system that
the individual experiences repeated infec- affects reabsorption of the interstitial fluid
tions or has difficulty in controlling acute and transportation of lymph (lymphatic
exacerbations.24 drainage). Cancer surgery and radiation
therapy are considered the most frequent
■ Secondary Lymphedema causes of secondary lymphedema. Diagnosis
Chief Clinical Characteristics of lymphedema is primarily a diagnosis of
This presentation typically includes heaviness, exclusion. All other causes of edema are
achiness, tightness, tingling and numbness of ruled out before this diagnosis is reached.
the involved extremity, and painless swelling Lymphoscintigraphy, bioelectrical impedance,
of a limb that initially fluctuates with rest or magnetic resonance imaging, and computed
elevation but with time does not dissipate. tomography can be used to assist in the diag-
There may or may not be open chronic wounds nosis. Treatment of this condition begins
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824 Chapter 40 Marjolin’s Ulcer

first with treating any infections that might with ulcers in this location. The wound edges are
FAILURE OF WOUNDS TO HEAL

be present. Treatment may include manual irregular and satellite ulcers are often present.
lymphatic drainage mobilization followed by
Background Information
compression bandages with short-stretch
This condition is more prevalent in females
materials, joint range of motion, proper skin
and rarely seen in African Americans. These
care, and exercise. Patient education regard-
ulcers occur in individuals who have uncon-
ing skin care, independent bandaging tech-
trolled hypertension, particularly diastolic
niques, and lymph drainage massage tech-
hypertension, and do not respond to standard
niques are critical in order for the individual
wound care until the hypertension is adequately
to control the lymphedema. The individual
managed. In addition, there may be a history
with this condition will require lifelong self-
of minor trauma. Pathophysiology is charac-
treatment and physical therapy intervention
terized by classic hyalinization and thickening
if the individual experiences repeated infec-
of the arteriole media (without atheroma and
tions or has difficulty in controlling acute
calcification) and a decrease in skin perfusion.29
exacerbations.
Referral to a medical specialist is imperative
■ Marjolin’s Ulcer for management underlying hypertension
in conjunction with nonsurgical wound man-
Chief Clinical Characteristics
agement. Aggressive wound management
This presentation involves history of repeated
(eg, surgical debridement and closure with
trauma, unusual location and no obvious eti-
flaps) may cause wound exacerbation.
ology, and active and abnormal cellular prolif-
eration that continues indefinitely if untreated. ■ Melanoma
It is characterized by a vague irregular outline.
Chief Clinical Characteristics
Background Information This presentation is defined by the ABCDE
Marjolin’s ulcer wounds usually occur secondary rule (Fig. 40-9):
to squamous cell carcinoma or basal cell carci- Asymmetry,
noma.26 Any chronic wound of 2 to 3 months’ Border irregularity,
duration that is nonresponsive to standard care Color variegation,
for a patient without identifiable comorbidities Diameter more than 5 mm, and
should be suspected of being malignant. Char- Evolution, indicating that the lesion changes
acteristics to alert the clinician to the possibility in size, color, and shape.30
of malignancy within a wound include the
Background Information
following: nonencapsulated with invasive fingers
This condition occurs when normal cutaneous
of abnormal tissue, systemically detrimental
melanocytes (the cells responsible for skin pig-
wounds, inflamed edges, a process that limits the
mentation) become malignant. The abnormal
growth into adjacent tissue and sometimes
causes a bulbous or cauliflower appearance,
or it may be vascular or necrotic, depending on
the blood supply in the tissue connecting
the neoplasm to the host.27,28 Individuals with
nonhealing wounds that are suspected of being
malignant should be referred to a primary care
physician. Debridement, modalities, and nega-
tive pressure therapy are contraindicated until
malignancy has been excluded.
■ Martorell’s Ulcer
Chief Clinical Characteristics
This presentation can be characterized by FIGURE 40-9 Melanoma has the following charac-
wounds on the anterolateral or posterior teristics: Asymmetry, Border irregularity, Color varie-
aspect of the lower third of the leg and cause mod- gation, Diameter more than 5 mm, and Evolution, in-
erate to severe pain, more than is usually expected dicating that the lesion changes in size, color, and shape.
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Chapter 40 Neuropathic Wounds 825

transformation of cells can occur in the basal bacterial toxins and enzymes and damage to the

FAILURE OF WOUNDS TO HEAL


layer of the epidermis where the melanocytes endothelial lining. This leads to fluid leakage
are located or in a preexisting nevus. Similar to into extravascular spaces, diminished blood
other skin cancers, risk factors for melanoma flow, tissue hypoxemia, and necrosis, as well as
include intense sun exposure with a history production of oxygen-free radicals and nitrous
of childhood sunburns. Other risk factors oxide. Shock, immunosuppression, depression
include fair skin, family history of melanoma, of myocardial function, and failure of multiple
multiple nevi, ethnicity (white vs. black), regular organs leading to death are consequences of the
use of tanning beds before age 30, and immuno- fascial inflammatory process. This condition is
suppressive therapy.17 Because this condition is classified into two types. Type 1 is a polymicro-
highly curable by surgical excision during the bial infection caused by aerobic and anaerobic
early stages and is usually fatal if undetected microbes (eg, Clostridium and Bacteroides
until the later stages when the abnormal cells species). Type 2 necrotizing fasciitis is caused by
penetrate the dermis and potentially metasta- group A Streptococcus, with or without Staphy-
size, early detection and expeditious treatment lococcus.20 Individuals with diabetes mellitus,
are necessary for optimal chances of survival. immunosuppression (eg, related to acquired
Therefore, individuals with any dermatologi- immunodeficiency syndrome, malignancy, and
cal changes suspicious of this condition should chronic corticosteroid use), perirectal abscess,
be immediately referred to a primary care obesity, malnutrition, hypertension, or smok-
physician or dermatologist. Physical therapy ing history are predisposed to developing this
intervention for wound management is not condition.32,33 Survival depends on immediate
indicated. diagnosis and treatment, consisting of wide-
spectrum intravenous antibiotics and thorough,
■ Necrotizing Fasciitis immediate debridement of all infected tissue.
Chief Clinical Characteristics Any individual suspected of having this condi-
This presentation involves a wound that is char- tion should be referred immediately to an
acterized by erythema and edema without de- emergency department or transported by emer-
marcated borders; severe pain; tense shiny skin gency medical services if needed.34 Standard
progressing to ischemic skin as tissue necrosis ad- wound care is indicated only after surgical
vances; clear or hemorrhagic blisters, progress- debridement and medical management have
ing to bullae filled with gray odiferous fluid been initiated.
(dishwater pus); separation of necrotic tissue
along fascial planes with myonecrosis; high ■ Neuropathic Wounds
fever and chills; decreased urinary output; Chief Clinical Characteristics
change in mental status; weakness and fatigue; This presentation may involve blisters, calluses,
and dry black eschar in advanced disease. punctures, skin tears, or cracks, usually on the
plantar surface of the foot, over the first and fifth
Background Information
metatarsal heads, on the distal digits, or in the
Necrotizing fasciitis usually occurs on the
interdigital spaces (Fig. 40-10).
extremities, especially the lower ones. The
abdominal wall, groin, perianal area, and post- Background Information
operative wounds are also common sites of This condition occurs as a result of abnormal
occurrence. If the affected area is the male gen- mechanical forces or minor trauma to the
italia, the disease is referred to as Fournier’s insensate foot. Bony abnormalities, diminished
gangrene.31 This condition is a life-threatening or absent protective sensation, decreased tissue
infection of the fascia underlying the skin and oxygen saturation, and anhidrosis caused by
subcutaneous tissue that is characterized by autonomic neuropathy in combination with
acute onset and rapid progression. It occurs poorly fitting shoes increase the risk of calluses
when bacteria enter the skin (from, eg, minor and blisters that become nonhealing wounds.
trauma, skin disorders, needlesticks, or any The most frequently associated medical comor-
existing ulceration) and elicit an intense inflam- bidity is type 2 diabetes mellitus; however,
matory response. The bacteria spread through- neuropathic wounds also occur in individuals
out the underlying tissue, causing release of with Hansen’s disease, spinal cord injury, spina
1528_Ch40_812-832 07/05/12 2:10 PM Page 826

826 Chapter 40 Osteomyelitis


FAILURE OF WOUNDS TO HEAL

FIGURE 40-10 Neuropathic wound. Wound caused


by mechanical trauma on a foot with sensory neu-
ropathy, most commonly seen in patients with diabetes.
FIGURE 40-11 Any wound that can be probed to
bone is suspicious of having osteomyelitis.
bifida, and other neuropathic conditions that
may be associated with vascular pathology. radiographs, magnetic resonance imaging,
This condition is best treated by redistribution radionuclide bone scan, or computed tomog-
of the foot pressures to eliminate the mechan- raphy. Therefore, immediate referral to the
ical forces causing the wound and with standard primary care physician or orthopedist is ad-
wound care. The most common complication vised. Local wound management, including
is infection, at which time the patient should irrigation, debridement, and absorbent antimi-
be referred to a primary care physician. crobial dressings, is indicated for immediate
■ Osteomyelitis care. The use of occlusive dressings is not
recommended while the infection is active.
Chief Clinical Characteristics Offloading of the involved area with accom-
This presentation includes pain, edema, ery- modative dressings, adaptive shoes, and assis-
thema, and drainage; limited range of motion; tive devices will facilitate wound closure.
pain or tenderness during palpation or weight
bearing; or draining sinus under superficial gran- ■ Pemphigus
ulation tissue (Fig. 40-11). Other symptoms and Chief Clinical Characteristics
signs include fever, fatigue and malaise, and This presentation typically includes intraepi-
reluctance to use the involved extremity. dermal bullae of the skin and mucous mem-
Background Information branes, which present as flaccid bullae and
This condition begins as an inflammatory crusted erosions of the skin and mucosal mem-
response in the bone secondary to the presence branes, accompanied by skin or oral pain and
of pyogenic organisms, such as bacteria, fungi, pruritus. The blisters are flaccid and can be
or viruses. Subsequently, edema and leukocytic transient (differentiating this condition from
activity result in abscess formation; small bullous pemphigoid) and result in diffuse
terminal vessels in the bones thrombose; exu- crusts or scabs.
dates seal the canaliculi and extend into the Background Information
metaphysis, marrow cavity, and cortex; and This condition refers to a group of autoimmune
infection weakens the cortex, predisposing the diseases. The blisters that characterize this con-
bone to fracture.6 When this condition is asso- dition are caused by binding of the antibodies
ciated with chronic wounds, it usually occurs to adhesion proteins in the epidermis and/or
in conjunction with neuropathic ulcers where mucosal membranes.35 Two prototypical pem-
the soft tissue between the skin and bone is less phigus diseases are pemphigus vulgaris and
dense. This condition should be suspected pemphigus foliaceus, both of which are chronic
in any chronic wound that can be probed disorders requiring long-term treatment. Indi-
to the bone.33 The diagnosis is confirmed by viduals suspected of having this condition
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Chapter 40 Pressure Ulcers 827

should be referred to a dermatologist for med- ● Stage V: Purple or maroon localized area of

FAILURE OF WOUNDS TO HEAL


ical management. The primary complication is discolored intact skin or blood-filled blister
infection of the open wounds. due to damage of underlying soft tissue
from pressure and/or shear. The area may
■ Pressure Ulcers be preceded by tissue that is painful, firm,
Chief Clinical Characteristics mushy, boggy, warmer or cooler as com-
This presentation varies based on the extent of pared to adjacent tissue. Further descrip-
tissue involvement, including the following tion: Deep tissue injury may be difficult to
six stages: detect in individuals with dark skin tones.
Evolution may include a thin blister over a
● Stage I: Intact skin with nonblanchable red- dark wound bed. The wound may further
ness of a localized area usually over a bony evolve and become covered by thin eschar.
prominence. Darkly pigmented skin may not Evolution may be rapid, exposing addi-
have visible blanching; its color may differ tional layers of tissue even with optimal
from the surrounding area. Further descrip- treatment (Fig. 40-12E).
tion: The area may be painful, firm, soft, ● Stage VI: Full-thickness tissue loss in which
warmer or cooler as compared to adjacent tis- the base of the ulcer is covered by slough
sue. Stage I may be difficult to detect in indi- (yellow, tan, gray, green, or brown) and/or
viduals with dark skin tones. May indicate eschar (tan, brown, or black) in the wound
“at-risk” persons (Fig. 40-12A). bed. Further description: Until enough slough
● Stage II: Partial-thickness loss of dermis pre- and/or eschar is removed to expose the base of
senting as a shiny or dry shallow open ulcer the wound, the true depth and, therefore, stage
with a red-pink wound bed, without slough or cannot be determined. Stable (dry, adherent,
bruising. Also may present as an intact or intact without erythema or fluctuance) eschar
open/ruptured serum-filled blister. This stage on the heels serves as “the body’s natural
should not be used to describe skin tears, tape (biological) cover” and should not be re-
burns, perineal dermatitis, maceration, or moved (Fig. 40-12F).36
excoriation (Fig. 40-12B).
● Stage III: Full-thickness tissue loss. Subcuta- Background Information
neous fat may be visible but bone, tendon, or Lesions occur over bony prominences where
muscle is not exposed. Slough may include there is minimal soft tissue to absorb the exter-
undermining and tunneling. Further descrip- nal forces that are applied to the skin, such as
tion: The depth of a Stage III pressure ulcer the sacrum, ischial tuberosities, greater
varies by anatomical location and subcuta- trochanters, heels, malleoli, medial femoral
neous tissue. Stage III ulcers can be shallow. In condyles, elbows, scapular spine, and vertebrae.
contrast, areas of significant adiposity can This condition results primarily from four
develop extremely deep Stage III pressure causative factors: shear forces on subcutaneous
ulcers. Bone/tendon is not visible or directly tissue, friction of the skin against a support
palpable (Fig. 40-12C). surface, external pressure that is greater than the
● Stage IV: Full-thickness loss with exposed capillary closing pressure, and excessive moisture
bone, tendon, or muscle. Slough or eschar may resulting in skin maceration. Because subcuta-
be present on some parts of the wound bed. neous tissue damage can occur before any
Often includes undermining and tunneling. external skin necrosis is visible, this condition is
Further description: The depth of Stage IV usually more extensive than initially visible with
pressure ulcers varies by anatomical location. skin inspection. Risk factors include immobility,
The bridge of the nose, ear, occiput, and malle- joint contractures, decreased or absent sensa-
olus do not have subcutaneous tissue and these tion, age, poor nutrition, incontinence, and
ulcers can be shallow. Stage IV ulcers can psychosocial factors (eg, altered mental status,
extend into muscle and/or supporting structures depression, stress, polypharmacy, drug and
(eg, fascia, tendon, or joint capsule), making alcohol abuse, and poor social support systems).
osteomyelitis possible. Exposed bone/tendon Standard care begins with offloading or reducing
is visible or directly palpable (Fig. 40-12D). the pressure to the affected area using specialty
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828 Chapter 40 Pyoderma Gangrenosum


FAILURE OF WOUNDS TO HEAL

A B

C D

E F
FIGURE 40-12 Pressure wounds: (A) Stage I on the greater trochanter; (B) Stage II on the sacrum; (C) Stage
III on the gluteal region; the dark purple area on the sacrum is a suspected deep tissue injury; (D) Stage IV
on the greater trochanter extends to the fascia and muscle; (E) suspected deep tissue injury on the lateral
malleolus; (F) wound on the heel that is considered an unstageable wound because the wound bed cannot
be visualized.

support surfaces and periodic repositioning, often coalesce to become a larger ulceration.
along with debridement of necrotic tissue The initial wounds may develop spontaneously
and moist wound dressings. If this condition or in response to minor trauma or to an oper-
is extensive, medical referral is indicated. ative procedure (pathergy) (Fig. 40-13).37
Complications include soft tissue infection,
osteomyelitis, and impaired mobility. Background Information
Usually occurring on the trunk or lower
■ Pyoderma Gangrenosum extremities, this condition occurs in conjunc-
Chief Clinical Characteristics tion with several systemic diseases, including
This presentation includes sterile pustules and irritable bowel syndrome, rheumatoid arthri-
rapidly progresses to painful, purplish, suppu- tis, hematologic diseases, or malignancies.38
rative, undermined ulcers. Smaller pustules will The exact pathogenesis is unknown; therefore,
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Chapter 40 Squamous Cell Carcinoma 829

Referral to a physician is indicated if there

FAILURE OF WOUNDS TO HEAL


are complications, including ulceration and
gangrene, in which case amputation may be
necessary.
■ Sickle Cell Disease
Chief Clinical Characteristics
This presentation typically includes lower
extremity wounds on the malleolar sites, hemo-
siderosis, prominent superficial veins, and lower
extremity edema. This condition may cause
FIGURE 40-13 Pyoderma gangrenosum usually complications in multiple organ systems, includ-
occurs in immunocompromised patients. ing stroke, skin ulcers, and blindness.
Background Information
this condition is a diagnosis of exclusion. This condition involves abnormal red blood
Biopsy results may include an interstitial neu- cell morphology that causes them to become
trophilic infiltrate expanding into the subcuta- rigid and sticky, disrupting blood flow to
neous tissue.39 Immediate medical referral bones, and resulting in painful bone infarcts.
is advised for treatment of any underlying Wounds are most common in individuals
systemic disease, immunosuppressive therapy, who are homozygous for this condition. The
anti-inflammatory medications such as corti- pathophysiology of wounds includes venous
costeroids, cytotoxic therapy, and pain manage- hypertension and vaso-occlusion that leads to
ment. Unlike the majority of wounds with skin infarct.38 This condition may be compli-
necrotic tissue, debridement is contraindicated cated by lower oxygen saturation levels due
because it causes further pathergy.40 Local to fewer red blood cells being oxygenated.
wound management includes topical antibiotic There is no cure for this condition; treatment
creams, topical steroids, and moisture-retentive is palliative and preventive. Local wound care
dressings. Use of sterile technique with dressing includes debridement, moist wound dressings,
changes is recommended to prevent infection, and compression therapy.42 Individuals with
the most common complication. this condition who have nonhealing wounds
are advised to see a hematologist for treatment
■ Raynaud’s Disease including blood transfusions and chelation of
Chief Clinical Characteristics high iron levels as a result of the transfusions.43
This presentation may involve pallor, numbness,
and cold sensations of the fingers and, less com- ■ Squamous Cell Carcinoma
monly, of the toes. As this condition progresses, Chief Clinical Characteristics
symptoms may include rubor, throbbing, and This presentation commonly involves gray to
paresthesias. yellow-brown hyperkeratosis and may progress
Background Information to crusty wart-like lesions that sometimes
The cause of this condition is vasospasm of the bleed, irregular red scaly patches, persistent
small arteries and arterioles, causing hypoxia of open wounds that crust or bleed, or raised
the digits, beginning with the tips and advanc- lesions with indented centers.44 This condition
ing to the proximal phalanges. Attacks are trig- is a malignant proliferation of the keratinocytes
gered by cold exposure or by emotional stress. in the epidermis (Fig. 40-14).45
This condition is more prevalent in women, Background Information
and it also can be a manifestation of collagen Squamous cell carcinoma most commonly
vascular diseases (eg, scleroderma and systemic occurs in areas that are exposed to the sun or in
lupus erythematosus), pulmonary hyperten- areas where the skin has been previously injured
sion, thoracic outlet syndrome, myxedema by burns, trauma, radiation, diabetes, chronic
trauma, serum sickness, or long-term exposure osteomyelitis, or chronic venous insufficiency.
to cold or vibrating machinery during work.41 Individuals suspected of having this condition
There is no indication for local wound care. should be referred to a primary care physician or
1528_Ch40_812-832 07/05/12 2:10 PM Page 830

830 Chapter 40 Traumatic Injury

black necrotic dots on the fingers and toes


FAILURE OF WOUNDS TO HEAL

(Fig. 40-15).46
Background Information
This condition is caused by an inflammation
of blood vessels, usually as a result of an
immune reaction in the vessel wall. This con-
dition may be cutaneous, involving the post-
capillary venules, or systemic, involving any
of the organs. Symptoms vary according to
the type of tissue involved and the extent of
the tissue involvement. Wounds related to
this condition frequently occur in conjunc-
FIGURE 40-14 Squamous cell carcinoma.
tion with other autoimmune connective
tissue diseases (eg, systemic lupus erythe-
dermatologist. Physical therapy intervention for matosus, scleroderma, rheumatoid arthritis,
wound management is not indicated. Wegener’s granulomatosis, temporal arteri-
■ Traumatic Injury tis, and cryoglobulinemia) or with some
malignancies (eg, leukemia or lymphoma).43
Chief Clinical Characteristics This condition also can occur without any
This presentation may take on many different obvious associated infection or other illness.
appearances depending on the mechanism of Referral to a medical specialist is recom-
injury and will follow a normal healing mended for diagnosis and interventions that
sequence unless untreated comorbidities exist may include immunosuppressants, systemic
(eg, diabetes, immunosuppressive conditions, corticosteroids, cytotoxic drugs if corticos-
protein energy malnutrition, or infection). An teroids are ineffective, and identification and
embolic or thrombotic arterial occlusion after treatment of any underlying infection. 47
a traumatic event will cause absent pulses, pain, Wound management includes standard care
paresthesias, loss of skin color, and possible and compression therapy if the wounds are
paralysis distal to the occlusion. on the lower extremity.
Background Information
The occlusion may result from an embolus that
breaks away from a proximal hematoma and
lodges in a peripheral artery or from a thrombus
that forms at the site of injury, leading to periph-
eral tissue ischemia that must be treated emer-
gently to avoid tissue necrosis and possible loss
of limb. Immediate referral to an emergency
department is indicated if hemostasis is not
achieved within 30 minutes, or if the individual
demonstrates symptoms and signs of acute
thrombosis. Referral to a primary care physician
is also recommended if a post-traumatic wound
fails to heal in a timely sequence. Physical
therapy intervention is indicated after normal
circulation has been restored.
■ Vasculitis
Chief Clinical Characteristics
This presentation typically includes palpable
purpura, petechiae, urticaria, painful or
tender nodules, patches of skin necrosis FIGURE 40-15 Vasculitis on the lower extremity of
(cutaneous necrotizing vasculitis), or small a patient with polyarteritis nodosa.
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Chapter 40 References 831

References 22. Lin P, Phillips TJ. Kaposi’s sarcoma. Wounds. 2001;

FAILURE OF WOUNDS TO HEAL


1. Lazarus GS, Cooper DM, Knighton DR, et al. 13(6):237–240.
Definitions and guidelines for assessment of wounds 23. Levinson KL, Feingold E, Ferrell RE, Glover TW,
and evaluation of healing. Arch Dermatol. Apr 1994; Traboulsi EI, Finegold DN. Age of onset in hereditary
130(4):489–493. lymphedema. J Pediatr. Jun 2003;142(6):704–708.
2. Enoch S, Harding K. Wound bed preparation: the 24. Rockson SG. Diagnosis and management of lym-
science behind the removal of barriers to healing. phatic vascular disease. J Am Coll Cardiol. Sep 2
Wounds. 2003;15(7):213–229. 2008;52(10):799–806.
3. Huether SE, Kravitz M. Structure, function, and disorders 25. Cheville AL. Current and future trends in lym-
of the integument. In: Huether SE, McCance KL, eds. phedema management: implications for women’s
Understanding Pathophysiology. St. Louis, MO: Mosby; health. Phys Med Rehabil Clin N Am. Aug 2007;
1996:1084–1121. 18(3):539–553, x.
4. Hagood CO, Wilson JR. Necrotic wounds produced by 26. Snyder RJ, Stillman RM, Weiss SD. Epidermoid cancers
spider bites. In: Sheffield PJ, Smith APS, Fife CE, eds. that masquerade as venous ulcer disease. Ostomy Wound
Wound Care Practice. Flagstaff, AZ: Best Publishing; Manage. Apr 2003;49(4):63–64, 65–66.
2004:389–403. 27. Fallis BD. Neoplasia. In: Robbins SL, ed. Textbook of
5. Wilson JR, Hagood CO Jr, Prather ID. Brown recluse Pathology. 2nd ed. Philadelphia, PA: W. B. Saunders;
spider bites: a complex problem wound. A brief review 1964:103–133.
and case study. Ostomy Wound Manage. Mar 2005; 28. Trent JT, Kirsner RS. Herpesvirus infections and
51(3):59–66. herpetic wounds. Adv Skin Wound Care. Sep–Oct
6. Mourad LA, McCance KL. Alterations of musculoskele- 2003;16(5):236–243.
tal function. In: Huether SE, McCance KL, eds. Under- 29. Davison S, Lee E, Newton ED. Martorell’s ulcer revis-
standing Pathophysiology. St. Louis, MO: Mosby; ited. Wounds. 2003;15(6):208–212.
1996:1036–1070. 30. Friedman RJ, Rigel DS, Kopf AW. Early detection of
7. Gordon RM. Skin cancer: more than skin deep. malignant melanoma: the role of physician examination
Adv Skin Wound Care. Dec 2009;22(12):574–580; quiz and self-examination of the skin. CA Cancer J Clin.
581–582. May–Jun 1985;35(3):130–151.
8. Foundation TSC. About basal cell carcinoma. 31. Ribo JC, Boucher B, Merwath D, Olive J. Case report:
http://www.skincancer.org/basal/index.php. Accessed implications for a patient diagnosed with Fournier’s
April 7, 2003. gangrene. Wounds. 2002;14(9):340–347.
9. Garcia EJ. Infections of the leg that mimic malignancy. 32. Norton KS, Johnson LW, Perry T, Perry KH, Sehon
Infect Med. 1998;15(8):557–567. JK, Zibari GB. Management of Fournier’s gangrene:
10. Hanley EJ. Buerger disease (thromboangiitis obliter- an eleven year retrospective analysis of early recogni-
ans). http://www.medscape.com. Accessed August 27, tion, diagnosis, and treatment. Am Surg. Aug 2002;
2005. 68(8):709–713.
11. Lin MD, Phillips TJ. Bullous pemphigoid. Wounds. 33. Armstong DG, Boulton AJM, Jospeh WS, Lipsky BL.
2002;14(5):36–41. Diagnosing and treating osteomyelitis. Podiatry Today.
12. Chan L. Bullous pemphigoid. http://emedicine.med- 2003:8–12.
scape.com/article/1062391-overview, 2002. Accessed 34. Yuen JC, Feng Z. Salvage of limb and function in necro-
October 12, 2008. tizing fasciitis of the hand: role of hyperbaric oxygen
13. Barillo DJ, Paulsen SM. Management of burns to the treatment and free muscle flap coverage. South Med J.
hand. Wounds. 2003;15(1):4–9. Feb 2002;95(2):255–257.
14. Polizzotto MN, Bryan T, Ashby MA, Martin P. Sympto- 35. Woldegiorgis S, Swerlick RA. Pemphigus in the south-
matic management of calciphylaxis: a case series eastern United States. South Med J. Jul 2001;94(7):
and review of the literature. J Pain Symptom Manage. 694–698.
Aug 2006;32(2):186–190. 36. National Pressure Ulcer Advisory Panel. Pressure ulcer
15. Burkhart CG, Mian A. Calciphylaxis: a case report and stages revised by NPUAP. http://www.npuap.org/pr2.htm.
review of literature. Wounds. 1999;11(2):58–61. Accessed 15 December, 2009.
16. Hirschmann JV. Fungal, bacterial, and viral infections 37. Walusimbi M, Mannari RJ, Payne WG, Ochs D, Blue
of the skin. http://www.medscape.com Accessed ML, Robson MC. Pyoderma gangrenosum: case report
March 9, 2003. of novel treatment with topical steroid and silver sulfa-
17. Beitz JM. Coumadin-induced skin necrosis. Wounds. diazine. Wounds. 2002;14(6):227–229.
2002;14(6):217–220. 38. Wollina U. Clinical management of pyoderma gan-
18. Lin MD, Phillips TJ. Type II cryoglobulinemia. Wounds. grenosum. Am J Clin Dermatol. 2002;3(3):149–158.
2002;14(5):85–91. 39. Chakrabaarty A, Phillips TJ. Pyoderma gangrenosum.
19. Shah JB. Approach to commonly misdiagnosed wound Wounds. 2002;14(8):302–305.
and unusual leg ulcers. In: Sheffield PJ, Smith APS, 40. Paparone PP, Paparone PW, Paparone P. Post-traumatic
Fife CE, eds. Wound Care Pract. Flagstaff, AZ: Best pyoderma gangrenosum. Wounds. 2009;21(4):89–94.
Publishing; 2004:405–422. 41. National Institutes of Health. Questions and answers
20. Trent JT, Kirsner RS. Necrotizing fasciitis. Wounds. about Reynaud’s phenomenon. http://www.niams.nih.gov.
2002;14(8):282–292. Accessed August 28, 2005.
21. Sakakibara S, Tosato G. Regulation of angiogenesis 42. Hamm RL, Rodrigues J, Weitz IC. Pathophysiology and
in malignancies associated with Epstein-Barr virus multidisciplinary management of leg wounds in sickle
and Kaposi’s sarcoma-associated herpes virus. Future cell disease: a case discussion and literature review.
Microbiol. Sep 2009;4:903–917. Wounds. 2006;18(10):277–285.
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832 Chapter 40 References

43. Hahn BH. Lupus and vasculitis. http://www.lupus.org. 46. Lee J, Phillips TJ. Livedoid vasculitis. Wounds.
FAILURE OF WOUNDS TO HEAL

Accessed August 24, 2005. 2001;13(4):132–134.


44. Foundation TSC. About squamous cell carcinoma. 47. de Araujo TS, Kirsner RS. Vasculitis. Wounds.
http://www.skincancer.org/squamous/index.php. Accessed 2001;13(3):99–112.
April 7, 2003.
45. Dasgeb B, Ghosn S, Phillips T. Well differentiated squa-
mous cell carcinoma with verrucous clinical presenta-
tion. Wounds. 2005;17(3):67–72.
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CHAPTER41
Case Demonstration: Fatigue
■ Kim Levenhagen, PT, DPT, WCC ■ Chris A. Sebelski, PT, DPT, OCS, CSCS

NOTE: This case demonstration was developed being inactive and losing 20 lbs recently dur-
using the diagnostic process described in ing his illness. He has noted excessive sweat-
Chapter 4 and demonstrated in Chapter 5. The ing especially at night. Past medical history
reader is encouraged to use this diagnostic includes human immunodeficiency virus
process in order to ensure thorough clinical rea- (HIV; 8 years) and hospitalization for Pneu-
soning. If additional elaboration is required on mocystis carinii pneumonia 1 month ago.
the information presented in this chapter, please Prior to this recent bout of pneumonia, Alex
consult Chapters 4 and 5. was active as a self-employed business owner
and participated in triathlons. He has been
THE DIAGNOSTIC PROCESS off work for the past month following a hos-
pitalization for pneumonia. Since that
Step 1 Identify the patient’s chief concern.
episode of care, he has been too tired and
Step 2 Identify barriers to communication.
short of breath to ride his bike or run. His
Step 3 Identify special concerns.
goals are to return to work and eventually
Step 4 Create a symptom timeline and sketch
return to triathlon training. The employees
the anatomy (if needed).
at his business and his family have not been
Step 5 Create a diagnostic hypothesis list
informed of his diagnosis of HIV. His most
considering all possible forms of remote and
recent blood testing indicates CD4+ T lym-
local pathology that could cause the
phocytes 450 cells/mm3. Alex’s medication
patient’s chief concern.
includes the highly active antiviral reactive
Step 6 Sort the diagnostic hypothesis list
therapy (HAART) regimen (for the past
by epidemiology and specific case
8 months), which includes tenofovir, emtric-
characteristics.
itabine, ritonavir, and a daily multivitamin.
Step 7 Ask specific questions to rule specific
conditions or pathological categories less
likely.
Step 8 Re-sort the diagnostic hypothesis list Teaching comments: The U.S. Depart-
based on the patient’s responses to specific ment of Health and Human Services Panel on
questioning. Clinical Practices for Treatment of HIV Infec-
Step 9 Perform tests to differentiate among tion sets guidelines for the treatment of HIV
the remaining diagnostic hypotheses. including initialization of HAART regimen.
Step 10 Re-sort the diagnostic hypothesis list In the question of “when to start” this ther-
based on the patient’s responses to specific apy, the panel has been unable to reach a
tests. two-thirds consensus for their recommenda-
Step 11 Decide on a diagnostic impression. tions. The 2009 guidelines stated: “Based on
Step 12 Determine the appropriate patient cumulative observational cohort data demon-
disposition. strating benefits of antiretroviral therapy
in reducing [acquired immune deficiency
syndrome] AIDS- and non-AIDS-associated
morbidity and mortality, the Panel now
Case Description recommends antiretroviral therapy for
patients with CD4 count between 350 and
Alex is a frail 42-year-old male presenting
500 cells/mm3. This recommendation is made
with concerns of fatigue and generalized
with 50% of the Panel in agreement.”1
weakness. He attributes his symptoms to
833
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834 Chapter 41 Case Demonstration: Fatigue

STEP #1: Identify the patient’s chief concern. topic be explored. Provision of support via
● Fatigue/generalized weakness. Note: This open dialogue can make a small but signifi-
symptom is his primary concern; however, cant effect on adherence. Questions regard-
the diagnostic category list below includes ing the medication, its side effects, and the
pathologies that involve the symptoms of prescription dosage should be referred to
shortness of breath due to his comment Alex’s pharmacist.2
regarding his perceived limitation in daily ● Gender issues with communication. Thera-
activities. pists of a different gender than Alex may feel
less comfortable asking sensitive questions.
STEP #2: Identify barriers to communication.
Conversely, Alex also may feel less comfort-
● Inability to collect information due to the able divulging sensitive information to a
patient’s eagerness to return to work. Alex therapist of another gender.
may be less likely to share information that
will lead to a diagnostic impression with a
prognosis that is inconsistent with his goals.
Teaching comments: Alex is a “nondis-
● Social stigma related to the diagnosis of closer.” He has not informed his family,
HIV. The therapist may require additional friends, and/or colleagues of his diagnosis.
sensitivity to Alex’s perception of how his There is conflicting evidence in the literature
condition is received socially, particularly regarding the overall effect of disclosing HIV
because of the documented nondisclosure to status to select people and the reasoning
family and coworkers regarding the underly- behind disclosing versus nondisclosing. In a
ing diagnosis of HIV. In addition, therapists meta-analysis of the literature, a small but
should examine their own perceptions and statistical relationship was shown between
beliefs about HIV in order to minimize the social support and disclosing. Additionally,
potential impact on the ability of the thera- the authors of the meta-analysis postulated
pist to fully engage in the diagnostic process. that the act of nondisclosure was driven by
● Medication adherence. Adherence to the the stigma surrounding the disease.3
HAART medications is necessary for long-
term viral suppression, yet it is known that
adherence to HAART medications over a STEP #3: Identify special concerns.
long period of time is often compromised. ● Recent profound weight loss. The weight
Financial burden, psychosocial stigma, side loss, in combination with fatigue and re-
effects, and the recent change in health sta- ports of excessive sweating, suggests an un-
tus indicate that reconfirmation of adher- derlying systemic disease process that must
ence to the medication regimen is necessary be investigated.
at this time. With inconsistent adherence,
● Presence of HIV. The presence of HIV is a
there is an increased threat of development
caution secondary to the complicated med-
of a strain of the virus that is resistant to
ical course and medication regimen.
known agents. Due to the importance of
compliance with the regimen of medica- STEP #4: Create a symptom timeline and
tions, it is strongly recommended that this sketch the anatomy (if needed).

8 years 8 months 1 month


ago ago ago Today

Onset of HIV Initiated Hospitalized Initial


diagnosis HAART x3 days for physical
medication pneumonia therapy
regimen evaluation
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Chapter 41 Case Demonstration: Fatigue 835

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.
T Trauma T Trauma
Exercise Exercise
Pneumothorax Pneumothorax (unassociated with night
sweats and extreme weight loss)
I Inflammation I Inflammation
Aseptic Aseptic
Cardiomyopathy Cardiomyopathy (unassociated with night
sweats and extreme weight loss)
Chronic inflammatory demyelinating Chronic inflammatory demyelinating
polyneuropathy polyneuropathy (unassociated with night
sweats and extreme weight loss)
Inflammatory myopathies: Inflammatory myopathies:
● Dermatomyositis ● Dermatomyositis (unassociated with

night sweats and extreme weight loss)


● Inclusion body myositis ● Inclusion body myositis (age;

unassociated with night sweats and


extreme weight loss)
● Multifocal motor neuropathy ● Multifocal motor neuropathy (time course)

● Polymyositis ● Polymyositis (unassociated with night

sweats and extreme weight loss)


Pleural effusion Pleural effusion
Pleurisy Pleurisy
Systemic lupus erythematosus Systemic lupus erythematosus (gender,
presentation)
Septic Septic
Cardiomyopathy Cardiomyopathy
Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis
Pleurisy Pleurisy
Pleural effusion Pleural effusion
Pneumonia Pneumonia
M Metabolic M Metabolic
Diabetes mellitus polyneuropathy Diabetes mellitus polyneuropathy
Diabetic myopathy Diabetic myopathy
Medication toxicity/toxic myopathy Medication toxicity/toxic myopathy
Myasthenia gravis Myasthenia gravis (unassociated with night
sweats and extreme weight loss)
Va Vascular Va Vascular
Anemia Anemia
Arteriovenous malformation within the spine Arteriovenous malformation within the
spine (unassociated with night sweats
and extreme weight loss)
Atherosclerosis Atherosclerosis
Cardiomyopathy Cardiomyopathy
Transient ischemic attack/stroke Transient ischemic attack/stroke (time course)
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836 Chapter 41 Case Demonstration: Fatigue

De Degenerative De Degenerative
Deconditioning Deconditioning
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
● AIDS- and non-AIDS-related malignancy ● AIDS- and non-AIDS-related malignancy
● Colon ● Colon
● Hematologic ● Hematologic

● Kidney ● Kidney

● Lung ● Lung

● Pancreas ● Pancreas

● Prostate ● Prostate

● Spinal tumor ● Spinal tumor

Teaching comments: This listing of ma-


lignant primary tumors is due to the multiple
comments of the patient including recent
weight loss, night sweats, and generalized
weakness or fatigue. Complicating the picture
is that these same symptoms are common in
the presentation of HIV secondary to both the
disease course and the medications used to
treat the disease course.

Malignant Metastatic, such as: Malignant Metastatic, such as:


● AIDS- and non-AIDS-related malignancy ● AIDS- and non-AIDS-related malignancy
● Colon metastases ● Colon metastases
● Kidney metastases ● Kidney metastases
● Pancreas metastases ● Pancreas metastases
● Prostate metastases ● Prostate metastases
● Spinal metastases ● Spinal metastases

Teaching comments: Per the Panel on


Clinical Practices for Treatment of HIV Infec-
tion, “The incidence of non-AIDS malignancy
in HIV-infected subjects is higher than in
matched HIV-uninfected controls. Large cohort
studies of mostly patients receiving antiretro-
viral treatment have reported a consistent
link between low CD4 counts (<350–500
cells/mm3) and the risk of AIDS- and/or non-
AIDS-defining malignancy. The ANRS C04
demonstrated a statistically significant relative
risk of all cancers evaluated (except for anal
carcinoma) in patients with CD4 counts <500
cells/mm3 compared with patients with
current CD4 counts >500 cells/mm3 and a
protective effect of antiretroviral therapy for
HIV-associated malignancies.”1
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Chapter 41 Case Demonstration: Fatigue 837

Benign: Benign:
Not applicable Not applicable
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Depression Depression
Neurological compromise due to acquired Neurological compromise due to
immunodeficiency virus acquired immunodeficiency virus
(unassociated with night sweats and
extreme weight loss)
Peripheral /myopathy due to HIV Peripheral neuropathy/myopathy due
to HIV

Teaching comments: Severity of depres-


sion and anxiety in patients with a diagnosis
of HIV are more related to the magnitude
of symptomatology rather than to the degree
of immunodeficiency or the number of oppor-
tunistic infections.4

STEP #7: Ask specific questions to rule Teaching comments: This is not in a der-
specific conditions or pathological matome pattern. The clinician should consider
categories less likely. pathologies with dermatome-type sensory
● Do you have shortness of breath at rest? “No,
changes as less likely than others. Oftentimes
I become short of breath and exhausted with the initial presentation of diabetes mellitus or
any activities that requires a lot of effort such peripheral neuropathy due to disease pro-
as climbing the stairs. I can catch my breath gression is a change of sensation at the
after sitting down for a few minutes.” This re- most distal aspects of the extremity. The clini-
sponse makes less likely primary cardiopul- cian should be suspicious of protective sensa-
monary pathology and pulmonary disorders tion loss as assessed by Semmes-Weinstein
such as pneumonia-related pleurisy, pul- monofilament testing.
monary embolism, and pulmonary fibrosis.
● Do you have back pain? “No.” This response
makes less likely conditions affecting the lum- STEP #8: Re-sort the diagnostic
bar region, kidney pathology, and pneumonia. hypothesis list based on the patient’s
● Have you noticed a change in frequency, responses to specific questioning.
color, or urgency with urination or bowel T Trauma
function? “No.” This response makes less
Not applicable
likely conditions affecting the kidney, dia-
betes mellitus, the colon, the pancreas, or I Inflammation
the prostate. Aseptic
● Do you experience numbness or tingling? Pleural effusion (no shortness of breath or
“Yes, my toes and the bottoms of my feet back pain)
tingle; at times, so do my hands. I have no- Pleurisy (no shortness of breath)
ticed in the past couple of years that I do not Septic
like to walk barefoot due to the sensitivity of Cardiomyopathy (no shortness of breath)
my feet.” This response makes more likely Idiopathic pulmonary fibrosis (no shortness
sources of symptoms that are associated of breath)
with sensory changes. Pleural effusion (no shortness of breath)
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838 Chapter 41 Case Demonstration: Fatigue

Pleurisy (no shortness of breath or back pain) The patient answers questions with appro-
Pneumonia (no shortness of breath or priate affect, making less likely conditions
back pain) associated with depressed mood. There are
M Metabolic bruises throughout the upper and lower ex-
Diabetes mellitus polyneuropathy
tremities, but none appear on the trunk.
Diabetic myopathy
Upon specific questioning, Alex acknowl-
Medication toxicity/toxic myopathy
edges that recently he has been bruising
more easily. This finding, although nonspe-
Va Vascular cific, may increase the index of suspicion for
Anemia AIDS-associated lymphoma. Palpation of
Atherosclerosis painless, swollen lymph nodes in the neck,
Cardiomyopathy (no shortness of breath) chest, axillary, or groin regions can further
De Degenerative increase the index of suspicion. There are
Deconditioning signs of lipodystrophy, including thinning
of the face and arms and increased bulk at
Tu Tumor
the back of the neck, which is a nonspecific
Malignant Primary, such as: finding associated with HAART adherence
● AIDS- and non-AIDS-related malignancy of greater than 6 months.5
● Colon (no change in bowel function)
● Auscultation. Lungs: No crackles or wheez-
● Hematologic
ing noted, confirming the suspicion based
● Kidney (no signs of blood in urine)
on history that acute restrictive or obstruc-
● Lung (no shortness of breath, no back
tive pulmonary pathologies are less likely.
pain)
Heart sounds: No murmur or S4 noted, con-
● Pancreas (no change in bowel function,
firming the initial impression that primary
no back pain)
pathology of the myocardium is less likely.
● Prostate (no back pain, no change in

frequency/urgency in urination)
● Vitals. Heart rate: 88 beats per minute at
● Spinal tumor (no back pain)
rest. Respiratory rate: 15 breaths/min at rest.
Malignant Metastatic, such as:
Body temperature: 99.8°. Blood pressure:
● AIDS- and non-AIDS-related malignancy
136/88 SpO2: 95% at rest on room air.
● Colon metastases (no change in bowel
STEP #10: Re-sort the diagnostic
function) hypothesis list based on the patient’s
● Kidney metastases (no change in color
responses to specific tests.
of urine)
● Pancreas metastases (no back pain, no
T Trauma
change in bowel function) Not applicable
● Prostate metastases (no change in I Inflammation
frequency/urgency in urination) Aseptic
● Spinal metastases (no back pain)
Not applicable
Benign:
Septic
Not applicable
Not applicable
Co Congenital
M Metabolic
Not applicable
Diabetic myopathy
Ne Neurogenic/Psychogenic Medication toxicity/toxic myopathy
Depression Va Vascular
Peripheral neuropathy/myopathy due to HIV
Anemia
STEP #9: Perform tests to differentiate Atherosclerosis
among the remaining diagnostic
De Degenerative
hypotheses.
● Observation. Patient is not in apparent dis-
Deconditioning
tress sitting at rest in the clinic, making less Tu Tumor
likely conditions associated with anxiety. AIDS- and non-AIDS-related malignancy
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Chapter 41 Case Demonstration: Fatigue 839

C Congenital STEP #12: Determine the appropriate


Not applicable patient disposition.
● Initiate physical therapy treatment with
Ne Neurogenic/Psychogenic
consult to address diagnostic impression.
Depression
Peripheral neuropathy/myopathy due to HIV
STEP #11: Decide on a diagnostic Teaching comment: Physical therapy in-
impression. terventions are seldom prescribed in isolation
for patients receiving HAART. The complexity
● AIDS and non-AIDS malignancy vs. med-
of the disease process coupled with the side
ication toxicity.
effects of the medications results in the need
for a multidisciplinary approach including
Teaching comment: Although certain physicians, psychologists, nutritionists, and
conditions could not be ruled less likely, this therapies.
should not preclude the clinician from using this
process for differential diagnosis with a com-
plex patient case. The conditions of atheroscle-
rosis, anemia, and depression are common Case Outcome
secondary conditions associated with a diagno- The consultation with the referring physician
sis of HIV. The clinician should attempt to fit all included a discussion on the results of Alex’s
of the patient’s concerns to the pathological recently completed blood tests, the results of
condition. Though one or two of Alex’s concerns which demonstrated anemia and an elevated
may meet the patient presentation of athero- white blood count. These results, in combina-
sclerosis, anemia, or depression, not all do. Al- tion with Alex’s symptoms, necessitated
though deconditioning secondary to recent bed putting a hold on therapy services for 4 weeks
rest and illness with pneumonia could not be for further investigation of his health status.
completely excluded based on the history find- The patient was diagnosed with AIDS-related
ings, the patient’s report of extreme weight loss lymphoma.
(20 lbs over 1 month), fatigue with daily activi- After confirmation of the diagnosis, initia-
ties, and excessive sweating at night, in combi- tion of chemotherapy and completion of an
nation with the physical findings of low-grade exercise stress test, Alex resumed physical ther-
temperature and bruising on the extremities, in- apy.6 An aerobic training program was initi-
dicates the need for referral to exclude tumors ated that involved intermittent bouts of short-
and metabolic issues associated with the pa- duration exercise and rest to accommodate the
tient’s underlying HIV infection and treatment.6 “down days” that are often associated with
chemotherapy treatment.8 Alex’s goals were
reevaluated based on the results of the exercise
Teaching comment: Lipodystrophy is a stress test. Several studies have documented
collection of symptoms seen since the advent of improvements in both the physical and psy-
HAART. Symptoms include metabolic changes, chosocial status of patients with a diagnosis of
insulin resistance, glucose intolerance, diabetes, lymphoma or HIV/AIDS and the interven-
and hyperlipidemia. Physical signs may occur tions of aerobic and progressive resistance
due to the redistribution of adipose tissue such training. Outcome expectations from this
as increased visceral fat in the abdomen, episode of physical therapy care for Alex will
breasts, and neck; a “buffalo hump” at the include an increased rate of completing the
back of the neck; and a decrease of fat on the planned chemotherapy treatment session,8 an
extremities. These metabolic and fat changes improvement in patient self-report of quality-
increase the risk in this population of athero- of-life measures, and physical improvements
sclerosis and cardiac conditions such as myocar- such as improved peak oxygen consumption.8
dial infarction affirming the importance for vital An outline of suggested training regimens is
sign monitoring for safe exercise prescription.6,7 given here:
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840 Chapter 41 Case Demonstration: Fatigue

DIAGNOSIS PRESCRIPTION OUTCOME


Progressive HIV/AIDS Up to four sets of eight Improved strength and
resistance exercise repetitions at 70%–80% of 1 psychological status.
repetition maximum (RM) were
utilized. Large and small muscle
groups were involved.9
Aerobic training HIV/AIDS 20-minute sessions three times Improved cardiorespiratory
per week for 4 weeks. fitness and psychological
status.10
Progressive Lymphoma 8–10 exercises of major muscle Improved quality of life
resistance exercise groups involving 10–15 reported.11
repetitions of from one to three
sets, 2 to 3 days per week at
60%–70% of 1 RM.6,11
Aerobic training Lymphoma Upright or recumbent cycle Improved cardiorespiratory
ergometer three times per week fitness and psychological
for 12 weeks. Duration was up status.
to 20 minutes at 60% peak Did not interfere with
power output until week 4; chemotherapy or
45 minutes by week 9 at 75% completion rate.8
of peak power output.

References Neuromuscular, Neoplastic, Immunologic, and Hemato-


1. U.S. Department of Health and Human Services, Panel logic Conditions. 2nd ed. ed. Philadelphia, PA: Lippin-
on Clinical Practices for Treatment of HIV Infection. cott Williams & Wilkins; 2010.
Guidelines for the use of antiretroviral agents in HIV-1- 7. Gopal M, Bhaskaran A, Khalife WI, Barbagelata A.
infected adults and adolescents. http://aidsinfo.nih. Heart disease in patients with HIV/AIDS—an emerging
gov/ContentFiles/AdultandAdolescentGL.pdf. Accessed clinical problem. Curr Cardiol Rev. May 2009;5(2):
May 17, 2010. 149–154.
2. Simoni JM, Amico KR, Smith L, Nelson K. Antiretroviral 8. Courneya KS, Sellar CM, Stevinson C, et al. Random-
adherence interventions: translating research findings to ized controlled trial of the effects of aerobic exercise on
the real world clinic. Curr HIV/AIDS Rep. Feb 2010;7(1): physical functioning and quality of life in lymphoma
44–51. patients. J Clin Oncol. Sep 20 2009;27(27):4605–4612.
3. Smith R, Rossetto K, Peterson BL. A meta-analysis of dis- 9. Dolan SE, Frontera W, Librizzi J, et al. Effects of a super-
closure of one’s HIV-positive status, stigma and social vised home-based aerobic and progressive resistance
support. AIDS Care. Nov 2008;20(10):1266–1275. training regimen in women infected with human im-
4. Perdices M, Dunbar N, Grunseit A, Hall W, Cooper DA. munodeficiency virus: a randomized trial. Arch Intern
Anxiety, depression and HIV related symptomatology Med. Jun 12, 2006;166(11):1225–1231.
across the spectrum of HIV disease. Aust N Z J Psychia- 10. Lucia A, Earnest C, Perez M. Cancer-related fatigue:
try. Dec 1992;26(4):560–566. can exercise physiology assist oncologists? Lancet Oncol.
5. Anuurad E, Bremer A, Berglund L. HIV protease in- Oct 2003;4(10):616–625.
hibitors and obesity. Curr Opin Endocrinol Diabetes Obes. 11. Cramp F, James A, Lambert J. The effects of resistance
Oct 2010;17(5):478–485. training on quality of life in cancer: a systematic litera-
6. American College of Sports Medicine, ed. ACSM’s ture review and meta-analysis. Support Care Cancer.
Resource for Clinical Exercise Physiology: Musculoskeletal, Nov 2010;18(11):1367–1376.
1528_Ch42_841-850 07/05/12 2:11 PM Page 841

SECTION IV
Children

CHAPTER 42
Special Diagnostic Issues in Children
■ Sharon K. DeMuth, PT, DPT, MS ■ Hugh G. Watts, MD

IN THIS CHAPTER: Introduction


■ Special features of history taking in children

■ Special features of the physical examination “Children are not small adults.”
in children This is a well-known, but often overlooked
■ Unique features of bone growth and injury
maxim. When pointed out, it is so obvious that
writing about it may seem unnecessary, but it
OUTLINE bears emphasis and should be kept in mind
Introduction 841 throughout the various discussions in this
Special Features of History Taking in chapter.
Children 841 Pediatric practice like all physical therapy
The Age of a Child is the Defining Feature in practice is constantly evolving. With the adop-
Differential Diagnosis 841 tion of Vision 2020 by the American Physical
The History is Generally Obtained Secondhand Therapy Association, physical therapists are
Through the Parent 842 expected to practice as doctors of physical ther-
Obtaining Information About a Child’s apy.1 Pediatric physical therapists serve children
Development 842 and their families in many environments where
Some Pitfalls 843 they may be the initial health care provider
Never Forget the Possibility of Child Abuse 844 evaluating a child’s or infant’s ability to move.
Special Features of the Physical Because pediatric physical therapy practice is
Examination of a Child 844 the only physical therapy practice that is feder-
Get to the Child’s Level 844 ally mandated (IDEA),2 the expectation that
Watch While the Child is Waiting to Be Seen 844 pediatric physical therapists will recognize signs
Potential Problems with Disrobing 844 and symptoms and accurately diagnose patients
Examine the Painful Area Last 845 as part of providing those services is crucial.
Confirmation of Discrepancies 845
Examining the Child to Obtain Information About Special Features of History Taking
the Child’s Development 845 in Children
Issues Related to Growth of Bones in
Children 846 The Age of a Child is the Defining
Growth of the Long Bones 846 Feature in Differential Diagnosis
Growth Disturbances Due to Abnormal Muscle
Many diseases only occur at specific ages or are
Activity 846
extremely rare at other ages. For example,
Leg-Length Discrepancy 846
Legg-Calvé-Perthes disease more commonly
Differences in Fractures Between Children occurs in the hip of boys ages 4 to 6, while a
and Adults 846 slipped capital femoral epiphysis would be
Epiphyseal Fractures 846
more commonly expected in a boy ages 12 to
Physiological Differences 848
15. If a boy presents with increasing weakness
Mechanical Differences 848
at age 7 or 8, Duchenne muscular dystrophy is
Other Considerations 849
a more likely diagnosis than spinal muscular
Conclusion 849 atrophy III.
841
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842 Chapter 42 Special Diagnostic Issues in Children

Exact age is important to establish. In if the question is asked in simple language and
some cultures, for example, in Saudi Arabia the answer interpreted with wisdom. If the
where birthdays are not celebrated, a child’s child states that she has a “tummy ache” and
exact age is not well documented. The ages of when asked to point to where it hurts she
children adopted from another country are of- points to her head, this information can be
ten only estimates. This has been the case in helpful.
children from orphanages in Romania, Russia, Asking a child questions may also give
and some parts of China. A healthy skepticism you important information about how the
is needed if such a child does not fit within the family interacts. Does the parent constantly
expected range of norms. Bone age radi- interrupt or insist on interpreting? If so, it is
ographs and standardized developmental test- important to give the child a chance to speak
ing may help to determine an approximate for herself and reassure the parent that he
age. Additionally, infants who are born prema- will get his chance to give information in a
turely (more than 3 weeks early) are evaluated minute. If the child always looks at the par-
based on “adjusted age” until they are 2 years ent first as if looking for permission to speak,
old. This means that a child born 2 months this can also provide an indication about the
early, who has now lived 4 months since deliv- family dynamics.
ery, would be regarded as a 2-month-old in
terms of developmental performance, not as a Obtaining Information About
4-month-old.3 a Child’s Development
A physical therapist may be the first health
The History is Generally Obtained care provider to interact with some children
Secondhand Through the Parent especially in settings such as “early interven-
tion” programs (serving children from birth
Especially when children are young, parents
to 3 years of age) or the school system.
provide all the information. It is easy to over-
The child may not have received a formal
look the fact that the information provided by
assessment from a primary care physician
a parent may be misinterpreted by the health
or pediatrician and the family may not have
care provider.
access to any health care services. Again the
PARENTS (OR THEIR SURROGATES) MAY age of the child and the setting direct the
BRING ASSUMPTIONS TO THE CLINICIAN evaluation.
“My child hurts when she....” This may be For an infant, activities such as feeding and
true, but it may not be true. Such a statement sleeping should be assessed as well as the in-
may well set you on the wrong track when fant’s gross- and fine-motor abilities. It is also
developing your list of possible diagnoses. important to screen the infant’s vision and
Cultural background may pose additional hearing. An infant with torticollis may keep
challenges, related to assumptions about dis- the head turned to one side because of a visual
ease etiology, privacy customs, and language loss as well as a tight sternocleidomastoid
barriers.4 muscle. Infants with hypotonia or hypertonia
The clinician should search for the original may have difficulty feeding. All infants go
problem that concerned the parent. For exam- through periods in their development when
ple, if parents state that they want their child they cry more than other periods, and an in-
evaluated for a leg-length difference, the clini- fant who has a central nervous system injury
cian may focus on that particular issue when may be hard to console or get to sleep, increas-
in reality the problem the parent initially was ing strain on the family and the risk for child
concerned about was a limp that a friend or abuse. Older children with known diagnoses
grandparent might have suggested was due to such as cerebral palsy or myelodysplasia may
the unequal leg length. be underweight or overweight and may need a
The clinician should try to get as much referral to a pediatrician and or a registered
history as possible from the child. Even a dietician in order to help the family provide
2-year-old can provide valuable information better care for the child.
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Chapter 42 Special Diagnostic Issues in Children 843

In the early intervention setting, the focus pediatrician, pediatric neurologist, or pedi-
is on the family5 and the use of open-ended atric orthopedist. If the family has no access to
questions such as “Tell me about your baby’s health care services, should they be referred to
typical schedule,” “What types of activities a social worker or to a state system such
does the baby enjoy?” and “Does the baby as Medicaid? The family may be unwilling to
sleep through the night?” may elicit more in- accept that there is anything really wrong with
formation than a question requiring only a the infant or child and it may take a number of
yes or no answer. Open-ended questions also visits by the physical therapist and/or referral
work well with older children in the pre- to another health care provider to help the
school or school setting because they allow family accept and begin to cope with their
the physical therapist to begin to assess the child’s diagnosis.
child’s cognitive ability and interests while
getting acquainted with the child and the Some Pitfalls
family and developing a mental list of poten-
A HISTORY OF A FALL IS ALWAYS SUSPECT
tial diagnoses.
Children fall frequently so it is easy to ascribe
Knowing when the child accomplished
the cause of some problem to a fall. If a
certain motor skills such as rolling over (4
mother notes a swollen knee when her
to 5 months), moving alone from lying
3-year-old is being given a bath, she may well
to sitting up (6 to 7 months), walking (12 to
think that it is due to the fall off of the sofa
15 months), and reaching for objects (3 to
she witnessed 2 days ago. On the other hand,
4 months) is important.5 However, some
the swollen knee could be due to juvenile
parents will be better historians than others.
rheumatoid arthritis. If there is a history of a
Often with a first child the family may be
fall, it is important to get the details. Was the
able to tell you exactly what happened but
child able to get up and walk or run right
they may not be able to give the same details
away? Was the crying inordinately prolonged?
for the fourth child.
Was there a wound or scrape? These details
In addition, certain cultures place value on
will give a better understanding of the seri-
certain motor activities, for example, inde-
ousness of the fall.
pendence in dressing, and will encourage those
activities. For this reason, the timeline for ac- A HISTORY OF FEVER ALWAYS NEEDS FULL
complishment of certain tasks will be different DETAILS
for different infants. For example, in some Children in their initial school years frequently
African countries children are not allowed or bring upper respiratory infections home with
encouraged to crawl but are carried until they them. These fevers can easily confuse the diag-
can walk alone, a task often achieved at 8 or nostic problem. A parent may, for example, in-
9 months of age.5 advertently overlook an important fever that
The “back to sleep movement,” which has heralds osteomyelitis by ascribing the fever to
been endorsed by the American Academy of a “cold.” Did the child have a runny nose or a
Pediatrics and others since 1966, encourages cough?
parents to place babies face up to sleep to
reduce the incidence of sudden infant death “TICKET OF EXIT” ISSUES
syndrome. This has altered the expected age Children often develop a strong wish to avoid
for accomplishment of crawling and walking unpleasant social situations by inventing ill-
by decreasing prone experiences.6,7 nesses. Gym class at school is commonly an
The clinician uses this knowledge about activity a child wants to avoid. A preteen girl
whether a child accomplished motor skills who is embarrassed about her delayed physical
within a range of expected norms to assess if development in comparison to her classmates
the child is behaving appropriately for his or may not want to participate in gym class if she
her age. If not, the clinician determines is required to change clothes in a locker room
whether the child needs to be referred to a spe- where she has to expose herself. A teenage boy
cialist such as a physical therapist who is a pedi- with gynecomastia may feel the same way.
atric certified specialist (PCS), developmental Unwilling to discuss the real issues with their
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844 Chapter 42 Special Diagnostic Issues in Children

parents, a backache or foot pain may become abuse is suspected. All local and state depart-
the means of getting an excuse from gym class. ments of health have systems in place for
Similarly, a child who is being pushed into reporting. It is incumbent on the physical
competing in an activity such as skating or therapist to know the procedures for the state
gymnastics may feel overwhelmed and look in which he or she is licensed to practice.
for a way to get out of the competition by
developing a malady. It may take particularly Special Features of the Physical
sensitive questioning to get to the root of such Examination of a Child
matters.
Get to the Child’s Level
THE “BY THE WAY” CONSULTATIONS
Parents often bring siblings along when one of Imagine yourself lying on an examining table
their other children is being seen in the clinic. in your underwear when a 10-foot-tall person
It is not unusual for the parent to take the op- comes in to examine you. That’s not unlike the
portunity to have one of the sibs evaluated at situation for a child. Lower yourself to the
the same time with the common “Oh, by the child’s level by sitting down or squatting.
way, what about my other child’s flat feet?” As
with any informal consultation (see Chapter 3), Watch While the Child is Waiting to
such interactions are fraught with potential Be Seen
problems. Time is limited since the appoint- Often the parent will bring a child in for eval-
ment has been scheduled for only one child; uation of a limp or in-toeing only to be frus-
the history is frequently hurried and superfi- trated when the child walks absolutely nor-
cial; and often no record of the visit is made. mally in front of the clinician. If possible, keep
While it is a nuisance for the mother to make a an eye on the waiting area and watch the chil-
separate appointment for the additional child, dren before you see them—that is, when they
your reasons for this need to be carefully are not putting on a studied gait for your eyes.
explained to the parent if disgruntlement is to Other tricks such as asking a child to rub his
be avoided. tummy with one hand while patting the top of
his head with the other hand, then asking him
Never Forget the Possibility of Child to walk, may fool the child into walking with
Abuse2 his more usual gait.
Child abuse can happen in what appear to be
Potential Problems with Disrobing
“the best” of families. Overlooking the possi-
bility may mean that the next time it happens, An adequate examination requires the child
the child may be badly maimed or, even worse, to disrobe. Feet and lower extremity prob-
killed. Question the details of any story that lems cannot be appropriately evaluated by
involves trauma, a fall off of a changing table, just pulling up the child’s trouser legs. Spinal
or a fall down the stairs. One does not need to deformities cannot be evaluated properly
sound accusatory while doing so. Look for without being able to see the child’s entire
bruises that appear to have different dates of trunk and pelvis. Many children are unhappy
occurrence—that is, some that are blue-black about undressing for an examination. They
while others are yellowish or greenish. Do the may be shy or afraid that this is the first step
bruises suggest finger or hand marks left to getting an injection. If this occurs, being
behind by a vigorous slap? Does the child raise arbitrary and insisting may lead to a totally
his arm as if to protect himself from a blow unsatisfactory examination. Some tech-
when you approach? For the school-age child niques that can work are to avoid eye contact
has there been a change in behavior at school, with the child and simply state to the parent
a change in academic performance, or fre- which clothes you want to have removed. It
quent absences that are unexplained? may also help to step out of room saying
Physical therapists, like all health care you’ll return when the child is ready for
providers, are mandated reporters if child examination.
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Chapter 42 Special Diagnostic Issues in Children 845

Examine the Painful Area Last of equipment such as a ride-on-toy or slide in


order to begin to “make friends” so you can
This seems obvious, but it is frequently perform your assessment.
forgotten. Evaluate all the normal areas first. To evaluate children successfully, you need
This may require a prior explanation to the to be familiar with the timing of typical devel-
parent who may think you are inept or forget- opmental activities. That information is be-
ful if they see you examining the left leg when yond the scope of this book but resources can
it is the right leg that is the problem. When be found in other excellent references.5,9–11
finally coming to the painful extremity, log The physical therapist is also expected
rolling the extremity gently should be done to provide accurate information about a
first before going to a range-of-motion child’s ROM, muscle strength, and functional
(ROM) evaluation. abilities. A child of 4 years can usually cooper-
ate with ROM testing and may even be able to
Confirmation of Discrepancies understand the concept of pushing against the
If in doubt about the extent or intensity of a therapist, allowing an active assessment of
pain, a child can be asked to roll prone. Exam- muscle strength by manual muscle testing
ine the opposite side as if it was the alleged (MMT). By 6 years of age children who are
painful side and ask the child if it hurts. cognitively at their appropriate age level
should be able to follow directions for ROM
and MMT. Therapists should be familiar with
Examining the Child to Obtain
changes in ROM at different ages5 and may
Information About the Child’s
need to be prepared to estimate muscle
Development
strength based on observation of various func-
As stated earlier, the physical therapist may be tional activities if the child is unable to coop-
the first health care provider a child has seen, so erate with a traditional examination.
there may not be a prior physician evaluation It is critical to be able to provide accurate
or history to review. In this situation it is im- records of strength and mobility for many
portant to try to estimate the child’s cognitive, childhood illnesses in order to determine if the
language, emotional, and self-help capabilities child is getting better or worse. The physical
as well as her motor skills. Is the child able to therapist usually has more time and more toys
talk to you? Can you understand what she is than other medical practitioners who only see
saying and does the vocabulary seem appropri- the child in an office and is thus able to gain a
ate for her age? You can expect that by 2 years better understanding of a child’s capabilities.
of age children will have a vocabulary of Providing care in the home or school settings
around 100 words.5 also allows the physical therapist to under-
If the child is shy and will not talk to you, stand how the child and family function most
listen while he talks to the family or be pre- of the time. This information can be extremely
pared to try again later in the evaluation after important in making an accurate diagnosis
he has gotten used to you. If the child is old and will need to be shared with the child’s
enough, ask him to take off his clothing. Chil- other health care providers.
dren ages 3 to 4 years usually can manage most We cannot stress enough the importance
of this.8 This gives you a chance to see how of the physical therapist in assessing for signs
they problem solve and it allows you to start to of child abuse. Look for the signs described
generate ideas about areas to focus on during above: bruises that appear to have different
your physical exam. If the child cannot remove dates of occurrence; bruises that suggest finger
her shoes or socks, if she cannot unbutton but- or hand marks left behind by a vigorous slap;
tons, or sit alone to even attempt these activi- does the child raise his arm as if to protect
ties you are gaining a lot of valuable informa- himself from a blow when you approach. Look
tion. This may be another area where cultural for additional signs of the loss of previously
expectations of independence differ. If the accomplished skills such as toilet training or
child appears very frightened, it might be help- problems with nightmares and excessive
ful to allow her to play with a toy or use a piece weight gain or loss.
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846 Chapter 42 Special Diagnostic Issues in Children

Issues Related to Growth of Bones healing with overlap or lengthening due to the
in Children stimulation of the healing fracture causing
longitudinal overgrowth. Other causes are dis-
Growth of the Long Bones location of the hip, osteomyelitis, traumatic
physeal injury, Legg-Calvé-Perthes disease,
This growth takes place at the physis, known in and congenital conditions.
the past more commonly as the epiphyseal The concern about leg-length discrepancy
growth plate. Abnormalities in longitudinal varies with the specialty of the person evaluating
growth in children most frequently result from the child—the more specialized a clinician, the
trauma, infection, or loss of vascular supply. less concerned he or she is about the lesser de-
On the other hand, growth can be stimulated, grees of leg-length difference. Pediatric orthope-
presumably due to increased blood supply, as a dic surgeons are not concerned with a leg-length
result of fracture healing and/or chronic in- difference if, at maturity, the difference will be
flammation as seen in juvenile rheumatoid less than 2 cm.
arthritis. The main issue with leg-length differ-
Differential inhibition of longitudinal ences in children is not the current differ-
growth (eg, medial versus lateral) may result ence, but what the difference will be at matu-
in angular deformity. This is commonly seen rity. A difference of 2 cm at the current time
after fractures of the physis medially at the may be of little concern if the difference is
ankle. Inhibition of growth of the physeal stable in absolute terms. However, if the
cells due to excessive asymmetric longitudi- difference is due to a congenital decrease in
nal loading is presumed to be the cause of longitudinal growth of the femur, for exam-
the progressive genu varum seen in Blount’s ple, a 10% difference in a 1-year-old boy, the
disease. ultimate difference at maturity could be an
additional 4 cm.
Growth Disturbances Due to The management of leg-length discrep-
Abnormal Muscle Activity ancy requires an adequate series of measure-
Bone growth responds to the muscle forces ap- ment data, usually at yearly intervals. Simple
plied to the bones. What is often overlooked is tape-measure evaluations are not adequate.
that the shape of the bones may be modified by Radiographs taken so that magnification is
the growth of the apophyses, for example, the eliminated are important (either scanograms
greater trochanter of the femur. In the past, or orthoroentgenograms). Because bone length
when poliomyelitis was much more common, norms have been established based on bone
the effects of lack of muscle pull on the growth age rather than calendar age, a simultaneous
in length and shape of the bones was seen fre- x-ray of the left hand is also taken and com-
quently by medical personnel caring for these pared to norms. Because of the importance of
children. Ewald and others12,13 showed that the having baseline measurements and appropri-
shape of the upper end of the femur, and espe- ate serial measurements, a child with this
cially the neck-shaft angle, was markedly de- problem should be given an early referral to an
creased in experimental animals when the hip orthopedic surgeon who is familiar with
abductor muscles were removed or defunc- leg-length discrepancies.
tioned. This effect is presumed to be the etiology
of the increased proximal femoral valgus seen in Differences in Fractures Between
children with cerebral palsy where their hip ab- Children and Adults
ductor muscles may be reciprocally inhibited by
the spasticity of the hip adductor muscles. Fractures in children are different from those
seen in adults in a number of ways.
Leg-Length Discrepancy
Epiphyseal Fractures
Causes for leg-length discrepancy are numer-
ous.14 Probably the commonest are due to the The most obvious anatomical difference is the
results of a fracture, either shortening due to presence of the physes (epiphyseal growth
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Chapter 42 Special Diagnostic Issues in Children 847

plates) responsible for longitudinal growth As with Salter-Harris I fractures, the line of
that are susceptible to injury. Furthermore, the separation along the physis is not always ex-
subarticular physis, which allows for the clusively at that level; however, the prolifer-
growth in width and in shape of the ends of ating cells are usually not injured, so growth
the bones, is also vulnerable to injury. disturbance is not a common feature of this
Nomenclature for fractures is not firmly fracture.
agreed on. Fractures that take place at the ● Salter-Harris III: In these fractures, the line
ends of the bones in children where the of the break goes across the zone of provi-
physis, the secondary center of ossification, sional mineralization, then turns toward the
and the subarticular physis may be involved joint and breaks the secondary center of
have generally been classified as epiphyseal ossification. It may, or may not continue
fractures. Although a number of different across the subarticular physis and articular
classification schemes are available, the most cartilage into the joint itself. If the fracture
commonly used is the Salter and Harris extends totally into the joint, the loose frag-
system (Fig. 42-1).15 ment can easily dislodge and result in an in-
● Salter-Harris I: In these fractures, the break congruous joint surface with long-term dire
takes place across the zone of provisional consequences for joint function and growth.
mineralization. Although the line of separa- Although the fracture extends along the
tion is not always exclusively at that level, zone of provisional mineralization initially,
the proliferating cells are usually not in- the turn toward the joint crosses the layer of
volved; hence, growth disturbance is not a proliferating cells in the physis with a con-
common feature. The issue with these frac- siderable probability of causing a growth
tures is recognition. There is no break in the disturbance. Such fractures often require
osseous tissue so there is nothing to be seen accurate replacement of the fragments and
on the bones on a plain radiograph. Deep fixation by surgery.
soft tissue swelling seen on the x-ray may be ● Salter-Harris IV: In this fracture, the break
the only clue. Recognition comes from the takes place at approximately right angles to
knowledge that such a lesion could take the physis, and extends from the metaph-
place and an understanding of the mecha- ysis into the joint. The free fragment con-
nism of injury. tains a piece of metaphysis, a segment of
● Salter-Harris II: In these fractures, the break the physis and of the bony secondary center
takes place across the zone of provisional of ossification.
mineralization, but as the fracture reaches Usually, the free fragment moves toward
the far side of the bone, the line of the break the joint, not only causing joint incongruity,
turns toward the metaphysis and leaves a but the bone of the metaphysis may heal
telltale small triangle of bone attached to the across to the bone of the secondary center of
physis. Because of this small fragment of ossification, which prevents the physis from
bone attached to the physis, the lesion is growing. Such fractures, seen most com-
usually readily recognized on an x-ray. monly at the ankle and elbow, result in an

A B C D E

FIGURE 42-1 Salter-Harris fracture classification: (A) Salter-Harris I; (B) Salter-Harris II; (C) Salter-Harris III;
(D) Salter-Harris IV; (E) Salter-Harris V.
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848 Chapter 42 Special Diagnostic Issues in Children

asymmetrical growth arrest that requires angular remodeling readily takes place, the
treatment. angulation between the line of the shaft and the
● Salter-Harris V: In this fracture, the mecha- line parallel to the physis does not necessarily
nism of injury is presumed to be excessive remodel. If the angulation of the physis is in the
axial loading that damages the proliferating plane of the joint motion (eg, flexion or exten-
cells of the physis. Injury to the blood sup- sion of the knee in a proximal tibial fracture),
ply may play a part. There is nothing to see remodeling is more likely. However, there is
on the x-ray, and the injury is recognized much less likely to be remodeling in the varus,
after the fact when the bone is discovered to valgus plane. At best, malrotation should be
have stopped growing. expected to show only minimal correction.

Physiological Differences Mechanical Differences


Children’s fractures also differ physiologically Children’s bones differ from those of adults in
from those in adults. They heal more rapidly, three important mechanical ways, as dis-
which leads to both good and bad results. cussed next.
Rapid healing, with less likelihood of delayed
union or nonunion, makes children’s fractures
PERIOSTEUM
easier to treat. However, if the bones are mal-
The periosteum is more of a structure in chil-
positioned, rapid healing may require that the
dren’s bones than in adults’. The tissue is
bones be refractured in order to gain appropri-
much stronger and may not tear when the
ate alignment if the malunited fracture is not
bone shaft breaks, thus leaving the broken
treated early enough.
bone more stable within an intact sleeve of
GROWTH IN LENGTH the soft tissue periosteum. This can help the
Growth in length may be partially or totally orthopedic surgeon because it can make the
inhibited by the trauma or by the injury to the job of aligning the bone fragments easier
vessels that supply nutrition to the multiply- through simple traction. The tough perios-
ing cells of the physis. However, as noted teum may allow a small child to continue
above, a healing fracture may stimulate a rap- walking after a spiral fracture of the shaft of
idly growing bone to grow longer. This is the tibia. This can present a diagnostic
particularly common with fractured femurs, dilemma because it may lead to health care
especially where the fracture is close to the workers assuming that a fracture could not be
rapidly growing physis at the distal femur. the cause of a limp because the child would
Recognition of this phenomenon may lead the ordinarily not be able to walk.
treating orthopedic surgeon to leave the ends
THE MECHANICAL PROPERTIES OF BONE IN
of the fracture intentionally overlapped by 1
CHILDREN ARE DIFFERENT
to 2 cm in anticipation of the overgrowth. It is
Bones can bend without breaking, a phenom-
important for all health care providers work-
enon that is sometimes called plastic deforma-
ing with children to understand these treat-
tion.16,17 Bones can crush longitudinally, cre-
ment principles because they may be ques-
ating a stable break that shows in an x-ray as a
tioned about issues like this by parents and
bulging ring around the bone at about the
patients.
junction of the metaphysis and diaphysis. This
is called a buckle or torus fracture because its
EFFECTS OF REMODELING
appearance on x-ray is similar to the ring at
Periosteal growth controls the increase in bone
the top of a Greek Doric column (Fig. 42-2).
diameter. This growth can result in remarkable
remodeling of a child’s bone following frac- LIGAMENTS ARE STRONGER THAN THE
tures. Judgment of this effect allows the ortho- ATTACHMENT OF THE PHYSES TO THE
pedic surgeon to accept considerable angula- METAPHYSIS
tion in a long bone fracture alignment in Ligamentous structures in adults are less
anticipation of the straightening. Although strong than the bones to which they are
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Chapter 42 Special Diagnostic Issues in Children 849

from those used with adults. The history is


generally obtained secondhand through the
parent and it is easy to overlook the fact that
the parent’s information may be misinter-
preted by the clinician. For example, a history
of a fall is always suspect (because children fall
so frequently), because it is easy to ascribe a
traumatic cause related to the fall when an-
other form of pathology may be responsible.
Cultural differences in childrearing may pose
A B C additional challenges. The physical therapist
FIGURE 42-2 (A, B) Buckle (torus) fractures share needs to be aware of the ages at which partic-
a resemblance to (C) the top of a Doric column. ular conditions affect a child, and the unique
features of growth and injury that should alter
how one assesses the likelihood of a diagnosis.
attached. Hence, they are the structures most Similarly, approaching children to examine
likely to be torn with a varus or valgus stress at them requires special techniques: get to the
the knee. However, in children, the opposite is child’s level; watch while the child is in the
true. The ligaments are stronger than the waiting area (and not afraid); and examine
bones. Hence, a valgus stress at the knee is the painful area last.
much more likely to result in a fracture at the
physeal-metaphyseal junction (the zone of References
provisional mineralization) than a torn liga- 1. American Physical Therapy Association. Vision 2020.
http://www.apta.org/vision2020. Accessed June 28,
ment. This difference becomes less noticeable 2011.
as the child grows toward the mid-teen years, 2. Phillips WE, Spotts ML. Medico-legal issues in the
at which time torn ligaments may be seen United States. In: Campbell SK, Vander Linden DW,
more frequently. Palisano RJ, eds. Physical Therapy for Children. 3rd ed.
Philadelphia, PA: Elsevier; 2006:1123–1139.
3. Carter S. Neonatology: frequently asked questions: cal-
Other Considerations culating corrected age. Emory University Department of
Pediatrics. http://www.pediatrics.emory.edu/divisions/
ACTIVITY AND CAUTION neonatology/dpc/faq.html. Accessed September 14, 2009.
Children are usually more active and less 4. Fadiman A. The Spirit Catches You and You Fall Down.
cautious than adults, making them more New York: Farrar, Straus and Giroux; 1998.
5. Long T, Toscano K. Handbook of Pediatric Physical Ther-
vulnerable to fractures. However, this in- apy. 2nd ed. Philadelphia, PA: Lippincott Williams &
creased activity and devil-may-care attitude Wilkins; 2002.
makes it less likely that their joints will 6. Dewey C, Fleming P, Golding J, Team AS. Does the
stiffen during the immobilization needed for supine sleeping position have any adverse effects on the
child? II. Development in the first 18 months. Pediatrics.
fracture healing. 1998;101(1, E5).
7. Davis BE, Moon RY, Sachs HC, Ottolini MC. Effects of
FREQUENT FRACTURES sleep position on infant motor development. Pediatrics.
Some children will be seen who seem to have 1998;102:1135–1140.
an inordinate number of fractures. Two po- 8. Ratliffe KT. The typically developing child. In Clinical
Pediatric Physical Therapy: A Guide for the Physical
tential causes come to mind: osteogenesis Therapy Team. Philadelphia, PA: Mosby; 1998:45–51.
imperfecta and child abuse. It is often diffi- 9. Adolph KE. Learning in the development of infant loco-
cult to differentiate between these, but they motion. Monogr Soc Res Child Dev. 1997;62(3):1–140.
must always be kept in mind and a proper 10. Adolph KE, Berger SE. Motor development. In: Damon
W, Lerner RM, eds. Handbook of Child Psychology. Vol II.
diagnosis is critical. 6th ed. New York: Wiley; 2006.
11. Campbell SK. Understanding motor performance in chil-
dren. In Campbell SK, Vander Linden DW, Palisano RJ,
Conclusion eds. Physical Therapy for Children. 3rd ed. Philadelphia,
PA: Elsevier; 2006:3–190.
Making a diagnosis for a child requires special 12. Hoyt WA, Troyer ML, Reef T. The proximal femoral
techniques in history taking that are different epiphysis. JBJS. 1966;48–A:1026.
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13. Ewald FC, Hirohashi K. Effect of distal transfer of the 16. Vorlat P, DeBroeck H. Bowing fractures of the forearm
greater trochanter in growing animals. JBJS. 1973;55(4): in children: a long term followup. Clin Orthop.
1064–1067. 2003;413:233–237.
14. Moseley CF. Leg-length discrepancy. In: Morrissy RT, 17. Sanders WE, Heckman JD. Traumatic plastic deforma-
Weinstein SL, eds. Lovell and Winter’s Pediatric Or- tion of the radius and ulna. A closed method of correc-
thopaedics. Vol 2. 6th ed. Philadelphia, PA: Lippincott tion of deformity. Clin Orthop. 1984;188:58–67.
Williams & Wilkins; 2006:1213–1256.
15. Salter R, Harris WR. Injuries involving the epiphyseal
plate. JBJS. 1963;45–A:587.
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CHAPTER43
Elbow Pain in a Child
■ Julia L. Burlette, PT, DPT, OCS ■ Jill S. Masutomi, PT, DPT, PCS

Description of the Symptom Remote causes are defined as occurring outside


this region.
This chapter describes possible causes of elbow
pain in a child. Local causes of elbow pain are Special Concerns
defined as occurring in the region bounded by ■ Decreased pulses
the distal one-third of the humerus, proximal ■ Recent change of status from previous
one-third of the radius and ulna, and corre- ■ Skin breakdown and wound problems
sponding articular and periarticular structures. ■ Warmth and swelling associated with a fever

851
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852 Chapter 43 Elbow Pain in a Child

CHAPTER PREVIEW: Conditions That May Lead to Elbow Pain in a Child


ELBOW PAIN IN A CHILD

T Trauma
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Acute brachial plexus Dislocations: Not applicable
injury 859 • Elbow dislocation 861
Fractures:
• Supracondylar fracture of the
humerus 863

UNCOMMON
Obstetric brachial Fractures: Nerve injury:
plexus injury 860 • Monteggia fracture-dislocation 863 • Median nerve injury 865
• Transphyseal fracture 864

RARE
Cervical radiculopathy 860 Not applicable Fractures:
• Coronoid process 862

I Inflammation
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
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Chapter 43 Elbow Pain in a Child 853

ELBOW PAIN IN A CHILD


LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Fractures: Not applicable Fractures:


• Lateral condyle 862 • Medial epicondyle 862
Osteochondritis/osteochondrosis:
• Osteochondritis of the capitellum
(Little Leaguer’s elbow) 866
• Osteochondrosis of the capitellum
(Panner’s disease) 867
Pulled elbow (nursemaid’s elbow,
acute radial head subluxation) 867

Muscle strains: Fractures: Medial collateral ligament


• Extensor muscle strain 865 • Olecranon 863 sprain/disruption or
Nerve injury: insufficiency 865
• Radial nerve injury 866 Muscle strains:
• Flexor/pronator muscle
strain 865
Nerve injury:
• Ulnar nerve injury 866

Dislocations: Not applicable Not applicable


• Radial head dislocation,
traumatic 861
(continued)
Fractures:
• Capitellar fracture 862

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Aseptic Aseptic Aseptic


Tendinopathies (tendinitis): Apophysitis: Apophysitis:
• Lateral epicondylitis (tennis • Olecranon 860 • Medial epicondyle (Little
elbow) 868 Leaguer’s elbow) 860
Septic Tendinopathies
Septic Not applicable (tendinitis):
Not applicable • Medial epicondylitis
(golfer’s elbow) 868
Septic
Not applicable
(continued)
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854 Chapter 43 Elbow Pain in a Child

Inflammation (continued)
ELBOW PAIN IN A CHILD

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


UNCOMMON
Not applicable Aseptic Not applicable
Juvenile idiopathic arthritis 864
Septic
Septic arthritis (pyogenic
arthritis) 867
RARE
Not applicable Aseptic Aseptic
Not applicable Tendinopathies (tendinitis):
• Distal biceps tendinitis 868
Septic
Osteomyelitis 867 Septic
Systemic fungal infection 868 Not applicable
Tuberculosis 869

M Metabolic
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Myositis ossificans 865 Not applicable
RARE
Not applicable Gout 864 Not applicable

Va Vascular
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Arterial injury 861 Not applicable
Compartment syndrome 861
RARE
Not applicable Hemophilia 864 Not applicable

De Degenerative
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
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Chapter 43 Elbow Pain in a Child 855

ELBOW PAIN IN A CHILD


LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Aseptic Not applicable


Tendinopathies (tendinitis):
• Triceps tendinitis 868
Septic
Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable


(continued)
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856 Chapter 43 Elbow Pain in a Child

Degenerative (continued)
ELBOW PAIN IN A CHILD

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


RARE
Not applicable Not applicable Not applicable

Tu Tumor
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Malignant Primary, such as: Not applicable
• Ewing’s sarcoma 869
• Osteosarcoma 870
• Rhabdomyosarcoma 870
Malignant Metastatic:
Not applicable
Benign, such as:
• Aneurysmal bone
cyst 869
• Fibromatosis 869
• Lipoma 869
• Osteoblastoma 870
• Osteochondroma 870
• Osteoid osteoma 870

Co Congenital
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
UNCOMMON
Not applicable Radioulnar synostosis 867 Not applicable

RARE
Not applicable Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL GENERALIZED LOCAL ANTERIOR
COMMON
Not applicable Not applicable Not applicable
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Chapter 43 Elbow Pain in a Child 857

ELBOW PAIN IN A CHILD


LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Dislocations: Not applicable Not applicable


• Radial head dislocation,
congenital 861

Not applicable Not applicable Not applicable

LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable


(continued)
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858 Chapter 43 Elbow Pain in a Child

Neurogenic/Psychogenic (continued)
ELBOW PAIN IN A CHILD

REMOTE LOCAL GENERALIZED LOCAL ANTERIOR


UNCOMMON
Not applicable Not applicable Not applicable
RARE
Not applicable Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Common Ages at Which Elbow Pain Presents in a Child


APPROXIMATE AGE CONDITION
Birth to 3 Years Juvenile idiopathic arthritis
Obstetric brachial plexus injury
Osteomyelitis
Pulled elbow
Septic arthritis
Transphyseal fracture
Preschool (3–5 Years) Juvenile idiopathic arthritis
Monteggia fracture-dislocation
Osteomyelitis
Pulled elbow
Radial head or neck fracture
Supracondylar humerus fracture
Transphyseal fracture
Traumatic radial head dislocation
Elementary School (6–11 Years) Aneurysmal bone cyst
Juvenile idiopathic arthritis
Lateral condyle fracture
Medial collateral ligament sprain/disruption
Medial epicondyle apophysitis
Medial epicondyle fracture
Monteggia fracture-dislocation
Olecranon apophysitis
Osteoblastoma
Osteochondrosis of the capitellum
Osteoid osteoma
Radial head or neck fracture
Supracondylar humerus fracture
Traumatic radial head dislocation
Middle School (12–14 Years) Aneurysmal bone cyst
Capitellar fracture
Elbow dislocation
Extensor muscle strain
Flexor/pronator muscle strain
Juvenile idiopathic arthritis
Lateral epicondylitis
Medial condyle fracture
Medial epicondyle fracture
Medial epicondylitis
Olecranon fracture
Osteoblastoma
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Chapter 43 Acute Brachial Plexus Injury 859

ELBOW PAIN IN A CHILD


LOCAL LATERAL LOCAL POSTERIOR LOCAL MEDIAL

Not applicable Not applicable Not applicable

Not applicable Not applicable Not applicable

Common Ages at Which Elbow Pain Presents in a Child—cont’d


APPROXIMATE AGE CONDITION
Osteochondritis of the capitellum
Osteochondrosis of the capitellum
Osteoid osteoma
Radial head or neck fracture
High School (15–18 Years) Acute brachial plexus injury
Aneurysmal bone cyst
Capitellar fracture
Elbow dislocation
Extensor muscle strain
Flexor/pronator muscle strain
Lateral epicondylitis
Medial collateral ligament sprain/disruption
Medial epicondylitis
Olecranon fracture
Osteoblastoma
Osteochondritis of the capitellum
Osteoid osteoma
Triceps tendinitis

Overview of Elbow Pain in a Child tissue is age dependent, and injury patterns are
commonly observed in association with the
The elbow is the most common joint injured skeletal developmental stage of the growing
in childhood. Elbow injuries in the skeletally child’s elbow. Tumors, infections, and arthri-
immature child have increased as participation tides are less likely to occur in the pediatric
in organized athletics has expanded for this elbow, given that much less longitudinal
age group. Juvenile and adolescent athletes are growth occurs at the distal humerus and prox-
participating at earlier ages—and at higher imal radius and ulna in relation to their oppo-
intensities of competition—in year-round site ends.
sports, placing excessive amounts of stress on
immature structures. There are several condi- Description of Conditions That
tions unique to the pediatric elbow that can be May Lead to Elbow Pain in a Child
attributed to the presence of the growth plates
or physes that distinguish these injuries from Remote
their adult counterpart. The high prevalence of
convoluted physes in the distal humerus, prox- ■ Acute Brachial Plexus Injury
imal radius, and proximal ulna make the Chief Clinical Characteristics
recognition of fractures difficult on plain radi- This presentation includes burning pain down
ographs. The strength of the musculoskeletal the arm, numbness, weakness, and dysesthesia.
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860 Chapter 43 Cervical Radiculopathy

Background Information recovery by 3 months of age is observed in up


ELBOW PAIN IN A CHILD

Also known as a burner, the mechanism of to 92% of injured infants.3 Additional in-
injury involves collision sports and typically volvement of the C7 nerve will result in the
includes traction to the brachial plexus from a classic “waiter tip deformity” with the shoul-
blow that depresses the shoulder. The upper der adducted and internally rotated, elbow ex-
trunks (C5–C6) are most commonly involved, tended, forearm pronated, and wrist and fingers
resulting in more numbness and pain in the flexed. Risk factors for obstetric brachial
C5 and C6 dermatomes and weakness in the plexus injury include high birth weight, pro-
deltoid and biceps. Assessment includes longed labor, and breech presentation.3 Treat-
screening for osseous injury and a thorough ment includes stretching and splinting to
neurological examination. Most acute brachial prevent further contracture. Microsurgery
plexus injuries from collision sports will re- may be beneficial if no recovery occurs by 3 to
solve with complete recovery within minutes 6 months. Tendon transfers and osteotomies
to hours. The athlete may return to sports may be indicated to increase function.
when neurological function is normal. The
athlete with a severe injury resulting in weak-
Local
ness lasting longer than 6 months should not
return to collision sports.1 APOPHYSITIS
■ Medial Epicondyle (Little
■ Cervical Radiculopathy
Leaguer’s Elbow)
Chief Clinical Characteristics
This presentation can be characterized by pain Chief Clinical Characteristics
in the cervical region and the corresponding This presentation may include pain, swelling,
dermatome for the involved nerve root. Pain is and localized tenderness over the medial epi-
typically increased with cervical movement, condyle. Reproduction of symptoms will occur
use of the involved upper extremity, and cough- with resisted wrist flexion, resisted forearm
ing or sneezing. This presentation may also pronation, and passive wrist extension with the
include motor loss, sensory disturbances, and elbow extended. Application of a valgus stress
diminished or absent reflexes corresponding to to an elbow flexed 20 degrees increases pain and
the involved nerve root. may also demonstrate laxity.4
Background Information
Background Information Medial epicondyle apophysitis is a repetitive
Cervical radiculopathy may be caused by stress injury typically found in the skeletally
trauma or other lesions (ie, space-occupying immature child and adolescent thrower, ages
lesion, infection, or hemorrhage). The diagno- 8 to 12.1,5 Its cause is excessive distractive force
sis is confirmed with magnetic resonance produced on the medial side of the elbow with
imaging. Progressive neurological deficits in- repetitive valgus stress leading to a series of
dicate surgical intervention. microavulsions at the bone–cartilage junc-
■ Obstetric Brachial Plexus Injury tion.6 It is seen most commonly in throwers
during the acceleration phases of throwing,
Chief Clinical Characteristics but can occur in gymnasts, tennis players,
This presentation can be characterized by weak- golfers, or with any repetitive valgus stress to
ness of the muscles innervated by the involved the elbow.6,7 Treatment includes rest until the
brachial plexus trunk. child is asymptomatic, followed by gradual
Background Information stretching and strengthening, with a slow pro-
The mechanism of injury is traction of the gression of throwing or return to sports.8
brachial plexus during delivery when the head
is pulled laterally away from the shoulder. The ■ Olecranon
injury is classified by the level of trunk in- Chief Clinical Characteristics
volvement. The upper trunk (C5 and C6) is This presentation may involve pain, mild
most commonly involved.2 It is also known as swelling, and tenderness at the insertion of the
upper plexus palsy or Erb’s palsy. Complete triceps at the tip of the olecranon. Pain is
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Chapter 43 Radial Head Dislocation, Traumatic 861

exacerbated by resisted elbow extension, and to prevent muscle ischemia. A decompressive

ELBOW PAIN IN A CHILD


a decrease in elbow range of motion may be fasciotomy is required to alleviate the pressure.
present.8 The major complication of compartment syn-
drome is a Volkmann contracture.10
Background Information
Traction apophysitis at the distal triceps in- DISLOCATIONS
sertion is the most common posterior elbow
■ Elbow Dislocation
injury in skeletally immature pitchers, 8 to
12 years of age. The olecranon is subject to Chief Clinical Characteristics
minor stress fractures in throwing secondary This presentation includes severe pain,
to the excessive triceps pull in the early accel- swelling, deformity, and inability to flex or
eration phase, coupled with the impaction of extend the elbow.
the olecranon into the humeral fossa in the Background Information
late follow-through phase.4,5,8,9 This condi- The mechanism of injury is typically a fall.
tion is associated with overuse4 in any move- Dislocations account for 5% of elbow in-
ment or task that involves repeated maximal juries in children.11 The most frequent pattern
extension of the elbow in a skeletally imma- of dislocation is posterior. Associated frac-
ture child.7 Treatment includes rest and ice tures are common. Other associated injuries
in the acute stage followed by stretching and include disruption of the anterior capsule,
strengthening. ulnar collateral ligament injury, and brachialis
■ Arterial Injury muscle tear. Arterial and nerve injuries are
uncommon. Peak incidence occurs in the sec-
Chief Clinical Characteristics ond decade, usually between 12 and 14 years
This presentation typically involves pain of age.5,11 Spontaneous reduction is common.12
out of proportion to the injury that increases Closed reduction with splinting for simple
with passive stretch of the involved muscles. dislocations usually results in good outcomes
Pulses are decreased or absent, skin temper- although loss of elbow motion may occur.
ature is decreased, and skin color is poor. This Dislocation with associated injury may re-
presentation may also include weakness and quire surgical intervention.
hypoesthesia.
■ Radial Head Dislocation,
Background Information
Congenital
The mechanism of injury is usually traumatic
and may include fracture or laceration. Surgical Chief Clinical Characteristics
repair is required. This presentation may involve restricted
pronation or supination and bony promi-
■ Compartment Syndrome nence at the location of the dislocation, usu-
Chief Clinical Characteristics ally posteriorly or laterally.13
This presentation involves pain, feelings of in- Background Information
creased pressure, tense compartment, weakness, This condition is uncommon and typically
numbness, and swelling. Pain is out of propor- occurs unilaterally. The radial head has a
tion than expected for injury and increases with rounded shape and the capitellum is under-
passive stretching of the muscles. Pain related to developed.14 It is usually not detected until
this condition is not relieved with immobiliza- later in childhood. Treatment consists of ob-
tion. Peripheral pulses are usually intact unless servation unless pain develops. Surgical in-
an arterial injury has occurred. tervention may be indicated once skeletal
Background Information maturation has been achieved.
The mechanisms of injury are numerous and
include fracture, crush injury, burns, and vas- ■ Radial Head Dislocation,
cular injury. Prolonged external pressure from Traumatic
a compressive cast or bandage may also cause Chief Clinical Characteristics
compartment syndrome. Immediate identifi- This presentation may be characterized by
cation of compartment syndrome is critical lateral elbow pain and restricted range of
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862 Chapter 43 Capitellar Fracture

motion. The radial head may be palpable in commonly unrecognized and may con-
ELBOW PAIN IN A CHILD

the dislocated position. tribute to continued instability following


elbow trauma.
Background Information
The mechanism of injury is typically a fall ■ Lateral Condyle
onto the hyperextended elbow or out- Chief Clinical Characteristics
stretched, pronated hand. Isolated radial This presentation is characterized by lateral
head dislocation is rare and often associated elbow pain and swelling.
with plastic deformation of the ulna.3 It is
considered a variant of the Monteggia lesion. Background Information
This injury is frequently missed and may re- The mechanism of injury is typically a varus
sult in future problems if left untreated. It stress with the elbow in extension. The frac-
may be treated with closed reduction and ture can also occur with an elbow dislocation
immobilization. Open reduction is indicated or a fall onto a flexed elbow. Lateral condyle
if closed reduction is unsuccessful, with de- fractures occur between the ages of 2 and
layed reduction greater than 3 weeks postin- 14 years, but are more common between the
jury,12 and with persistent dislocation.3 ages of 6 and 10 years.16 These fractures are
Complications are uncommon; they include relatively common, accounting for 15% to
posterior interosseous nerve lesion and de- 20% of distal humerus fractures in chil-
creased elbow range of motion. dren,17 and they occur more frequently in
boys. This fracture usually requires internal
FRACTURES fixation. An undisplaced and stable fracture
■ Capitellar Fracture may be treated with external immobilization
or percutaneous pinning. Complications of
Chief Clinical Characteristics lateral condyle fracture include nonunion,
This presentation can involve pain and avascular necrosis, ulnar nerve palsy, cubitus
swelling at the lateral elbow and antecubital valgus deformity, and physeal arrest.5,11
fossa. Range of motion is restricted, especially
with flexion. The lateral elbow may be tender. ■ Medial Condyle
Background Information Chief Clinical Characteristics
The mechanism of injury is typically a fall, This presentation may include medial elbow
with the elbow extended, onto an out- pain and swelling.
stretched arm. The fracture may also occur Background Information
with a fall onto a flexed elbow.12 Capitellar The mechanism of injury is typically a fall
fractures are rare and usually occur in chil- onto the point of a flexed elbow or a valgus
dren older than 12 years.15 Cubitus recurva- stress on an extended elbow causing an
tum and cubitus valgus may facilitate this avulsion fracture.16 Isolated medial condyle
injury. One-third of these injuries are associ- fractures are rare. Surgical intervention is
ated with a radial head fracture.15 Capitellar indicated for displaced fractures. Nondis-
fractures usually require internal fixation; placed fractures may be managed with
however, minimally displaced fractures may immobilization only.
be managed with closed reduction.
■ Medial Epicondyle
■ Coronoid Process Chief Clinical Characteristics
Chief Clinical Characteristics This presentation can be characterized by me-
This presentation includes pain and swelling dial elbow pain, tenderness, and valgus insta-
at the antecubital fossa. Crepitus may occur bility. Mild to moderate swelling may occur.
with end-range elbow extension. Isolated The fracture may result in mild to severe
injury is rare. ulnar nerve injury.
Background Information Background Information
This fracture may occur with elbow disloca- The mechanism of injury is typically a fall on
tion or severe trauma. This condition is an outstretched hand, but may also occur
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Chapter 43 Supracondylar Fracture of the Humerus 863

with a direct blow to the medial elbow. Fifty with a direct blow or twisting injury.11,21 The

ELBOW PAIN IN A CHILD


percent of cases are associated with elbow fracture is uncommon, accounting for 4% to
dislocation.5,18 Dislocation with spontaneous 6% of elbow fractures in children.11,22 Thirty-
reduction may occur. Medial epicondyle two percent of olecranon fractures have as-
fractures typically occur between the ages of sociated elbow injuries including radial neck
10 and 14 years.5,17 It is a relatively common fracture, medial epicondyle fracture, coro-
fracture, comprising 12% of elbow fractures.19 noid fracture, and osteochondral fracture.11
Indications for surgical intervention include Treatment is determined by the fracture
valgus instability, ulnar neuropathy, entrapped site and pattern, amount of displacement,
fragment in the elbow joint, and displace- stability, and associated injuries. Most of
ment greater than 5 mm. Athletes will usually these injuries are nondisplaced or minimally
require surgical fixation. displaced requiring immobilization only.
Complications are uncommon and out-
■ Monteggia Fracture-Dislocation comes are good with the majority of children
Chief Clinical Characteristics regaining functional elbow range. Between
This presentation may involve pain, tender- 10% and 20% olecranon fractures require
ness, swelling, and angulation of the ulna. open reduction.11
Elbow range of motion is restricted. The
dislocation of the radial head may be visual-
■ Radial Head or Neck
ized on the lateral aspect of the elbow. Chief Clinical Characteristics
This presentation may involve pain, swelling,
Background Information and tenderness along the lateral aspect of the
The mechanism of injury is typically a fall elbow over the proximal radius. Pronation
onto the hyperextended elbow but less com- and supination are limited and painful with
monly may occur from a direct blow or a pain often referred to the wrist.
hyperpronation injury. This condition refers
to dislocation of the radial head associated Background Information
with ulnar fracture or bowing. The Mon- The mechanism of injury is typically a fall
teggia lesion is classified according to the on the outstretched extremity with a valgus
direction of the radial head dislocation and moment at the elbow, resulting in a fracture
the trauma to the ulna. It is an uncommon just distal to, or at the epiphyseal plate, or a
injury in children accounting for less than fracture of the neck of the radius.21 These
1% of all pediatric forearm fractures.20 It fractures may be associated with a rupture of
occurs most often in children younger than the medial collateral ligament, avulsion of
10 years of age.20 The posterior interosseous the medial epicondylar apophysis, olecranon
nerve may be injured with an anterior dislo- fracture, and rarely with dislocation. A stress
cation; however, associated nerve injuries are fracture of the radial neck with angular
less common than in adults. Additional com- deformity may occur in pediatric athletes.
plications include failure or loss of reduction, This injury typically occurs in children ages
elbow stiffness, avascular necrosis of the 4 to 14 years.11,17 Fracture management is
radial head, and radioulnar synostosis.20 The determined by the degree of angulation,
injury is usually treated by closed methods translation, elbow motion, and any con-
but may require surgical intervention if the comitant injuries.23 Treatment options in-
elbow is unstable. The prognosis is good. clude immobilization without reduction,
closed reduction, or open reduction with or
■ Olecranon without internal fixation.
Chief Clinical Characteristics ■ Supracondylar Fracture of the
This presentation includes posterior elbow pain, Humerus
tenderness, swelling, and decreased elbow range.
Chief Clinical Characteristics
Background Information This presentation typically includes pain,
The mechanism of injury is typically a fall on tenderness, and swelling medially and lat-
an outstretched hand, but may also occur erally in the supracondylar area. Swelling
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864 Chapter 43 Transphyseal Fracture

may be present posteriorly and anteriorly and tenderness of the joint or bursal cavity.
ELBOW PAIN IN A CHILD

following high-energy injuries with greater Advanced cases may have palpable firm tophi.
displacement.
Background Information
Background Information Gout commonly manifests as acute olecranon
This fracture is the most common elbow frac- bursitis. The olecranon bursa is a common lo-
ture in children, and accounts for 60% of cation for tophi. The toes should also be as-
elbow injuries.11 The mechanism of injury is sessed for metatarsal joint involvement. Gout
typically a fall on the outstretched arm or is usually associated with elevated uric acid
with the elbow positioned a little flexed with levels. Gout is rare in children, although it may
the forearm pronated. The incidence of this be associated with inherited conditions that
injury is greatest in the first decade of life with are, in turn, associated with hyperuricemia.
a peak between the ages of 5 and 10 years.5 Polarized microscopic examination of aspi-
Injuries to the anterior interosseus nerve, rated synovial fluid confirms the diagnosis.
radial nerve, median nerve, and brachial ar- Treatment includes inflammation control,
tery can occur in up to 18% of displaced frac- medication, and diet modification.
tures.5 Treatment of these fractures is guided
by the amount of displacement. Nondisplaced ■ Hemophilia
to mildly displaced fractures are treated by Chief Clinical Characteristics
cast immobilization or through closed reduc- This presentation can be characterized by
tion followed by cast immobilization.17 With swelling, pain, warmth, stiffness, and the child
greater degrees of displacement, these injuries holding the elbow in a flexed position.
necessitate surgical intervention with either
closed reduction and percutaneous pin fixa- Background Information
tion or open reduction.5,11 This condition is an inherited, recessive, sex-
linked disorder that affects blood clotting
■ Transphyseal Fracture predominantly in males. Hemorrhages may
occur anywhere in the body, but are most of-
Chief Clinical Characteristics ten found in joints; typically the knee, elbow,
This presentation involves swelling, deforma- and ankle.24 Muscles are the second most
tion, muffled crepitus, and instability.17 common site of bleeding, with the iliopsoas,
Background Information gastrocnemius, and forearm flexor muscle
This injury is a fracture-separation of the compartment most frequently involved.25
distal humeral epiphysis that is observed in Depending on the severity of the disorder,
children from birth to 7 years of age, with a bleeding may occur spontaneously, or more
peak occurring before the child is 21/2 years of commonly, secondary to a minor trauma such
age.11 Multiple mechanisms of injury have as a sprain or muscle pull. Infants or toddlers
been reported, with the most common a ro- may develop subcutaneous ecchymosis over
tatory shear force, either at birth secondary bony prominences or large hematomas after
to traumatic delivery, child abuse, or from a receiving intramuscular vaccinations. Factor
fall onto an extended arm.17 Closed reduc- replacement therapy, given intravenously, is
tion is performed under anesthesia followed the mainstay of treatment for an acute
by cast immobilization if necessary. Percuta- bleeding episode.25 In addition to factor re-
neous pin stabilization is used to prevent loss placement, management of an acute bleed
of reduction and malunion with postsurgical includes rest, ice, compression, and elevation,
cast immobilization for 3 weeks. Damage to pain-free movement, splinting, and pain
the physis is rare and cubitus varus is an un- medication as needed (aspirin is contraindi-
common complication.11,17 cated in this patient population).24

■ Gout ■ Juvenile Idiopathic Arthritis


Chief Clinical Characteristics Chief Clinical Characteristics
This presentation involves a sudden onset of This presentation typically includes insidious
pain, swelling, redness of the superficial tissues, onset of multiple peripheral joint swelling,
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Chapter 43 Median Nerve Injury 865

heat, decreased extension greater than flex- symptoms may occur with passive wrist

ELBOW PAIN IN A CHILD


ion range of motion, and pain and tender- flexion.
ness with joint motion. Joint stiffness is most
Background Information
marked in the morning or after a period of
The mechanism of injury is typically in-
inactivity and improves with movement. The
creased wrist extensor use 1 to 2 days prior to
muscles surrounding the elbow may show
the onset of symptoms. Resolution of symp-
marked wasting, especially when the more
toms will usually occur in 1 to 3 days with
acute painful stage of the disease is subsiding.
avoidance of aggravating activities.
With chronic conditions, the pain may subside;
however, the joint is left with limited move- ■ Flexor/Pronator Muscle Strain
ment and possibly long-term deformity.
Chief Clinical Characteristics
Background Information This presentation may include muscle soreness
This condition is one of the most common and tenderness of the wrist flexor/pronator
rheumatic diseases of childhood, and is one musculature. Reproduction of symptoms will
of the more frequent chronic illnesses of chil- occur with active wrist flexion and forearm
dren.26 There is a peak age of onset between pronation with increased symptoms when
1 and 3 years of age and a secondary peak just resistance is applied. Symptoms may be re-
before puberty.26,27 Treatment includes med- produced with passive wrist extension and
ications, activity modification, general condi- forearm supination.
tioning, splinting, and casting. Surgical inter-
Background Information
vention may be necessary to improve long-term
The mechanism of injury is typically in-
joint function.
creased forearm use 1 to 2 days prior to the
■ Medial Collateral Ligament onset of symptoms. Symptoms will typically
Sprain/Disruption or resolve in 1 to 3 days with rest.
Insufficiency
■ Myositis Ossificans
Chief Clinical Characteristics
This presentation can involve medial elbow Chief Clinical Characteristics
pain, tenderness, and laxity with valgus stress This presentation can involve swelling, tender-
testing. ness, and warmth over the involved muscle.

Background Information Background Information


The mechanism of injury typically includes The mechanism of injury is typically a direct
a history of repetitive overhead throwing blow to the muscle mass that causes a signifi-
activities but may also occur from a fall on cant contusion. The etiology of this condition
the outstretched hand. Valgus force at the is theorized to be due to a hematoma organi-
elbow may cause complete or partial tearing zation involving a progressive transformation
of the medial collateral ligament. Ligamentous of fibrous tissue to cartilage and then bone;
injury in children occurs more commonly hematoma calcification; intramuscular bone
before the growth spurt and after growth formation after detachment of periosteal flaps,
plate closure in adolescents.6 Medial collat- periosteal rupture, metaplasia of intramuscu-
eral ligament disruption usually requires lar connective tissue into cartilage and bone,
surgical repair to allow a full return to sports. and an individual predisposition to myosi-
tis.28,29 Radiographic changes may occur 3 to
MUSCLE STRAINS 4 weeks following the injury. Surgical resection
■ Extensor Muscle Strain is rarely indicated unless function is impaired.
Chief Clinical Characteristics
NERVE INJURY
This presentation may be characterized by
muscle soreness and tenderness of the wrist ■ Median Nerve Injury
extensor musculature. Symptoms are repro- Chief Clinical Characteristics
duced with active wrist extension and increased This presentation may include aching pain
when resistance is applied. Reproduction of in the anterior forearm and palpable pain
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866 Chapter 43 Radial Nerve Injury

along the course of the nerve. Reproduction Microtrauma may be caused by chronic
ELBOW PAIN IN A CHILD

of symptoms may occur with resisted forearm, subluxation, valgus stress, and flexor carpi
elbow, or finger motions. The patient may ulnaris muscle compression.4 Ulnar nerve
present with weakness in the muscles inner- injury is associated with medial condyle
vated by the median nerve and numbness fracture and flexion type supracondylar
in the hand, primarily the first and second fracture.30 Ulnar neuritis may occur in
digits. young athletes from repeated throwing and
may be associated with medial instability.9
Background Information
Treatment for this condition includes rest
The mechanism of injury may be acute
and inflammation control. Strengthening is
trauma or chronic, repetitive microtrauma.
initiated when the acute phase resolves.
Repetitive, resisted pronation and supina-
Traumatic nerve injuries from fractures or
tion of the forearm may contribute to micro-
following surgery usually resolve sponta-
trauma of the median nerve. Median nerve
neously.31
compression should initially be managed
conservatively with inflammation control
and activity modification. Traumatic median OSTEOCHONDRITIS/
nerve injuries from fractures usually resolve OSTEOCHONDROSIS
spontaneously.30 ■ Osteochondritis of the
Capitellum (Little Leaguer’s
■ Radial Nerve Injury Elbow)
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation involves minimal to severe This presentation can involve dull pain, ten-
aching pain in the lateral forearm and derness over the lateral elbow, effusion, and
tenderness in the supinator region. Resisted loss of elbow range, most commonly extension.
wrist extension with the elbow extended or Catching, locking, and crepitus may also
resisted supination of the forearm may re- occur with elbow motion.32 Symptoms are
produce symptoms. The child suspected of aggravated with use of the involved extrem-
this condition may present with partial or ity and relieved with rest.
complete paralysis of the muscles innervated
Background Information
by the radial nerve.
Osteochondritis of the capitellum is asso-
Background Information ciated with frequent repetitive overuse of
Radial nerve injury may occur from the elbow and is most common in young
trauma, space-occupying lesions, inflam- throwing athletes between the ages of
mation, excessive muscular activity, and is 13 and 16 years. It is also associated with
associated with extension type supracondylar loose body formation. Treatment is guided
fracture.30 by clinical findings and radiographs.
Magnetic resonance imaging may aid in
■ Ulnar Nerve Injury early detection of this condition.33 Non-
surgical intervention includes rest and
Chief Clinical Characteristics
activity modification. Surgical interven-
This presentation may involve aching pain
tion may be indicated to remove loose
in the medial elbow and forearm and pares-
bodies. Prognosis depends on the size
thesias in the sensory dermatome of the
and extent of the lesion and the patient’s
ulnar nerve. Weakness and atrophy of the
age. Younger patients have a better prog-
muscles innervated by the ulnar nerve may
nosis but functional limitations are com-
be found depending on the extent of the
mon and a return to sports is not always
injury.
achieved. A poor outcome has been corre-
Background Information lated with advanced lesions, a large osteo-
The mechanism of injury may be traumatic chondral defect, and development of
or secondary to microtrauma to the nerve. osteoarthritis.34
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Chapter 43 Septic Arthritis (Pyogenic Arthritis) 867

■ Osteochondrosis of the ■ Pulled Elbow (Nursemaid’s Elbow,

ELBOW PAIN IN A CHILD


Capitellum (Panner’s Disease) Acute Radial Head Subluxation)
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes aching pain, ten- This presentation can be characterized by pain,
derness over the lateral side of the elbow, local tenderness, and the forearm positioned in
local swelling, and decreased range, typically pronation. The child is typically unwilling to
a loss of 5 to 20 degrees of extension. Symp- move the elbow and rotation of the forearm
toms are aggravated with activity and will provoke pain.
relieved with rest.
Background Information
Background Information The mechanism of injury is typically axial
Osteochondrosis of the capitellum is associ- traction to the extended elbow but less com-
ated with repetitive microtrauma to the lat- monly occurs secondary to a fall. Pulled el-
eral elbow from valgus stress or increased ax- bow is common in children less than 5 years
ial load as observed with throwing athletes of age, but occurs most frequently between
and gymnasts. It occurs more commonly in the ages of 6 months to 3 years. The injury is
boys, in the dominant extremity, and usually observed more frequently in girls. Treatment
between the ages of 7 and 12 years.35 Radi- consists of a simple reduction maneuver and
ographs demonstrate abnormalities of the immobilization is typically not indicated un-
capitellum. Treatment includes inflamma- less the child has persistent pain.12 The rate of
tion control, rest, and activity modification. recurrence ranges around 5%.37
The prognosis is good but healing may re-
■ Radioulnar Synostosis
quire a prolonged period. Residual defor-
mity is rare. Chief Clinical Characteristics
This presentation involves restricted forearm
pronation and supination. The elbow is typically
■ Osteomyelitis
positioned in midpronation or hyperpronation.
Chief Clinical Characteristics Pain is usually not a feature of this condition.
This presentation typically involves a rapid
onset of symptoms including general malaise, Background Information
high fever, chills, severe constant pain, swelling, This condition occurs equally in males and
and tenderness over the metaphysis of the females and over half of cases are bilateral.
involved bone. The limb is painful with any Congenital radioulnar synostosis is an uncom-
movement, which leads to an appearance of mon condition with a hereditary predisposi-
paralysis in infants. The older child with this tion.13 Unilateral cases present with minimal
condition may refuse to use the affected functional disability. Bilateral cases may require
limb.26,27 surgical intervention to improve pronation/
supination position to enhance function.
Background Information Although rare, synostosis can also occur be-
Osteomyelitis occurs most frequently from tween the humerus, radius, and ulna.
birth to 5 years old and is rarely found in the
elbow. The disease occurs 2.5 times more ■ Septic Arthritis (Pyogenic
commonly in males than in females.36 In Arthritis)
some cases the infection starts in the bony Chief Clinical Characteristics
metaphysis and spreads to an adjacent joint, This presentation can be characterized by acute
creating a septic arthritis. It is caused by an in- onset and by symptoms that include a high-
fection in the bone secondary to a minor in- grade fever, malaise, and pain, with joint involve-
jury with the most common causative bac- ment including swelling, intra-articular effusion,
terium being Staphylococcus aureus.27,36 and warmth. The child will typically hold the
Treatment includes aspiration, antibiotics, elbow in approximately 30 to 60 degrees of flex-
immobilization of the affected area, and sur- ion with reports of severe pain and muscle
gical drainage if necessary. spasm on attempting any slight movement.26,27
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868 Chapter 43 Systemic Fungal Infection

Background Information epicondyle. Tenderness may also be present just


ELBOW PAIN IN A CHILD

Septic arthritis is an infection of the joint anterior and distal to the lateral epicondyle
usually caused by a cocci bacterial organism, at the origin of the extensor carpi radialis
most commonly Staphylococcus aureus.27 Sep- brevis. Reproduction of symptoms will occur
tic arthritis can occur at any age but is most with resisted wrist extension and passive wrist
frequently seen in children younger than flexion.
3 years of age and is two to three times
Background Information
more frequent in boys than girls.26,27 The
This condition is found frequently in adoles-
elbow is the third most commonly affected
cent tennis players secondary to the repeti-
joint and is treated by immediate aspiration
tive loading to the extensor carpi radialis
and drainage and intravenous administration
brevis tendon, but can also be found in
of antibiotics.27,36
young throwers and swimmers.4,38 Lateral
■ Systemic Fungal Infection epicondylitis occurs after skeletal matura-
tion, which happens at approximately four-
Chief Clinical Characteristics teen years of age in males and thirteen
This presentation can include pain, tenderness, years of age in females.9 Treatment includes
and swelling over the elbow or the surrounding activity modification, inflammation control,
soft tissues. stretching, bracing, and strengthening when
Background Information inflammation has resolved.
Exposure and subsequent infection may be in-
sidious in nature, with the majority of those ■ Medial Epicondylitis
infected asymptomatic. Musculoskeletal in- (Golfer’s Elbow)
volvement is rare; however, lesions of bones Chief Clinical Characteristics
and joints result in diffuse soft tissue swelling, This presentation involves medial elbow pain
bone destruction, and sinus formation. Sys- and localized tenderness at the common flexor
temic fungal infections occur most frequently origin. Tenderness may also occur at the prox-
in the young, old, and immunocompromised imal flexor and pronator teres muscle bellies.
populations.27 Treatment includes long-term Reproduction of symptoms will occur with
antibiotics, irrigation, and debridement of the resisted wrist flexion, resisted forearm prona-
affected area. tion, and passive wrist extension.
TENDINOPATHIES Background Information
(TENDINITIS) This condition typically occurs in adoles-
■ Distal Biceps Tendinitis cent throwers and pitchers secondary to
overuse and repetitive valgus stress to the
Chief Clinical Characteristics elbow during the acceleration phase of their
This presentation includes pain and tender- delivery, however it is also found in golfers,
ness in the anterior elbow. Reproduction tennis players, and gymnasts.7,9 Epicondylitis
of symptoms will occur with resisted elbow versus apophysitis is more likely to occur
flexion and forearm supination. after closure of the medial epicondyle physis,
Background Information which occurs at approximately 17 years of
This is a rare injury in adolescents that may age in males and 14 years of age in females.4
present secondary to weight lifting or carry- Treatment includes activity modification,
ing heavy loads with flexed elbows. Treat- inflammation control, and stretching and
ment includes activity modification and strengthening when the acute symptoms
inflammation control. have subsided.

■ Lateral Epicondylitis ■ Triceps Tendinitis


(Tennis Elbow) Chief Clinical Characteristics
Chief Clinical Characteristics This presentation involves pain, inflammation,
This presentation includes pain, inflamma- and localized tenderness at the insertion of
tion, and localized tenderness at the lateral the distal triceps tendon. Reproduction of
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Chapter 43 Lipoma 869

symptoms will occur with resisted elbow and 25% may require additional surgery

ELBOW PAIN IN A CHILD


extension and passive elbow flexion. secondary to recurrence.41
Background Information
■ Ewing’s Sarcoma
Triceps tendinitis is more common in older
throwers but can present with any repetitive Chief Clinical Characteristics
elbow extension in adolescents after skeletal This presentation includes pain and local
maturation.4,9 Triceps tendinitis is often as- swelling at the site of the tumor. Constitu-
sociated with other injuries such as loose tional symptoms such as fever and malaise may
bodies and osteochondral defects and is be present specifically when metastatic disease
typically a result of overuse rather than poor is present.42
throwing mechanics.4 Treatment includes Background Information
activity modification and inflammation Ewing’s sarcoma is the second most com-
control in the acute stage followed by mon malignant bone tumor in children and
strengthening. adolescents. These tumors most commonly
develop in the axial skeleton, but may arise
■ Tuberculosis in any bone, particularly the diaphyses of the
Chief Clinical Characteristics humerus and femur. Approximately 25% of
This presentation includes a period of general patients with this condition may present
malaise, poor appetite, loss of weight, and low- with overt metastasis, typically in the lungs,
grade fever prior to any local symptoms. The bone marrow, or bone.43 Ewing’s tumors are
initial local symptoms include swelling or inter- typically sensitive to both chemotherapy and
ference with function rather than pain. Pain radiotherapy; surgical excision is utilized
develops gradually in the affected joint and is as well.44
aggravated by movement and later becomes
severe at night.27 ■ Fibromatosis
Background Information Chief Clinical Characteristics
The infecting organism is usually Mycobac- This presentation, although highly variable,
terium tuberculosis and the primary infection typically includes an enlarging soft tissue mass
takes place through the lungs, tonsils, or ali- associated with localized pain.45
mentary tract.27 Unlike acute osteomyelitis, Background Information
infection is more likely to affect a synovial Fibromatoses represent a group of rare fi-
joint than the shaft of the bone.27 Tuberculo- broproliferative disorders comprising 12%
sis of the elbow usually responds to antibi- of soft tissue tumors identified in child-
otic treatment; however, sometimes clear- hood.46 Although benign, they are locally in-
ance of the disease by surgical excision vasive and prone to recurrence.47 Loss of
involving the humerus or the ulna may be function has been frequently observed in the
needed. affected extremity.45 Surgical excision with a
wide margin of resection remains the pri-
TUMORS mary treatment for this condition.
■ Aneurysmal Bone Cyst
Chief Clinical Characteristics ■ Lipoma
This presentation typically involves dull pain Chief Clinical Characteristics
and swelling. This presentation involves a small, asympto-
matic soft tissue mass.
Background Information
An aneurysmal bone is a benign, vascular le- Background Information
sion. It is frequently seen in individuals dur- A lipoma is a benign soft tissue neoplasm
ing the second decade of life.39 In 30% of comprised of adipose tissue. Surgical inter-
cases, aneurysmal bone cysts occur in associ- vention is indicated in cases where the
ation with other benign and malignant tu- lipoma interferes with function or becomes
mors.40 Surgical excision is usually required symptomatic.
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870 Chapter 43 Osteoblastoma

■ Osteoblastoma caused by microfractures through the in-


ELBOW PAIN IN A CHILD

Chief Clinical Characteristics volved area, and in severe cases secondary


This presentation may include pain that is to stretching and compression of the sur-
not significantly relieved by aspirin. rounding structures.50

Background Information Background Information


Osteoblastoma is an uncommon, benign tu- Osteosarcoma is the most common primary
mor and usually occurs between the ages of malignancy of bone in children, but it is rare
10 and 25 years.37 It is more likely to occur in in the elbow. It typically occurs between 10
males. The tumor is locally aggressive and and 20 years of age, with the peak age inci-
requires surgical excision. Recurrence is dence during the adolescent growth spurt.51
common. The onset of symptoms is insidious in nature
and often related to a minor trauma such as
■ Osteochondroma a sports-related injury.50 Treatment of the
Chief Clinical Characteristics tumor requires surgical ablation and adju-
This presentation commonly includes hard vant chemotherapy.
swelling adjacent to the involved joint.37
■ Rhabdomyosarcoma
Elbow motion may be impaired and irritation
of the involved tissues may cause pain. Chief Clinical Characteristics
This presentation includes a mass in an ex-
Background Information tremity that may or may not be painful. Pain
Osteochondroma is a very common benign or tenderness may be present if the tumor is
tumor, but is uncommon in the elbow. It can displacing a peripheral nerve.
occur at any age but is usually diagnosed in
patients younger than 21 years of age.48 Background Information
Growth of the tumor usually ceases after the Rhabdomyosarcoma is the most common
patient has reached skeletal maturity. Surgi- pediatric soft tissue sarcoma, and two-
cal excision is indicated for lesions that cause thirds of patients are less than 10 years of
pain or impair motion. age.50,52 They can develop anywhere in the
body but are often found in the extremities.
■ Osteoid Osteoma Approximately half the tumors of the limbs
Chief Clinical Characteristics are alveolar in nature and are therefore
This presentation can involve sharp pain that highly metastatic.53 Treatment typically in-
is worse at night and relieved significantly cludes presurgical neoadjuvant chemother-
with aspirin. apy, surgical excision, and postsurgical
chemotherapy; radiation therapy is used
Background Information if needed.
Osteoid osteoma is a relatively common, be-
nign tumor and usually occurs between the References
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CHAPTER44
Back Pain in a Child
■ Munesha Ramsinghani Lona, PT, DPT, PCS ■ Josiane Stickles, PT, DPT

Description of the Symptom ■ Night pain, pain at rest, or unremitting pain


■ Unexplained weight loss
This chapter describes possible causes of ■ New or evolving neurological symptoms:
back pain in a child, including the region ■ Abnormal reflexes/posturing
of the posterior body between the eighth ■ Loss of bowel and/or bladder function

rib and posterior superior iliac spines. ■ Sensory changes


■ Radiating pain

Special Concerns ■ Abdominal tenderness


■ Changes in functional mobility ■ Skin rash, tenderness on palpation
■ Fever ■ Postural changes

CHAPTER PREVIEW: Conditions That May Lead to Back Pain in a Child

T Trauma
REMOTE LOCAL
COMMON
Not applicable Muscle strain/overuse syndrome 885
Scheuermann’s disease (idiopathic/structural kyphosis) 888
Spondylolisthesis 890
Spondylolysis 890
UNCOMMON
Not applicable Fractures:
• Burst 881
• Compression 881
• Flexion-distraction 882
• Fracture-dislocations 882
Lumbar radiculopathy 884
RARE
Not applicable Slipped vertebral apophysis 889

I Inflammation
REMOTE LOCAL
COMMON
Not applicable Aseptic
Juvenile rheumatoid arthritis 883
Septic
Not applicable
(continued)

873
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874 Chapter 44 Back Pain in a Child

Inflammation (continued)
BACK PAIN IN A CHILD

REMOTE LOCAL
UNCOMMON
Not applicable Aseptic
Rheumatoid arthritis–like conditions of the spine:
• Juvenile ankylosing spondylitis 886
Septic
Discitis 880
Tuberculous spondylitis (spinal tuberculosis) 891
RARE
Aseptic Aseptic
Not applicable Rheumatoid arthritis–like conditions of the spine:
• Arthritis with inflammatory bowel disease 886
Septic • Juvenile dermatomyositis 887
Acute pelvic inflammatory • Psoriatic arthritis 887
disease 878 • Systemic lupus erythematosus 888
Appendicitis 879 Transverse myelitis 891
Cholecystitis 879
Pancreatitis 879 Septic
Pleuritis (pleurisy) 879 Meningitis 884
Pyelonephritis 880 Spinal epidural abscess 889
Vertebral osteomyelitis 895

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Idiopathic juvenile osteoporosis 883
Metabolic bone disease 885
Osteogenesis imperfecta (brittle bone disease) 885

Va Vascular
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Hemophilia 882
Sickle cell anemia (crisis) 889
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Chapter 44 Back Pain in a Child 875

BACK PAIN IN A CHILD


De Degenerative
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Malignant Primary, such as:
• Ewing’s sarcoma 892
• Leukemia 893
• Lymphoma 893
• Osteosarcoma (osteogenic sarcoma) 895
Malignant Metastatic:
Not applicable
Benign:
Not applicable
RARE
Malignant Primary, such as: Malignant Primary:
• Nephroblastoma (Wilms’ Not applicable
tumor) 880 Malignant Metastatic, such as:
Malignant Metastatic: • Astrocytoma 892
Not applicable • Neuroblastoma 894
Benign: • Rhabdomyosarcoma 895
Not applicable Benign, such as:
• Aneurysmal bone cyst 891
• Langerhans’ cell histiocytosis 892
• Osteoblastoma 894
• Osteoid osteoma 894

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Joint hypermobility syndrome 883
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876 Chapter 44 Back Pain in a Child


BACK PAIN IN A CHILD

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Psychosomatic back pain 886
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Common Ages at Which Back Pain Presents in a Child


APPROXIMATE AGE CONDITION
Birth to 3 Years Discitis
Hemophilia
Langerhans’ cell histiocytosis
Meningitis
Metabolic bone disease
Neuroblastoma
Pleuritis
Pyelonephritis
Spinal epidural abscess
Transverse myelitis
Tuberculous spondylitis
Vertebral osteomyelitis
Wilms’ tumor
Preschool (3–5 Years) Appendicitis
Astrocytoma
Discitis
Joint hypermobility syndrome
Langerhans’ cell histiocytosis
Leukemia
Lymphoma
Meningitis
Neuroblastoma
Pleuritis
Pyelonephritis
Rhabdomyosarcoma
Vertebral osteomyelitis
Wilms’ tumor
Elementary School (6–11 Years) Aneurysmal bone cyst
Appendicitis
Astrocytoma
Discitis
Ewing’s sarcoma
Fractures
Hemophilia
Idiopathic juvenile osteoporosis
Joint hypermobility syndrome
Juvenile dermatomyositis
Langerhans’ cell histiocytosis
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Chapter 44 Back Pain in a Child 877

BACK PAIN IN A CHILD


Common Ages at Which Back Pain Presents in a Child—cont’d
APPROXIMATE AGE CONDITION
Leukemia
Lymphoma
Meningitis
Osteoblastoma
Osteogenesis imperfecta
Osteoid osteoma
Osteosarcoma
Pleuritis
Rhabdomyosarcoma
Sickle cell anemia
Spondylolisthesis
Spondylolysis
Systemic lupus erythematosus
Middle School (12–14 Years) Aneurysmal bone cyst
Appendicitis
Ewing’s sarcoma
Fractures
Hemophilia
Lumbar radiculopathy
Idiopathic juvenile osteomyelitis
Joint hypermobility syndrome
Juvenile dermatomyositis
Juvenile rheumatoid arthritis
Langerhans’ cell histiocytosis
Leukemia
Lymphoma
Muscle strain/overuse syndrome
Osteoblastoma
Osteogenesis imperfecta
Osteoid osteoma
Osteosarcoma
Pancreatitis
Psychosomatic back pain
Scheuermann kyphosis
Sickle cell anemia
Slipped vertebral apophysis
Spondylolisthesis
Spondylolysis
Transverse myelitis
High School (15–18 Years) Acute pelvic inflammatory disease
Aneurysmal bone cyst
Appendicitis
Cholecystitis
Ewing’s sarcoma
Fractures
Hemophilia
Lumbar radiculopathy
Idiopathic juvenile osteomyelitis
Joint hypermobility syndrome
Juvenile ankylosing spondylitis
Juvenile dermatomyositis
Juvenile rheumatoid arthritis
Leukemia
Lymphoma
Continued
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878 Chapter 44 Acute Pelvic Inflammatory Disease


BACK PAIN IN A CHILD

Common Ages at Which Back Pain Presents in a Child—cont’d


APPROXIMATE AGE CONDITION
Meningitis
Muscle strain/overuse syndrome
Psoriatic arthritis
Osteoblastoma
Osteogenesis imperfecta
Osteoid osteoma
Osteosarcoma
Pancreatitis
Psychosomatic back pain
Scheuermann’s kyphosis
Sickle cell anemia
Slipped vertebral apophysis
Spondylolisthesis
Spondylolysis
Transverse myelitis

Overview of Back Pain in a Child Description of Conditions That


May Lead to Back Pain in a Child
There are differing points of view on whether
reports of back pain in children should be taken Remote
seriously. Studies have shown that back pain is ■ Acute Pelvic Inflammatory
common in about 34% to 60% of children.1,2 Disease
Some research has shown that at least 50% of
children with back pain have a significant spinal Chief Clinical Characteristics
disease,3 while other research4 has found that This presentation commonly includes lower
less than 30% are diagnosed with spinal disease. abdominal pain in adolescents, associated with
Reports of back pain are generally not as serious dull, constant, and poorly localized pain. Many
as previously thought; however, they still re- adolescents will also demonstrate emotional dis-
quire careful consideration. The patient’s his- tress because of the unknown source or cause
tory and pain pattern are extremely important. of pain.7 Other symptoms such as abnormal
The spine and related structures of a child vaginal discharge, fever, pain in the right up-
differ significantly from those of an adult and per quadrant, painful intercourse, and irreg-
this should affect how the physical therapist ular menstrual bleeding can occur as well.
evaluates and treats the child with back pain. Background Information
Unlike in adults, vascular channels are present The U.S. Centers for Disease Control and Pre-
in children that connect the cartilaginous vention now recommends that only one of the
growth plate to the nucleus pulposus, allowing following criteria be identified to confirm the
direct invasion of infectious organisms.5 The diagnosis: adnexal (pertaining to the uterus
pediatric spine has increased hydration of the appendages), cervical motion, or uterine
intervertebral disks, incomplete ossification of tenderness.8 This condition is caused by a
vertebrae, the presence of ring apophyses, bacterial infection in the upper genital tract,
and increased elasticity and ligamentous laxity most commonly associated with sexually
resulting in greater flexibility. Younger children transmitted infections such as gonorrhea and
also tend to be relatively weaker than adults, chlamydia. Each year 1 million adolescents in
especially in the abdominal region, resulting in the United States will become infected, with
increased lordosis. In trauma the increased the most prevalence in women between 15 and
elasticity of the ligaments and muscles may 23 years of age.9 Treatment typically includes
allow for neurological damage even in the aggressive antibiotics and hospitalization. If
presence of normal radiographs.6 left untreated, this condition often leads to
1528_Ch44_873-897 08/05/12 6:02 PM Page 879

Chapter 44 Pleuritis (Pleurisy) 879

more serious problems such as infertility, prolonged fasting or rapid weight reduction,

BACK PAIN IN A CHILD


ectopic pregnancy, and chronic pelvic pain. treatment for childhood cancer, abdominal
surgery, and pregnancy. Laparoscopic chole-
■ Appendicitis cystectomy is commonly performed in symp-
Chief Clinical Characteristics tomatic infants and children. In cases of
This presentation typically involves pain in obstructive jaundice and pancreatitis, endo-
the middle of the abdomen, near the navel, that scopic retrograde cholangiography with stone
gradually moves to the lower right area of extractions is also performed.11
the abdomen. Pain is often accompanied by
■ Pancreatitis
nausea with vomiting and fever. Tenderness
in the right lower quadrant, specifically Chief Clinical Characteristics
rebound tenderness, is a component for diag- This presentation can include back pain in
nosis confirmation. combination with abdominal pain, persistent
vomiting, and fever. Thoracolumbar pain is
Background Information more commonly reported in older children and
Diagnosis is difficult in young children, a adolescents with this condition, and younger
factor contributing to perforation rates of 30% children commonly report primary symptoms
to 60% in this population. Approximately of abdominal pain and vomiting. The pain is
80,000 children experience this condition in epigastric and steady, often resulting in the
the United States annually, a rate of 4 per child assuming an antalgic position with hip and
1,000 children younger than 14 years of age. knees flexed, sitting upright, or lying on the
The incidence of this condition increases with side to alleviate pain. Lying supine and exten-
age, peaking in adolescence and rarely occur- sion of the spine aggravate the symptoms of
ring in children younger than 1 year old. Diag- pain. Children with this condition will also be
nosis is confirmed by detailed abdominal exam- very uncomfortable and irritable. The abdomen
ination. Laboratory evaluation may include may be distended and tender; a mass may be
complete blood cell count and urinalysis. Ultra- palpable.
sound and computed tomography will reveal an
enlarged appendix. Treatment requires imme- Background Information
diate surgical removal of the appendix.10 This condition is the most common pancreatic
disorder in children. Blunt abdominal injuries,
■ Cholecystitis mumps and other viral illnesses, multisystem
Chief Clinical Characteristics disease, congenital anomalies, and biliary
This presentation typically involves right upper microlithiasis account for most known etiolo-
quadrant or epigastric pain, nausea, vomiting, gies. Many cases are of unknown etiology or
fever, and jaundice. Right upper quadrant are secondary to a systemic disease process.
guarding and tenderness are present. Pain may Diagnosis can be established by measuring the
radiate to an area just below the right scapula. serum amylase and amylase isozyme levels.
Treatment is focused on alleviating pain and
Background Information restoring metabolic homeostasis. Pain medica-
This condition refers to inflammation or in- tion is prescribed and fluid electrolyte and
fection of the gallbladder. In children, it is mineral balance should be maintained.12
usually caused by an underlying infection. It
can also be a result of gallstones causing an ■ Pleuritis (Pleurisy)
obstruction of the gallbladder ducts. This Chief Clinical Characteristics
condition is rare in otherwise healthy children. This presentation typically involves a dull ache
It occurs more commonly in children with localized over the chest wall and is referred to
various other predisposing conditions such as the shoulder or the back. If pleuritis is caused
chronic hemolytic disease (ie, sickle cell ane- by pleural effusion, the primary symptom is
mia), obesity, ileac disease, cystic fibrosis, pain, which is exaggerated by deep breathing,
chronic liver disease, Crohn’s disease, pro- coughing, and straining. Pain with breathing
longed parenteral nutrition, premature birth accounts for grunting and guarding of respira-
with complicated medical or surgical course, tion, and children with this condition will
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880 Chapter 44 Pyelonephritis

often lie on the affected side in an attempt to first year of life. The prevalence of urinary
BACK PAIN IN A CHILD

decrease respiratory excursions. tract infections varies with age. Diagnosis


is confirmed usually by urine culture and
Background Information
common course of treatment is with a wide
The most common causes of this condition
spectrum of antibiotics.14
in children are bacterial pneumonia, heart fail-
ure, rheumatological causes, and metastatic TUMORS
intrathoracic malignancy. A variety of other
■ Nephroblastoma
diseases account for the remaining cases, in-
(Wilms’ Tumor)
cluding tuberculosis, lupus erythematosus,
aspiration pneumonitis, uremia, pancreatitis, Chief Clinical Characteristics
subdiaphragmatic abscess, and rheumatoid This presentation typically includes an
arthritis. Boys and girls are affected equally. asymptomatic, nontender mass in the
Diagnosis is confirmed upon radiographic abdomen. Associated symptoms may be ab-
examination, and often a pleural tap is per- dominal pain, fever, nausea, and vomiting.
formed to examine the exudates of pleural Hematuria, anemia, and hypertension can
effusion. Treatment is dependent on the under- occur, since the kidneys are instrumental in
lying disease. Antibiotics are often prescribed controlling blood pressure. Shortness of breath
when the underlying pleural effusion is caused may be present if metastases to the lungs are
by bacterial pneumonia. When large amounts present.15,16
of fluid are present, the fluid can be drained to Background Information
make the patient more comfortable.13 This condition involves a tumor that origi-
nates from renal precursor cells. It is the sec-
■ Pyelonephritis ond most common abdominal tumor in
Chief Clinical Characteristics infants and children up to 5 years old and
This presentation commonly includes chills, is the most common primary malignant
fever, pain in the lower part of the back on tumor of the kidney. Multiple laboratory
either side, malaise, nausea, vomiting, and tests, including complete blood count, ultra-
occasionally diarrhea. Some newborns and sound, magnetic resonance imaging, or
infants may show nonspecific symptoms such as computed tomography, are indicated. Treat-
jaundice, poor feeding, irritability, and weight ment includes removal of the kidney con-
loss. About one-third of individuals with this con- taining the tumor followed by chemother-
dition also have symptoms of cystitis, including apy and/or radiation. The survival rate is
frequent, painful urination. One or both typically 70% to 95%, especially in young
kidneys may be enlarged and tender, with ten- children, or those with stage I, II, or III
derness felt in the flank on the affected side. tumors.15 Recurrences are usually seen
within 3 years. Postradiation scoliosis may
Background Information follow in the second decade.
This condition is a form of urinary tract infec-
tion occurring in the upper urinary tract. In- Local
volvement of the renal parenchyma is called
acute pyelonephritis, whereas if there is no ■ Discitis
parenchymal involvement, the condition may Chief Clinical Characteristics
be called pyelitis. The term cystitis is used This presentation consists of back pain as a pre-
when there is bladder involvement. Urinary senting symptom in approximately 50% of chil-
tract infections occur in 3% to 5% of girls and dren with discitis. Children under 3 years of
1% of boys. In girls, the first urinary tract age often will refuse to bear weight on lower
infection usually occurs by the age of 5 years, extremities or will limp during ambulation.
with peaks during infancy and toilet training. Infants may refuse to crawl or sit up. Children
After the first urinary tract infection, 60% to ages 3 to 8 years of age may also avoid other
80% of girls will develop a second urinary activities and report having vague abdominal
tract infection within 18 months. In boys, and back pain. Older children and adolescents
most urinary tract infections occur during the may report abdominal pain that is usually
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Chapter 44 Compression 881

localized to the thoracolumbar region of the causing sepsis and to stabilize structures at risk

BACK PAIN IN A CHILD


lumbar spine (although discitis is rare in this par- for deformity causing neurological damage.5
ticular age group).17 They may also report leg
and buttock pain due to nerve root irritation. FRACTURES
In all age groups, a low-grade fever may be ■ Burst
present. Commonly there are spasms of the Chief Clinical Characteristics
paravertebral musculature, decreased range of This presentation includes acute back pain in
motion of the spine, and tightness of the ham- the region of injury that will depend on the
strings. Children will tend to keep their back stiff severity of the injury, limitations in move-
and rigid during walking and squatting activ- ment, and tenderness on palpation. With se-
ities, avoiding fluid motion of the spine. Chil- vere injury neurological signs may be present
dren will often maintain a flexed position. If left as a result of spinal cord compression.
untreated, the pain can progress such that the
only position of relief is supine.5 Background Information
These fractures are rare in children, accounting
Background Information for approximately 10% of thoracolumbar frac-
This condition is an inflammation, irritation, tures with a mean age of 14 years.20 Burst frac-
and swelling of the intervertebral disk space or tures are a result of axial compression com-
vertebral end plate. This is an uncommon bined with flexion and involve the anterior and
condition usually seen in children younger middle column structures. With severe injury
than 10 years of age. It can be caused by a bac- the posterior longitudinal ligament can be
terial or viral infection or other inflammatory damaged, resulting in instability and potential
process, such as autoimmune diseases. The neurological injury.21,22 Radiographs will show
lumbar disks are most commonly affected, less than 50% of anterior vertebral body
followed by the thoracic spine.18 Laboratory collapse and less than 35-degree kyphosis with
studies include a complete blood workup; a stable injury.22 There may also be bone frag-
however, children with discitis will often have ment in the spinal canal from the middle
normal white blood cell counts and be column and a vertical laminar fracture. A com-
afebrile, demonstrating no systemic symp- puted tomography scan will show disruption
toms.19 Staphylococcus aureus is the most com- of the anterior and posterior vertebral ring. For
mon source of infection for children with stable fractures treatment includes immobi-
discitis. X-rays will reveal changes in the disk lization with hyperextension casting or brac-
space and end plates of vertebral bodies. Mag- ing. Unstable injuries and the presence of neu-
netic resonance imaging will assist with early rological symptoms may require surgical
diagnosis and assist in differentiating discitis intervention including internal fixation and
from other infectious disease processes. Non- decompression.
surgical treatment includes immobilization
with a thoracolumbar orthosis and rest. An- ■ Compression
tibiotic treatment is often added to ensure
Chief Clinical Characteristics
eradication of the infection. If the child con-
This presentation includes acute back pain in
tinues to have symptoms and is not showing
the region of injury that will depend on the
signs of improvement, surgical drainage may
severity of the injury, limitations in move-
be necessary. If left untreated, the infection can
ment, and tenderness on palpation. With se-
spread to adjacent vertebral bodies, progress-
vere injury neurological signs may be present
ing to vertebral osteomyelitis. Children pre-
as a result of spinal cord compression.
senting with symptoms of septic discitis
should be referred to a physician for evalua- Background Information
tion and treatment. Treatment includes an in- These fractures are uncommon injuries in
tensive course of antibiotics, immobilization children and adolescents, but child abuse
of the affected spine, and rest. Immobilization results in about 3% of spinal fractures in
with a brace, cervicothoracic orthosis, or halo children, typically at the thoracolumbar junc-
may help prevent deformity and aid in pain tion.20,23 An axial compression fracture can
relief. Surgery is indicated to drain abscesses occur at any age and is typically a result of
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882 Chapter 44 Flexion-Distraction

severe trauma such as a fall on the buttocks. In surgical intervention, progressive kyphosis
BACK PAIN IN A CHILD

the absence of trauma, vertebral compression can occur.20


fractures may also be found in children
with osteopenia and hepatoblastoma.14 Radi- ■ Fracture-Dislocations
ographs will show decreased anterior vertebral Chief Clinical Characteristics
height and computed tomography scan may This presentation includes acute back pain in
show anterior end-plate fracture with intact the region of injury that will depend on the
posterior wall. Symptoms may be relieved severity of the injury, limitations in move-
with casting or bracing in extension to allow ment, and tenderness on palpation. With
healing. severe injury neurological signs may be pres-
ent as a result of spinal cord compression.
■ Flexion-Distraction Fracture-dislocations are highly unstable in-
Chief Clinical Characteristics juries that involve failure of all three columns
This presentation includes acute back pain in of the spine, which are disrupted by any com-
the region of injury that will depend on the bination of compression, tension, rotation,
severity of the injury, limitations in movement, or shear.21,22
and tenderness on palpation. With severe
Background Information
injury neurological signs may be present as
The most common method of injury is flex-
a result of spinal cord compression.
ion with axial compression. The child may
Background Information present with neurological signs if there is
Flexion-distortion fractures are more com- cord compression following severe fracture-
mon in children than burst and compression dislocation. The child should undergo a
fractures, and result in tension failure of the complete neurological examination and a
middle and posterior columns with intact computed tomography or magnetic reso-
anterior longitudinal ligament.21 A variant nance imaging scan to determine the extent
of this injury is a Chance fracture, which is a of central cord involvement if any due to the
transverse fracture through the posterior and malalignment of the vertebral bodies. Life-
middle vertebral body. Flexion-distraction threatening intra-abdominal injuries and
injuries typically occur as a result of motor rib fractures are typically present with
vehicle accidents secondary to seat-belt fracture-dislocation and should be assessed
restraints that are designed for adults. In a and treated appropriately. Treatment can
child the lap restraint tends to rest across the include immobilization, traction, internal
abdomen, creating a fulcrum for distraction fixation into hyperextension, or vertebral
when the child is thrown forward into flex- fusion if the fracture-dislocation is unstable.
ion during a head-on collision.20,22 Inci- If there is injury to the spinal cord, the child
dence of this injury is unknown; however, should be managed with an intensive multi-
occurrences are increasing with mandatory disciplinary rehabilitation approach.20–22,24
use of restraints. If the child presents with
the classic lap-belt sign across the abdomen, ■ Hemophilia
flexion-distraction should be suspected. Chief Clinical Characteristics
Posterior element injury may not be appreci- This presentation commonly involves pain and
ated in plain radiographs and therefore the restricted movement in the lumbar spine in
child may be diagnosed only with a com- combination with symptoms in the hinge joints
pression fracture.20 A high incidence of of the elbow, knee, and ankle; muscle pain in the
intra-abdominal trauma is associated with a gastrocnemius, iliopsoas, or forearm; and an
flexion-distraction injury and should be absence of fever. Neurological deficits may
assessed and treated accordingly. Treatment occur several hours to days after hemorrhage
includes hyperextension casting or bracing. depending on the severity of the hemorrhage.
Surgical intervention is reserved for verte-
bral body involvement and typically involves Background Information
posterior fusion with compression instru- Usually a known diagnosis of hemophilia will
mentation for stability. For those without prompt clinicians to investigate this condition as
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Chapter 44 Juvenile Rheumatoid Arthritis 883

a diagnostic possibility. However, in cases pain. Developmental history may reveal delayed

BACK PAIN IN A CHILD


of a mild form of the disease process or in walking (~15 months of age). Children may
younger children who have not yet been diag- be considered “clumsy” or having poor coordi-
nosed, a careful history that may lead a clinician nation.29 Examination of joints may reveal
to believe the child may have a blood clotting hypermobility, most commonly in knees, elbows,
disorder should also make the clinician suspi- wrist, hand metacarpophalangeal joints, and
cious of the possibility of spontaneous spinal ankles.
epidural hematoma (SSEH) when a child pres-
Background Information
ents with back pain. This term is used to collec-
This condition may result from ligamentous
tively identify several X-linked disorders of
laxity, which is inherent and determined
blood coagulation. The most common of these
by genes that encode for collagen, elastin,
are Factor VIII deficiency, or hemophilia A,
fibrillin, and tenascin. Most people who pres-
comprising 80% to 90% of the population with
ent with only joint hypermobility have no
hemophilia. The next most common is Factor
symptoms or ill effects. Children are diag-
IX deficiency, also known as hemophilia B.25
nosed with this condition when the muscu-
These factors are necessary precursors for the
loskeletal symptoms of pain persist greater
blood clotting process to occur to stop bleeding.
than 12 weeks and when there is exercise-
Uncontrolled bleeding can occur in organ
induced pain and exercise intolerance with
systems, the central nervous system, muscle
joint hypermobility. Benign joint hypermobil-
tissue, and most frequently in joint spaces.
ity syndrome is said to exist provided that
The most common hemorrhage is spinal
patients do not have signs of any other
epidural hematoma. Spontaneous spinal
rheumatic, neurological, skeletal, or metabolic
epidural hematoma is rare in children; the cervi-
disease, such as Ehlers-Danlos syndrome or
cothoracic region is the most common site.26
Marfan’s syndrome. Physical therapy is indi-
The diagnosis of SSEH is confirmed by mag-
cated, including strengthening of musculature
netic resonance imaging. Treatment includes
surrounding hypermobile joints, education
administration of factor replacement and rest.
about joint protection, and pain management
Generally, children have good neurological
techniques.30
outcome and the hematoma resolves after factor
therapy is administered. Surgical treatment is
■ Juvenile Rheumatoid Arthritis
reserved for severe cases and is considered for
intractable or progressive cases.27 Chief Clinical Characteristics
This presentation may include generalized back
■ Idiopathic Juvenile Osteoporosis pain, accompanied by peripheral joint pain of
Chief Clinical Characteristics affected joints: knees, ankles, elbows, wrist, and
This presentation may be characterized by low hands. Initial symptoms often include morning
back pain in combination with long bone and stiffness of affected joints; easy fatigability, par-
joint pain due to spontaneous vertebral fractures ticularly after school in the early afternoon;
and to spinal deformities, primarily kyphosis. joint pain later in the day; and joint swelling.
Arthritis is evidenced by joint swelling or
Background Information effusion and the presence of two or more of the
This condition is very rare, with multiple following signs: heat, limitation of range of
causes related to consequences of primary motion, and tenderness or pain on motion.
endocrine, gastrointestinal, bone marrow, con- Symptoms must persist for a minimum of
nective tissue, or developmental diseases. Onset 6 weeks. Typically, joints of the extremities
is typically before puberty.28 This condition is a are affected; however, the spine may also be
diagnosis of exclusion, and it is made when no affected. Back pain associated with this condi-
obvious underlying cause can be detected. tion may be the result of spinal involvement or
■ Joint Hypermobility Syndrome habitual posture secondary to pain.
Chief Clinical Characteristics Background Information
This presentation commonly includes arthral- This condition is one of the most common
gia, abnormal gait, joint deformity, and back rheumatic diseases of children and a major
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884 Chapter 44 Lumbar Radiculopathy

cause of chronic disability. The etiology of juve- radiculopathies rarely occur in children
BACK PAIN IN A CHILD

nile rheumatoid arthritis (JRA) is unknown; and adolescents, and are usually the result
however, it is believed to be an autoimmune of cumulative trauma rather than a single
response against cells in the joint fluid. The episode as in adults. Adolescents account for
end result of the autoimmune response can only 1% to 4% of reported herniations.1,35
include adhesions and osteophyte prolifera- Often there is a family history of lumbar disk
tion in the joint space, fibrosis of surrounding disease. Sacralization of the fifth lumbar verte-
tissue (tendons and ligaments) resulting in brae is frequently accompanied by low back
contracture, and scarring of the underlying pain and disk herniation proximal to the tran-
bone, causing the joint shape to be irregular.31 sitional vertebra. Radiographs rarely show loss
It affects the cervical spine more often than it of intervertebral disk height and, therefore,
affects the joints of the thoracic and lumbar a magnetic resonance imaging or computed
spine.32 The age of onset is set younger than tomography scan should be obtained. The
16 years to distinguish this condition from most common levels are L4–L5 and L5–S1.1,36
adult-onset disease. Complications of major Most patients respond well to conservative
concern are acute iritis and uveitis leading to treatment that includes rest, nonsteroidal
blindness. These complications are seen most anti-inflammatory drugs, and gradual return
often in those children with the pauciarticular to activities. Surgical treatment is usually
form of this condition. Because no definitive reserved for those with positive nerve tension
laboratory tests are available to identify JRA, signs, persistent abnormal sensation and
the diagnosis is made by exclusion of other reflexes, lower extremity weakness, and sciatic
rheumatic diseases or diagnoses such as pain. Excision of the intervertebral disk may
joint infections, trauma, malignancies, or sys- be necessary, and in rare cases of spinal insta-
temic illnesses, depending on the presenting bility, fusion may be indicated.34
symptoms. Medical management includes a
variety of oral medications used to control ■ Meningitis
joint inflammation such as nonsteroidal anti- Chief Clinical Characteristics
inflammatory drugs, antirheumatic drugs, This presentation can be characterized by back
and glucocorticoids. Intra-articular injections pain associated with systemic infection and
of glucocorticoids may be used to target single meningeal irritation including fever, nausea,
joints. In some severe cases, surgical interven- altered level of consciousness, irritability,
tion may be indicated for soft tissue releases, lethargy, anorexia, poor appetite, symptoms of
synovectomies, osteotomies, and arthroplasty. upper respiratory tract infection, myalgias,
■ Lumbar Radiculopathy arthralgias, tachycardia, hypotension, and
various cutaneous signs. Most commonly, this
Chief Clinical Characteristics
condition is preceded by several days of fever
This presentation includes back pain with
accompanied by upper respiratory tract or gas-
radiation to the lower extremity, positive
trointestinal symptoms, followed by the previ-
Lasègue’s sign,33 decreased lumbar range of mo-
ously mentioned, nonspecific symptoms.37
tion, lumbar muscle spasm, gait abnormalities,
Other possible signs include nuchal rigidity
scoliosis, hyperlordosis, and lower extremity
(stiff neck), positive Kernig’s sign (flexion of
sensory changes.33,34 Unlike adults, children do
the hip to 90 degrees with subsequent pain upon
not typically present with abnormal reflexes or
passive extension of the hip), and positive
lower extremity weakness.
Brudzinski sign (involuntary flexion of the knees
Background Information and hips after passive flexion of the neck in
Lumbar radiculopathy varies in severity with supine). Positive Brudzinski and Kernig’s signs
the severity of disk herniation. The direction are not consistently present in all children,
of herniation is usually posterolateral or poste- especially children younger than 12 to 18 months
rior. In adolescents, the protruding disk mate- old. Acute forms of this condition can present
rial is more fibrous and secured to the carti- in a dramatic fashion with sudden onset and
laginous plate and, therefore, appears more rapid progression, frequently leading to death
like a fracture of fibrocartilage.33 Lumbar within 24 hours. Rarely, the presentation involves
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Chapter 44 Osteogenesis Imperfecta (Brittle Bone Disease) 885

decreased level of consciousness, seizure activ- infants (<1,500 g) and in infants born preterm

BACK PAIN IN A CHILD


ity, and purpura (unblanchable lesion caused (<32 weeks gestation). The rate of osteopenia is
by bleeding in the skin). inversely related to birth weight and gestational
age and correlates to rate of fractures.40 Infants
Background Information
suspected of having this condition are adminis-
This condition is an infection of the meninges,
tered nutritional supplements in the neonatal
the outer covering of the spinal cord and brain.
intensive care units to improve bone strength,
The infection can be due to bacteria, virus, or
which further decreases incidence of fractures.
protozoa. In 1986, there was a significant
Decreased bone mineral density in the lumbar
decrease in meningitis caused by certain strains
vertebrae usually resolves by age 3 to 4 years.41
due to the implementation of universal immu-
nization against these certain bacterium. Major ■ Muscle Strain/Overuse Syndrome
risk factors are lack of immunity to specific
Chief Clinical Characteristics
pathogens associated with young age, nonim-
This presentation includes pain, muscle spasms,
munization, and being in close contact with
and tenderness. Pain may increase with exces-
infected people (household, day care centers,
sive flexion or extension and rarely radiates.
and schools). Transmission of the disease is via
person-to-person contact with respiratory tract Background Information
secretions or droplets. Fifty-five percent of Muscle strains can occur as acute injuries,
those diagnosed with meningitis are younger often from improper technique or from
than 19 years of age and incidence is higher chronic overuse. Injuries occur frequently in
among children younger than 2 years of age.38,39 children who carry heavy backpacks or wear
Diagnosis is confirmed by blood culture analy- them improperly, participate in sports requir-
sis and cerebrospinal fluid collected by lumbar ing excessive motion such as gymnastics,
puncture. Electroencephalography, magnetic or practice improper body mechanics. Diag-
resonance imaging, and computed tomography nostic tests will be negative and x-rays will be
give valuable information regarding brain normal. Both resisted contraction and stretch-
function and swelling. Antibiotics are most ing of the affected muscle may reproduce
commonly used as treatment for meningitis characteristic pain. Treatment includes rest,
originating from bacteria. Corticosteroids may nonsteroidal anti-inflammatory and muscle
also be used in conjunction to reduce the result- relaxant medications, gradual return to activi-
ing edema that may produce additional neuro- ties, and physical therapy.
logical injury. A large portion of the treatment
will focus on supportive care of the secondary ■ Osteogenesis Imperfecta (Brittle
problems resulting from the infection (pain, Bone Disease)
cardiovascular insufficiency, and decreased kid- Chief Clinical Characteristics
ney function). Children and adolescents pre- This presentation commonly involves a spectrum
senting with back pain and other symptoms of of disablement ranging from mild to lethal.
this condition should immediately be referred Children with milder forms (osteogenesis im-
to their primary care physician. perfecta [OI] type I) sustain vertebral fractures
usually after mild to moderate trauma, such as
■ Metabolic Bone Disease falls when learning to walk. This risk of fracture
Chief Clinical Characteristics decreases after puberty. Children with moder-
This presentation commonly includes radial ate to severe forms (OI types III and IV) may
metaphysic, femoral neck, and lumbar vertebrae be born with vertebral fractures or will sustain
fractures in an infant. several vertebral fractures over their lifetime.
Children with this condition commonly have blue
Background Information
sclera, which distinguishes it from other meta-
The risk for fracture is high; however, very
bolic disease processes.
few infants present with these fractures due to
decreased amounts of movement at this age. Background Information
This condition is also known as osteopenia of This condition is the most common cause of
prematurity; it occurs in very low birth weight genetically mediated osteoporosis. Treatment
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886 Chapter 44 Psychosomatic Back Pain

is aimed at fracture and deformity manage- RHEUMATOID ARTHRITIS–LIKE


BACK PAIN IN A CHILD

ment. Individuals with this condition are CONDITIONS OF THE SPINE


also treated with bisphosphonate agents and ■ Arthritis with Inflammatory
intravenous pamidronate to enhance bone Bowel Disease
mineralization.42
Chief Clinical Characteristics
■ Psychosomatic Back Pain This presentation involves sacroiliac joint or
Chief Clinical Characteristics low back pain and stiffness associated with an
This presentation includes back pain in the acute episode of gastrointestinal upset.
absence of a definitive diagnosis of inclusion or Background Information
exclusion. In children and adolescents whose Children with this disease are likely to be
symptoms seem to exceed the findings and who HLA-B27 positive, suggesting an underly-
may be subject to other home or school factors, ing genetic predisposition. In some cases,
psychosomatic reactions must be considered. when the inflammatory bowel disease is
These patients generally report severe symp- latent, the course of arthritis may be similar
toms but rarely have positive physical findings. to the course of juvenile ankylosing
They may report a relatively minor preceding spondylitis.48,49
trauma. The patients often report other nonspe-
cific symptoms including headaches, neck pain, ■ Juvenile Ankylosing Spondylitis
shoulder pain, chest pain, fatigue, abdominal Chief Clinical Characteristics
pain, limb pain, and difficulty breathing.26 This presentation includes an insidious onset
Background Information of sacroiliac and/or low back pain and stiff-
Of children and adolescents reporting symp- ness that may be unilateral or bilateral and
toms, 10% to 25% can be of psychosomatic difficult to localize initially. Sacroiliitis is the
origin.26,43 The prevalence of this disorder hallmark sign, however, along with spinal
tends to increase with age; adolescents ages pain and stiffness, usually does not manifest
16 to 18 years are more likely to have psycho- until 2 to 5 years after onset. The pain will
somatic symptoms. Some studies have also become more persistent over time, along with
shown a higher prevalence in girls than in gradual loss of spinal mobility and a reduc-
boys.44 The symptoms of neck, shoulder, and tion in normal lumbar lordosis. Symptoms
back pain were earlier thought to be the result are typically worse at night or in the morning
of carrying weight and the frequency of use of and last at least 30 minutes. In ankylosing
backpacks and school bags; however, psycho- spondylitis pain may be relieved with rest
somatic factors appear to be more strongly or only mild activity. During active disease,
related to the occurrence of neck, shoulder, systemic symptoms such as fever and weight
and/or back pain complaints than weight and loss may be present, and the child may refuse
type of school bag.45 Diagnostic studies often to walk. Associated complications may
include administering standardized tests such include atlantoaxial subluxation, uveitis
as the Family Environmental Scale, Brief leading to blindness, acute iritis, aortic valve
Symptom Inventory, and Children’s Depres- insufficiency, decreased vital capacity, neuro-
sion Inventory to obtain objective measures logical complications, and, rarely, gastrointesti-
and establish a diagnosis. It is also not uncom- nal and renal problems.
mon to find that another family member has Background Information
similar problems and that the child has This condition is the most prevalent spondy-
subconsciously taken on those symptoms.46 A loarthropathy in children, found primarily
detailed medical evaluation must be com- in adolescent boys and young men. Onset is
pleted to rule out organic causes of back pain usually in the second or third decade of life,
before the consideration of psychosocial prob- but 10% to 20% of those affected will have
lems as the cause of symptoms. Treatment of onset prior to age 16. Only about 25% of
these patients includes aggressive psychologi- children with this disease will present ini-
cal treatment and physical therapy.47 tially with sacroiliac or spinal symptoms.
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Chapter 44 Psoriatic Arthritis 887

Peripheral joints are involved in 30% to 50% myositis with subsequent calcinosis occurs,

BACK PAIN IN A CHILD


of patients, usually within the first 10 years causing severe limitation of range of motion
of disease. Ankylosing spondylitis is a of involved joints. The onset of proximal
chronic disease with intermittent periods of weakness is insidious and difficult to recog-
remission; it is rarely active persistently. nize. It is often detected by difficulty in
There is no specific laboratory test for anky- everyday activities such as climbing stairs,
losing spondylitis; however, most patients reaching overhead, going from sitting to
have the presence of HLA-B27 (human standing, and going from the floor to stand-
leukocyte antigen), elevated erythrocyte ing.52 This condition occurs in approxi-
sedimentation rate and C-reactive protein mately 1 to 3 children per million every year.
level, and absence of rheumatoid factor. The average age of onset is between 6 and
Radiographic changes include erosion, scle- 7 years of age with a higher prevalence in
rosis, and eventually fusion of the sacroiliac girls. Laboratory investigations include
joint and vertebrae. Magnetic resonance im- blood tests, muscle biopsy, and specific anti-
aging and computed tomography of the body tests. Treatment includes protection
sacroiliac joint may assist with early diagno- from the sun and a possible course of corti-
sis. Enthesitis distinguishes this condition costeroids. Physical therapy is indicated with
from juvenile rheumatoid arthritis. Individ- close supervision from the child’s rheuma-
uals with this condition typically begin a tologist and pediatrician. Physical therapy
course of nonsteroidal anti-inflammatory should include passive range of motion
medications upon diagnosis for pain con- and stretching during the disease course.
trol. Corticosteroids are used only short term Once active inflammation has resolved,
due to associated problems including osteo- treatment should focus on strengthening
porosis. Research indicates that treatment deconditioned muscle, regaining range of
with tumor necrosis factor can significantly motion, and performing weight-bearing
improve symptoms.50 activities to ensure adequate bone mineral-
ization. Bed rest and immobilization are
■ Juvenile Dermatomyositis contraindicated.
Chief Clinical Characteristics
This presentation is characterized by a skin ■ Psoriatic Arthritis
rash that may be located on the torso, low Chief Clinical Characteristics
back, and buttocks area early on in the This presentation involves psoriatic rash,
disease process. The characteristic skin rash arthritis, nail pitting, dactylitis (or “sausage
may also be found around the shoulder digit”), and occasional fatigue. As the disease
girdle, extensor surfaces of the arms and legs, progresses sacroiliitis is present, resulting in
medial malleoli of the ankles, and face. hip and low back pain. In children arthritis
Disease onset is often insidious and may may manifest prior to psoriasis, or onset of
include fatigue, low-grade fever, weight loss, arthritis and psoriasis is concurrent.
irritability, arthralgia, and abdominal pain.
Background Information
Proximal muscle weakness is usually recog-
Chronic iridocyclitis is seen in approxi-
nized after a median of 2 months.51 Back
mately 15% of children.48,49 This condition
pain, although an uncommon symptom of
typically occurs in young girls, usually man-
this condition, is often reproduced with pal-
ifesting between 7 and 13 years of age, but
pation of the low back. Although this condi-
always occurring prior to age 16 years. There
tion is rare in children, it is the most common
is almost always a family history of psoriasis.
of its type.
Radiographs and computed tomography
Background Information scan will determine extent of involvement of
The disease process is triggered by an anti- the spine, hips, and sacroiliac joint. Treat-
gen-driven response, which manifests as a ment includes nonsteroidal anti-inflammatory
skin rash. In the absence of prompt diagno- medications, corticosteroids, and topical
sis and treatment, the development of or systemic medications for psoriasis.
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888 Chapter 44 Systemic Lupus Erythematosus

Modalities can also be used to control in- ■ Scheuermann’s Disease


BACK PAIN IN A CHILD

flammation and pain. (Idiopathic/Structural Kyphosis)


■ Systemic Lupus Erythematosus Chief Clinical Characteristics
This presentation includes pain and mild to
Chief Clinical Characteristics severe rigid thoracic kyphosis with inability to
This presentation may initially include mus- actively correct the deformity.34 There is often
culoskeletal pain in any or all regions of the point tenderness over the apex of deformity,
body including the back in 74% of children,53 which is usually localized between T7 and T9.
in the presence of fever, fatigue, arthralgia, Neurological complications are rare.
malar rash, anorexia, lymphadenopathy,
pleuritic pain, and seizures. Background Information
This condition results in an increase in the
Background Information normal kyphotic curvature of the thoracic
This condition is a rheumatic disease of un- spine that is rigid. It results in wedge-shaped
known cause characterized by autoantibod- vertebrae with loss of anterior height.6,15,34
ies directed against self-antigens and result- The problem is typically cosmetic, with mild
ing in inflammatory damage to target organs pain that is localized to the area of the defor-
including kidneys, blood-forming cells, and mity during forward flexion.1 In many cases of
the central nervous system. The incidence this condition, there is compensatory lumbar
varies by ethnicity and location. Prevalence lordosis that can be painful. Due to increased
rates vary from 4 to 250 in 100,000 children. lordosis there is a high incidence of spondylol-
The median age at diagnosis is approxi- ysis due to increased strain on the L5 pars
mately 10 years, and onset before age 8 is interarticularis.1,22 Etiology is unknown; theo-
uncommon.54 Diagnosis is confirmed by the ries include heredity, increased anterior pres-
combination of clinical and laboratory man- sure on the vertebrae impairing anterior
ifestations revealing multisystem disease. growth, mechanical weakening, and collagen
Treatment regimen depends on the affected defects.34,36 Incidence is approximately 8%,
target organs. Nonsteroidal anti-inflammatory and is most common in adolescent males.
agents are used to treat arthralgia and Diagnosis is confirmed with lateral standing
arthritic symptoms. Corticosteroids have radiographs that show the vertebral deformi-
been demonstrated to control symptoms ties of decreased anterior height, reduced disk
and autoantibody production in systemic space, end-plate irregularities, and Schmorl’s
lupus erythematosus. Patients with severe nodes, which are thought to be disk herniation
disease may require cytotoxic therapy. When into the vertebral body. A consistent criterion
the child is cleared for activity, physical ther- is wedging of 5 degrees in three or more
apy should focus on stretching, joint protec- adjacent vertebra.6,55 Radiographs will differ-
tion, and relaxation techniques to manage entiate Scheuermann kyphosis from postural
joint and muscle pain. During periods of round back, in which there are no structural
remission, an exercise program of strength- changes and which is correctable with hyper-
ening and cardiovascular exercise may be extension.6,34,55 Treatment will vary depending
implemented to maintain the strength of on the degree of deformity and the age of the
surrounding musculature and to prevent the child. Skeletally mature asymptomatic individ-
detrimental effects of bone demineralization uals do not require treatment. Younger chil-
due to prolonged use of corticosteroids.54 dren with mild deformity may respond favor-
Physical therapy treatment should be pro- ably to activity modification and an exercise
vided under supervision of the child’s program emphasizing hyperextension activi-
physician. The natural history is unpre- ties to relieve pain and increase flexibility.
dictable; patients may present with a history Physical therapy will not change the structural
of many years of symptoms or with acute, deformities. Extension bracing may be benefi-
life-threatening disease. Left untreated, this cial in those who are skeletally immature. Sur-
condition may result in spontaneous remis- gical correction is reserved for individuals who
sion, years of disease, or rapid death. are skeletally mature and who are symptomatic
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Chapter 44 Spinal Epidural Abscess 889

and have not responded to conservative treat- and bone grafting.59 Treatment of pain caused

BACK PAIN IN A CHILD


ment. This may include anterior and/or poste- by sickle cell disease focuses on pain manage-
rior spinal fusion with instrumentation.34,56 ment using nonsteroidal anti-inflammatory
drugs and limited use of opiate medication.
■ Sickle Cell Anemia (Crisis) Other treatments targeting the disease process
Chief Clinical Characteristics are aimed at decreasing red blood cell adhe-
This presentation includes joint pain with sions, increasing cellular hydration, lowering
possible physical signs in the spine including blood viscosity, and elevating hemoglobin
local tenderness over the spinous process and levels. Bone marrow transplantation is the
decreased range of back motion.57 Bone changes only cure for sickle cell disease, but is only
cause pain due to marrow hyperplasia, tissue is- an option for children younger than 16 years
chemia, and infarction due to vaso-occlusion. of age.
Background Information ■ Slipped Vertebral Apophysis
This condition results from a point mutation
in the genetic code that causes structural Chief Clinical Characteristics
changes to the hemoglobin molecule. This This presentation involves low back pain, a pos-
structural change decreases the affinity of oxy- itive Lasègue’s sign, and motor and sensory signs
gen to red blood cells, thereby decreasing that correspond with a spinal root distribu-
delivery of oxygen to structures of the body. tion.20,36 This injury is most common in adoles-
There are different strains of the disease, cent boys participating in sports that require
depending on the component of genetic code repeated heavy lifting.
affected, which causes various levels of severity Background Information
and involvement expressed in the disease Injury is usually the result of repetitive axial
process. Newborn infants seldom exhibit loading causing the ring apophysis to slip, or
features of sickle cell disease; however, it can even separate, and move into the spinal canal.
be diagnosed by blood tests. Clinical manifes- The majority of these injuries occur at L4–L5
tations are uncommon prior to age 5 to or L5–S1. In young adolescents the ring
6 months. Infants with sickle cell anemia have apophysis is not yet fully ossified and adhered
abnormal immune function, making bacterial to the vertebral body. Radiographs may show
sepsis the greatest risk for morbidity and mor- the apophysis fragment within the spinal
tality. By age 5, most children will have a canal, but this is best viewed with computed
painful episode of the small bones of the tomography or magnetic resonance imaging.
hands and feet that is believed to be caused Treatment typically requires surgical excision
by choking off of blood supply as a result of of the fragment, and most patients respond
rapidly expanding bone marrow. Depending well.34
on the type of sickle cell disease, children may
have symptoms ranging from never experienc- ■ Spinal Epidural Abscess
ing any painful episodes to experiencing some Chief Clinical Characteristics
pain on nearly a daily basis. Episodes of severe This presentation commonly includes fever, back
pain requiring hospitalization occur on aver- pain, and radiculopathy.60 The radicular symp-
age one time per year. Young children usually toms can progress to weakness and paralysis.
experience pain of the extremities and older
children/adolescents are more likely to experi- Background Information
ence pain in the head, chest, abdomen, and Staphylococcus aureus is the bacteria most
back. Sickle cell anemia affects most of the commonly responsible for this condition.
other organ systems of the body, leading to a Children at higher risk include those with dia-
variety of complications.58 Avascular necrosis betes mellitus, and those who have suffered a
leading to collapse of vertebral bodies and in- recent trauma or have recently had a lumbar
fective spondylitis are some of the more seri- puncture. This condition most commonly
ous complications of the spine, the majority of occurs in adults, but also can occur in young
which require anterolateral decompression infants who have suppressed immune systems.
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890 Chapter 44 Spondylolisthesis

The main challenge with this condition is common site for slippage is the fifth lumbar
BACK PAIN IN A CHILD

achieving early diagnosis before these neuro- vertebra on the sacrum. Standing anteroposte-
logical symptoms occur. Diagnosis in infants rior and lateral radiographs are needed to
can be difficult due to the inconsistent presen- determine the grade of slippage, and an
tation. Older infants and toddlers who are oblique view is necessary to appreciate the pars
ambulatory may limp or refuse to bear weight defect. In high-grade slips with neurological
on an extremity because of weakness, paraple- deficits, a complete neurological exam is indi-
gia, or paraparesis, indicative of neurological cated. Treatment is determined by symptoms,
deficits. Magnetic resonance imaging displays clinical signs, and the grade of slip. Asympto-
the greatest diagnostic accuracy. Lumbar matic patients do not require intervention.
puncture to determine cerebrospinal fluid Those with low-grade slips and pain tend to do
protein concentration is not indicated for well with conservative treatment of activity
diagnosis because it could lead to bacteria restriction, bracing, and exercises. Fusion is
spreading into the subarachnoid space, placing recommended for grade II slips or greater even
the child at risk for meningitis.61 Nonsurgical if they are asymptomatic because they are at
management involves treatment with antibi- risk for further slippage during growth
otics, and this approach often is effective in spurts.22,36 Reduction of the slip is not recom-
children diagnosed early in the disease process. mended because of the high risk of neurologi-
In the later stages of the disease process, chil- cal injury.6,22,36 Patients are more prone to
dren with neurological deficits may require recurrent symptoms and clinical deformity if
surgical intervention in conjunction with local forward slipping is allowed to progress. Pro-
antibiotics. Laminectomy is the most common gression of slip, delayed union, neuropathy of
surgical procedure used to decompress the the fifth nerve root, and cauda equina syn-
lesion on the spinal cord. Children suspected drome have all been reported as postsurgical
of having this condition should immediately complications.22,36
be referred for medical evaluation and treat-
ment by a physician. ■ Spondylolysis
Chief Clinical Characteristics
■ Spondylolisthesis This presentation can include low back pain
Chief Clinical Characteristics with hyperextension that rarely radiates, scol-
This presentation includes low back pain that iosis, decreased lumbar lordosis, and increased
may radiate to the lower extremities, limitations lumbosacral kyphosis.34 The child will often
in range of motion, a positive Lasègue’s sign, and have hamstring tightness that can affect gait.1,6
tenderness over the lumbar spinous processes Adolescents participating in sports that require
with or without a palpable step. Significant repetitive hyperextension, such as gymnastics,
hamstring tightness or spasm may result in an wrestling, and football, appear to be at increased
abnormal gait pattern with short stride length, risk.6 Most children with spondylolysis are
flexed knees, and pelvic waddle. Decreased lum- asymptomatic or may have symptoms that are
bar lordosis, scoliosis, and flattened buttocks mild and therefore often overlooked.
may also be present. Neurological signs are rare,
Background Information
but may be present even in low-grade slips
This condition is a congenital or acquired
secondary to nerve root compression.6,22,36,62
defect of the pars interarticularis, the bone
As with spondylolysis (see next entry), symptoms
between the superior and inferior facets of the
are usually aggravated by activities that require
vertebrae. An acquired defect would arise as a
repetitive flexion and extension such as
stress or fatigue fracture of the pars.1,36 The
gymnastics.
pars interarticularis defect can occur at any
Background Information level, most commonly at L5, and can occur
This condition involves the forward displace- unilaterally or bilaterally. A bilateral defect no
ment of a proximal vertebra on the one below longer provides posterior stability and may
it, typically as a result of bilateral pars interar- result in forward slippage, especially of L5 over
ticularis defect. The pars defect is not present S1, known as spondylolisthesis. Spondylolysis
in congenital spondylolisthesis. The most can occur at any age, but is rarely seen before
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Chapter 44 Aneurysmal Bone Cyst 891

age 10; it appears to be more prevalent in boys. months. High doses of corticosteroids have

BACK PAIN IN A CHILD


An oblique standing view is the best view to also been used, however, there is no evidence
show the pars defect that can easily be over- that they significantly affect outcome.15,24
looked if only anteroposterior and lateral
views are taken.1,22,63 Computed tomography ■ Tuberculous Spondylitis (Spinal
or bone scan is used if radiographs are incon- Tuberculosis)
clusive, and single-photon emitted computed Chief Clinical Characteristics
tomography will determine if the lesion is This presentation may include pain, tender-
acute or chronic.36 Treatment depends on the ness, and weakness in the presence of possible
degree of the defect and whether the patient is systemic signs including fever, night sweats,
symptomatic. If pain is present in the absence anorexia, weight loss, and sensory changes.
of spondylolisthesis, immobilization with a Neurological deficits are uncommon in chil-
thoracic-lumbar-sacral orthosis for 3 to dren, but if present, may be due to pressure from
6 months may be most beneficial. Physical paravertebral abscess.
therapy should focus on hamstring and lumbar Background Information
musculature stretching, abdominal strengthen- This condition originates and affects the ante-
ing, and monitoring of gradual return to activ- rior vertebral bodies in the lower thoracic and
ity. With persistent pain unrelieved by nons- upper lumbar spine at onset and may affect the
teroidal anti-inflammatory medication, rest, disks in later stages of the disease. In some
and physical therapy, surgical stabilization can cases vertebral collapse occurs, resulting in
be achieved through posterior spinal fusion.6,34 kyphosis. Diagnostic testing includes skin
Any child with this condition should be tests, erythrocyte sedimentation rate, histol-
followed closely, because they are at risk for ogy, and computed tomography. Treatment in-
progression to spondylolisthesis. Girls tend to cludes antibiotics, antituberculosis therapy
have a higher rate of progression. such as pyrazinamide, isoniazid, and rifampin,
■ Transverse Myelitis and in some cases, chemotherapy. Bracing may
be necessary to prevent progression of kypho-
Chief Clinical Characteristics
sis. Surgical treatment is usually indicated for
This presentation involves sudden onset of
abscesses resulting in cord compression, chil-
severe back pain and progressive symmetric
dren younger than 15 years of age with kypho-
lower extremity numbness and weakness that
sis of at least 30 degrees, and children younger
begins distally. Progression to complete paral-
than 10 years with destruction of the vertebral
ysis may take several hours to several days
bodies.22
depending on the severity and location of the
inflammation. There may also be loss of deep TUMORS
tendon reflexes and bowel and bladder control.
Patients often experience tightness in a belt- ■ Aneurysmal Bone Cyst
like fashion around the stomach at the level Chief Clinical Characteristics
affected. This presentation may include chronic back
pain and neurological signs due to cord com-
Background Information
pression; however, these tumors are typically
Onset of symptoms typically follows fever,
asymptomatic and are usually discovered
malaise, and a viral infection, often Lyme dis-
when a pathological fracture or hemorrhage
ease, or influenza, rubella, mumps, varicella,
occurs.
herpes, or Epstein-Barr viruses. Computed
tomography or magnetic resonance imaging Background Information
is indicated to rule out cord compression, This condition involves a highly vascularized
abscess, or neoplasm, and cerebrospinal fluid mass with fibrous connective tissue.64
exam will differentiate transverse myelitis Locally, the lesion can be aggressive and
from Guillain-Barré syndrome, meningitis, destructive. Rarely, it is found in the
poliomyelitis, and other infectious diseases. sacrum.65 Peak age of diagnosis is typically
Partial or full spontaneous recovery occurs in between 10 and 20 years of age with a
most patients, but can take up to several slight female predominance.64,65 Radiographs
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892 Chapter 44 Astrocytoma

suggestive of this condition will show an swelling, palpable mass, tenderness, and
BACK PAIN IN A CHILD

expansile, lytic lesion that can involve the decreased range of motion. Other symptoms
entire bone, extending into the vertebral can include fever, malaise, and weight loss
body. There may also be involvement of when there are metastases.71
adjacent vertebral bodies, the intervertebral
Background Information
disks, posterior ribs, and paravertebral soft
This condition is the most common malig-
tissue.65 Differential diagnosis includes uni-
nant primary tumor found in the pediatric
cameral bone cyst, giant cell tumor, and
spine56 and the second most common pedi-
malignancy. Treatment can include excision
atric malignancy overall.72 Approximately
or curettage with bone graft, and possible
2.1 children per million are diagnosed with
fusion with care to preserve nerve roots.1,66
this condition each year with predominance
Complete resection is not always achieved
in males. Diagnosis is usually between the
due to proximity to neurovascular struc-
ages of 10 and 20 years, and is uncommon
tures. Radiation may also be necessary, but
before age 5.72 Treatment includes a combi-
this poses a risk for future radiation-induced
nation of multiagent chemotherapy, fol-
sarcomas. Recurrence is generally 20% to
lowed by radiation and resection for local
30% and this increases with incomplete
control. Prognosis for cure is approximately
resection.65,67
75% in those who present without metasta-
■ Astrocytoma sis, and less than 30% for those with
metastasis.
Chief Clinical Characteristics
This presentation includes localized pain, ■ Langerhans’ Cell Histiocytosis
motor weakness, gait abnormalities, stiffness,
scoliosis, and sphincter dysfunction. Paresthe- Chief Clinical Characteristics
sia may occur with disease progression. Pain may This presentation commonly consists of pain
be exacerbated with coughing or sneezing. that is usually relieved with nonsteroidal
anti-inflammatory medications, rest, and, if
Background Information necessary, bracing. Associated symptoms may
This condition refers to a neoplasm of astro- be hepatosplenomegaly, anemia, leukopenia,
cytal origin, which is a type of glial cell. and thrombocytopenia. Neurological symp-
Astrocytomas account for about half of all toms may manifest if there is cord compression.
childhood brain tumors with peak age
around 5 to 9 years. These tumors become Background Information
widespread, infiltrating via cerebrospinal This condition is commonly found in the
fluid pathways, and therefore are difficult to anterior elements of the spine as a single
cure. They are rarely found outside of the lesion or is multifocal. A single, localized
central nervous system.68,69 Approximately lesion is also known as eosinophilic granu-
15% of individuals with this condition loma. This condition is generally found in
become hydrocephalic.36,70 A biopsy is nec- children 3 to 15 years of age with a predom-
essary to grade an infiltrating astrocytoma so inance for white males, but can manifest at
growth rate can be determined.68 Spinal any age.34,64,73 Radiographs will show a well-
lesions are best viewed with magnetic reso- defined lesion, with focal bone destruction
nance imaging. Low-grade forms of this and possible vertebral collapse, or vertebral
condition can be surgically excised, although plana. This condition appears to be the most
complete resection is difficult. The 5-year common cause of vertebral plana.73 The
survival rate is approximately 65% to 70%. intervertebral disks are not affected.73 Treat-
High-grade lesions are best treated with ment is usually reserved for those with organ
radiation and chemotherapy.26 dysfunction, unrelieved symptoms, or
osseous deformity, because this condition is
■ Ewing’s Sarcoma usually self-limiting.73 Treatment can in-
Chief Clinical Characteristics clude a combination of any or all of radia-
This presentation typically manifests as severe, tion, chemotherapy, steroid therapy and
persistent pain especially at night, localized curettage, and bracing.64,66
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Chapter 44 Lymphoma 893

■ Leukemia better success rate if performed in remis-

BACK PAIN IN A CHILD


Chief Clinical Characteristics sion.76 With current advances in treatment
This presentation commonly involves reports the 5-year survival rate, without relapse is
of aches and pains in the back, extremities, and reaching 80% to 90%.77
joints; fatigue; and anorexia. There may also ■ Lymphoma
be intermittent low-grade fever, enlarged
Chief Clinical Characteristics
lymph nodes, weight loss, petechiae, lethargy,
This presentation initially involves asympto-
shortness of breath, bruises, and excessive
matic, chronically swollen lymph nodes, most
bleeding.
commonly in the neck, axillae, or groin. Other
Background Information symptoms may include neurological symp-
Leukemia is the most common malignancy toms due to cord compression, fever, itching
in children and adolescents, with a higher in- or irritation of the skin, weight loss, night
cidence in males.16,74 It is typically diagnosed sweats, cough, or shortness of breath.75 Symp-
within the first decade of life and accounts toms depend on the type of lymphoma pres-
for about 40% of all childhood malignan- ent and the proximity to other structures and
cies. The two most common types account organs.
for approximately 90% of all leukemias: Background Information
acute lymphoblastic leukemia (ALL), 77% to Lymphoma is one of the most prevalent
80%, and acute myelogenous leukemia malignant tumors of childhood, and can
(AML), 10% to 13%. Some symptoms may manifest quickly. The two general types of
be consistent with a viral infection, but the lymphoma are Hodgkin’s lymphoma and
symptoms persist longer. Other symptoms non-Hodgkin’s lymphoma (NHL), and each
are a result of progressive disease and bone has its own subtypes. Hodgkin’s disease is
marrow failure as a result of bone marrow distinguished from non-Hodgkin’s by the
infiltration by malignant cells, leading to presence of specific cancer cells in the biopsy
anemia and decreased production of clotting material, termed Reed-Sternberg cells.75,78
factors.74 Diagnostic evaluation of patients Approximately 40% of pediatric cases are of
with leukemia includes complete blood the Hodgkin’s type, and 60% are non-
count and bone marrow biopsy. Examina- Hodgkins.16 Approximately 10% to 15%
tion of bone marrow aspirate is necessary for of all cases of Hodgkin’s disease, and about
confirmation, and a spinal tap determines if 5% of all NHL cases, are children under age
leukemia is present in the central nervous 19, both with a higher prevalence in boys.
system.75 Infiltration of bone and bone mar- NHL is more common in children under
row may also be viewed on magnetic reso- age 5.78,79 Biopsy, radiographs, computed to-
nance imaging.64 Rarely radiographs may mography, and blood tests are performed for
show osteopenia and compression fractures. definitive diagnosis and to assess the stage
Analysis of chromosome abnormalities in of disease. Bone marrow biopsy is done
the leukemia cells is very important to assess to evaluate bone marrow involvement. Radi-
prognosis and formulate treatment. Treat- ographs and computed tomography typi-
ment is specific to the type of leukemia pres- cally show enlarged lymph nodes in the
ent and typically has several phases. It in- affected areas and can show extensive,
volves multiple courses of chemotherapy destructive lesions in patients with osseous
and antibiotics to prevent infection, and involvement. Magnetic resonance imaging is
often a child will require blood transfusions. indicated to evaluate soft tissue involve-
Side effects of chemotherapy and steroid ment.71 Laboratory tests may show anemia
treatment include neutropenia, thrombocy- and elevated sedimentation rate. Treatment
topenia, and risk for infection. Long-term includes chemotherapy and radiation, the
complications can include osteoporosis, and course of which depends on the type of lym-
avascular necrosis. Bone marrow or stem cell phoma, the stage of disease, and if there is
transplant is reserved for situations where bone marrow metastasis. Bone marrow
standard chemotherapy has failed and has a transplant may be indicated if there is failure
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894 Chapter 44 Neuroblastoma

to respond to standard chemotherapy and suggesting cord compression. Scoliosis is rare,


BACK PAIN IN A CHILD

radiation. Survival rates are 90 to 95% for but when present, it is typically convex
those with local lesions, and 75 to 80% for toward the site of the tumor.
those with more diffuse disease.16
Background Information
■ Neuroblastoma Although rare, this condition is one of the
Chief Clinical Characteristic more common lesions found in the spine,
This presentation may include severe localized typically in the posterior elements of the
back pain, weakness, scoliosis, and neurologi- lumbar and thoracic vertebrae. They tend to
cal signs secondary to spinal cord compression. be large, destructive tumors, and may extend
Other symptoms may include a palpable into the vertebral bodies and infiltrate soft
mass, hepatomegaly, constipation, abdominal tissue.71 Diagnosis is usually between 10 and
pain, bladder dysfunction, and ecchymoses. 20 years of age and there is a 2:1 male pre-
It is one of the few cancers that cause dominance.65 Radiographs show a lucent
diarrhea.16,68,71,80 area with a sclerotic border, and may include
multifocal calcifications. Computed tomog-
Background Information raphy will more clearly define the sclerotic
This condition is a solid tumor that develops borders, while magnetic resonance imaging
in neural crest tissue, most commonly in the is optimal for showing central cord compres-
abdomen, chest, or adrenal glands.16 It rarely sion. Treatment consists of surgical resection
originates in the brain or spinal cord. If the with an approximately 10% to 15% recur-
primary lesion originates somewhere other rence rate.
than the spinal cord, metastases is hemato-
genic in nature, and can spread to bone, ■ Osteoid Osteoma
bone marrow, skin, lungs, and the brain. Chief Clinical Characteristics
This is the most common extracranial cancer This presentation involves predominantly
of childhood and the third most common intense nocturnal pain that may be relieved
pediatric neoplasm overall. Approximately with nonsteroidal anti-inflammatory medica-
500 new cases are diagnosed each year. It is tions or aspirin.1,64 The pain may be referred
usually diagnosed in infancy, almost always or localized. Gait deviations and muscle
by 5 years of age, and is rarely seen in chil- atrophy are often present. About 50% of
dren over 10 years of age.16,68,71,80 Low-risk children present with nonstructural, painful
tumors (stage I) in infants treated with scoliosis,56 which should be a red flag because
surgical excision and observation have an idiopathic scoliosis is not painful.
approximately 90% survival rate without
relapse. Children over 3 years of age with Background Information
stage IV tumors only have a 10% to 15% sur- This condition includes highly vascularized
vival rate.16 In infants the tumor may regress connective tissue bundles, usually sclerotic,
completely with spontaneous cell death or surrounding an osteoid nidus or growth
mature to normal ganglion cells and become center. They are small round or ovate tumors
benign. In children over 1 year of age, the that are usually less than 1 cm in diame-
disease progression may be rapidly metasta- ter.71,81 Spinal osteoid lesions are found
tic and often fatal.16,68 primarily in the posterior elements of the
lumbar vertebra.34,56,64,71,81 Soft tissue
■ Osteoblastoma changes are rare. This condition is typically
Chief Clinical Characteristics found in children between the ages of 5 and
This presentation produces night pain that 20, with an approximate 2:1 male-to-female
demonstrates limited responsiveness to non- ratio.65,71 Erythrocyte sedimentation rate,
steroidal anti-inflammatory medications or leukocyte count, and protein electrophoresis
aspirin.1,71 The pain is dull and localized should be normal. Radiographs and com-
to the affected region of the lumbar spine.65 puted tomography will also differentiate
In addition neurological signs such as para- osteoid osteomas from osteoblastomas, in-
paresis and paresthesia may be present, fection, lymphoma, and facet abnormalities
1528_Ch44_873-897 08/05/12 6:03 PM Page 895

Chapter 44 Vertebral Osteomyelitis 895

such as spondylolysis.65 Spinal lesions typi- diagnosed prior to age 6 and is most preva-

BACK PAIN IN A CHILD


cally require open surgical excision. In most lent in male infants.67,71,75 Diagnostic tests
cases, the scoliosis will resolve and pain will include biopsy, computed tomography, and
subside completely.62 magnetic resonance imaging.71,75 Treatment
includes chemotherapy, radiation, and, if
■ Osteosarcoma (Osteogenic necessary, complete resection. For those
Sarcoma) diagnosed and treated in the early stages,
Chief Clinical Characteristics the survival rate is approximately 80% to
This presentation may be characterized by 90%.67,75 Prognosis is poor for those with the
persistent night pain. alveolar subtype, larger tumors, or who are
over 10 years of age.67,71,75
Background Information
This condition is related to highly malignant ■ Vertebral Osteomyelitis
and locally aggressive lesions.82 It rarely origi-
Chief Clinical Characteristics
nates in the axial skeleton, but when it does it
This presentation commonly includes back pain
is usually found in the vertebral bodies.66,67,82
as a presenting symptom. Children with this
Vertebral sarcomas comprise about 10%
condition will tend to keep their back stiff and
of all cases.56 These tumors are typically
rigid during walking and squatting activities,
found in the metaphyses of long bones and
avoiding fluid motion of the spine. Children
may metastasize to the spine. Diagnosis
with this condition will often maintain a flexed
usually occurs between 10 and 20 years of
position. If left untreated, the pain can progress
age, and adolescent growth spurts appear to
such that the only position of relief is supine.5
be the greatest risk periods for develop-
Neurological abnormalities may be present due
ment.64,83 Children with certain hereditary
to cord compression after vertebral collapse.
conditions such as retinoblastoma have a
When cervical spine is involved, the child may
significant predisposition for developing this
have symptoms of torticollis or dysphagia.
condition. Treatment includes local control
with surgical excision and radiation or Background Information
chemotherapy for metastatic lesions. If Although this condition and discitis can occur
neurological signs are present, surgical at any age, this condition is more likely to
decompression and laminectomy may be occur in adolescents, and discitis is more
necessary.22 Without metastasis approxi- commonly seen in children <3 years of age.
mately 70% of cases are cured with surgery The final diagnosis is made by radiographic
and chemotherapy. If metastases are present evaluation of the spine and blood work.17 Also,
upon diagnosis, the survival rate is less children who have history of immunocompro-
than 20%. mise after organ transplant or chemotherapy
may be at higher risk for infections such as this.
■ Rhabdomyosarcoma
Chief Clinical Characteristics References
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CHAPTER 45
Hip Pain in a Child
■ Stephanie A. Jones, PT, DPT, OCS, NCS

Description of the Symptom ■ Severe hip pain with an acute onset associ-
ated with an inability to bear weight, de-
This chapter describes possible causes of hip creased range of motion, and stiffness
pain in a child between the proximal one-third ■ Any post-traumatic hip pain
of the thigh, inguinal region, and buttock. ■ Hip pain associated with local swelling,
fever, and chills
Special Concerns
■ Any hip pain associated with rapidly ascend-
ing symmetrical weakness and hyporeflexia

CHAPTER PREVIEW: Conditions That May Lead to Hip Pain in a Child

T Trauma
REMOTE LOCAL
COMMON
Spondylolisthesis 905 Muscle strain 911
Slipped femoral capital epiphyses 917
Tendinitis 918
UNCOMMON
Spondylolysis 906 Chondral injury 908
Contusion 908
Snapping hip syndrome 917
RARE
Lumbar disk herniation/radiculopathy 905 Avulsion injuries:
• Psoas off lesser trochanter 907
• Rectus femoris off the anterior inferior iliac
spine 907
• Sartorius or tensor fascia lata off the anterior
superior iliac spine 907
Fracture 908
Labral tear 910
Meralgia paresthetica 911
Traumatic dislocation 918

I Inflammation
REMOTE LOCAL
COMMON
Not applicable Aseptic
Transient synovitis 918

898
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Chapter 45 Hip Pain in a Child 899

Inflammation (continued)

HIP PAIN IN A CHILD


REMOTE LOCAL
COMMON
Septic
Osteomyelitis (pyogenic or granulomatous) 912
Septic arthritis 916
UNCOMMON
Not applicable Aseptic
Bursitis 907
Osteochondritis dissecans 912
Septic
Lyme disease (tick paralysis) 911
RARE
Aseptic Aseptic
Not applicable Apophysitis 907
Demyelinating polyneuropathies:
Septic • Acute demyelinating polyradiculoneuropathy
Appendicitis 904 (Guillain-Barré syndrome) 908
Chronic granulomatous disease 905 • Chronic demyelinating polyradiculoneuropathy 908
Discitis 905 Juvenile dermatomyositis 909
Juvenile rheumatoid arthritis 909
Osteitis pubis 912
Rheumatic fever 913
Rheumatoid arthritis–like diseases of the hip:
• Arthritis with inflammatory bowel disease 913
• Juvenile ankylosing spondylitis 914
• Juvenile dermatomyositis 914
• Psoriatic arthritis 915
• Reactive arthritis (Reiter’s syndrome) 915
• Systemic lupus erythematosus 915
Scleroderma (focal or systemic sclerosis) 916
Septic
Poliomyelitis 913
Pyomyositis 913
Tuberculosis arthritis 918

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Osteogenesis imperfecta 912
Rickets, vitamin D–resistant (hypophosphatasia) 916
RARE
Metabolic myopathy 905 Ehlers-Danlos syndrome 908
Gaucher’s disease 909
(continued)
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900 Chapter 45 Hip Pain in a Child

Metabolic (continued)
HIP PAIN IN A CHILD

REMOTE LOCAL
RARE
Lesch-Nyhan syndrome 910
Myositis ossificans (heterotopic ossification) 912
Osteopetrosis 913
Rickets, vitamin D–dependent (nutritional) 916

Va Vascular
REMOTE LOCAL
COMMON
Not applicable Avascular necrosis of the hip 907
Legg-Calvé-Perthes disease 910
Sickle cell anemia 917
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Hemophilic arthropathy 909
Henoch-Schönlein purpura (small-vessel
vasculitis) 909
Polyarteritis nodosa (medium- and large-vessel
vasculitis) 913

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Malignant Primary, such as:
• Nephroblastoma (Wilms’ tumor) 920
Malignant Metastatic:
Not applicable
Benign, such as:
• Osteoid osteoma 921
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Chapter 45 Hip Pain in a Child 901

Tumor (continued)

HIP PAIN IN A CHILD


REMOTE LOCAL
RARE
Not applicable Malignant Primary, such as:
• Ewing’s sarcoma 919
• Leukemia 919
• Lymphoma 920
• Neuroblastoma 920
• Osteosarcoma (osteogenic sarcoma) 921
• Rhabdomyosarcoma 922
• Synovial sarcoma 922
Malignant Metastatic:
Not applicable
Benign, such as:
• Langerhans’ cell histiocytosis 919
• Osteochondroma 921

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Acetabular dysplasia 906
RARE
Not applicable Metaphyseal chondrodysplasia 911
Multiple epiphyseal dysplasia 911
Myelodysplasia (spina bifida) 911

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Meralgia paresthetica 911
Spastic cerebral palsy 918

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Common Ages at Which Hip Pain Presents in a Child


APPROXIMATE AGE CONDITION
Birth to 3 Years Chronic inflammatory demyelinating polyneuropathy
Discitis
Juvenile rheumatoid arthritis (polyarticular)
(continued)
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902 Chapter 45 Hip Pain in a Child


HIP PAIN IN A CHILD

Common Ages at Which Hip Pain Presents in a Child—cont’d


APPROXIMATE AGE CONDITION
Lesch-Nyhan syndrome
Leukemia
Lyme disease (tick paralysis)
Metabolic myopathy
Myelodysplasia (spina bifida)
Nephroblastoma
Neuroblastoma
Osteochondroma
Osteogenesis imperfecta
Osteomyelitis
Osteopetrosis
Poliomyelitis
Polyarteritis nodosa
Rhabdomyosarcoma
Rickets
Scleroderma
Septic arthritis
Transient synovitis
Tuberculosis arthritis
Preschool (3–5 Years) Acute transient synovitis
Discitis
Gaucher’s disease
Henoch-Schönlein purpura
Legg-Calvé-Perthes disease
Leukemia
Lyme disease (tick paralysis)
Metabolic myopathy
Nephroblastoma
Neuroblastoma
Osteochondroma
Osteomyelitis
Rhabdomyosarcoma
Scleroderma
Septic arthritis
Tuberculosis arthritis
Elementary School (6–11 Years) Chronic granulomatous disease
Ewing’s sarcoma
Guillain-Barré syndrome
Hemophilic arthropathy
Henoch-Schönlein purpura
Hip dislocation (traumatic)
Juvenile dermatomyositis
Juvenile rheumatoid arthritis (pauciarticular)
Legg-Calvé-Perthes disease
Leukemia
Lyme disease (tick paralysis)
Lymphoma
Metaphyseal chondrodysplasia
Multiple epiphyseal dysplasia
Osteochondritis dissecans
Osteochondroma
Osteoid osteoma
Osteomyelitis
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Chapter 45 Hip Pain in a Child 903

HIP PAIN IN A CHILD


Common Ages at Which Hip Pain Presents in a Child—cont’d
APPROXIMATE AGE CONDITION
Osteosarcoma
Psoriatic arthritis
Pyomyositis
Rhabdomyosarcoma
Scleroderma
Sickle cell anemia
Spondylolysis
Middle School (12–14 Years) Acetabular dysplasia
Avascular necrosis
Epiphyseal dysplasias
Ewing’s sarcoma
Hip bursitis
Hip contusion
Hip fractures
Juvenile ankylosing spondylitis
Juvenile chronic arthritis
Juvenile dermatomyositis
Juvenile rheumatoid arthritis (polyarticular)
Leukemia
Lyme disease (tick paralysis)
Lymphoma
Metabolic myopathy
Muscle strain
Myelodysplasia (spina bifida)
Myositis ossificans
Osteochondritis dissecans
Osteoid osteoma
Osteosarcoma
Psoriatic arthritis
Reactive arthritis (Reiter’s syndrome)
Rhabdomyosarcoma
Rheumatic fever
Scleroderma
Slipped femoral capital epiphysis
Snapping hip syndrome
Spondylolysis
Tendinitis
High School (15–18 Years) Apophysitis
Appendicitis
Avulsion injury
Cerebral palsy (spastic)
Chondral injury
Ehlers-Danlos syndrome
Ewing’s sarcoma
Heterotopic ossification
Hip bursitis
Hip contusion
Hip fractures
Juvenile ankylosing spondylitis
Labral tears
Lumbar disk herniation/radiculopathy
Lyme disease (tick paralysis)
(continued)
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904 Chapter 45 Appendicitis


HIP PAIN IN A CHILD

Common Ages at Which Hip Pain Presents in a Child—cont’d


APPROXIMATE AGE CONDITION
Meralgia paresthetica
Metaphyseal chondrodysplasia
Muscle strain
Myelodysplasia (spinal bifida)
Myositis ossificans
Osteitis pubis
Osteochondritis dissecans
Osteochondroma
Osteoid osteoma
Osteosarcoma
Rhabdomyosarcoma
Rheumatic fever
Scleroderma
Septic arthritis
Sickle cell anemia
Slipped capital femoral epiphysis
Snapping hip syndrome
Spondylolisthesis
Spondylolysis
Synovial sarcoma
Systemic lupus erythematosus
Tendinitis
Tuberculosis arthritis

Overview of Hip Pain in a Child The most common childhood diseases re-
sulting in hip pain include slipped capital
Hip pain is common in children. A child with femoral epiphysis, Legg-Calvé-Perthes disease,
a painful hip will commonly present with dif- acetabular dysplasia, septic arthritis, transient
ficulty weight bearing, a limp if at an age old synovitis, and muscular injuries.1–3 For infants
enough to walk, and pain-limited range of and toddlers transient synovitis of the hip and
motion. Young children in particular might septic arthritis are the most common causes
have difficulty describing their symptoms or of hip pain.4 In children under the age of
cooperating with the examination. This same 10 years, Legg-Calvé-Perthes disease, septic
clinical presentation occurs in a wide variety arthritis, transient synovitis, and various can-
of underlying pathologies. The correct diag- cers are the most likely sources of hip pain. In
nosis is important due to the potential for adolescence, as sports participation increases,
long-term dysfunction from hip disease if traumatic injuries such as slipped capital
proper treatment is delayed or absent. This is femoral epiphysis, muscle strains, tendinitis,
particularly relevant in diseases such as septic and spondylolisthesis are more frequent.
arthritis and slipped capital femoral epiph-
ysis, which can lead to severe joint destruc- Description of Conditions That
tion if not treated expeditiously. Since the May Lead to Hip Pain in a Child
presenting symptoms for fairly benign disor-
ders such as transient synovitis are similar to Remote
those that require urgent medical attention
such as septic arthritis, prompt evaluation of ■ Appendicitis
any acute onset of hip pain in a child is criti- Chief Clinical Characteristics
cal. Referred pain to the medial knee joint This presentation can include tenderness
results in the frequent misdiagnosis of pri- or pain in the right anterior pelvis or psoas
mary hip disease. region.5
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Chapter 45 Spondylolisthesis 905

Background Information Background Information

HIP PAIN IN A CHILD


Retrocecal appendicitis typically presents with Onset can be insidious or related to mechani-
right lower abdominal or lumbar pain with cal trauma. Bending, lifting, and prolonged sit-
tenderness of the right ilium or lumbar areas.6 ting often increase symptoms. Damage of the
If appendiceal rupture occurs there may also intervertebral disk due to a disruption in the
be an ilial abscess causing right flank or cos- annulus fibrosus or vertebral end plate is an
tovertebral pain.6 These symptoms might also uncommon phenomenon in adolescents.9
be accompanied by anorexia, low-grade fever, Most cases are managed nonsurgically. Related
and leukocytosis.6 Treatment includes surgery saddle numbness or bowel and bladder dys-
and antibiotic medications. function indicate a need for referral to a spine
surgeon in order to consider urgent surgical
■ Chronic Granulomatous Disease decompression.
Chief Clinical Characteristics
This presentation commonly involves thigh ■ Metabolic Myopathy
or hip pain, painful limp, limited hip range of Chief Clinical Characteristics
motion, and recurrent abscesses.7 The classic presentation can include pain,
Background Information cramping, stiffness, and fatigue during intense
This disorder includes recurrent purulent bac- exercise.10
terial and fungal infections, which are the most Background Information
common underlying condition in extracranial Myophosphorylase deficiency (McArdle’s syn-
osteomyelitis.7 It may present as an osseous drome) is the most common of these nine
infection.7 Imaging tests or biopsy is used to glycogen defect related disorders.10 The onset
differentiate the disease.7 Treatment includes is in childhood to early adolescence.10 Diag-
antibiotic medications and chemotherapy.7 nosis is made with muscle biopsy and the
■ Discitis forearm ischemic exercise test.10 Another
presentation of this disease includes progres-
Chief Clinical Characteristics sive weakness similar to that in the dystro-
This presentation for infants or toddlers may phies. This presentation most commonly
include a limp, avoidance of ambulation, or occurs in infancy to early childhood.11
abdominal pain.8 Treatment may include diet modification and
Background Information exercise.12,13
Pain or spasm may be present when the hip
is placed in extension (log-roll test).8 This ■ Spondylolisthesis
infection of the disk space is common in chil- Chief Clinical Characteristics
dren. Moderately elevated erythrocyte sedi- This presentation includes hip and low back
mentation rate, disk space narrowing with ir- pain that may radiate to the lower extremities,
regular vertebral end plates on imaging limitations in range of motion, a positive
studies, and local tenderness to palpation can Lasègue’s sign, and tenderness over the lumbar
be used to confirm the diagnosis.2,8 Treatment spinous processes with or without a palpable step.
is with antibiotic medication.8 Significant hamstring tightness or spasm may
result in an abnormal gait pattern with short
■ Lumbar Disk Herniation/ stride length, flexed knees, and pelvic waddle.
Radiculopathy Decreased lumbar lordosis, scoliosis, and flat-
Chief Clinical Characteristics tened buttocks may also be present. Neurolog-
This presentation includes local or referred ical signs are rare, but may be present even in
pain from the disk injury as well as radicular low-grade slips secondary to nerve root compres-
symptoms due to encroachment on a nerve sion.14–17 As with spondylolysis (see next
root that can refer to the buttocks, groin, or else- entry), symptoms are usually aggravated by
where in the lower extremity in a dermatomal activities that require repetitive flexion and
pattern. extension such as gymnastics.
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906 Chapter 45 Spondylolysis

Background Information level, most commonly at L5, and can occur


HIP PAIN IN A CHILD

This condition involves the forward displace- unilaterally or bilaterally. A bilateral defect no
ment of a proximal vertebra on the one below longer provides posterior stability and may re-
it, typically as a result of bilateral pars interar- sult in forward slippage, especially of L5 over
ticularis defect. The pars defect is not present S1, known as spondylolisthesis. Spondylolysis
in congenital spondylolisthesis. The most can occur at any age, but is rarely seen before
common site for slippage is the fifth lumbar age ten; it appears to be more prevalent in
vertebra on the sacrum. Standing anteroposte- boys. An oblique standing view is the best view
rior and lateral radiographs are needed to de- to show the pars defect that can easily be over-
termine the grade of slippage, and an oblique looked if only anteroposterior and lateral
view is necessary to appreciate the pars defect. views are taken.17,19,20 Computed tomography
In high-grade slips with neurological deficits a or bone scan is used if radiographs are incon-
complete neurological exam is indicated. clusive, and single-photon emitted computed
Treatment is determined by symptoms, clinical tomography will determine if the lesion is
signs, and the grade of slip. Asymptomatic pa- acute or chronic.16 Treatment depends on the
tients do not require intervention. Those with degree of the defect and whether the patient is
low-grade slips and pain tend to do well with symptomatic. If pain is present in the absence
conservative treatment of activity restriction, of spondylolisthesis, immobilization with a
bracing, and exercises. Fusion is recommended thoracic-lumbar-sacral orthosis for 3 to
for grade II slips or greater even if they are 6 months may be most beneficial. Physical
asymptomatic because they are at risk for fur- therapy should focus on hamstring and lum-
ther slippage during growth spurts.16,17 Reduc- bar musculature stretching, abdominal strength-
tion of the slip is not recommended because ening, and monitoring of gradual return to
of the high risk of neurological injury.15–17 activity. With persistent pain unrelieved by
Patients are more prone to recurrent symp- nonsteroidal anti-inflammatory medication,
toms and clinical deformity if forward slipping rest, and physical therapy, surgical stabilization
is allowed to progress. Progression of slip, de- can be achieved through posterior spinal fu-
layed union, neuropathy of the fifth nerve sion.15,18 Any child with this condition should
root, and cauda equina syndrome have all been be followed closely, because they are at risk for
reported as postsurgical complications.16,17 progression to spondylolisthesis. Girls tend to
have a higher rate of progression.
■ Spondylolysis
Chief Clinical Characteristics Local
This presentation can include hip and low back
pain with hyperextension that rarely radiates, ■ Acetabular Dysplasia
scoliosis, decreased lumbar lordosis and in- Chief Clinical Characteristics
creased lumbosacral kyphosis.18 The child will This presentation involves pain in the hip that
often have hamstring tightness that can affect may be associated with a limp. Usually the range
gait.15,19 Adolescents participating in sports of motion is unchanged. While dysplasia of
that require repetitive hyperextension, such as the acetabulum is seen as an infant, it is not a
gymnastics, wrestling, and football appear to cause of pain until the early to middle teenage
be at increased risk.15 Most children with years.
spondylolysis are asymptomatic or may have
Background Information
symptoms that are mild and therefore often
This condition is characterized by a shallow
overlooked.
acetabulum or slanted acetabular roof
Background Information (Fig. 45-1).21 Overall developmental dyspla-
This condition is a congenital or acquired de- sia is far more common in females with a
fect of the pars interarticularis, the bone be- ratio of 6:1.21 Acetabular dysplasia has also
tween the superior and inferior facets of the been associated with labral tears due to the
vertebrae. An acquired defect would arise as a hypertrophy of the labrum from impinge-
stress or fatigue fracture of the pars.16,19 The ment by the acetabulum, resulting in pain as
pars interarticularis defect can occur at any well as osteoarthritis later in adulthood.22
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Chapter 45 Bursitis 907

separate disorder from Legg-Calvé-Perthes

HIP PAIN IN A CHILD


disease. It may require surgical management.26
AVULSION INJURIES
Chief Clinical Characteristics
This presentation includes a variable clinical
picture that depends on the affected muscle
group.
Background Information
Sprinting or sports activities are the most
common mechanism of injury for avulsion
injuries.24,25 The force of the muscle contrac-
tion is beyond of the strength of the apophy-
FIGURE 45-1 Developmental dysplasia of the left hip seal muscle attachment, resulting in an avul-
with dislocation (anteroposterior view). (Image cour- sion fracture.28 Recovery is expected in 4 to
tesy of Nishant Verma, MD, diagnostic radiology 6 weeks with rehabilitation.24
resident, Indiana University School of Medicine.) ■ Psoas Off Lesser Trochanter
Chief Clinical Characteristics
This presentation may include anterior hip
pain.28
The diagnosis is confirmed with x-rays.
Treatment in infants may include bracing ■ Rectus Femoris Off the Anterior
such as with a Pavlik harness. Other inter- Inferior Iliac Spine
ventions include physical therapy for Chief Clinical Characteristics
strengthening and positioning or in more se- This presentation can be characterized by
vere cases surgery such as an osteotomy.23 groin pain, weakness with active hip flexion
■ Apophysitis and knee extension, limited hip extension
motion, and difficulty weight bearing.24
Chief Clinical Characteristics
This presentation involves the gradual onset of ■ Sartorius or Tensor Fascia Lata
mild pain at the muscle attachment sites.24 Off the Anterior Superior Iliac
Spine
Background Information
Pain and inflammation are due to excessive Chief Clinical Characteristics
traction to the apophysis, attachment site, This presentation includes anterior hip pain,
or insertion of muscle into bone.24 This condi- weakness with hip flexion, external rotation
tion is managed with rest and progressive and abduction, limited hip extension, and in-
rehabilitation.25 ternal rotation motion as well as difficulty
weight bearing.24
■ Avascular Necrosis of the Hip
■ Bursitis
Chief Clinical Characteristics
This presentation involves groin pain, difficulty Chief Clinical Characteristics
weight bearing, limited hip extension, and This presentation commonly includes pain
internal rotation.26 localized over the bursa that might radiate
distally.24
Background Information
The vascular supply to the femoral head can Background Information
be disturbed by trauma, treatments requiring This condition involves inflammation and
very high-dose steroids (such as in post–kidney swelling of the synovial fluid-filled sacs
allograft rejection), and disorders including called bursas.24 The most common location
endocrine dysfunction, human immunodefi- is the trochanteric bursa followed by the is-
ciency virus, and sickle cell disease.27 This is a chial, iliopsoas, and iliopectineal bursae.24
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908 Chapter 45 Chondral Injury

This disorder can be caused by a direct blow Background Information


HIP PAIN IN A CHILD

to the bursae, prolonged pressure to the area, This condition is a rapidly progressive lower
friction over the bursae from muscle imbal- motor neuron disease that affects boys more
ances, bony alignment, and/or environmen- commonly than girls.31 It can rapidly lead to
tal factors in sports activity.24 Treatment respiratory failure in acute stages if not
includes rest, anti-inflammatory medica- treated. Intravenous immunoglobulin has
tions, ultrasound, and therapeutic exercise to been shown to be the most effective medical
restore muscle balance.25 Sometimes corti- therapy but plasma exchange and corticos-
costeroid injections are used for the inflam- teroids are also utilized.31 Most patients
mation, as well. undergo extensive rehabilitation to regain
strength and restore function.
■ Chondral Injury
■ Chronic Demyelinating
Chief Clinical Characteristics Polyradiculoneuropathy
This presentation includes reports of hip joint
Chief Clinical Characteristics
locking.22,29
This presentation involves ascending weakness
Background Information and muscle pain with a subacute onset over
This condition is frequently associated with a at least 2 months.32
jumping or twisting mechanism.29 Surgery Background Information
may be indicated if nonsurgical management The course of disease may include chronic
does not alleviate symptoms.29 progression or periodic relapses. Intravenous
immunoglobulin has been shown to be the
■ Contusion most effective medical therapy but plasma ex-
Chief Clinical Characteristics change and corticosteroids are also utilized.31
This presentation includes pain, spasm, and Most patients undergo extensive rehabilita-
transient weakness of affected muscles.24,25 tion to regain strength and restore function.

Background Information ■ Ehlers-Danlos Syndrome


This injury results in hematoma formation Chief Clinical Characteristics
from a blow to the musculature surrounding This presentation includes joint hypermobility that
the hip.25 Hip pointer is the term used to indi- can lead to recurrent hip dislocations and joint
cate a contusion to the musculature that at- effusion in these children.10,33 Individuals with this
taches to the iliac crest.24 In severe cases this condition are also prone to experiencing disloca-
can lead to the development of myositis ossi- tions at the knee, shoulder, elbow, and clavicle and
ficans.24 Treatment includes ice, ultrasound, typically have cardiac defects.
anti-inflammatory medications, or steroid
injection.25 Background Information
This is an inherited connective tissue disorder
DEMYELINATING with 10 distinct variations.33 Management
POLYNEUROPATHIES may include bracing or physical therapy for
■ Acute Demyelinating
strengthening.
Polyradiculoneuropathy
■ Fracture
(Guillain-Barré Syndrome)
Chief Clinical Characteristics
Chief Clinical Characteristics
This presentation involves an acute onset of
This presentation commonly involves ascend-
activity-limiting hip pain and swelling.24
ing weakness and muscle pain.30 Leg pain
often precedes symptoms of weakness. 3 Background Information
Prodromal symptoms or events such as gas- This would include any fracture to the pelvis
troenteritis, upper respiratory infection, surgery, or proximal femur of the following types:
or immunization 1 to 4 weeks prior to weak- traumatic or stress.24 Traumatic fractures due
ness may be reported.30,31 to compression or a blow in children are most
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Chapter 45 Juvenile Rheumatoid Arthritis 909

commonly a result of a motor vehicle acci- ■ Henoch-Schönlein Purpura

HIP PAIN IN A CHILD


dent, fall from height, or sports activity.24 (Small-Vessel Vasculitis)
Traumatic avulsion fractures result from trac- Chief Clinical Characteristics
tion to a muscle origin or attachment, usually This presentation often includes abdominal
in ballistic sport activities.24 This is the most pain after an upper respiratory infection followed
common fracture type in children and typi- by a rash (petechiae) and edema in dependent
cally occurs at the anterior superior iliac areas of the extremities.10
spine, anterior inferior iliac spine, ischial
tuberosity, or lesser trochanter.24 Stress frac- Background Information
tures are of insidious onset and typically Arthritis and nephritis can develop.10 The
related to overuse from sports activities such course of the disease usually resolves in 2 weeks
as running. Overall the incidence of pediatric without intervention.10 Nonsteroidal anti-
hip fractures is rare.24 Management ranges inflammatory medication may be used to
from surgical fixation of fracture segments to manage symptoms.
rehabilitation.
■ Juvenile Dermatomyositis
■ Gaucher’s Disease Chief Clinical Characteristics
Chief Clinical Characteristics This presentation includes a vasculitic rash on
This presentation typically involves muscu- the face, elbows, and knees and the slow onset
loskeletal symptoms such as a recurrent ache in of signs of proximal weakness such as difficulty
the hip, knee, or shoulder joint and bone crisis walking and clumsiness.10
of the femur or tibia, which can include severe Background Information
pain, swelling, and erythema.10 Muscle pain due to the inflammation of the
Background Information blood vessels may also occur.10 This condi-
This disorder is deficiency of a fat metabo- tion should be excluded in cases of chronic
lizing enzyme that results in fatty infiltra- arthritis and muscle inflammation. Muscle
tion of tissues including bone marrow. Os- enzyme testing, electromyography, and mus-
teonecrosis of the femoral head and cle biopsy are used to confirm the diagnosis.10
pathological long bone fractures may also Corticosteroid therapy will reverse the dis-
occur.10 The juvenile form of this disease ease in 6 months for most cases.10 Complica-
(type 3) has an onset in early childhood.10 tions include dysphagia, chronic abdominal
Treatment includes enzyme replacement pain leading to perforation, cerebritis, and
therapy and in some cases bone marrow diffuse calcification of muscle or subcuta-
transplantation.34 neous tissues.10
■ Hemophilic Arthropathy ■ Juvenile Rheumatoid Arthritis
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes physical signs of in- This presentation commonly involves the consis-
flammation due to only minor joint trauma tent presence for 6 or more weeks of joint swelling,
that results in hemarthrosis.10 or the presence of two or more of the following:
limited joint range of motion, palpable tender-
Background Information
ness, painful movement, or joint warmth.10
Synovium proliferation or pannus usually oc-
curs in the knee, ankle, and elbow joints of Background Information
children with this blood clotting disorder.35 Hip involvement may present 1 to 6 years after
Hip osteonecrosis may develop from vascular initial disease onset in 30% to 50% of affected
occlusion due to bleeding or synovial hyper- children and typically is bilateral.37 Other asso-
plasia.35 Hemophilia is almost exclusive to ciated problems include osteoporosis, growth
males.10 Treatment of the hemophilia may in- disturbance due to epiphyseal plate damage,
clude infusion of treated plasma-derived fac- coxa magna, and less commonly hip subluxa-
tor or recombinant factor. The joint damage tions, avascular necrosis, and, rarely, fusion.37
may necessitate joint arthroplasty surgery.36 Hip involvement is usually also associated with
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910 Chapter 45 Labral Tear

the more severe forms requiring aggressive Background Information


HIP PAIN IN A CHILD

medical management with long-term remission- This disease can have an acute traumatic or in-
inducing drugs such as methotrexate and inten- sidious onset over months. The femoral head
sive physical therapy for contracture manage- ceases to grow due to a disturbance in blood
ment, bracing, strengthening, and gait training.37 supply. The secondary center of ossification
Functional problems include altered gait possi- becomes fragmented as revascularization occurs
bly with a limp and difficulty with stair climbing and then reossification follows (Fig. 45-2).21 It is
and rising from a seated position.37 The three more common in males by a 4:1 ratio.21
subtypes of this condition are systemic, pol- Smaller, highly active boys with delayed skele-
yarticular, and pauciarticular. Pauciarticular or tal maturity may be predisposed to developing
oligoarticular must affect five or fewer joints.21 this disorder.21 Between 8% and 24% of cases
Late-onset pauciarthritis occurs mostly in boys are bilateral.25 Most cases are self-limiting
after age 8.21 It involves concomitant enthesitis but need monitoring with radiographs and
or tendinitis and arthritis that asymmetrically periodic clinical assessments.25 More severe
affects the knees, shoulders, spine, or hips.10 The cases might require strengthening, splinting,
polyarticular type involves more than five joints weight-bearing limitations, and even surgery.21
symmetrically and usually occurs in small joints It is important to avoid weight bearing for a
but can involve the hips.23 This type is more period to allow for revascularization and pre-
common in females. Systemic arthritis is charac- vent permanent bony deformity.25
terized by fever and rash but may also include
thrombocytosis, leukocytosis, anemia, pleuritis, ■ Lesch-Nyhan Syndrome
pericarditis, osteopenia, delayed growth, he- Chief Clinical Characteristics
patosplenomegaly, and lymphadenopathy.10,23 This presentation includes gouty arthritis,
Patients with monoarticular disease report mental retardation, hypertonicity resulting
morning stiffness, joint pain, and swelling in the in hip dislocation, movement disorders, and
initial stages.10 The joints are warm and limited self-mutilating behaviors.21,38
in range of motion.
Background Information
■ Labral Tear This is a rare genetic disorder characterized by
Chief Clinical Characteristics an enzyme deficiency that results in uric acid
This presentation includes catching, clicking, giv- accumulation.38 This disorder only occurs in
ing way, pain, and decreased range of motion boys.21 Death due to renal failure typically oc-
of the hip.22,29 curs in the first two decades. Medical manage-
ment may include allopurinol to address the
Background Information excess uric acid and various medications to
These tears are associated with sports activi- manage the neurological symptoms.38
ties that involve running, pivoting, jumping,
and kicking such as in tennis, soccer, martial
arts, dance, and football.29 Children may de-
scribe the mechanism of injury as twisting or
an axial load on a flexed hip if they recall an
acute event.29 Hip dysplasia can also result in
labral tears most commonly occurring ante-
riorly.22 Such tears can sometimes be man-
aged nonsurgically; otherwise surgery might
be indicated.

■ Legg-Calvé-Perthes Disease
Chief Clinical Characteristics
This presentation involves groin, thigh, and/or FIGURE 45-2 Legg-Calvé-Perthes disease of the
knee pain, a limp (typically with the femur right hip (anteroposterior view). (Image courtesy of
externally rotated), limited hip abduction, and Nishant Verma, MD, diagnostic radiology resident,
internal rotation.23 Indiana University School of Medicine.)
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Chapter 45 Myelodysplasia (Spina Bifida) 911

■ Lyme Disease (Tick Paralysis) ■ Multiple Epiphyseal Dysplasia

HIP PAIN IN A CHILD


Chief Clinical Characteristics Chief Clinical Characteristics
This presentation involves a rash commonly This presentation is characterized by abnor-
with central clearing, fever, fatigue, malaise, mal ossification of multiple physes, resulting
headache, myalgias, and polyarthritis in the in lower limb stiffness and pain, usually sym-
early stage.10 metrically and most often in the hips and knees.
Irregular epiphyseal and physeal development
Background Information
may also result in skeletal deformities such as
This condition involves infection by a tick-
coxa vara.41
borne spirochete Borrelia burgdorferi. The
joint pain is secondary to an autoimmune Background Information
reaction rather than a septic arthritis due to The Fairbank and Ribbing types have been
the organism. Ninety percent of cases in the further differentiated. Fairbank-type dysplasia
United States have been in the states of Con- is more severe and includes short extremities,
necticut, Massachusetts, Minnesota, New Jersey, waddling gait, and joint pain.10 The Ribbing
New York, Pennsylvania, Rhode Island, and type may be difficult to distinguish from sym-
Wisconsin.10 It is treated with antibiotic metrical Legg-Calvé-Perthes with its proximal
medication.39 femur involvement.10 This is a predominantly
autosomal dominant inherited disorder. Treat-
■ Meralgia Paresthetica ment may include activity modification or
Chief Clinical Characteristics surgery.41
This presentation includes numbness, burning,
tingling, and/or pain of the anterolateral ■ Muscle Strain
thigh.24 This condition results from an Chief Clinical Characteristics
entrapment or compression of the superficial This presentation includes an acute onset of
branch of the lateral femoral cutaneous local pain, spasm, swelling, weakness, and/or
nerve.24 echymosis.24,25
Background Information Background Information
This condition is typically due to obesity, a di- This injury is common in the hip adductor, il-
rect blow, or the wearing of tight garments or iopsoas, and hamstring muscle groups.24,25 It
heavy accessories (such as a tool belt) over the results from an overstretching or tearing of the
anterior hip. It can also follow prolonged oper- muscle.24 Strains commonly occur in sports
ations done in the prone position, such as in such as soccer, basketball, and sprinting.24
posterior spine fusions in children.24 Manage- Management includes ice, compression, rest,
ment is nonsurgical, consisting of measures to anti-inflammatory medications, ultrasound,
provide relief from mechanical compression of and gradual return to exercise and sport.25
the nerve.
■ Myelodysplasia (Spina Bifida)
■ Metaphyseal Chondrodysplasia Chief Clinical Characteristics
Chief Clinical Characteristics This presentation includes muscle imbalances
This presentation of this metaphyseal that result from weakness due to spinal cord
pathology can produce hip pain, short/ injury and can result in hip dislocation.21
bowed legs, and a waddling gait with the
Background Information
Schmid type.10
In rare cases children with this condition
Background Information might also develop neuropathic arthropathy of
This autosomal dominant group of disorders their hips or a monoarthritis resulting in pain
may present in infancy or childhood. Radio- and swelling.10,42 This is a group of congenital
logic examination will demonstrate metaphy- disorders that results in neural tube defects
seal abnormalities with limb shortening or that alter the development of the spinal cord,
deformity.40 Treatment may include surgery spine, or brain. It includes meningocele,
for correction of deformity. myelomeningocele, and spina bifida occulta.
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912 Chapter 45 Myositis Ossificans (Heterotopic Ossification)

Surgical intervention is typically indicated to Background Information


HIP PAIN IN A CHILD

close the spinal canal defect. Other interven- This overuse disorder is associated with sports
tions may include bracing and rehabilitation such as running, soccer, and football.25 It is
to address the weakness that results from the treated with rest, anti-inflammatory medica-
spinal cord injury.43 tions, and progressive return to sport.25

■ Myositis Ossificans (Heterotopic ■ Osteochondritis Dissecans


Ossification) Chief Clinical Characteristics
Chief Clinical Characteristics This presentation includes hip pain and
This presentation may include localized pain and difficulty weight bearing.21
limited joint range of motion following the Background Information
resolution of a soft tissue trauma.24 This is a subchondral bone defect that is rarely
Background Information seen in the proximal femur except in children
Heterotopic ossification is the laying down with healed Legg-Calvé-Perthes disease.21 It
of bone at an abnormal site.44 Hip fractures may be caused by minor trauma or ischemia
especially those followed by surgery are asso- and often resolves without intervention.21
ciated with the development of this disorder
in the periarticular space.44 In the pediatric
■ Osteogenesis Imperfecta
population such fractures are most likely as- Chief Clinical Characteristics
sociated with high-velocity injuries such as This presentation includes fracture due to
motor vehicle accidents or a fall from a brittle bones as a potential cause of hip pain
height.44 The incidence for this disorder is ap- in these patients. 10 This is an autosomal
proximately 20% in patients with brain or dominant disorder resulting in poor collagen
spinal cord injury with the hip being the most synthesis and brittle bones that develop abnor-
common site.44 Children with cerebral palsy mally, resulting in short stature and bowing
undergoing hip surgery are at higher risk for of long bones. Many children with this condi-
this complication. The three primary sites of tion have bone pain relating to old fractures
involvement in this region are inferomedial to as well as muscle weakness and ligamentous
the hip, between the anterosuperior iliac laxity.21
spine and the proximolateral femur, and the Background Information
posterior hip.44 Treatment is controversial and Management may include bracing, rehabilita-
may include therapy to maintain ROM, tion, and possibly surgery for stabilization of
diphosphonate treatment, manipulation, and the joints.
surgical excision.44 When the formation of
heterotopic bone follows direct trauma to a ■ Osteomyelitis (Pyogenic or
muscle, especially in the quadriceps or adduc- Granulomatous)
tors, the term myositis ossificans is often used, Chief Clinical Characteristics
but the biological process is the same. Early This presentation may include a sudden onset
return to activity, soft tissue mobilization, ul- of throbbing pain and swelling over the site
trasound, or use of heat soon after a contusion of infection, along with fever, chills, and
may increase the risk for developing this dis- malaise.5,21,45
order.25 Surgical excision is performed if the
pain and range-of-motion restriction limit Background Information
function, but surgery is not recommended This is an inflammatory disease of bone and
until at least 1 year postinjury to prevent bone marrow, most commonly due to a bacte-
recurrence.25 rial infection from a preceding trauma, sys-
temic illness, or surgery.5,8,21 In children the le-
■ Osteitis Pubis sions are most common in the metaphyses of
Chief Clinical Characteristics long, tubular bones and less often at the
This presentation is characterized by an insid- greater trochanter, femoral neck, or innomi-
ious onset of pain over the pubic symphysis nate of the pelvis.21,46 Osteomyelitis is more
sometimes radiating to the groin.24 common in boys and can be misdiagnosed as
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Chapter 45 Arthritis with Inflammatory Bowel Disease 913

septic arthritis.45,47 Delay in treatment of os- ■ Pyomyositis

HIP PAIN IN A CHILD


teomyelitis with antibiotic medications can Chief Clinical Characteristics
result in destruction of the involved bone.21 This presentation may include limited, painful
Lack of response to antibiotic medications hip movements with an inability to bear
may necessitate surgical debridement.8 weight.51,52

■ Osteopetrosis Background Information


This can be difficult to differentiate from septic
Chief Clinical Characteristics arthritis.51 It is more common in tropical cli-
This presentation commonly results in epiphy- mates or warm North American climates dur-
seal separations and femur fractures.48 ing summer.52 An abscess is a subcategory of
Background Information pyomyositis that has been described in the
Osteoclast dysfunction results in excessive psoas, obturator externus or internus muscles,
bone density in this rare congenital disor- and retroperitoneal space in children.52,53 A pri-
der.48 The goal of medical management is mary psoas abscess is formed without any
osteoclast stimulation through calcium re- detectable underlying infection and is more
striction, steroids, parathyroid hormone, and common in children.53 A secondary abscess
interferon.49 Children may also undergo a relates to an underlying infection such as tuber-
bone marrow transplant in certain forms of culosis, appendicitis, or osteomyelitis.53 This
this condition.49 condition may also be related to a history of
trauma or strenuous exercise.53 Treatment may
include surgical drainage, aspiration, and/or a
■ Poliomyelitis course of antibiotic medications.52
Chief Clinical Characteristics
This presentation includes muscle tenderness ■ Rheumatic Fever
and pain especially hamstring muscle spasm Chief Clinical Characteristics
or tightness that may precede the onset of This presentation may be characterized by rapid
weakness. onset of migrating pain and inflammation.10
Background Information Background Information
This is a viral infection involving the motor This is an inflammatory disease that results
neurons of the central nervous system, espe- from a Streptococcus bacterial infection. Inci-
cially in the spinal cord.50 While the incidence dence is higher in non-Western countries.54
has been reduced dramatically during the past It is associated with a transient form of pol-
decade, the disease is still an important one in yarthritis with lower extremity involvement
India and parts of Africa. Treatment includes preceding upper extremity early in the
supportive care and rehabilitation.50 course of the illness.10 Treatment is with
anti-inflammatory medications.10
■ Polyarteritis Nodosa
(Medium- and Large-Vessel RHEUMATOID ARTHRITIS–LIKE
Vasculitis) DISEASES OF THE HIP
Chief Clinical Characteristics ■ Arthritis with Inflammatory
This presentation at onset is variable, but may Bowel Disease
include abdominal or extremity pain, rash,
Chief Clinical Characteristics
hypertension, hematuria, and/or fever.10
This presentation involves hip, sacroiliac joint,
Background Information or low back pain and stiffness associated with
It can be difficult to distinguish from Kawasaki an acute episode of gastrointestinal upset.
disease, systemic-onset juvenile rheumatoid There are two forms of peripheral arthropa-
arthritis, small-vessel vasculitis, infection, neo- thy associated with inflammatory bowel
plasm, and inflammatory bowel disease.10 This disease that occur in about 25% of patients.55
disease is rare in children. Treatment includes The type I arthropathy associated with
steroid therapy.10 inflammatory bowel disease presents in a
1528_Ch45_898-924 07/05/12 2:14 PM Page 914

914 Chapter 45 Juvenile Ankylosing Spondylitis

pauciarticular distribution and it is consid- involved in 30% to 50% of patients, usually


HIP PAIN IN A CHILD

ered self-limiting.55 within the first 10 years of disease. Ankylos-


ing spondylitis is a chronic disease with in-
Background Information termittent periods of remission; it is rarely
Inflammatory bowel disease is an idiopathic active persistently. There is no specific labo-
immune disorder affecting the intestinal ratory test for ankylosing spondylitis, how-
tract. The most common types are Crohn’s ever, most patients have the presence of
disease and ulcerative colitis.56 Children with HLA-B27 (human leukocyte antigen), ele-
this disease are likely to be HLA-B27 posi- vated erythrocyte sedimentation rate and
tive, suggesting an underlying genetic pre- C-reactive protein level, and absence of
disposition. In some cases, when the inflam- rheumatoid factor. Radiographic changes in-
matory bowel disease is latent, the course of clude erosion, sclerosis, and eventually fu-
arthritis may be similar to the course of juve- sion of the sacroiliac joint and vertebrae.
nile ankylosing spondylitis.57,58 Treatment Magnetic resonance imaging and computed
may include aminosalicylate or steroid med- tomography of the sacroiliac joint may assist
ications and bowel resection depending on with early diagnosis. Enthesitis distinguishes
the severity of the disorder.56 this condition from juvenile rheumatoid
arthritis. Individuals with this condition
■ Juvenile Ankylosing Spondylitis typically begin a course of nonsteroidal anti-
Chief Clinical Characteristics inflammatory medications upon diagnosis
This presentation includes an insidious onset for pain control. Corticosteroids are used
of hip, sacroiliac, and/or low back pain and only short term due to associated problems
stiffness that may be unilateral or bilateral and including osteoporosis. Research indicates
difficult to localize initially. Sacroiliitis is the that treatment with tumor necrosis factor
hallmark sign, however, along with spinal can significantly improve symptoms.59
pain and stiffness, it usually does not mani-
fest until 2 to 5 years after onset. The pain will ■ Juvenile Dermatomyositis
become more persistent over time along with Chief Clinical Characteristics
gradual loss of spinal mobility and a reduc- This presentation is characterized by a skin
tion in normal lumbar lordosis. Symptoms rash that may be located on the torso, low
are typically worse at night or in the morn- back, and buttocks area early on in the disease
ing and lasting at least 30 minutes. In anky- process. The characteristic skin rash may also
losing spondylitis pain may be relieved with be found around the shoulder girdle, exten-
rest or only mild activity. During active dis- sor surfaces of the arms and legs, medial
ease, systemic symptoms such as fever and malleoli of the ankles, and face. Disease on-
weight loss may be present, and the child may set is often insidious and may include fatigue,
refuse to walk. Associated complications may low-grade fever, weight loss, irritability,
include atlantoaxial subluxation, uveitis arthralgia, and abdominal pain. Proximal
leading to blindness, acute iritis, aortic valve muscle weakness is usually recognized after a
insufficiency, decreased vital capacity, neuro- median of 2 months.60 Hip pain, although an
logical complications, and, rarely, gastrointesti- uncommon symptom of this condition, is
nal and renal problems. often reproduced with palpation of the af-
fected region. Although this condition is rare
Background Information
in children, it is the most common of its type.
This condition is the most prevalent spondy-
loarthropathy in children, found primarily Background Information
in adolescent boys and young men. Onset is The disease process is triggered by an antigen-
usually in the second or third decade of life, driven response, which manifests as a skin
but 10% to 20% will have onset prior to rash. In the absence of prompt diagnosis and
age 16. Only about 25% of children with this treatment, the development of myositis with
disease will present initially with sacroiliac subsequent calcinosis occurs, causing severe
or spinal symptoms. Peripheral joints are limitation of range of motion of involved
1528_Ch45_898-924 07/05/12 2:14 PM Page 915

Chapter 45 Systemic Lupus Erythematosus 915

joints. The onset of proximal weakness is in- accompanies discoloration (dusky blue or red-

HIP PAIN IN A CHILD


sidious and difficult to recognize. It is often ness), enthesitis, swelling, warmth, and ten-
detected by difficulty in everyday activities derness to palpation of the involved joints.11
such as climbing stairs, reaching overhead,
Background Information
going from sitting to standing, and going
It is asymmetrical, affecting an average of
from the floor to standing.61 This condition
four joints and predominantly the lower
occurs in approximately 1 to 3 children per
extremities.10 Hip involvement occurs un-
million every year. The average age of onset is
commonly. It is most common in Caucasian
between 6 and 7 years of age with a higher
males.10 The onset of symptoms is usually
prevalence in girls. Laboratory investigations
preceded by a genitourinary or gastroin-
include blood tests, muscle biopsy, and
testinal infection.10 While the underlying
specific antibody tests. Treatment should in-
cause is an infection, the condition itself is a
clude passive range-of-motion exercises
reactive arthritis to the bacterial proteins,
and stretching during the disease course.
not an infection. It has also been associated
Once active inflammation has resolved, treat-
with human immunodeficiency virus. In
ment should focus on strengthening decondi-
children a genitourinary infection may be
tioned muscle, regaining range of motion,
an indication of sexual abuse. 10 This
and performing weight-bearing activities to
condition is medically managed with anti-
ensure adequate bone mineralization. Bed
inflammatory medications or in severe cases
rest and immobilization are contraindicated.
with low-dose corticosteroids.10
■ Psoriatic Arthritis ■ Systemic Lupus Erythematosus
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation involves psoriatic rash, This presentation may initially include mus-
arthritis, nail pitting, dactylitis (or “sausage culoskeletal pain in any or all regions of the
digit”), and occasional fatigue. As the disease body including the back in 74% of children.62
progresses sacroiliitis is present, resulting in This may be accompained by the presence of
hip and low back pain. In children arthritis fever, fatigue, arthralgia, malar (butterfly)
may manifest prior to psoriasis, or onset of rash, anorexia, lymphadenopathy, pleuritic
arthritis and psoriasis is concurrent. pain, and seizures.
Background Information Background Information
Chronic iridocyclitis is seen in approximately This condition is a rheumatic disease of un-
15% of children.57,58 This condition typically known cause characterized by autoantibodies
occurs in young girls, usually manifesting directed against self-antigens and resulting in
between 7 and 13 years of age, but always oc- inflammatory damage to target organs in-
curring prior to age 16 years. There is almost cluding kidneys, blood-forming cells, and the
always a family history of psoriasis. Radi- central nervous system. The incidence varies
ographs and computed tomography scan will by ethnicity and location. Prevalence rates
determine extent of involvement of the spine, vary from 4 to 250 per 100,000 children. The
hips, and sacroiliac joint. Treatment includes median age at diagnosis is approximately
nonsteroidal anti-inflammatory medica- 10 years, and onset before age 8 is uncom-
tions, corticosteroids, and topical or systemic mon.63 Diagnosis is confirmed by the combi-
medications for psoriasis. Modalities can also nation of clinical and laboratory manifesta-
be used to control inflammation and pain. tions revealing multisystem disease. Treatment
regimen depends on the affected target or-
■ Reactive Arthritis (Reiter’s gans. Nonsteroidal anti-inflammatory agents
Syndrome) are used to treat arthralgia and arthritic
Chief Clinical Characteristics symptoms. Corticosteroids have been demon-
This presentation of reactive arthritis or strated to control symptoms and autoanti-
Reiter’s syndrome can be characterized by body production in systemic lupus erythe-
pain with passive and active movement that matosus. Patients with severe disease may
1528_Ch45_898-924 07/05/12 2:14 PM Page 916

916 Chapter 45 Rickets, Vitamin D–Dependent (Nutritional)

require cytotoxic therapy. When the child is uremia, and prolonged treatment with certain
HIP PAIN IN A CHILD

cleared for activity, physical therapy should anticonvulsants.5 This condition is an autoso-
focus on stretching, joint protection, and mal dominant inherited disorder. It can be as-
relaxation techniques to manage joint and sociated with renal osteodystrophy, another
muscle pain. During periods of remission, an metabolic disease found in children with renal
exercise program of strengthening and car- disease.64 Supplements and medications are
diovascular exercise may be implemented to not indicated for this form of rickets. Surgery,
maintain the strength of surrounding muscu- bracing and rehabilitation may be necessary to
lature and to prevent the detrimental effects of address orthopedic complications.
bone demineralization due to prolonged use
of corticosteroids.63 Physical therapy treat- ■ Scleroderma (Focal or Systemic
ment should be provided under supervision of Sclerosis)
the child’s physician. The natural history is Chief Clinical Characteristics
unpredictable; patients may present with a This presentation includes fatigue, arthralgias
history of many years of symptoms or with or myalgias, and Raynaud’s syndrome (va-
acute, life-threatening disease. Left untreated, sospasm of arteries in response to cold).10 It
this condition may result in spontaneous also can result in arthritis, tendinitis, weak-
remission, years of disease, or rapid death. ness, and joint contractures.10
■ Rickets, Vitamin D–Dependent Background Information
(Nutritional) This rare, chronic multisystem connective tissue
disease results in fibrosis, small-vessel vasculitis,
Chief Clinical Characteristics
and an autoimmune response.10 Pharmacologic
This presentation includes profound weakness,
therapy is the primary means of management
skeletal deformity such as bowing of long bones,
with therapy involved for management of con-
and an inability to ambulate.64
tractures and systemic effects.10
Background Information
In the underdeveloped world, children who ■ Septic Arthritis
are undernourished may have a vitamin D Chief Clinical Characteristics
deficiency resulting in osteomalacia that can This presentation commonly includes severe
lead to hip pain due to stress fracture or pain and spasm with all hip movements, limp
slipped capital femoral epiphysis.64 Lack of or inability to bear weight, fever, tenderness to
exposure to sunlight can also be a precipitat- palpation, local warmth, and edema.10,51
ing factor.5 Vitamin D supplements to ad-
Background Information
dress the specific deficiency can resolve this
Children often maintain their hip in a position
disorder.
of flexion, abduction, and external rotation.10
■ Rickets, Vitamin D–Resistant The majority of cases in children are monoartic-
(Hypophosphatasia) ular, affect the knee or hip, and result from a
bacterial infection.10 It occurs less commonly
Chief Clinical Characteristics
due to tuberculosis or fungal infections such as
This presentation involves spine, pelvic, and
coccidioidomycosis.10 An elevated erythrocyte
long bone deformity due to poor bone miner-
sedimentation rate helps to confirm the diagno-
alization and overgrowth of unmineralized
sis.10 In neonates or infants anorexia, irritability,
bone matrix.5
and failure to thrive may be the only indications
Background Information of pathology.10 In sexually active adolescents the
In the industrialized world vitamin D–resistant infection may be due to gonococcus and pres-
rickets or hypophosphatasia is the most com- ents as a migratory mono- or polyarticular
mon form of rickets. This form is not related arthritis with accompanying hemorrhagic or
to dietary deficiency but instead due to pustular skin lesions.10 Complications of the
vitamin D metabolism derangement or mal- disease include osteomyelitis and osteonecrosis
absorption, or homeostasis of calcium or of the hip.10 Treatment includes antibiotic
phosphorus due to kidney or liver disease, therapy, surgical drainage, and debridement.10
1528_Ch45_898-924 07/05/12 2:14 PM Page 917

Chapter 45 Snapping Hip Syndrome 917

■ Sickle Cell Anemia head on the neck of the femur (Fig. 45-3).66,67

HIP PAIN IN A CHILD


Chief Clinical Characteristics This disorder occurs bilaterally in 18% to 50%
This presentation is characterized by painful of children with higher frequency in the African
vaso-occlusive crisis triggered by infection, American population.67 Overall this disorder is
dehydration, or acidosis that results in diffuse most common in males, and is more common
joint pain.10 in Pacific Islanders and African Americans than
other ethnic groups.67 It is also associated with
Background Information an obese, hypogonadal, or tall and thin body
Complications of the vaso-occlusion that type.25,68 Prevalence is higher in children with
occurs in this disease include osteonecrosis of hypothyroidism on growth hormone therapy
the femoral head, osteomyelitis, and septic and and is associated in rare cases with radiation
reactive arthritis.5,10 Slipped capital femoral therapy.68 Complications of this disorder in-
epiphysis should be excluded in these children clude avascular necrosis and chondrolysis.67 In-
due to similar age and presentation.65 Manage- tervention options include immobilization with
ment includes folic acid supplements and pain a hip-spica cast and various types of surgical
management during acute crises. fixation.67
■ Slipped Femoral Capital ■ Snapping Hip Syndrome
Epiphyses Chief Clinical Characteristics
Chief Clinical Characteristics This presentation includes clicking and pain
This presentation involves acute or insidious located over the greater trochanter or the groin
onset of groin pain, which may radiate into the depending on the structure involved.24
anteromedial thigh or knee, increased pain with
Background Information
weight bearing, and a passive limitation of
This phenomenon of clicking of the hip is typ-
abduction, flexion, and internal rotation.25
ically due to the iliotibial band snapping over
Background Information the greater trochanter, but it also can occur
The pathology is due to a disruption of the cap- from the iliopsoas over the iliopectineal
ital epiphyseal plate resulting in a slipping of the eminence, biceps femoris long head over the

Offset of epiphysis
and metaphysis
indicated with
black arrows

Widened physis when compared


with the normal other side

FIGURE 45-3 Slipped capital


femoral epiphysis of the leg hip, an-
teroposterior view. Inset: frog leg
view. (Images courtesy of Keven
Preston, MD, diagnostic radiology
resident, Indiana University School
of Medicine.)
1528_Ch45_898-924 07/05/12 2:14 PM Page 918

918 Chapter 45 Spastic Cerebral Palsy

ischial tuberosity, and the iliofemoral liga- to a prior viral infection, microtrauma, or
HIP PAIN IN A CHILD

ments over the femoral head.24 These extra- allergic reaction but is largely a diagnosis of
articular disorders can progress into bursitis.24 exclusion with diseases such as bacterial
Intra-articular problems such as loose bodies synovitis, septic arthritis, osteomyelitis, and
can also cause the same symptoms.24 Snapping pyarthrosis being ruled out.71 This is usually a
hip is typically associated with activities self-limiting disorder treated with rest and
such as dancing and running.25 Management anti-inflammatory medications.71
includes inflammation management and
restoring of muscle balance.24,25 ■ Traumatic Dislocation
Chief Clinical Characteristics
■ Spastic Cerebral Palsy This presentation is characterized by a range
Chief Clinical Characteristics of presentations that correspond with the
This presentation may include hip subluxation direction of dislocation. A child with a poste-
or dislocation as a result of the muscle imbal- rior dislocation will present with a flexed, ad-
ance between spastic hip adductors and other ducted, and internally rotated limb that appears
weak hip musculature.69 shortened. The posturing is the opposite for
an anterior dislocation without shortening.
Background Information
An inferiorly dislocated hip will present in
A child with this condition may also have in-
hyperflexion.72
creased femoral anteversion and femoral neck-
to-shaft angle, decreasing the stability of the Background Information
hip articulation.70 Subluxations or disloca- Hip dislocation due to trauma is rare in the
tions are more common in nonambulatory pediatric population with most cases due to a
children with cerebral palsy and may be more motor vehicle accident, a fall from height, or a
likely during growth spurts and do not tend to sports activity.73 The hip is dislocated in the
be a problem until their second decade of posterior direction in the majority of cases
age.21 Nonsurgical management may include with a few being anterior or inferior.24 The dis-
positioning and stretching. location is sometimes associated with a con-
comitant hip fracture (17%).73 Complications
■ Tendinitis after this injury include avascular necrosis
Chief Clinical Characteristics (3% to 15%), heterotopic ossification, coxa
This presentation includes a gradual onset of magna, and instability.72,73 There is a signifi-
local pain and weakness associated with cant association between the time from dislo-
activity.24 cation to reduction and the incidence of
avascular necrosis.72 Management includes re-
Background Information
duction of dislocation and rehabilitation.
This inflammation of tendons of the hip is
most common in the iliopsoas and ham- ■ Tuberculosis Arthritis
string muscle groups. It is usually associated
with overuse and sports activities. Treatment Chief Clinical Characteristics
can include rest, inflammation management, This presentation includes joint pain and
taping, strengthening, and gradual return to swelling.10 This form of arthritis is most com-
activity. mon in the hips and knees.10

■ Transient Synovitis Background Information


This condition can be related to an adjacent os-
Chief Clinical Characteristics
teomyelitis involving the hands or feet in chil-
This presentation commonly involves unilateral
dren.10 This is a chronic, slowly progressive dis-
hip joint pain and a limp.71 Often, hip motion
order. It is confirmed by a tissue and synovial
is limited and children maintain a position of
fluid culture that is positive for Mycobacterium
hip flexion and external rotation.71
tuberculosis due to joint invasion.74 Treatment
Background Information may include combination chemotherapy and
Boys are more commonly affected between the surgery for debridement, stabilization, or
ages of 3 and 8.71 This condition may be linked synovectomy.10
1528_Ch45_898-924 07/05/12 2:14 PM Page 919

Chapter 45 Leukemia 919

TUMORS osseous deformity, because this condition is

HIP PAIN IN A CHILD


■ Ewing’s Sarcoma usually self-limiting.78 Treatment can in-
clude a combination of any or all of radia-
Chief Clinical Characteristics tion, chemotherapy, steroid therapy and
This presentation typically manifests as severe, curettage, and bracing.79,80
persistent pain especially at night, localized
swelling, palpable mass, tenderness, and de-
■ Leukemia
creased range of motion. Other symptoms can
include fever, malaise, and weight loss when Chief Clinical Characteristics
there are metastases.75 This presentation commonly involves reports
of aches and pains in the extremities, back, and
Background Information joints; fatigue; and anorexia. There may also
This condition is the most common malig- be intermittent low-grade fever, enlarged
nant primary tumor found in the pediatric lymph nodes, weight loss, petechiae, lethargy,
spine76 and the second most common pedi- shortness of breath, bruises, and excessive
atric malignancy overall.77 Approximately 2.1 bleeding.
children per million are diagnosed with this
condition each year with predominance in Background Information
males. Diagnosis is usually between the ages Leukemia is the most common malignancy
of 10 and 20 years, and is uncommon before in children and adolescents, with a higher in-
age 5.77 Treatment includes a combination of cidence in males.81,82 It is typically diagnosed
multiagent chemotherapy, followed by radi- within the first decade of life and accounts
ation and resection for local control. Prog- for about 40% of all childhood malignan-
nosis for cure is approximately 75% in those cies. The two most common types account
who present without metastasis, and less for approximately 90% of all leukemias:
than 30% for those with metastasis. acute lymphoblastic leukemia (ALL), 77% to
80%, and acute myelogenous leukemia
■ Langerhans’ Cell Histiocytosis (AML), 10% to 13%. Some symptoms may
be consistent with a viral infection, but the
Chief Clinical Characteristics
symptoms persist longer. Other symptoms
This presentation commonly consists of pain
are a result of progressive disease and bone
that is usually relieved with nonsteroidal anti-
marrow failure as a result of bone marrow
inflammatory medications, rest, and, if nec-
infiltration by malignant cells, leading to
essary, bracing. Associated symptoms may be
anemia and decreased production of clotting
hepatosplenomegaly, anemia, leukopenia, and
factors.82 Diagnostic evaluation of patients
thrombocytopenia. Neurological symptoms
with leukemia includes complete blood
may manifest if there is cord compression.
count and bone marrow biopsy. Examina-
Background Information tion of bone marrow aspirate is necessary for
This condition is commonly found in the confirmation, and a spinal tap determines if
anterior elements of the spine as a single leukemia is present in the central nervous
lesion or is multifocal. A single, localized le- system.83 Infiltration of bone and bone mar-
sion is also known as eosinophilic granu- row may also be viewed on magnetic reso-
loma. This condition is generally found in nance imaging.79 Rarely radiographs may
children 3 to 15 years of age with a predom- show osteopenia and compression fractures.
inance for white males, but can manifest at Analysis of chromosome abnormalities in
any age.18,78,79 Radiographs will show a well- the leukemia cells is very important to assess
defined lesion, with focal bone destruction prognosis and formulate treatment. Treat-
and possible vertebral collapse, or vertebral ment is specific to the type of leukemia pres-
plana. This condition appears to be the most ent and typically has several phases. It in-
common cause of vertebral plana.78 The volves multiple courses of chemotherapy
intervertebral disks are not affected.78 and antibiotics to prevent infection, and of-
Treatment is usually reserved for those with ten a child will require blood transfusions.
organ dysfunction, unrelieved symptoms, or Side effects of chemotherapy and steroid
1528_Ch45_898-924 07/05/12 2:14 PM Page 920

920 Chapter 45 Lymphoma

treatment include neutropenia, thrombocy- includes chemotherapy and radiation, the


HIP PAIN IN A CHILD

topenia, and risk for infection. Long-term course of which depends on the type of lym-
complications can include osteoporosis, and phoma, the stage of disease, and if there is
avascular necrosis. Bone marrow or stem cell bone marrow metastasis. Bone marrow
transplant is reserved for situations where transplant may be indicated if there is failure
standard chemotherapy has failed and has a to respond to standard chemotherapy and
better success rate if performed in remis- radiation. Survival rates are 90 to 95% for
sion.83 With current advances in treatment those with local lesions, and 75 to 80% for
the 5-year survival rate, without relapse is those with more diffuse disease.81
reaching 80% to 90%.84
■ Nephroblastoma (Wilms’
■ Lymphoma Tumor)
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation initially involves asympto- This presentation typically includes an
matic, chronically swollen lymph nodes, most asymptomatic, nontender mass in the ab-
commonly in the neck, axillae, or groin. Other domen. Associated symptoms may be ab-
symptoms may include neurological symptoms dominal pain, fever, nausea, and vomiting.
due to cord compression, fever, itching or irri- Hematuria, anemia, and hypertension can
tation of the skin, weight loss, night sweats, occur, since the kidneys are instrumental in
cough, or shortness of breath.83 Symptoms controlling blood pressure. Shortness of
depend on the type of lymphoma present and breath may be present if metastases to the
the proximity to other structures and organs. lungs are present.81,87
Background Information
Lymphoma is one of the most prevalent Background Information
malignant tumors of childhood, and can This condition involves a tumor that origi-
manifest quickly. The two general types of nates from renal precursor cells. It is the
lymphoma are Hodgkin’s lymphoma and second most common abdominal tumor in
non-Hodgkin’s lymphoma (NHL), and each infants and children up to 5 years old and is
has its own subtypes. Hodgkin’s disease is the most common primary malignant
distinguished from non-Hodgkin’s by the tumor of the kidney. Multiple laboratory
presence of specific cancer cells in the biopsy tests, including complete blood count,
material, termed Reed-Sternberg cells.83,85 ultrasound, magnetic resonance imaging,
Approximately 40% of pediatric cases are of or computed tomography, are indicated.
the Hodgkin’s type, and 60% are non- Treatment includes removal of the kidney
Hodgkin’s.81 Approximately 10% to 15% of containing the tumor followed by
all cases of Hodgkin’s disease, and about chemotherapy and or radiation. The sur-
5% of all NHL cases, are children under vival rate is typically 70% to 95%, especially
age 19, both with a higher prevalence in in young children, or those with stage I, II,
boys. NHL is more common in children un- or III tumors.87 Recurrences are usually seen
der age 5.85,86 Biopsy, radiographs, computed within 3 years. Postradiation scoliosis may
tomography, and blood tests are performed follow in the second decade.
for definitive diagnosis and to assess the
stage of disease. Bone marrow biopsy is done ■ Neuroblastoma
to evaluate bone marrow involvement. Radi- Chief Clinical Characteristics
ographs and computed tomography typi- This presentation may include severe localized
cally show enlarged lymph nodes in the af- back pain, weakness, scoliosis, and neurolog-
fected areas and can show extensive, ical signs secondary to spinal cord compres-
destructive lesions in patients with osseous sion. Other symptoms may include a palpa-
involvement. Magnetic resonance imaging is ble mass, hepatomegaly, constipation,
indicated to evaluate soft tissue involve- abdominal pain, bladder dysfunction, and
ment.75 Laboratory tests may show anemia ecchymoses. It is one of the few cancers that
and elevated sedimentation rate. Treatment cause diarrhea.75,81,88,89
1528_Ch45_898-924 07/05/12 2:14 PM Page 921

Chapter 45 Osteosarcoma (Osteogenic Sarcoma) 921

Background Information often present. About 50% of children present

HIP PAIN IN A CHILD


This condition is a solid tumor that develops with nonstructural, painful scoliosis,76 which
in neural crest tissue, most commonly in the should be a red flag because idiopathic scolio-
abdomen, chest, or adrenal glands.81 It rarely sis is not painful.
originates in the brain or spinal cord. If the
primary lesion originates somewhere other Background Information
than the spinal cord, metastases is hemato- This condition includes highly vascularized
genic in nature, and can spread to bone, connective tissue bundles, usually sclerotic,
bone marrow, live, skin, lungs, and the brain. surrounding an osteoid nidus or growth
This is the most common extracranial cancer center. They are small round or ovate tu-
of childhood and the third most common mors that are usually less than 1 cm in
pediatric neoplasm overall. Approximately diameter.75,90 Spinal osteoid lesions are
500 new cases are diagnosed each year. It is found primarily in the posterior elements of
usually diagnosed in infancy, almost always the lumbar vertebra.18,75,76,79,90 Soft tissue
by 5 years of age, and is rarely seen in chil- changes are rare. This condition is typically
dren over 10 years of age.75,81,88,89 Low-risk found in children between the ages of 5 and
tumors (stage I) in infants treated with 20, with an approximate 2:1 male-to-female
surgical excision and observation have an ratio.75,91 Erythrocyte sedimentation rate,
approximately 90% survival rate without leukocyte count and protein electrophoresis
relapse. Children over 3 years of age with should be normal. Radiographs and com-
stage IV tumors only have a 10% to 15% sur- puted tomography will also differentiate
vival rate.81 In infants the tumor may regress osteoid osteomas from osteoblastomas, in-
completely with spontaneous cell death, or fection, lymphoma, and facet abnormalities
mature to normal ganglion cells and become such as spondylolysis.91 Spinal lesions typi-
benign. In children over 1 year of age, the cally require open surgical excision. In most
disease progression may be rapidly metasta- cases, the scoliosis will resolve and pain will
tic and often fatal.81,88 subside completely.14

■ Osteochondroma ■ Osteosarcoma (Osteogenic


Chief Clinical Characteristics Sarcoma)
This presentation involves hip pain that can Chief Clinical Characteristics
result from compression of a nerve by or frac- This presentation may be characterized by
ture of the exostosis.5 persistent night pain.
Background Information Background Information
This cartilaginous exostosis is more com- This condition is related to highly malignant
mon in males and sometimes occurs in the and locally aggressive lesions.92 It rarely orig-
pelvis.5 It is more common in late adoles- inates in the axial skeleton, but when it does
cence to young adulthood.5 Familial osteo- it is usually found in the vertebral bod-
chondromatosis or multiple hereditary exos- ies.80,92,93 Vertebral sarcomas comprise about
tosis is an autosomal dominant inherited 10% of all cases.76 These tumors are typically
variant in which more than two osteochon- found in the metaphyses of long bones and
dromas are present.5 This form is more com- may metastasize to the spine. Diagnosis usu-
mon in males during childhood and is more ally occurs between 10 and 20 years of age,
likely to involve the hip region.5 and adolescent growth spurts appear to be
the greatest risk periods for development.79,94
■ Osteoid Osteoma Children with certain hereditary conditions
Chief Clinical Characteristics such as retinoblastoma have a significant pre-
This presentation involves predominantly in- disposition for developing this condition.
tense nocturnal pain that may be relieved with Treatment includes local control with surgi-
nonsteroidal anti-inflammatory medications cal excision and radiation or chemotherapy
or aspirin.19,79 The pain may be referred or lo- for metastatic lesions. If neurological signs
calized. Gait deviations and muscle atrophy are are present, surgical decompression and
1528_Ch45_898-924 07/05/12 2:14 PM Page 922

922 Chapter 45 Rhabdomyosarcoma

laminectomy may be necessary.17 Without References


HIP PAIN IN A CHILD

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■ Synovial Sarcoma 14. Hosalkar H, Dormans JP. Back pain in children requires
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CHAPTER46
Case Demonstration: Hip Pain in a Child
■ Hugh G. Watts, MD ■ Chris A. Sebelski, PT, DPT, OCS, CSCS

NOTE: This case demonstration was developed University of Southern California. Her son has
using the diagnostic process described in mentioned that he has pain in his right hip.
Chapter 4 and demonstrated in Chapter 5. The Your friend is unsure of exactly where the pain
reader is encouraged to use this diagnostic is. She’s hoping that you will check out her
process in order to ensure thorough clinical child to see if an expensive journey through
reasoning. If additional elaboration is required the medical system can be avoided.
on the information presented in this chapter, Billy is 7 years old and is a good student
please consult Chapters 4 and 5. in the second grade. Questions directed to
your friend indicate that Billy’s pain came
THE DIAGNOSTIC PROCESS on gradually about 2 months ago, a few days
after he fell off his bicycle. He is a healthy
Step 1 Identify the patient’s chief concern.
boy overall and has had all of his immuniza-
Step 2 Identify barriers to communication.
tions. She admits that he has had several
Step 3 Identify special concerns.
intermittent low-grade fevers, but there have
Step 4 Create a symptom timeline and sketch
been a lot of “bugs” going around his school.
the anatomy (if needed).
Billy has a 5-year-old sister who is developing
Step 5 Create a diagnostic hypothesis list
typically.
considering all possible forms of remote and
You agree to see him. Billy walks into the
local pathology that could cause the
clinic with a right antalgic gait pattern. When
patient’s chief concern.
asked, Billy points vaguely to his right hip
Step 6 Sort the diagnostic hypothesis list by
above the greater trochanter and in front of his
epidemiology and specific case
upper thigh.
characteristics.
Step 7 Ask specific questions to rule specific STEP #1: Identify the patient’s chief
conditions or pathological categories less concern.
likely. ● Right anterolateral hip pain
Step 8 Re-sort the diagnostic hypothesis list
based on the patient’s responses to specific STEP #2: Identify barriers to
questioning. communication.
Step 9 Perform tests to differentiate among ● This is a personal referral from a friend.

the remaining diagnostic hypotheses. ● Patient is a 7-year-old male, which suggests

Step 10 Re-sort the diagnostic hypothesis list associated issues with communication.
based on the patient’s responses to specific STEP #3: Identify special concerns.
tests.
● Family history. Though no details are
Step 11 Decide on a diagnostic impression.
known, the mother has identified a possible
Step 12 Determine the appropriate patient
family history of a bleeding disorder.
disposition.
● Recent illness. This may raise the index of
clinical suspicion for causes of pain that are
associated with illness.
Case Description
STEP #4: Create a symptom timeline and
You receive a phone call from a good friend sketch the anatomy (if needed).
of yours who is a graduate student at the

925
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926 Chapter 46 Case Demonstration: Hip Pain in a Child

2 months 2 days
ago ago Today

Fell off Complaints Intermittent fevers Phone call Initial


bike of R hip pain from friend physical
therapy
evaluation

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.
Remote Remote
T Trauma T Trauma
Lumbar disk herniation/radiculopathy Lumbar disc herniation/radiculopathy
(would require major trauma)
Spondylolisthesis Spondylolisthesis (too young; congenital
type is painless)
Spondylolysis Spondylolysis (too young)
I Inflammation I Inflammation
Aseptic Aseptic
Not applicable Not applicable
Septic Septic
Appendicitis (especially retrocecal) Appendicitis (especially retrocecal) (timeline
too long)
Chronic granulomatous disease Chronic granulomatous disease
Discitis Discitis
Osteomyelitis of the lumbar vertebra Osteomyelitis of the lumbar vertebra
Septic arthritis of sacroiliac joint Septic arthritis of sacroiliac joint

M Metabolic M Metabolic
Metabolic myopathy Metabolic myopathy (not associated with
fever)
Va Vascular Va Vascular
Polyarteritis nodosa Polyarteritis nodosa (rare in children)
De Degenerative De Degenerative
Not applicable Not applicable
Tu Tumor Tu Tumor
Lymphoma Lymphoma
Neuroblastoma Neuroblastoma (too old)
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Not applicable Not applicable
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Chapter 46 Case Demonstration: Hip Pain in a Child 927

Local Local
T Trauma T Trauma
Avulsion injuries: Avulsion injuries:
● Psoas off the lesser trochanter ● Psoas off the lesser trochanter (too young)

● Rectus off the AIIS ● Rectus off the AIIS (too young)

● Sartorius off the ASIS ● Sartorius off the ASIS (too young)

Chondral injury Chondral injury (not associated with fever)


Delayed-onset muscle soreness Delayed-onset muscle soreness (not
associated with fever)
Heterotopic ossification due to trauma Heterotopic ossification due to trauma (not
associated with fever)
Hip contusion Hip contusion (not associated with fever)
Hip dislocation Hip dislocation (can walk; not associated
with fever)
Hip fracture Hip fracture (can walk)
Labral tear Labral tear (too young)
Meralgia paresthetica Meralgia paresthetica (not associated with
fever)
Muscle strain Muscle strain (not associated with fever)
Myositis ossificans Myositis ossificans (too young)
Slipped capital femoral epiphyses Slipped capital femoral epiphyses (too
young, unless major metabolic,
endocrine, renal problem)
Snapping hip syndrome Snapping hip syndrome (not associated
with fever)
I Inflammation I Inflammation
Aseptic Aseptic
Apophysitis of greater trochanter Apophysitis (not associated with fever)
Chronic inflammatory demyelinating Chronic inflammatory demyelinating
polyneuropathy polyneuropathy
Guillain-Barré syndrome Guillain-Barré syndrome
Hip bursitis Hip bursitis (not associated with fever)
Juvenile rheumatoid arthritis (and related Juvenile rheumatoid arthritis (and related
diseases): diseases):
● Juvenile dermatomyositis ● Juvenile dermatomyositis

● Juvenile spondyloarthropathies ● Juvenile spondyloarthropathies

● Scleroderma ● Scleroderma

● Systemic lupus erythematosus ● Systemic lupus erythematosus

Osteitis pubis Osteitis pubis (not associated with fever)


Reactive arthritis Reactive arthritis (too young)
Rheumatic fever Rheumatic fever (timeline too long)
Tendinitis Tendinitis (not associated with fever)
Transient synovitis Transient synovitis (too old)
Septic Septic
Acute: Acute:
Lyme disease (tick paralysis) Lyme disease (tick paralysis)
Osteomyelitis of femoral neck Osteomyelitis of femoral neck
Poliomyelitis Poliomyelitis (would be known by this time)
Pyomyositis Pyomyositis (not sick enough)
Septic arthritis of the hip Septic arthritis of the hip joint (timeline too
long)
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928 Chapter 46 Case Demonstration: Hip Pain in a Child

Chronic: Chronic:
Brucellosis of the proximal femur or pelvis Brucellosis of the proximal femur or pelvis
(wrong country)
Fungal infection of the proximal femur or Fungal infection of the proximal femur or
pelvis (such as actinomycosis, pelvis (such as actinomycosis
coccidioidomycosis, or histoplasmosis) coccidioidomycosis, or histoplasmosis)
(wrong geography)
Tuberculosis of the proximal femur or Tuberculosis of the proximal femur or
pelvis pelvis
M Metabolic M Metabolic
Ehlers-Danlos syndrome Ehlers-Danlos syndrome (not associated
with fever)
Gaucher’s disease Gaucher’s disease (not specifically
associated with fever, otherwise normal
growth, development, and appearance to
date)
Gout Gout (possible but unlikely at this age)
Lesch-Nyhan syndrome Lesch-Nyhan syndrome (would be known
by this time)
Osteogenesis imperfecta Osteogenesis imperfecta (would be known
by this time; not associated with fever)
Osteopetrosis Osteopetrosis (would be known by this
time; not associated with fever)
Rickets: Rickets:
● Vitamin D–dependent (nutritional) ● Vitamin D–dependent (nutritional) (not

associated with fever)


● Vitamin D–resistant (hypophosphatasia) ● Vitamin D–resistant (hypophosphatasia)

(not associated with fever)


Va Vascular Va Vascular
Hemophilic arthropathy Hemophilic arthropathy (would be known
by this time)
Legg-Calvé-Perthes/osteonecrosis Legg-Calvé-Perthes/osteonecrosis (not
associated with fever)
Henoch-Schönlein purpura Henoch-Schönlein purpura (would be
known by this time)
Osteochondritis dissecans Osteochondritis dissecans (not associated
with fever)
Sickle cell anemia Sickle cell anemia (would be known by this
time)
De Degenerative De Degenerative
Not applicable Not applicable
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
● Ewing’s sarcoma ● Ewing’s sarcoma

● Leukemia ● Leukemia

● Osteosarcoma ● Osteosarcoma

● Rhabdomyosarcoma ● Rhabdomyosarcoma

● Synovial sarcoma ● Synovial sarcoma


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Chapter 46 Case Demonstration: Hip Pain in a Child 929

Malignant Metastatic Malignant Metastatic (unlikely at this


age)
Benign, such as: Benign, such as:
● Langerhans’ cell histiocytosis ● Langerhans’ cell histiocytosis

(eosinophilic granuloma) (eosinophilic granuloma)


● Osteochondroma ● Osteoid osteoma (not associated with

fever)
● Osteoid osteoma ● Osteochondroma (not associated with

fever)
Co Congenital Co Congenital
Acetabular dysplasia Acetabular dysplasia (not associated with
fever)
Epiphyseal dysplasias Epiphyseal dysplasias (not associated with
fever)
Myelodysplasia (spina bifida/ Myelodysplasia (spina bifida/
myelomeningocele) myelomeningocele) (would be known by
this time)
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
School phobia School phobia (not associated with fever)
Spastic cerebral palsy Spastic cerebral palsy (not associated with
fever)

STEP #7: Ask specific questions to rule I Inflammation


specific conditions or pathological Aseptic
categories less likely. Not applicable
● Have you had any weakness in your legs or
Septic
arms? No, the patient denies any symptoms Chronic granulomatous disease
of weakness. This would exclude diagnostic Discitis (no back pain)
categories associated with weakness such as Osteomyelitis of the lumbar vertebra (no
Guillain-Barré and chronic demyelinating back pain)
polyradiculoneuropathy. Septic arthritis of sacroiliac joint
● Have you been more tired than usual? No,
M Metabolic
making forms of cancer, such as lymphoma
and metastatic disease, less likely. Not applicable
● Have you had any back pain? No, making Va Vascular
osteomyelitis of lumbar vertebrae and disci- Not applicable
tis unlikely. De Degenerative
● Have you been hiking in the woods (or oth-
Not applicable
erwise exposed to ticks)? No, making Lyme
disease less likely. Tu Tumor
Lymphoma (absence of fatigue)
STEP #8: Re-sort the diagnostic
hypothesis list based on the patient’s Co Congenital
responses to specific questioning. Not applicable
Remote Ne Neurogenic/Psychogenic
T Trauma Not applicable
Not applicable
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930 Chapter 46 Case Demonstration: Hip Pain in a Child

Local STEP #9: Perform tests to differentiate


T Trauma among the remaining diagnostic
hypotheses.
Not applicable
● Oral temperature: 100°F.
I Inflammation
Aseptic
Chronic inflammatory demyelinating Teaching Comments: Body temperature
polyneuropathy (no symptoms of weakness) is the most discriminating test at this point for
Guillain-Barré syndrome (no symptoms of further differentiation of the remaining diag-
weakness) nostic categories. A positive finding allows
Juvenile rheumatoid arthritis (and related the clinician to be more efficient with the
diseases): remaining physical exam measures. Addition-
● Juvenile dermatomyositis
ally, a positive finding with the temperature
● Juvenile spondyloarthropathies
narrows the diagnostic categories, demon-
● Scleroderma
strating that Billy will require referral to a
● Systemic lupus erythematosus
physician because the remaining categories
Septic are not appropriate for direct access care by a
Acute: physical therapist.
Lyme disease (tick paralysis) (no chance for
exposure to ticks)
Osteomyelitis of femoral neck ● Palpation/percussion of the lumbar spine:
Chronic: There was no pain or limitation in segmen-
Fungi (such as actinomycosis or tal mobility found during this exam, mak-
coccidioidomycosis) ing chronic granulomatous disease and
Tuberculosis of the proximal femur or pelvis discitis less likely.
M Metabolic ● Active range of motion of the hip: Normal
and pain free, making primary hip patholo-
Not applicable gies less likely.
Va Vascular ● Compression of the sacroiliac joint via the
Not applicable ilial wings: Acute pain, making septic arthri-
De Degenerative tis of the sacroiliac joint more likely.
Not applicable
Tu Tumor
Teaching Comments: Many physical ther-
Malignant Primary, such as: apists would continue the physical exam in
● Ewing’s sarcoma order to collect further data to share with the
● Leukemia (absence of fatigue) patient and with the health care practitioner
● Osteosarcoma
to whom they will refer the patient. Interpre-
● Rhabdomyosarcoma
tation of test results should be based on the
● Synovial sarcoma
keys of evidence-based practice: patient view-
Malignant Metastatic: point, clinician’s experience, and research. The
Not applicable sacroiliac compression test has been studied
Benign, such as: extensively with mixed reviews on reliability.1
● Langerhans’ cell histiocytosis
However; with consideration of all three
(eosinophilic granuloma) components of evidence-based practice, the
Co Congenital information received from the physical
Not applicable exam techniques in combination with that of
the temperature strengthens the diagnostic
Ne Neurogenic/Psychogenic
impression.
Not applicable
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Chapter 46 Case Demonstration: Hip Pain in a Child 931

STEP #10: Re-sort the diagnostic Septic


hypothesis list based on patient’s Acute:
responses to specific tests. Osteomyelitis of femoral neck (no pain with
Remote hip active range of motion)
Chronic:
T Trauma
Fungi (such as actinomycosis or
Not applicable coccidioidomycosis) (no pain with hip
I Inflammation active range of motion)
Aseptic Tuberculosis of the proximal femur or pelvis
Not applicable (no pain with hip active range of motion)
Septic M Metabolic
Chronic granulomatous disease (no pain Not applicable
with palpation) Va Vascular
Discitis (no pain with palpation and
Not applicable
percussion)
Septic arthritis of sacroiliac joint De Degenerative
M Metabolic Not applicable
Not applicable Tu Tumor
Va Vascular Malignant Primary, such as:
● Ewing’s sarcoma (no pain with hip active
Not applicable
range of motion)
De Degenerative ● Leukemia (no pain with hip active range

Not applicable of motion)


● Osteosarcoma (no pain with hip active
Tu Tumor
range of motion)
Not applicable ● Rhabdomyosarcoma (no pain with hip
Co Congenital active range of motion)
Not applicable ● Synovial sarcoma (no pain with hip active

Ne Neurogenic/Psychogenic range of motion)


Malignant Metastatic:
Not applicable
Not applicable
Local Benign, such as:
T Trauma ● Langerhans’ cell histiocytosis

Not applicable (eosinophilic granuloma) (no pain with


hip active range of motion)
I Inflammation
Co Congenital
Aseptic
Juvenile rheumatoid arthritis (and related Not applicable
diseases): Ne Neurogenic/Psychogenic
● Juvenile dermatomyositis (pain is not
Not applicable
reproduced with ileal wing compression)
● Juvenile spondyloarthropathies (pain is STEP #11: Decide on a diagnostic
not reproduced with ileal wing impression.
compression) ● Septic arthritis of the sacroiliac joint

● Scleroderma (pain is not reproduced with


STEP #12: Determine the appropriate
ileal wing compression) patient disposition.
● Systemic lupus erythematosus (pain is
● Refer Billy to physician urgently for addi-
not reproduced with ileal wing
tional evaluation.
compression)
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932 Chapter 46 Case Demonstration: Hip Pain in a Child

Case Outcome high-dose oral agents on an outpatient basis.


As a result of this intervention, Billy’s condi-
Billy was referred to a pediatric orthopedic tion resolved.
surgeon, who confirmed the diagnosis of sep-
tic arthritis of the sacroiliac joint. Billy was Reference
treated with a course of antibiotic therapy that 1. van der Wurff P, Hagmeijer RH, Meyne W. Clinical tests
began with high-dose intravenous agents of the sacroiliac joint. A systematic methodological
administered on an inpatient basis followed by review. Part 1: reliability. Man Ther. 2000;5(1):30–36.
1528_Ch47_933-958 07/05/12 2:15 PM Page 933

CHAPTER47
Knee Pain in a Child
■ Jennifer Lundberg, PT, DPT ■ Cassandra Sanders-Holly, PT, DPT, PCS

Description of the Symptom ■ Knee pain associated with fever, flu-like


symptoms, skin changes in appearance and
This chapter describes possible causes of knee temperature
pain in a child. Local causes are defined as ■ Knee pain that is worse at night, unrelent-
occurring between the distal one-third of the ing pain, pain associated with night sweats,
thigh and proximal one-third of the lower leg rapid unexplained weight changes
in a child. Remote causes are defined as occur- ■ Pain that persists for several weeks with no
ring outside this region. known mechanical etiology

Special Concerns
■ Sudden onset of knee pain in the absence of
trauma

CHAPTER PREVIEW: Conditions That May Lead to Knee Pain in a Child

T Trauma
REMOTE LOCAL
COMMON
Slipped capital femoral Contusion (bone/muscle) 943
epiphysis 941 Fat pad impingement 944
Fractures:
• Avulsion, supracondylar (Segond fracture) 944
• Epiphyseal injuries 944
• Stress fracture 944
Growing pains 945
Iliotibial band friction syndrome 945
Ligament sprains/tears:
• Anterior cruciate ligament sprain/rupture 946
• Lateral collateral ligament sprain/rupture 946
• Medial collateral ligament sprain/rupture 947
• Posterior cruciate ligament sprain/rupture 947
Muscle strain (eg, quadriceps, hamstrings, adductors) 948
Osgood-Schlatter disease 949
Patellar dislocation 950
Patellofemoral pain syndrome (chondromalacia patella) 951
Sinding-Larsen-Johansson disease 952
UNCOMMON
Not applicable Blount’s disease 942
Meniscus tears:
• Lateral meniscus tear 947
• Medial meniscus tear 948
(continued)

933
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934 Chapter 47 Knee Pain in a Child

Trauma (continued)
KNEE PAIN IN A CHILD

REMOTE LOCAL
RARE
Lumbar radiculopathies: Nerve entrapments:
• L4 radiculopathy 940 • Common peroneal nerve at the fibular head 948
• L5 radiculopathy 941 • Saphenous nerve 949
• S1 radiculopathy 941 Plica syndrome 951

I Inflammation
REMOTE LOCAL
COMMON
Not applicable Aseptic
Bursitis:
• Infrapatellar 942
• Pes anserine (Voshell’s bursitis) 942
• Prepatellar 943
Patellar tendonitis 951
Transient synovitis 953
Septic
Lyme disease (Lyme arthritis) 947
Osteomyelitis of the distal femur, proximal tibia, or fibula 950
Pyogenic arthritis (septic arthritis) 952
UNCOMMON
Aseptic Aseptic
Ankylosing spondylitis 939 Erythema nodosum 944
Juvenile rheumatoid arthritis 946
Septic Rheumatic fever 952
Epidural abscess 940 Systemic lupus erythematosus 953
Septic hip 941
Septic
Not applicable
RARE
Not applicable Not applicable

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Osteogenesis imperfecta 949
RARE
Not applicable Gout 944
Myositis ossificans 948
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Chapter 47 Knee Pain in a Child 935

KNEE PAIN IN A CHILD


Va Vascular
REMOTE LOCAL
COMMON
Avascular necrosis of the Osteochondritis desiccans 949
femoral head (Legg-
Calvé-Perthes disease) 940
UNCOMMON
Not applicable Hemophilia 945
Sickle cell disease 952
RARE
Not applicable Henoch-Schönlein purpura 945
Thrombus (venous or arterial) 953
Vascular malformation 956

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Popliteal cyst 951

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Malignant Primary, such as:
• Leukemia 954
Malignant Metastatic:
Not applicable
Benign, such as:
• Osteochondroma 954
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Malignant Primary, such as:
• Ewing’s sarcoma 953
• Lymphoma 954
• Osteosarcoma (osteogenic sarcoma) 955
• Synovial sarcoma 956
Malignant Metastatic, such as:
• Neuroblastoma 954
Benign, such as:
• Ganglion cysts 954
(continued)
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936 Chapter 47 Knee Pain in a Child

Tumor (continued)
KNEE PAIN IN A CHILD

REMOTE LOCAL
RARE
• Osteoblastoma 954
• Osteoid osteoma 955
• Pigmented villonodular synovitis 955
• Synovial chondromatosis 955

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Patella alta 950
Patellar subluxation 950
UNCOMMON
Not applicable Discoid meniscus 943
RARE
Not applicable Bipartite patella 942
Congenital dislocated patella 943

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Conversion syndrome 940 Complex regional pain syndrome (reflex sympathetic
Somatization (psychogenic dystrophy) 943
rheumatism) 941

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Common Ages at Which Knee Pain Presents in a Child


APPROXIMATE AGE CONDITION
Birth to 3 Years Hematogenous septic arthritis
Hemophilia
Neuroblastoma
Osteogenesis imperfecta
Osteomyelitis
Pyogenic arthritis
Septic hip
Sickle cell anemia
Thrombus
Transient synovitis
Venous malformation
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Chapter 47 Knee Pain in a Child 937

KNEE PAIN IN A CHILD


Common Ages at Which Knee Pain Presents in a Child—cont’d
APPROXIMATE AGE CONDITION
Preschool (3–5 Years) Avascular necrosis of the proximal femur
Contusion
Discoid meniscus
Epiphyseal injuries
Ewing’s sarcoma
Fracture
Hemophilia
Henoch-Schönlein purpura
Juvenile rheumatoid arthritis
Leukemia
Muscle strain
Myositis ossificans
Neuroblastoma (metastasis)
Osteogenesis imperfecta
Osteomyelitis
Patellar subluxation
Popliteal cyst
Pyogenic arthritis
Septic hip
Sickle cell
Stress fracture
Transient synovitis
Venous malformation
Elementary School (6–11 Years) Avascular necrosis of the proximal femur
Bursitis
Congenital dislocated patella
Contusion
Discoid meniscus
Epiphyseal injuries
Ewing’s sarcoma
Hemophilia
Henoch-Schönlein purpura
Juvenile rheumatoid arthritis
Ligamentous sprain/tear
Muscle strain
Osgood-Schlatter disease
Osteochondritis desiccans
Osteomyelitis
Popliteal cyst
Rheumatic fever
Septic hip
Sickle cell anemia
Sinding-Larsen-Johannson disease
Slipped capital femoral epiphysis
Stress fracture
Synovitis
Tendonitis
Thrombus
Transient synovitis
Venous malformation
(continued)
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938 Chapter 47 Knee Pain in a Child


KNEE PAIN IN A CHILD

Common Ages at Which Knee Pain Presents in a Child—cont’d


APPROXIMATE AGE CONDITION
Middle School (12–14 Years) Ankylosing spondylitis
Bursitis
Contusion
Conversion syndrome
Discoid meniscus
Embolism (venous or arterial)
Epiphyseal injuries
Ewing’s sarcoma
Fat pad impingement
Fracture
Gout
Hemophilia
Henoch-Schönlein purpura
Juvenile rheumatoid arthritis
Ligamentous sprain/tear
Lyme disease
Meniscus tear
Muscle strain
Myositis ossificans
Osgood-Schlatter disease
Osteoblastoma
Osteochondritis desiccans
Osteoid osteoma
Osteomyelitis
Osteosarcoma
Patellar dislocation
Popliteal cyst
Rheumatic fever
Saphenous nerve entrapments
Septic hip
Sickle cell anemia
Sinding-Larsen-Johansson disease
Slipped capital femoral epiphysis
Stress fracture
Supracondylar cortical avulsion
Systemic lupus erythematosus
Tendonitis
Thrombus
Venous malformation
High school (15–18 Years) Ankylosing spondylitis
Bursitis
Contusion
Conversion syndrome
Epiphyseal injuries
Ewing’s sarcoma
Fat pad impingement
Hemophilia
Juvenile rheumatoid arthritis
Ligamentous sprain/tear
Lyme disease
Meniscus tear
Muscle strain
Myositis ossificans
Osgood-Schlatter disease
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Chapter 47 Ankylosing Spondylitis 939

KNEE PAIN IN A CHILD


Common Ages at Which Knee Pain Presents in a Child—cont’d
APPROXIMATE AGE CONDITION
Osteoblastoma
Osteochondritis desiccans
Osteoid osteoma
Osteomyelitis
Osteosarcoma
Patellar dislocation
Rheumatic fever
Saphenous nerve entrapment
Sickle cell anemia
Slipped capital femoral epiphysis
Stress fracture
Supracondylar cortical avulsion
Synovitis
Systemic lupus erythematosus
Tendonitis
Thrombus

Overview of Knee Pain in a Child painful treatments, such as an injection in


young children.
In 1998, de Inocencio analyzed 1,000 consecu- When the cause of the knee pain is trau-
tive pediatric clinic visits made by children at matic, there would likely be a known mecha-
least 3 years of age and less than 15 years of nism of injury and the patient has typically
age.1 The study found that 61 of those 1,000 sought intervention from a medical doctor. It is
visits were related to musculoskeletal pain. not uncommon, however, for a physical thera-
Furthermore, the study found that the greatest pist to receive a diagnosis from a physician that
number of these reports (33%) were due to is general, such as “knee pain,” and requires fur-
knee pain. In the physical therapy setting, the ther evaluation. Many of these diagnoses need
patients present with the primary complaint of to be confirmed by radiographic evidence or
musculoskeletal dysfunction, and reports due other special tests that are outside of the scope
to knee pain are common. This makes differ- of physical therapy, and if suspected should be
ential diagnosis of the pediatric knee of critical referred back to a physician. Another fact to be
importance. aware of is that prepubescent or skeletally im-
Knee pain in children can be difficult to mature children who participate in sports are
diagnose. An infant or a young child cannot more prone to injury involving the growth
often describe, quantify, or localize the pain plates of the tibia and femur.
he or she feels. The clinician must be skilled
in observation and palpation of the child, as Description of Conditions That
well as adept at interviewing the child’s par- May Lead to Knee Pain in a Child
ent(s). Physiological pain signs are important
to recognize in nonverbal children. Often Remote
parents will cite a change in the child’s gait, or
a “limp,” as their chief complaint. Parents ■ Ankylosing Spondylitis
may note that their infant or toddler is refus- Chief Clinical Characteristics
ing to move a leg or bear weight. Parents may This presentation includes dull and diffuse pain
also be concerned that the infant is delayed in or stiffness in the lumbar spine and buttock
walking. Children often will not mention region that persists for several months. The pain
pain for various reasons such as difficulty and stiffness is usually described as worse in
describing it, or fear of losing the ability to the morning and at night and can be allevi-
participate in play, sports, and/or fear of ated by a warm shower and light activity.
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940 Chapter 47 Avascular Necrosis of the Femoral Head (Legg-Calvé-Perthes Disease)

Background Information behaviors.4 The child may also appear indiffer-


KNEE PAIN IN A CHILD

Symptoms can begin in a peripheral joint ent to the symptoms described.


such as the hip or knee caused by an inflam-
Background Information
mation of the site of bony attachment of ten-
This psychosomatic pain condition is usually
dons and ligaments in the joint. Chronic
related to secondary gain for the child with
inflammation in the vertebral joints can lead
family and/or friends. It is also likely to help
to bony overgrowth with fusion and immobi-
him or her deal with the environment. It may
lization of these joints. Inflammation of
be related to stress in the child’s life as well.
the eye can accompany this diagnosis. This
This syndrome may assist the child in dealing
condition is often referred to as “bamboo
with stress in his or her life and thus decrease
spine” because of the characteristic appear-
anxiety.5 In a study by Fritz and colleagues, at
ance on x-ray. Physical therapy intervention is
least two of the following factors must be
directed at impairments such as pain, limited
present in a child for the diagnosis of conver-
range of motion, weakness, and gait devia-
sion syndrome:
tions. Gentle aerobic activity or aquatherapy
may also be indicated. 1. History of symptoms varied significantly in
the telling.
■ Avascular Necrosis of the Femoral 2. Pain was out of proportion to exam findings.
Head (Legg-Calvé-Perthes 3. Only vague adjectives used to describe the
Disease) pain.
Chief Clinical Characteristics 4. Evidence of emotional difficulties in other
This presentation typically includes pain of an areas.6
insidious onset that radiates to the anterome-
Children suspected of having this condi-
dial aspect of the knee. Often a Trendelenburg
tion should be referred to a psychiatrist or
limp is the first sign with no known history of
other mental health diagnostician for addi-
injury. Common reports also include muscle
tional evaluation and management.
stiffness that may increase with activity. Clin-
ical findings include mild limitation of hip range ■ Epidural Abscess
of motion, especially extension, abduction, and
Chief Clinical Characteristics
internal rotation.
The child presents with motor and/or sensory
Background Information signs and symptoms in a dermatomal/
This condition most commonly presents in myotomal distribution. Orbital inflammation
boys ages 5 to 7 years, though it can range with forehead edema and headache are clini-
from 2 years old to adolescence. It is a self- cal signs of an infection in the cranium.
limiting disease characterized by avascular
necrosis of the femoral head. Revasculariza- Background Information
tion occurs almost invariably without treat- This type of abscess is an infection of the
ment. Complications such as femoral head epidural space that can encroach on the spinal
deformation have been identified.2,3 Physical cord and compromise the vascular supply of
therapy intervention is directed at impair- the region. Epidural abscess can be a complica-
ments such as pain, limited range of motion, tion of severe or chronic sinus infections in
weakness, and gait deviations. children. Medical management includes an-
tibiotic therapy and surgical drainage. Physical
■ Conversion Syndrome therapy management is important for any
Chief Clinical Characteristics resultant impairments.7
This presentation consists of knee pain in which LUMBAR RADICULOPATHIES
history and examination data are inconsistent
with any predictable pattern of underlying ■ L4 Radiculopathy
anatomy and physiology. Adolescents suspected Chief Clinical Characteristics
of having this condition are often emotionally This presentation can be characterized by
labile, dramatic, sexually provocative, and ego- pain in the lumbar spine and paresthesias
centric, and demonstrate attention-seeking radiating from the anterior aspect of the hip,
1528_Ch47_933-958 07/05/12 2:15 PM Page 941

Chapter 47 Somatization (Psychogenic Rheumatism) 941

thigh, and knee, sometimes ending antero- ■ Septic Hip

KNEE PAIN IN A CHILD


medially from the knee to the foot. Depend- Chief Clinical Characteristics
ing on the severity, this presentation may also This presentation includes painful, restricted
include a decreased or absent patellar ten- active and passive motion of the hip, but may
don reflex and motor loss in the muscles present as anteromedial knee pain. Difficulty
innervated by the L4 nerve. Prone knee bend with weight bearing and a limp with gait are
may reproduce symptoms. common. Typically this condition is accompa-
Background Information nied by fever.8
A lumbar disk herniation is the most com-
Background Information
mon cause for this condition. The diagnosis
Such a problem requires immediate referral
is confirmed with magnetic resonance imag-
to an orthopedic surgeon. This may also be
ing. Surgical intervention may be indicated
referred to as septic arthritis or infectious
in severe cases of lower extremity pain
arthritis. Surgical or medical intervention is
accompanied by neurological signs.
typically indicated. Following medical man
■ L5 Radiculopathy agement, physical therapy intervention is
directed at impairments such as pain, limited
Chief Clinical Characteristics range of motion, weakness, and gait deviations.
This presentation includes pain in the lum-
bar spine and paresthesias radiating from the ■ Slipped Capital Femoral
lateral aspect of the hip and buttock to the lat- Epiphysis
eral aspect of the knee, extending anterolat-
Chief Clinical Characteristics
erally down to the foot. Depending on the
This presentation involves hip, knee, and
severity, this presentation may also include
medial thigh pain that occurs insidiously or
motor loss in the muscles innervated by the L5
suddenly, and can be unilateral or bilateral.
nerve root.
Clinical signs include a slight limp with foot in
Background Information external rotation and limited hip abduction,
A lumbar disk herniation is the most com- internal rotation, and flexion. There may or
mon cause for this condition. The diagnosis may not be a history of trauma. Pain is aggra-
is confirmed with magnetic resonance imag- vated with activity and is alleviated with rest.
ing. Surgical intervention may be indicated
Background Information
in severe cases of lower extremity pain
This condition most commonly occurs
accompanied by neurological signs.
between the ages of 10 and 15 years with a
■ S1 Radiculopathy greater incidence in obese male children. This
condition requires immediate surgical atten-
Chief Clinical Characteristics tion.9,10 The physical therapy intervention that
This presentation typically includes pain in the follows is directed at impairments such as
lumbar spine and paresthesias radiating from pain, limited range of motion, weakness, and
the buttock to the posterior aspect of the knee gait deviations.
and extending posterolaterally from the knee
to the foot. Depending on the severity, this ■ Somatization (Psychogenic
presentation may also include a decreased or Rheumatism)
absent Achilles tendon reflex and motor loss
in the muscles innervated by the S1 nerve. Chief Clinical Characteristics
This presentation consists of history and
Background Information examination data that are inconsistent with
A lumbar disk herniation is a common cause anatomy and physiology. Adolescents suspected
for this condition. The diagnosis is con- of having this condition are often emotionally
firmed with magnetic resonance imaging. labile, dramatic, sexually provocative, and ego-
Surgical intervention may be indicated in centric and demonstrate attention-seeking
severe cases of lower extremity pain accom- behaviors.4 The child may also appear indiffer-
panied by neurological signs. ent to the symptoms described.
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942 Chapter 47 Bipartite Patella

Background Information but is classified based on ages: infantile (0 to


KNEE PAIN IN A CHILD

In contrast to conversion syndrome, children 3 years), juvenile (4 to 10 years), and adoles-


with this condition may demonstrate symp- cent (11 years and older). The infantile form is
toms of psychosis and severe mental illness the most common and is seen more in females,
such as hallucinations, strange symptom early walkers (<1 year), and African Americans,
explanations, and an inability to relate to his or and it presents with bilateral involvement in
her peers.4 80% of cases and a prominent medial meta-
physeal “beak.”12,13 The late-onset groups also
Local show obesity, and African American predomi-
nance, but in contrast to the infantile form,
■ Bipartite Patella males are affected more frequently and only
Chief Clinical Characteristics 50% have bilateral involvement.13 Treatment
This presentation involves tenderness at the in the infantile group begins with nonsurgical
superior pole of the patella. Children suspected bracing utilizing knee-ankle-foot orthoses.
of having this condition may report activity- Treatment in the late-onset groups is surgical
related aching.11 There may be a palpable ridge with a proximal tibial valgus osteotomy and a
on the patella. Pain is felt on the patella and range fibular diaphyseal osteotomy.
of motion may be uncomfortable.
BURSITIS
Background Information ■ Infrapatellar
This condition is seen most commonly in ado-
lescents, and boys are affected more often than Chief Clinical Characteristics
girls. It is usually found incidentally in very ac- This presentation commonly includes local-
tive children. History of a single event causing ized edema over the inferior aspect of the
the pain is not usual as there is often a minor patellofemoral joint and pain in the infrap-
trauma to the area, which then reveals the atellar region with palpation.
bipartite patella. It occurs when the secondary Background Information
ossification centers do not fuse to the primary With malalignment of the extensor mecha-
ossification centers, causing bipartite patella. nism or irritation to the infrapatellar fat pad,
Treatment is nonsurgical with modification of the infrapatellar bursa may become enlarged,
activity, aspirin, and splinting if necessary.11 inflamed, and painful. Pain is often associ-
ated with hyperextension or extension
■ Blount’s Disease overpressure. Treatment includes rest and
Chief Clinical Characteristics inflammation control.
This presentation consists of bowing of the lower
extremities in the infantile form. The bowing ■ Pes Anserine (Voshell’s Bursitis)
is asymmetrical, though usually bilateral, and Chief Clinical Characteristics
the child is frequently obese and has internal This presentation includes pain, tenderness,
tibial torsion and leg-length discrepancy. and localized edema at the anteromedial
In-toeing is commonly seen along with the tib- aspect of the knee. Patients may report pain
ial torsion. In children 4 years of age and older, with ascending stairs and tenderness to pal-
the primary complaint is often pain and then pation at the insertion site of the three tendons
bowing of the lower extremity. The child is that comprise the pes anserine group (semi-
frequently obese.12 tendinosus, gracilis, and sartorius). Pain is
typically located approximately 4 cm below the
Background Information joint line at the anteromedial aspect of the
This condition is an abnormal growth of the knee.14
medial proximal tibial epiphysis where it
begins to wedge and cause increased varus. It is Background Information
thought to be due to growth suppression at the Activities that involve repetitive cutting or
medial proximal tibia caused by compressive side-to-side stepping may also result in pes
trauma. It is the most common pathology that anserine bursitis. Chronic bursitis has been
causes tibial varus.12 It can occur at any age, associated with degenerative joint disease of
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Chapter 47 Discoid Meniscus 943

the knee or rheumatoid arthritis.15,16 Treat- usually results in a rotary subluxation of the

KNEE PAIN IN A CHILD


ment includes rest, control of inflammation, tibia on the femur. The child will also likely
orthotic therapy, and strengthening to have a positive apprehension test. This condi-
reduce stress on the medial structures of the tion requires surgical correction.
knee.
■ Contusion (Bone/Muscle)
■ Prepatellar Chief Clinical Characteristics
Chief Clinical Characteristics This presentation typically involves swelling,
This presentation is characterized by super- pain, and limited joint range of motion near the
ficial edema and diffuse pain over the ante- injury. Bruising is noted and pain and stiffness
rior aspect of the knee with palpation. with muscle activation can also occur.
Background Information Background Information
The mechanism of injury typically involves Contusions occur from direct or repeated
repeated minor trauma or kneeling, inciting blows from a blunt object that damage the
inflammation of the subcutaneous bursa muscle fibers or bone without breaking the
over the patella. Treatment includes avoid- skin. Bone marrow contusions can frequently
ance of kneeling and inflammation control. be identified on magnetic resonance imaging
Inflamed bursae need to be monitored for and result from a direct blow to the bone,
infection. compressive forces from adjacent bones, or
traction forces as in an avulsion injury. Com-
■ Complex Regional Pain Syndrome mon contusions in the knee joint include
(Reflex Sympathetic Dystrophy) dashboard injuries, pivot shift injury (valgus
Chief Clinical Characteristics load applied to the knee in flexion combined
This presentation typically includes pain that with external rotation of the tibia or internal
is disproportionate to any causative or trau- rotation of the femur), hyperextension injury,
matic event (allodynia) and excessive sensitiv- and lateral patellar dislocation.18 Physical ther-
ity (hyperalgesia), edema, skin temperature apy intervention is directed at impairments
changes, trophic changes, and impaired motor such as pain, limited range of motion, weak-
function. ness, and gait deviations.
Background Information ■ Discoid Meniscus
Diagnosis is by exclusion of other conditions.
In children, complex regional pain syndrome Chief Clinical Characteristics
is seen in the adolescent population. This con- This presentation typically includes symptoms
dition is usually treated nonsurgically with de- indicative of a torn meniscus: dull, aching
sensitization and restoration of normal use of pain, edema, and locking.19 It may cause a
the lower extremity. palpable and audible click and tenderness over
the joint line on the side of meniscus. “Snap-
■ Congenital Dislocated Patella ping” at the knee is also common. Many chil-
Chief Clinical Characteristics dren are asymptomatic.
This presentation is characterized by quadriceps
Background Information
weakness. Pain may or may not be a signifi-
This condition is an abnormally shaped
cant feature. The child will often present with
meniscus that is thicker and rounder and
a knee flexion contracture and/or a patella that
covers more of the tibial plateau than does
is held laterally.17
the normal semilunar-shaped meniscus. A
Background Information positive McMurray’s test and joint line ten-
Infants may demonstrate a delay in gait.17 This derness help with diagnosis. However, the
condition is commonly found bilaterally. This symptoms of a torn discoid meniscus may
condition is quite distinct from a subluxating not present with the typical mechanism of
or dislocating patella. The patella is firmly injury and, in fact, the child may not have a
fixed laterally, making knee extension difficult. recent history of trauma. The lateral menis-
The lateral pull of the quadriceps mechanism cus is most commonly affected. Treatment
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944 Chapter 47 Erythema Nodosum

usually includes saucerization (debridement) and varus stress of the knee and is frequently
KNEE PAIN IN A CHILD

of the meniscus.19 comorbid with anterior cruciate ligament or


meniscal tears.21 It is typically diagnosed by
■ Erythema Nodosum x-ray examination. Stabilization through
Chief Clinical Characteristics bracing, casting, or surgical intervention is
This presentation involves sudden onset of typically indicated by a physician. Growth
swollen, red, and painful nodules on the anterior disturbances are a potential complication of
aspect of the bilateral lower extremities. The fractures in children. Physical therapy inter-
child may also demonstrate fever and malaise. vention is directed at impairments such as
pain, limited range of motion, weakness, and
Background Information gait deviations.
The etiology is unknown but precipitating fac-
tors include streptococcal infection, tubercu- ■ Epiphyseal Injuries
losis, and drug reactions. The inflammation is Chief Clinical Characteristics
of the fat cells under the skin. The treatment is This presentation can be characterized by local-
medical and attends to the precipitating fac- ized tenderness to palpation over the plate that
tors. This condition usually lasts approxi- is often aggravated by contraction of the hip ad-
mately 2 weeks.13 However, it may take up to ductors. Pain may also be over the posterome-
6 weeks to alleviate. dial aspect of knee proximal to the joint line.
■ Fat Pad Impingement Background Information
Chief Clinical Characteristics These injuries typically occur at the epiphy-
This presentation includes anterior knee pain seal plate of children and adolescents and
that is typically localized near the inferior pole have been classified by type by Salter and
of the patella. It often occurs in the absence of Harris. Type I is a fracture along the plate,
eccentric loading, may be sudden onset follow- type II extends into the metaphysis on one
ing a hyperextension injury, and is aggravated side, type III extends into the epiphysis on
by prolonged standing or stairs and passive one side, and type IV extends through the
overpressure with extension.20 epiphyseal plate into the metaphysis.
Background Information ■ Stress Fracture
This condition may be caused by a direct blow Chief Clinical Characteristics
or can be due to chronic irritation. Pain with This presentation involves pain at and sur-
palpation is primarily along either side of the rounding the fracture site with impaired
patellar tendon and at the inferior pole. Physi- ability to bear weight. Children with this con-
cal therapy intervention may be indicated to dition will demonstrate a limp with attempts
address muscle imbalances and symptoms to weight bear.
such as pain. Taping the superior patella may
also be used to decrease the angle of the infe- Background Information
rior pole of the patella into the fat pad. This condition is frequently seen in the pedi-
atric athletic population, because they are
FRACTURES skeletally immature and susceptible to the
■ Avulsion, Supracondylar stress and strain of high-intensity muscle
(Segond Fracture) activity. This condition is common at the
epiphyseal growth plates of the long bones
Chief Clinical Characteristics
and the shaft of the proximal tibia. Treat-
This presentation typically involves pain with
ment includes rest and splinting.
adduction located at the posterior lateral knee,
proximal to the joint line. ■ Gout
Background Information Chief Clinical Characteristics
This condition is an avulsion of the tibia at This presentation includes acute, recurrent
the insertion of the lateral capsular ligament. episodes of severely painful arthritis with inflam-
It results from excessive internal rotation mation of the joints.
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Chapter 47 Iliotibial Band Friction Syndrome 945

Background Information mobility.23 Medical treatment is necessary and

KNEE PAIN IN A CHILD


This condition is primarily a disease of adults typically includes clotting factor replacement
and is rare in children. Children with Type I therapy. Physical therapy consists of range
glycogen storage disorder can have gouty and strength restoration after a joint bleed.
arthritis, typically during the adolescent years. Family and patient education on joint protec-
If gout occurs in a child, it is almost always sec- tion precautions is imperative to help mini-
ondary to another condition. At least 95% of mize bleeds.
gout happens in postpubertal men. It is a con-
dition of purine metabolism characterized by ■ Henoch-Schönlein Purpura
elevation of levels of uric acid in serum. Surgi- Chief Clinical Characteristics
cal intervention may be indicated including This presentation can be characterized by joint
amputation of the toes, partial resection of swelling, redness, tenderness, pain, periarticu-
tendons or joints, and arthroplasty or lar swelling, and warmth. The child develops a
arthrodesis of symptomatic joints.12,22 skin rash and abdominal pain, in addition to
the arthritic pain. The rash is palpable and pur-
■ Growing Pains puric and is noted on the legs and buttocks;
Chief Clinical Characteristics however, it may occur days to weeks after the first
This presentation typically involves diffuse pain symptoms are noted. According to Hoekelman,
that is deep, intermittent, and poorly localized. the most common feature is the rash, followed
It is most frequently felt bilaterally and at by arthritis.4 The child may also exhibit a low-
night.8,17 The pain is not articular, nor is the joint grade fever, malaise, headache, nosebleeds, and/
tender. or scrotal and scalp swelling.
Background Information Background Information
The child may report a “cramping” sensation. This condition is a hypersensitivity vasculitis
It is theorized to be the result of fatigue of the syndrome, which is an inflammatory process
muscles in the thighs and calves, usually bilat- of the blood vessels. This inflammation can
eral. The theory is that the longitudinal growth cause damage to the parts of the body supplied
of the bones is greater than the longitudinal by the affected vessels. It is often seen after a
muscle-tendon unit growth and this leads to respiratory viral illness. A definitive etiology is
tightness around the joints, causing an over- not known. The knee and the ankle are the two
use-type injury and subsequent pain.5 Grow- most common sites where arthritic pain is
ing pains are most common in 4- to 12-year- experienced. The physician needs to be in-
old children.17 Treatment includes massage volved to monitor renal function and the
and stretching. arthritis. The physical therapist will determine
impairments and limitations once the child is
■ Hemophilia cleared by the physician.
Chief Clinical Characteristics
This presentation typically includes pain in the ■ Iliotibial Band Friction Syndrome
knee with a red, swollen, and warm joint that Chief Clinical Characteristics
has decreased range of motion. This presentation involves localized pain over
the lateral femoral epicondyle that occurs dur-
Background Information ing activity. Symptoms are aggravated by knee
This condition is a genetic condition caused flexion and ambulation, typically alleviated
by a sex-linked recessive gene (seen in boys) with rest and a position of full knee extension.
that results in impaired blood clotting abilities
where the child bleeds into the joint. The knee Background Information
is one of the most frequently affected joints. This condition is an overuse syndrome in
The child may also have deep soft tissue hem- which friction of the iliotibial band causes
orrhages that occur most frequently in the inflammation of the underlying structures.
calf, thigh, iliopsoas, and upper extremities. Physical therapy intervention includes address-
Additionally, muscle weakness and atrophy ing muscle imbalances surrounding the knee
may be present due to the child’s decreased and hip joints.
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946 Chapter 47 Juvenile Rheumatoid Arthritis

■ Juvenile Rheumatoid Arthritis management of the inflammation and its


KNEE PAIN IN A CHILD

Chief Clinical Characteristics symptoms. Treatment includes aerobic activity


This presentation includes fever, abdominal pain, to help decrease the frequency and severity of
and joint swelling and heat with decreased range the joint flares as well as modalities to control
of motion. Local pain presents with a gradual symptoms during a flare. Consistent stretching
onset. Occasionally, a rash may also develop; this and strengthening are beneficial as well as
is evident only in the systemic type of juvenile patient and family education on management
rheumatoid arthritis (JRA). Stiffness is usually techniques and joint protection precautions.
worse in the morning and after rest. In addition LIGAMENT SPRAINS/TEARS
to the above-mentioned signs/symptoms, the
child will also demonstrate muscle atrophy due ■ Anterior Cruciate Ligament
to the chronic inflammation of the joints, caus- Sprain/Rupture
ing immobility and thus loss of muscle. Chief Clinical Characteristics
This presentation includes hypermobility of
Background Information the accessory motions at the knee joints, which
The cause of JRA is unknown. It is thought to is clinically tested with special tests such as the
be autoimmune in response to an unknown anterior drawer test, Lachman test, and oth-
infectious agent with a possible genetic predis- ers. Typically edema is observed and tender-
position. Criteria for diagnosis include greater ness to palpation is present.
than 6 weeks of inflammation of joints (syn-
Background Information
ovial), less than or equal to 16 years of age, and
Patients may note an audible pop or click
a determination of the onset type and number
during the injurious event that is typically a
of joints involved. Traditionally, three types
“plant and twist” or cut-and-pivot type of
were described; now several additional pat-
motion, or sudden deceleration. In children,
terns have been described.23 The three tradi-
the ligaments are usually stronger than the
tional types include pauciarticular, polyarticu-
bone at the physis, so Salter I and II fractures
lar, and systemic. The pauciarticular type
are more likely to occur than ligament tears.
demonstrates involvement in less than or
As children reach their teen-aged years, the
equal to four joints and is the most common
likelihood of ligament tears becomes more
of the three types. The knee joint is the most
like that of adults.25 Nonsurgical manage-
frequently involved in the pauciarticular sub-
ment of this condition in children has not
type.24 Here the findings are usually a swollen
demonstrated good outcomes; hence, surgi-
knee without redness and may be accompa-
cal treatment is recommended. Physical
nied by a small leg-length discrepancy due to
therapy intervention is directed at impair-
overgrowth from the inflammatory stimula-
ments such as pain, limited range of motion,
tion of the physes. The polyarticular type
weakness, and gait deviations.
demonstrates involvement in greater than or
equal to five joints, and is usually symmetric. ■ Lateral Collateral Ligament
Both the pauciarticular and polyarticular Sprain/Rupture
types are seen more frequently in females and
Chief Clinical Characteristics
children who are 1 to 4 years of age at onset.
This presentation involves pain, localized
The systemic type of JRA is characterized by
edema along the lateral aspect of the knee,
spiking fevers that peak in the night and return
and lateral joint line tenderness. Pain and/or
to normal by the morning. This group demon-
laxity is present with varus stress testing at
strates a rash and has fevers one to two times
30 degrees of knee flexion. This injury gener-
daily for greater than/equal to 2 weeks.24 Males
ally results in minimal effusion and pain with
and females are affected equally. Chronic
walking; however, the patient may report dif-
inflammation at the knee joint may lead to an
ficulty with running and cutting activities.
overgrowth of the distal femoral and proximal
tibial physes, resulting in a leg-length differ- Background Information
ence of up to 2 to 3 cm. All patients with this The mechanism of injury is typically from a
condition need to see a physician for medical varus stress applied to the knee, such as a
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Chapter 47 Lateral Meniscus Tear 947

direct blow to the medial aspect of the knee. condition occurs with falls onto a flexed

KNEE PAIN IN A CHILD


During varus stress testing, pain with no knee with the foot in plantarflexion, causing
joint laxity is a Grade I (stretch) injury. Lax- the tibial tubercle to contact the ground first,
ity with a firm end-feel is a Grade II (partial and in motor vehicle accidents resulting
tear) injury, and no firm end-feel is a Grade from contact with the dashboard. Magnetic
III (complete tear) injury.26 Clinical exami- resonance imaging confirms the diagnosis.
nation and magnetic resonance imaging Most tears are treated conservatively, but
confirm the diagnosis. Grade I, II, and III in- may be surgically repaired in an adolescent
juries are managed nonsurgically.26,27 Surgi- who is skeletally mature.
cal repair may be necessary to address asso-
ciated meniscal or combined ligament tears. ■ Lyme Disease (Lyme Arthritis)
Chief Clinical Characteristics
■ Medial Collateral Ligament This presentation may be characterized by an
Sprain/Rupture achiness in the joints, and joint stiffness in
Chief Clinical Characteristics addition to malaise and fever. Children with this
This presentation includes pain, localized condition may have edema, pain, and warmth,
edema along the medial aspect of the knee, and but frequently the child is without erythema
medial joint line tenderness. Pain and/or lax- about the knee.28 The pattern has remissions and
ity is present with valgus stress testing at recurrences. If a rash is present, it typically
30 degrees of knee flexion. This injury gener- shows “central clearing.”
ally results in minimal effusion and pain with
Background Information
walking; however, it also may cause difficulty
This condition involves a history of tick bite
with running and cutting activities.
months or up to 2 years prior to symptoms.
Background Information The tick bite may have demonstrated the
The mechanism of injury involves a valgus circular-type rash around the bite. In compar-
stress applied to the knee, such as a direct ison to children with other causes of arthritis,
blow to the lateral aspect of the knee. The patients with Lyme arthritis had a higher fre-
incidence of medial meniscus tears increases quency of episodic arthritis and initial knee
with increased severity of the sprain because joint arthritis, reported tick bites more fre-
of its attachment to the medial collateral quently, were older, had a lower frequency of
ligament. With valgus stress testing, pain initial arthralgias, and fewer large joints were
with no joint laxity is a Grade I injury involved.29 According to Hoekelman, 85% of
(stretch). Laxity with a firm end-feel is a cases involve the knee and the arthritis lasts
Grade II injury (partial tear), and no firm 1 to 2 weeks.4 Medical treatment is necessary.
end-feel is a Grade III injury (complete Physical therapy treatment is based on the
tear).26 Clinical examination and magnetic child’s impairments and functional limitations
resonance imaging confirm the diagnosis. that result from the disease.

■ Posterior Cruciate Ligament MENISCUS TEARS


Sprain/Rupture ■ Lateral Meniscus Tear
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation typically includes pain, This presentation may involve pain, lateral
edema, and tenderness in the region of the joint line tenderness, and reports of catching,
popliteal fossa. Positive posterior sag sign clicking, and locking. Mild joint line effusion
of the tibia with the hip and knee flexed to is present, and pain or a palpable click may
90 degrees and a positive posterior drawer be provoked with McMurray’s and Apley’s
test may be present. compression tests.
Background Information Background Information
The mechanism of injury is typically from a Mechanism of injury involves an acute, non-
hyperextension force or a direct anterior contact rotatory force with the knee flexed
blow to the knee in a flexed position. This and the foot planted. Meniscal compromise
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948 Chapter 47 Medial Meniscus Tear

leads to increased stress on the articular car- ■ Myositis Ossificans


KNEE PAIN IN A CHILD

tilage and early degenerative changes. Mag- Chief Clinical Characteristics


netic resonance imaging confirms this diag- This presentation involves pain, focal swelling,
nosis.30–32 Tears located in the peripheral and limited joint range of motion, commonly
one-third of the meniscus respond well to occurring in the arm or thigh. In the progres-
surgical intervention. Some researchers sive form, the patient presents with painful
advocate that tears in the middle one-third lumps in the muscle and stiffness in adjacent
zone also be repaired.33,34 joints.
■ Medial Meniscus Tear Background Information
Chief Clinical Characteristics Symptoms progress from proximal to distal.
This presentation can be characterized by This condition is an aberrant reparative
pain, medial joint line tenderness, and process that causes benign heterotopic ossifi-
reports of catching, clicking, and locking. Mild cation of the soft tissue. There are two forms;
joint line effusion is present, and pain or a pal- one can develop in response to a soft tissue
pable click may be provoked with McMurray’s injury or in the absence of injury, the other is
and Apley’s compression tests. a progressive form that is an autosomal domi-
nant genetic condition.
Background Information
Mechanism of injury involves an acute, non-
NERVE ENTRAPMENTS
contact rotatory force with the knee flexed and ■ Common Peroneal Nerve at the
the foot planted. The medial meniscus is more Fibular Head
commonly injured than the lateral meniscus Chief Clinical Characteristics
because it is less mobile.33 This condition This presentation may be characterized by a
often is associated with medial collateral partial or total loss of sensation in the distri-
ligament injuries because of its rigid attach- bution of the peroneal nerve. Weakness with
ment to the ligament and the joint capsule. ankle dorsiflexion and extension of the toes and
Meniscal compromise leads to increased stress a positive Tinel’s sign at the fibular head may
on the articular cartilage and early degenera- also be present.
tive changes. Magnetic resonance imaging
confirms the diagnosis.30–32 Typically, tears Background Information
located in the peripheral one-third of the Pain is an uncommon feature unless it is
meniscus heal well with surgical intervention, related to the specific cause of the nerve
and some researchers also advocate repair of entrapment, such as entrapment secondary
tears in the middle one-third zone.33,34 to soft tissue swelling and inflammation
from direct trauma. Causes include sitting
■ Muscle Strain (eg, Quadriceps, crossed-legged, prolonged immobility in bed
Hamstrings, Adductors) against bedrails or firm mattresses, trauma,
squatting, crouching, kneeling, and idio-
Chief Clinical Characteristics
pathic origins. Dynamic entrapments also
This presentation includes pain or tenderness
may occur during activities such as running.
with localized edema over the muscle belly or
The common peroneal nerve may be injured
tendon that is aggravated by contraction or
at any location along the nerve; however, en-
stretching of the muscle.
trapment most frequently occurs at the fibu-
Background Information lar head. The nerve may become compressed
Children with this condition often cite an under the fibrous arch in the region where
audible “pop” with a tear of the muscle. A the bifurcation of the nerve into its deep and
strain occurs when a muscle or tendon is over- superficial branches occurs.35,36 An electro-
stretched or torn. Physical therapy interven- diagnostic evaluation, including nerve con-
tion such as gentle stretching and strengthen- duction velocity and needle electromyogra-
ing of the surrounding musculature may be phy may confirm the diagnosis. Treatment is
indicated as well as edema management and generally conservative; however, surgical de-
pain control. compression may be indicated in recalcitrant
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Chapter 47 Osteogenesis Imperfecta 949

cases in which the anatomical site of entrap- an increase in pain with activity and a

KNEE PAIN IN A CHILD


ment is well characterized. decrease in pain with rest. Onset is usually
insidious and eventually quad atrophy is
■ Saphenous Nerve present.21
Chief Clinical Characteristics
This presentation involves pain and/or pares- Background Information
thesias in the medial thigh and knee, tender- This condition is necrosis of bone and carti-
ness to palpation over the adductor canal, lage with an unknown cause. Theories include
and normal motor function of the affected abnormal vascular anatomy causing necrosis,
extremity. Symptoms include a deep ache that the failure of an ossification center to fuse,
may radiate into the foot along the saphe- trauma, or constitutional factors.5 The most
nous nerve distribution. Symptoms are exac- common site is the lateral middle-to-posterior
erbated by prolonged walking or standing. portion of the medial femoral condyle, but the
lateral femoral condyle can be affected as well.
Background Information A positive Wilson’s test is indicative of this
Entrapment typically occurs where the saphe- condition. Williams and colleagues advocate
nous nerve pierces the fascia of the adductor non–weight-bearing activities in a knee
canal, resulting in inflammation. Mechanisms immobilizer and range of motion during the
for saphenous nerve entrapment may be trau- nonsurgical management of this process.39
matic, nontraumatic, or iatrogenic (eg, fol- The pain is gone in 6 to 18 months usually
lowing knee surgery or saphenous vein har- with full return to previous activity.40 If a loose
vest). Diagnosis may be confirmed with body is present, surgical resection of the loose
injection of local anesthetic. Symptoms typi- body may be indicated.
cally improve following an injection with a
local anesthetic and steroids and avoiding ag- ■ Osteogenesis Imperfecta
gravating activities. Neurolysis or neurectomy Chief Clinical Characteristics
may be performed if nonsurgical treatment This presentation commonly involves deformi-
fails in recalcitrant cases in which the anatom- ties of the long bones and ligamentous laxity. The
ical site of entrapment is well characterized.37 child may have a history of a few fractures to
many fractures, depending on the severity of
■ Osgood-Schlatter Disease the disease. The child often has short stature, blue
Chief Clinical Characteristics sclerae, and thin dentin.
This presentation includes swelling of the tib-
ial tubercle with tenderness and sometimes Background Information
warmth; the pain may increase with activity. Osteogenesis imperfecta is a genetic condition
and most forms of this condition result from
Background Information a defect in Type I collagen. This defect makes
This condition is caused by stress on the patel- the child’s bones osteoporotic and fragile.
lar ligament at its insertion on the tibia, with This disease is often called brittle bone disease
microfractures of the apophysis of the tibial because the children frequently sustain multi-
tubercle causing pain.17 Children with this con- ple fractures from seemingly mild trauma.
dition often demonstrate a slight limp. Pain can There are four major types of this condition
be elicited with resisted quadriceps contrac- varying from death in the prenatal/perinatal
tion. This condition is more commonly seen in period to mild bone fragility.28 However, in
boys.38 Physical therapy intervention may 2003, Zeitlin and colleagues described three
include stretching and strengthening of the additional types of this condition that are
surrounding musculature, especially the noncollagenous types.41 Hearing may also be
quadriceps and antagonists. impaired due to problems with the bones of
the middle ear. It is critical to rule out child
■ Osteochondritis Desiccans abuse on children who present with multiple
Chief Clinical Characteristics fractures. The multiple deformities and liga-
This presentation involves pain, edema, and a mentous laxity can be a clue for the clinician.
limp in gait. The clinician usually appreciates The current treatment is frequently medical
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950 Chapter 47 Osteomyelitis of the Distal Femur, Proximal Tibia, or Fibula

intravenous treatments along with surgical ■ Patellar Dislocation


KNEE PAIN IN A CHILD

rodding of the lower extremities as well as Chief Clinical Characteristics


physical therapy for pregait and gait once fix- This presentation includes a displaced patella.
ation is stable.41 The direction of displacement is almost always
■ Osteomyelitis of the Distal Femur, lateral. Effusion is usually present.
Proximal Tibia, or Fibula Background Information
Chief Clinical Characteristics Ligamentous laxity is often found in patients
This presentation is acute with extreme bone who have recurrences. Malalignments such
pain and includes a limp and/or refusal to as genu valgum and external tibial torsional
walk on affected leg. Additionally, warmth, deformities can additionally contribute to
edema, and redness will likely be present near disruption of the quadriceps mechanism
the knee joint. An infant may demonstrate and recurrent dislocation. Patella alta with
lack of movement of the involved leg elongation of the patellar tendon can predis-
(pseudoparalysis) and may refuse feeding. A pose an individual to patellar dislocation
fever is usually present. The pain is constant because the normal support within the
and progressively more severe and is aggra- trochlear groove is gone. This condition usu-
vated by movement. ally occurs with a blow to the medial side of
the leg, or more frequently to an indirect
Background Information blow, such as a fall.17 It is the most common
This condition is an inflammation of bone musculoskeletal dislocation in the young ath-
that is primarily due to infection. It is most lete.4 In a study by Neitosvaara and col-
frequently a response to infection of the bone leagues, 9 to 15 years of age was the most
and of the medullary canal that may follow common age group to experience disloca-
trauma to that area or result from remote tions of the patella.43 With knee flexion and
seeding. However, it is most commonly due to extension, recurrent patellar dislocation can
a hematogenous source in children due to slow cause patellar arthritis and other degenerative
blood flow near the metaphysis. Up to 70% of patellofemoral joint changes.22 Additionally,
hematogenous osteomyelitis is found in the fe- fracture incidence after patellar dislocation
mur or tibia.23 A history of recent illness, inoc- was 39%. Treatment consists of rest and
ulations, and so on may indicate an infectious edema/pain control, patellar taping, transcu-
agent that traveled via the blood to the long taneous electrical nerve stimulation, and
bones adjacent to the knee (ie, metaphyseal quadriceps and gluteal strengthening.
region). This condition requires immediate re-
ferral to medical care for antibiotics and likely ■ Patellar Subluxation
surgical excision. Chief Clinical Characteristics
This presentation involves tenderness on the
■ Patella Alta anterior knee at the medial side of the patella
Chief Clinical Characteristics and edema. Children with this condition may
This presentation commonly includes a high- report a “going out” sensation.5 Often, medial
riding and usually small patella. The patella does knee pain is present due to the stretch/tear of
not glide in the patellar groove. medial retinaculum.
Background Information Background Information
This condition is seen most commonly in The patella usually dislocates laterally. The
kids with cerebral palsy due to the continu- positive apprehension test is an indicator of
ous muscle forces on the patella.42 Children this issue. This condition may be associated
with this condition typically demonstrate a with an avulsion fracture of the lateral femoral
crouch position in gait. It can also be found condyle or the medial portion of the patella.
during rapid growth spurts in the adoles- Physical therapy treatment includes rest and
cent population or after trauma.42 Treat- edema/pain control, patellar taping, transcuta-
ment may consist of quadriceps stretching neous electrical nerve stimulation, and quadri-
and bracing. ceps and gluteal strengthening.
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Chapter 47 Popliteal Cyst 951

■ Patellar Tendonitis most often, the patella deviates laterally with

KNEE PAIN IN A CHILD


Chief Clinical Characteristics quadriceps contractions. Patella alta, joint
This presentation may involve anterior knee hypermobility, increased quadriceps angle,
pain with flexion and extension of the knee; it increased femoral anteversion, increased exter-
is often worse at night. Children with this con- nal tibial torsion, abnormal iliotibial band
dition may have swelling, pain with activity, and attachment, genu valgum, and genu recurva-
restricted motion at the knee. The tenderness is tum may all lead to this malalignment.42,44,45
usually at the lower pole of the patella and on Pronated feet are another finding seen with
the patellar tendon.21 patellofemoral pain syndrome. It is most com-
monly seen after an adolescent growth spurt. It
Background Information may also be due to overuse leading to an
This condition is an inflammation of the inflammatory response that causes pain.28
patellar tendon caused by overuse or repetitive Treatment includes rest, elevation, and ice fol-
activities such as jumping or running. It is lowed by restoration of range of motion,
common in the active adolescent age group. strengthening of gluteals and quadriceps,
Treatment includes stretching and strengthen- patellofemoral taping, and orthotics.
ing the surrounding musculature and control-
ling the symptoms of inflammation. ■ Plica Syndrome
Chief Clinical Characteristics
■ Patellofemoral Pain Syndrome
This presentation typically involves anterior or
(Chondromalacia Patella)
anteromedial pain that is intermittent and
Chief Clinical Characteristics often reported with clicking, snapping, catch-
This presentation is such that pain begins ing, or buckling sensations.
insidiously, most commonly in adolescent
females, with feelings of grinding, clicking, pop- Background Information
ping, or buckling. The pain may follow trauma It is typically aggravated by squatting, stair
as well, but this is not the most common presen- climbing, and prolonged sitting and standing.
tation. Pain is anterior and may be described as Tenderness along the inferior and medial
aching around the patella. The pain is usually patella can be elicited, and often hypertrophied
exacerbated by activities requiring flexion and tissue can be palpated. This condition is an
extension of the knee, such as the stairs. The child overuse injury in which inflammation leads to a
may also indicate that he or she feels the pain with hypertrophied synovial membrane in the knee
kneeling, squatting, running, and/or sitting as well. that loses elasticity. The medial plica is a band of
The physical therapist may be able to palpate tissue originating medially at the undersurface
crepitus as the child flexes/extends knee, though of the quadriceps tendon and attaches distally
point pain with palpation is not usually present. to the synovium, covering the infrapatellar fat
Muscle atrophy and patellar hypermobility may pad. Plica syndrome is a rare cause of knee pain,
be seen upon examination. Pain is activity related but more common in adolescents.46,47
and relieved by rest. One does not usually palpate
edema, warmth, or redness with patellofemoral ■ Popliteal Cyst
pain syndrome, and most patients have full range Chief Clinical Characteristics
of motion at the knee. The pain is often felt This presentation involves a cyst behind the
bilaterally. An additional finding may be that the knee between the semimembranosus and the
child has a painful or unstable single limb stance medial head of the gastrocnemius muscle.
on the involved leg and a limp with gait when The swelling is not midline or lateral in position.
symptomatic. The mass transluminates and is not tender. It
does not limit motion. Pain does not typically
Background Information increase with activity.
The patella does not track in the femoral groove
as it should and may be related to increased Background Information
stress and pain. It can be congenital or acquired A popliteal cyst is a form of bursitis. The cyst is
and has been thought to be due to malalign- caused by distention of the gastrocnemius
ment of the patellofemoral mechanism where, and semimembranosus bursa that is fed by the
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952 Chapter 47 Pyogenic Arthritis (Septic Arthritis)

muscle tenosynovium.12 It is thought to be from may also have a rash on his or her upper arms,
KNEE PAIN IN A CHILD

chronic irritation and is seen more in boys thighs, and trunk (though this is not common).
than girls.28 It is often misnamed a Baker cyst;
however, this condition should not be confused Background Information
with the “Baker’s cyst” that is found in adults. This condition is due to an abnormal immune
This condition is a connection of the popliteal response to a streptococcal infection. It causes
mass with the knee joint and follows an effusion an arthritic joint that is painful. This condition
into the joint.5 It is usually self-limiting. may not often show symptoms for 10 to 20 days
after the previous streptococcal infection (strep
■ Pyogenic Arthritis (Septic throat and/or tonsillitis). This condition is seen
Arthritis) most commonly in the 5- to 15-year-old
Chief Clinical Characteristics range.12,23 Medical treatment is required. Physi-
This presentation includes a child who is acutely cal therapy follows to address the child’s
ill with fever, chills, irritability, and malaise. impairments and functional limitations.
Children with this condition will report pain and ■ Sickle Cell Disease
local tenderness over the affected joint. The
child will limp with weight bearing or refuse to Chief Clinical Characteristics
use the leg. Redness, warmth, edema, muscle This presentation commonly includes edema,
spasm, and limited range of motion are also fever, erythema, warmth, and severe pain in
signs of this infection. Additionally, slight flex- the long bones and surrounding muscles that lasts
ion of the knee may be preferred as pain in- 3 to 5 days. There may be mild swelling and lim-
creases with motion. More so than other causes ited range of motion as well as a low-grade
of knee pain, severe pain on attempted movement fever. Initial crisis typically occurs around ages
of the joint is possible. The joint is extremely 3 to 4 years of age, The child is often smaller and
painful, hot, and very red in its presentation.28 weighs less than his or her peers when under
2 years of age. Symptoms may be migratory
Background Information and are recurrent.50
This condition is an infection of the joint
space caused by transient bacteremia, or a Background Information
direct wound. The bacteria then colonizes the This condition contains a subset of vascular
vascular synovium of the knee.17 This infec- conditions that are more prevalent in children
tion can cause destruction of the articular car- of African American descent. Pain due to sickle
tilage and long-term bony growth problems. cell disease is caused by excessive clustering
The knee is the second-most common joint of the sickled red blood cells, which lead to
involved, just after the hip.48 This condition bone infarcts and a secondary inflammatory
requires immediate referral to medical care for response. It can also cause avascular necrosis,
antibiotics and surgical drainage. According to septic and reactive arthritis, and leg ulcers, all
Hamer, urgent surgical draining is critical.49 of which can cause pain. Roger found the knee
to be the most common site of bony involve-
■ Rheumatic Fever ment in sickle cell anemia, whereas Hanissian
Chief Clinical Characteristics and Silverman showed the elbow to be the
This presentation may be characterized by an most common site, and the knee the second
acute onset of a very painful knee joint that most common.50,51 Treatment is multifactor-
may not be edematous. The pain usually becomes ial, though the pain crises are treated with flu-
migratory after 2 to 3 days, and responds well ids, pain medication, and oxygen. Medical
to aspirin. Joint involvement is sequential and management is required and cardiorespiratory
this fact, along with the extreme pain that challenges should be avoided.47,52
appears out of proportion to what is found
physically, can help differentiate rheumatic ■ Sinding-Larsen-Johansson
fever from other conditions.28 Finberg notes Disease
that the pain is so great that at times the child Chief Clinical Characteristics
may not be able to tolerate a sheet or blanket cov- This presentation involves tenderness at the
ering the knee.23 Children with this condition inferior pole of the patella, peripatellar
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Chapter 47 Ewing’s Sarcoma 953

swelling, and pain that increases with activity- have used vascular access devices. Greenway

KNEE PAIN IN A CHILD


related pain. and colleagues indicated that 90% of all neona-
tal thromboses were seen in neonates that had
Background Information
such devices.53 If the child is in the age range of
This condition is common in the pediatric/
10 to 17 years, another risk has been noted in
adolescent athletic population. It occurs due to
the literature. In these ages, girls are affected
microfractures at the inferior pole of the
2:1 over boys. Nguyen et al. studied 10 children
patella at the patellar tendon.17 Often the
diagnosed in this age range and found 7/10
quadriceps muscle is shortened and weak.
females were taking oral contraception.54 The
Radiographic evidence may include fragmen-
deep venous thromboses were most commonly
tation of the distal patellar pole.38
found on the left iliofemoral vein (11/15).54,55
■ Systemic Lupus Erythematosus Treatment for this condition is medical, involv-
ing the administration of anticoagulant
Chief Clinical Characteristics
medications.
This presentation typically is characterized by
transient and migratory pain, redness, swelling, ■ Transient Synovitis
and stiffness, as well as a malar (butterfly)
Chief Clinical Characteristics
rash and symmetric joint inflammation.
This presentation can involve unilateral hip,
Fever, weight loss, and decreased energy are
groin, medial thigh, or anteromedial knee
also reported. Joint movements are more
pain. Clinically, the child is able to move the
painful, but have an intermittent pattern
joint, but stiffness is present. Often the com-
as compared to children with juvenile
plaint of pain follows a recent respiratory
rheumatoid arthritis.28 Additionally, edema
infection.
is slight compared to the pain the children
experience.28 Background Information
Background Information The condition causes arthralgia and arthritis
The cause is unknown. Theories include an au- secondary to transient inflammation of the
toimmune disease process and/or B-lymphocyte synovium of the hip joint that typically
hyperactivity with genetic and environmental resolves. It is the most common cause of acute
relationships. This condition may recur with hip pain in children ages 3 to 10 years and it
exacerbations.23 Girls are five times more likely occurs twice as often in boys than girls.8 Phys-
than boys to have the disease, and children of ical therapy intervention is directed at impair-
African American descent are more frequently ments such as pain, limited range of motion,
affected than Caucasian children. Children weakness, and gait deviations.
suspected of having this condition must be TUMORS
evaluated by their physician for treatment to
■ Ewing’s Sarcoma
control organ and joint inflammation. Physical
therapy focuses on the patient’s impairments Chief Clinical Characteristics
and limitations. This presentation typically includes an
insidious onset with night and rest pain.
■ Thrombus (Venous or Arterial) Children with this condition may have a
Chief Clinical Characteristics fever. A soft tissue mass is common as is se-
This presentation includes swelling of the leg, vere pain and edema. Erythema and de-
warmth and redness, and pain worse with creased energy are also symptoms noted with
standing or walking. this disease.56
Background Information Background Information
This condition develops typically following This condition is a cancerous lesion that can
prolonged immobility, trauma to the area, and start anywhere in the body but develops in the
most frequently after a vascular access device bone. It tends to locate in weight-bearing
was used. A clot in the vessel obstructs blood bones and typically affects boys in the teenage
flow and causes pain. This condition is rare in years. It is found most frequently in the
children. Most cases are found in neonates who femur, then the tibia, then the humerus, then
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954 Chapter 47 Ganglion Cysts

the fibula (diaphysis of long bones).56 It is the ■ Lymphoma


KNEE PAIN IN A CHILD

second most common primary malignant Chief Clinical Characteristics


bone tumor seen in children.28 Treatment This presentation at the knee can involve
includes surgical resection and chemother- pain caused by the lymph nodes in the
apy; with this regimen, survival rates are now popliteal fossa or inguinal area, which causes
65% to 70% with localized disease.57 Physical knee pain. However, the most common pres-
therapy focuses on the child’s postoperative entation is cervical or supraclavicular lym-
impairments. phadenopathy that is progressive. Additional
findings include fever, night sweats, and
■ Ganglion Cysts weight loss.
Chief Clinical Characteristics
Background Information
This presentation involves a nonpainful or
This condition is a cancer of the lymph sys-
minimally painful soft tissue mass.
tem and is present in boys 3:1 over girls.12
Background Information Medical management is necessary for
Patients may report pain, clicking, or lock- chemotherapy and radiation.
ing with range of motion. This condition
is frequently found at the insertion sites of ■ Neuroblastoma
ligaments, near the region of the epiphysis. Chief Clinical Characteristics
Intra-articular cysts typically present with This presentation can involve pain; infants
greater losses of range of motion compared present with refusal to walk, irritability, and
to intraosseous cysts.58 Ganglion cysts are limping in ambulatory children.13
more common in women and 70% occur Background Information
in people between the ages of 20 and At the knee, this condition is a malignant,
40 years, but they are not frequently metastatic tumor that consists mainly of
located in the knee. Treatment typically immature nerve cells. It is most common in
involves aspiration or surgical excision; children under the age of 4 to 5 years.
however, most cysts do not require any
intervention. ■ Osteoblastoma
■ Leukemia Chief Clinical Characteristics
This presentation typically includes mild pain
Chief Clinical Characteristics that is not well defined. Pain is usually worse
This presentation typically includes fever, at night and may be relieved by aspirin.
weight loss, malaise, fatigue, swollen lymph Osteoblastoma is more common in older male
nodes, and joint swelling. Limping is fre- teens. Pain is progressive though mild.
quently an early sign. The child has diffuse,
nonspecific pain that is asymmetric. Chil- Background Information
dren with this condition appear systemically Treatment is surgical excision if symptomatic.60
ill with fever, pallor, bruises, and excessive
bleeding. ■ Osteochondroma
Chief Clinical Characteristics
Background Information
This presentation can involve a visible and/or
This condition is a cancer of the white blood
palpable firm, bony mass that is typically
cells and is the most common type of child-
painless. Pain can occur in the presence of a
hood cancer. The two major types include
fracture at the lesion site.12 Children with this
acute lymphoblastic leukemia (ALL) and
condition are generally asymptomatic, but a
acute myeloid or (nonlymphoblastic) lym-
decrease in range of motion may present
phoma. Although ALL predominantly affects
depending on the location of the lesion.
children ages 1 to 4 years, the peak age of
incidence is 2 to 3 years.23,59 Medical man- Background Information
agement with chemotherapy is the primary This condition is the most common benign
treatment course at this time. tumor of bone. It originates near the ends of
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Chapter 47 Synovial Chondromatosis 955

long bones and typically grows away from ■ Pigmented Villonodular

KNEE PAIN IN A CHILD


the joint. Growth of these lesions is typically Synovitis
during childhood and ceases with epiphyseal Chief Clinical Characteristics
closure. They typically occur as single lesions, This presentation can involve diffuse, re-
with most occurring at the knee at the ends current edema that is initially pain free,
of the femur and tibia.12 This condition is repeated hemarthrosis in the absence of
typically asymptomatic, and it is often inci- trauma, progressive and insidious onset of
dentally discovered on plain radiographs pain, palpable nodules, and decreased range
obtained for some other reason. Treatment of motion. Symptoms may include locking
generally only involves surgical resection of and catching.
the lesion.
Background Information
■ Osteoid Osteoma This condition is a benign, proliferative
Chief Clinical Characteristics pathology of unknown etiology that affects
This presentation typically includes bone pain synovial tissue. The condition results in var-
that is often worse at night. Pain is frequently ious degrees of villous and/or nodular
relieved with aspirin and does not typically changes in the joints, with the knee being the
increase with activity. The onset is usually most commonly involved.19,63–65 The two
insidious and described as diffuse, aching, or forms of the condition are diffuse and focal.
throbbing. May also see joint effusion and a The diffuse form typically involves large
flexion contracture.61 joints and involves the entire synovial lining,
resulting in destructive changes in the joint.
Background Information In contrast, the focal form involves small
In a study by Torg and colleagues, the major joints, such as the hands and feet, and results
physical finding was quadriceps atrophy.62 in mechanical symptoms like locking and
None of their subjects demonstrated effu- catching. Surgical intervention is the optimal
sion, decreased range of motion, tenderness choice of treatment and the best results are
at the knee, signs of internal derangement, most often observed with the focal form.30
or neurological deficits. Surgical excision For the focal form, resection of the localized
may be performed if symptomatic. synovium often results in success. For the
diffuse form, partial or complete removal of
■ Osteosarcoma (Osteogenic the entire synovial lining is required, how-
Sarcoma) ever, the recurrence rate is high.
Chief Clinical Characteristics
This presentation includes deep pain, especially ■ Synovial Chondromatosis
at night, that is not related to activity. Chief Clinical Characteristics
Children with this condition may have a pal- This presentation can involve a chronic
pable mass that is firm and tender. history of pain, swelling, stiffness, progressive
loss of range of motion, and joint locking.
Background Information Palpable nodules may also be present.
This condition is a cancerous lesion near the
epiphyseal plates of the long bones (eg, distal Background Information
femur, proximal tibia). There is no known This condition is a benign condition of
cause. Pain usually is present for more than unknown etiology that is characterized by
6 weeks. It is a rare disease that is more com- synovial membrane proliferation and meta-
mon in males between the ages of 10 and plasia. Nodular proliferation of the synovial
25 years during rapid growth spurts. This lining occurs and fragments may break off
condition is the most common primary bone into the joint to calcify and grow, resulting in
malignancy in children. Fifty percent are gradual joint degeneration and secondary
located in the distal femur, proximal tibia, or osteoarthritis. Almost any joint can be
metaphysis of fibula.61 Treatment includes affected, however the knee is affected in more
chemotherapy and surgical excision. than half of all reported cases. The condition
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956 Chapter 47 Synovial Sarcoma

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KNEE PAIN IN A CHILD

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iewicz TL. Arthroscopic meniscal repair with fibrin coma: current concepts in diagnosis and treatment.
clot of complete radial tears of the lateral meniscus Curr Opin Pediatr. Feb 2003;15(1):53–57.
in the avascular zone. Arthroscopy. May–Jun 1998;14 57. Marec-Berard P, Philip T. Ewing’s sarcoma: the pediatri-
(4):360–365. cian’s point of view. Pediatr Blood Cancer. May
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neuropathies. Baltimore, MD: Williams and Wilkins; 58. Gunter P, Schwellnus MP. Local corticosteroid injection
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36. Maudsley RH. Fibular tunnel syndrome. In proceedings domised controlled trial. Br J Sports Med. Jun
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letes. Orthop Clin North Am. Jul 2003;34(3):405–415. icopathologic features, diagnosis, and treatment of os-
39. Williams JS Jr, Bush-Joseph CA, Bach BR Jr. Osteochon- teoblastoma. Orthopedics. Feb 1999;22(2):244–247; quiz
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1998;11(4):221–232. 61. Gebhardt MC, Ready JE, Mankin HJ. Tumors about the
40. Sales de Gauzy J, Mansat C, Darodes PH, Cahuzac JP. knee in children. Clin Orthop Relat Res. Jun 1990(255):
Natural course of osteochondritis dissecans in children. 86–110.
J Pediatr Orthop B. Jan 1999;8(1):26–28. 62. Torg JS, Loughran T, Pavlov H, et al. Osteoid osteoma.
41. Zeitlin L, Fassier F, Glorieux FH. Modern approach to Distant, periarticular, and subarticular lesions as a cause
children with osteogenesis imperfecta. J Pediatr Orthop of knee pain. Sports Med. Jul–Aug 1985;2(4):296–304.
B. Mar 2003;12(2):77–87. 63. Handy JR. Popliteal cysts in adults: a review. Semin
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65. Nishihara RM, Helmstedter CS. Chondroblastoma: an 67. Hein KD, Mulliken JB, Kozakewich HP, Upton J, Bur-
KNEE PAIN IN A CHILD

unusual cause of knee pain in the adolescent. J Adolesc rows PE. Venous malformations of skeletal muscle. Plast
Health. Jan 2000;26(1):49–52. Reconstr Surg. Dec 2002;110(7):1625–1635.
66. Mann HA, Goddard NJ, Lee CA, Brown SA. Periarticu- 68. Theruvil B, Kapoor V, Thalava R, Nag HL, Kotwal
lar aneurysm following total knee replacement in he- PP. Vascular malformations in muscles around
mophilic arthropathy. A case report. J Bone Joint Surg the knee presenting as knee pain. Knee. Apr 2004;11
Am. Dec 2003;85-A(12):2437–2440. (2):155–158.
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CHAPTER48
Case Demonstration: Knee Pain and
Limping in a Child
■ Todd E. Davenport, PT, DPT, OCS ■ Sharon K. DeMuth, PT, DPT, MS

NOTE: This case demonstration was developed and friend of yours that you have been treating
using the diagnostic process described in for a while. Sophie’s mother is at the clinic for
Chapter 4 and demonstrated in Chapter 5. The her usual appointment, but she also would like
reader is encouraged to use this diagnostic your quick advice about Sophie. Sophie’s
process in order to ensure thorough clinical mother was unable to schedule an appoint-
reasoning. If additional elaboration is required ment with Sophie’s pediatrician, so no physi-
on the information presented in this chapter, cian referral is available for consultation.
please consult Chapters 4 and 5. Sophie has not been playing outside and her
teachers have told Sophie’s mother that Sophie
THE DIAGNOSTIC PROCESS has preferred to stay inside at her desk at recess
for the past 3 months. Your daughter, who is a
Step 1 Identify the patient’s chief concern.
classmate and friend of Sophie’s, also has
Step 2 Identify barriers to communication.
talked about the fact that Sophie has not been
Step 3 Identify special concerns.
playing as much lately. Sophie’s mother re-
Step 4 Create a symptom timeline and sketch
ports that Sophie has reported pain in her left
the anatomy (if needed).
knee and feeling tired. This keeps her from
Step 5 Create a diagnostic hypothesis list
playing games outside with her friends.
considering all possible forms of remote
Sophie’s mother thought a physical therapist’s
and local pathology that could cause the
advice would be especially useful. Sophie’s
patient’s chief concern.
mother reports she cannot recall Sophie talk-
Step 6 Sort the diagnostic hypothesis list by
ing about specifically injuring her knee, but
epidemiology and specific case characteristics.
Sophie’s mother knows that at Sophie’s age
Step 7 Ask specific questions to rule specific
falls are common on the playground.
conditions or pathological categories less likely.
Step 8 Re-sort the diagnostic hypothesis list STEP #1: Identify the patient’s chief
based on the patient’s responses to specific concern.
questioning. ● Left knee pain and fatigue that precludes
Step 9 Perform tests to differentiate among usual play
the remaining diagnostic hypotheses.
Step 10 Re-sort the diagnostic hypothesis list STEP #2: Identify barriers to
based on the patient’s responses to specific communication.
tests. ● Informal referral. This route of referral of-
Step 11 Decide on a diagnostic impression. ten requires that rapid assessments be made
Step 12 Determine the appropriate patient without the routine documentation. The
disposition. clinician should be careful to take the time
necessary to be thorough and generate
follow-up documentation for the patient
Case Description and patient’s family, clinic, and the pediatri-
cian as needed. Legal issues also may exist
Sophie is a 6-year-old female who presents to given the request to evaluate this patient
your clinic with her mother, a current patient without physician referral.

959
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960 Chapter 48 Case Demonstration: Knee Pain and Limping in a Child

● Patient and patient’s mother are acquain- ● Patient is a child. Obtaining history and
tances. The clinician should be careful to physical examination information from
collect all necessary information in order children requires special consideration.
to arrive at a diagnostic conclusion, since
STEP #3: Identify special concerns.
some information unconsciously might be
considered “too personal” to be collected. ● Knee pain without specific mechanism of
Acquaintance with the patient and her injury. Potentially raises the index of clini-
mother also might bias the clinician away cal suspicion for nontraumatic etiology.
from collecting information that could be STEP #4: Create a symptom timeline and
useful to exclude or confirm the presence of sketch the anatomy (if needed).
pathology that is serious or carries social
stigma.

3 months
ago Today

Onset of knee pain Initial


and associated activity physical
and participation therapy
restrictions evaluation

STEP #5: Create a diagnostic hypothesis STEP #6: Sort the diagnostic hypothesis
list considering all possible forms of list by epidemiology and specific case
remote and local pathology that could characteristics.
cause the patient’s chief concern.
Remote Remote
T Trauma T Trauma
Lumbar radiculopathies: Lumbar radiculopathies:
● L3 radiculopathy ● L3 radiculopathy

● L4 radiculopathy ● L4 radiculopathy

● L5 radiculopathy ● L5 radiculopathy

Slipped capital femoral epiphysis Slipped capital femoral epiphysis (patient age)
I Inflammation I Inflammation
Aseptic Aseptic
Transient synovitis of the hip Transient synovitis of the hip
Septic Septic
Epidural abscess Epidural abscess
Septic hip Septic hip
M Metabolic M Metabolic
Not applicable Not applicable
Va Vascular Va Vascular
Avascular necrosis of the proximal femur Avascular necrosis of the proximal femur
(absence of significant trauma; patient age)
Legg-Calvé-Perthes disease Legg-Calvé-Perthes disease
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Chapter 48 Case Demonstration: Knee Pain and Limping in a Child 961

De Degenerative De Degenerative
Not applicable Not applicable
Tu Tumor Tu Tumor
Not applicable Not applicable
Co Congenital Co Congenital
Not applicable Not applicable
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Not applicable Not applicable
Local Local
T Trauma T Trauma
Blount’s disease Blount’s disease
Contusion Contusion (absence of significant trauma)
Fat pad impingement Fat pad impingement
Foreign body in the knee Foreign body in the knee
Fractures: Fractures:
● Avulsion ● Avulsion (absence of significant trauma)

● Epiphyseal ● Epiphyseal (absence of significant trauma)

● Standard ● Standard (absence of significant trauma)

● Stress ● Stress (absence of significant trauma)

Growing pains Growing pains


Iliotibial band syndrome Iliotibial band syndrome (absence of
significant trauma; not associated with
fatigue)
Ligament sprain/tear Ligament sprain/tear (absence of significant
trauma; not associated with fatigue)
Meniscus tear Meniscus tear (absence of significant
trauma; not associated with fatigue)
Muscle strain Muscle strain (absence of significant
trauma; not associated with fatigue)
Osgood-Schlatter disease Osgood-Schlatter disease (patient age)
Patellar dislocation Patellar dislocation (absence of significant
trauma)
Patellofemoral pain syndrome Patellofemoral pain syndrome (not
associated with fatigue)
Plica syndrome Plica syndrome (not associated with fatigue)
Saphenous nerve entrapment Saphenous nerve entrapment (not
associated with fatigue)
Sinding-Larsen-Johansson disease Sinding-Larsen-Johansson disease (patient
age)
I Inflammation I Inflammation
Aseptic Aseptic
Bursitis: Bursitis:
● Infrapatellar ● Infrapatellar

● Pes anserinus ● Pes anserinus

● Prepatellar ● Prepatellar

Dermatomyositis Dermatomyositis
Erythema nodosum Erythema nodosum (unilateral involvement;
duration of symptoms)
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962 Chapter 48 Case Demonstration: Knee Pain and Limping in a Child

Juvenile ankylosing spondylitis Juvenile ankylosing spondylitis (patient age


and sex, uncommon initial presentation
in the knee)
Juvenile psoriatic arthritis Juvenile psoriatic arthritis
Juvenile rheumatoid arthritis Juvenile rheumatoid arthritis
Juvenile scleroderma Juvenile scleroderma
Patellar tendonitis Patellar tendonitis (not associated with
fatigue)
Rheumatic fever Rheumatic fever (duration of symptoms;
lack of migratory pains)
Systemic lupus erythematosus Systemic lupus erythematosus
Septic Septic
Lyme disease Lyme disease
Osteomyelitis Osteomyelitis
Pyogenic arthritis Pyogenic arthritis
Systemic fungal infection (eg, Systemic fungal infection (eg,
actinomycosis, blastomycosis, actinomycosis, blastomycosis,
coccidiomycosis, histoplasmosis) coccidiomycosis, histoplasmosis)
Tuberculosis Tuberculosis
M Metabolic M Metabolic
Gout Gout (patient age, uncommon first
presentation in the knee)
Myositis ossificans Myositis ossificans (absence of significant
trauma)
Va Vascular Va Vascular
Hemophilia Hemophilia (patient sex)
Henoch-Schönlein purpura Henoch-Schönlein purpura
Osteochondritis desiccans Osteochondritis desiccans (not associated
with fatigue)
Sickle cell anemia Sickle cell anemia (duration of symptoms)
Thrombus (venous or arterial) Thrombus (venous or arterial) (symptom
location)
Vascular malformation Vascular malformation
De Degenerative De Degenerative
Not applicable Not applicable
Tu Tumor Tu Tumor
Malignant Primary, such as: Malignant Primary, such as:
● Ewing’s sarcoma ● Ewing’s sarcoma
● Leukemia ● Leukemia
● Lymphoma ● Lymphoma

● Osteosarcoma ● Osteosarcoma

● Synovial sarcoma ● Synovial sarcoma

Malignant Metastatic, such as: Malignant Metastatic, such as:


● Neuroblastoma ● Neuroblastoma (patient age)

Benign, such as: Benign, such as:


● Ganglion cyst ● Ganglion cyst

● Osteoblastoma ● Osteoblastoma

● Osteochondroma ● Osteochondroma

● Osteoid osteoma ● Osteoid osteoma

● Pigmented villonodular synovitis ● Pigmented villonodular synovitis


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Chapter 48 Case Demonstration: Knee Pain and Limping in a Child 963

● Popliteal cyst ● Popliteal cyst


● Synovial chondromatosis ● Synovial chondromatosis (patient age)
Co Congenital Co Congenital
Bipartite patella Bipartite patella (not associated with
fatigue)
Congenital dislocated patella Congenital dislocated patella
Discoid meniscus Discoid meniscus
Osteogenesis imperfecta Osteogenesis imperfecta (patient age at
presentation; depends on OI type)
Patella alta Patella alta
Patellar subluxation Patellar subluxation
Ne Neurogenic/Psychogenic Ne Neurogenic/Psychogenic
Conversion syndrome Conversion syndrome
Complex regional pain syndrome Complex regional pain syndrome
(absence of significant trauma)
Somatization Somatization

STEP #7: Ask specific questions to rule


Teaching Comment: Sophie provides non-
specific conditions or pathological
verbal answers to the questions about pain
categories less likely.
and sensation. This is common for children
until they become comfortable with the
Teaching Comment: Sophie’s mother examiner; if a child is very shy, this may be all
may be more helpful in providing many as- they are able to do. It is safer to ask similar
pects of the history, but it is important for the questions in different ways to be more certain
clinician to address Sophie as much as possi- of the answers.
ble to solicit her perspectives and gain rap-
port for the physical examination.
Questions for Sophie’s Mother:
Questions for Sophie: ● Has Sophie gained or lost weight recently?
No, making less likely primary and malig-
● Where does it hurt? Sophie rubs her left leg nant metastatic disease.
vaguely between her thigh and her shin an- ● Is Sophie’s pain worse at night? No, making
teriorly. (This shows you that Sophie is will- less likely osteoid osteoma.
ing to provide information but needs help
to be specific enough for your evaluation.) STEP #8: Re-sort the diagnostic
Makes less likely popliteal cyst due to the hypothesis list based on the patient’s
location of the pain since Sophie has indi- responses to specific questioning.
cated anteriorly not posteriorly. Remote
● Can you show me with one finger where it T Trauma
hurts? Sophie points to left knee. (This
Lumbar radiculopathies:
localizes the pain to the left knee.) Makes
● L3 radiculopathy (no numbness and
less likely bilateral conditions.
tingling)
● Does your leg ever feel funny, like it is going ● L4 radiculopathy (no numbness and
to sleep or does it tingle? Sophie shakes her tingling)
head no. Makes less likely spinal pathology. ● L5 radiculopathy (no numbness and tingling)
● Does your leg ever feel numb or dead so
I Inflammation
you can’t feel things that touch the skin of
your legs or feet? Sophie shakes her head no. Aseptic
Makes less likely spinal pathology. Transient synovitis of the hip
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964 Chapter 48 Case Demonstration: Knee Pain and Limping in a Child

Septic De Degenerative
Epidural abscess (no numbness and Not applicable
tingling)
Tu Tumor
Septic hip
Malignant Primary, such as:
M Metabolic
● Ewing’s sarcoma (no weight changes)
Not applicable ● Leukemia (no weight changes)
Va Vascular ● Lymphoma (no weight changes)

● Osteosarcoma (no weight changes)


Legg-Calvé-Perthes disease
● Synovial sarcoma (no weight changes)
De Degenerative
Malignant Metastatic:
Not applicable Not applicable
Tu Tumor Benign, such as:
● Ganglion cyst
Not applicable
● Osteoblastoma
Co Congenital
● Osteochondroma
Not applicable ● Osteoid osteoma (no increase in pain at

Ne Neurogenic/Psychogenic night)
● Pigmented villonodular synovitis
Not applicable
● Popliteal cyst (pain location anterior not
Local
posterior)
T Trauma Co Congenital
Blount’s disease
Congenital dislocated patella
Fat pad impingement
Discoid meniscus
Foreign body in the knee
Patella alta
Growing pains (unilateral pain; no pain at
Patellar subluxation
night)
Ne Neurogenic/Psychogenic
I Inflammation
Conversion syndrome
Aseptic Somatization
Bursitis:
● Infrapatellar STEP #9: Perform tests to differentiate
● Pes anserinus among the remaining diagnostic
● Prepatellar hypotheses.
Dermatomyositis ● Inspection and palpation. The affected knee
Juvenile psoriatic arthritis is edematous with increased surface temper-
Juvenile rheumatoid arthritis ature. Makes less likely benign tumors.
Juvenile scleroderma There are no rashes or skin lesions
Systemic lupus erythematosus present on the lower extremities or face.
Septic Makes less likely dermatomyositis, Henoch-
Lyme disease Schönlein purpura, juvenile psoriatic
Osteomyelitis arthritis, juvenile scleroderma, systemic
Pyogenic arthritis lupus erythematosus.
Systemic fungal infection (eg, actinomycosis, There are no local masses or areas of fo-
blastomycosis, coccidiomycosis, cal tenderness in the knee. Makes less likely
histoplasmosis) bursitis, discoid meniscus, fat pad impinge-
Tuberculosis ment, ganglion cyst, osteochondroma, pig-
M Metabolic mented villonodular synovitis.
The knee and patella have normal align-
Not applicable
ment and tracking. Makes less likely con-
Va Vascular genital dislocated patella, patella alta, patel-
Henoch-Schönlein purpura lar subluxation, and Blount’s disease.
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Chapter 48 Case Demonstration: Knee Pain and Limping in a Child 965

● Log rolling of the hips. Unremarkable I Inflammation


for pain, guarding, and reproduction of Aseptic
pain. Makes less likely transient synovitis Bursitis:
of the hip, septic hip, Legg-Calvé-Perthes ● Infrapatellar (no focal tenderness)
disease. ● Pes anserinus (no focal tenderness)
● Oral temperature. Normal, ruling less ● Prepatellar (no focal tenderness)
likely pyrogenic conditions. Makes less Dermatomyositis (no skin lesions)
likely osteomyelitis, pyogenic arthritis, sys- Juvenile psoriatic arthritis (no skin
temic fungal infections, tuberculosis. lesions)
STEP #10: Re-sort the diagnostic Juvenile rheumatoid arthritis
hypothesis list based on the patient’s Juvenile scleroderma (no skin lesions)
responses to specific tests. Systemic lupus erythematosus (no skin
lesions; no facial rash)
Remote
Septic
T Trauma Lyme disease
Not applicable Osteomyelitis (no fever)
I Inflammation Pyogenic arthritis (no fever)
Aseptic Systemic fungal infection (eg,
Transient synovitis of the hip (no actinomycosis, blastomycosis,
reproduction with hip provocation coccidiomycosis, histoplasmosis) (no
tests) fever)
Tuberculosis (no fever)
Septic
Septic hip (no reproduction with hip M Metabolic
provocation tests; no fever) Not applicable
M Metabolic Va Vascular
Not applicable Henoch-Schönlein purpura (no skin
Va Vascular lesions)
Legg-Calvé-Perthes disease (no De Degenerative
reproduction with hip provocation Not applicable
tests) Tu Tumor
De Degenerative Malignant Primary:
Not applicable Not applicable
Tu Tumor Malignant Metastatic:
Not applicable
Not applicable Benign, such as:
Co Congenital ● Ganglion cyst (no masses; no focal

Not applicable tenderness)


● Osteoblastoma (no masses; no focal
Ne Neurogenic/Psychogenic
tenderness)
Not applicable ● Osteochondroma (no masses; no focal
Local tenderness)
T Trauma ● Pigmented villonodular synovitis (no

Blount’s disease (normal knee alignment) masses; no focal tenderness)


Fat pad impingement (no local masses; no Co Congenital
focal tenderness) Congenital dislocated patella (normal
Foreign body in the knee (no local masses; position)
no focal tenderness) Discoid meniscus (no focal tenderness)
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966 Chapter 48 Case Demonstration: Knee Pain and Limping in a Child

Patella alta (normal position) ● Educate Sophie and Sophie’s mother that
Patellar subluxation (normal position) the appointment should take place urgently.
Ne Neurogenic/Psychogenic
Conversion syndrome (not associated with Case Outcome
objective signs of apparent knee Sophie was referred to a pediatric rheuma-
inflammation) tology clinic where x-rays of her knees con-
Somatization (not associated with objective firmed the absence of a primary malignant
signs of apparent knee inflammation) or benign tumor. A blood test was taken that
STEP #11: Decide on a diagnostic was negative for Lyme disease and positive
impression. for an elevated sedimentation rate. A diagno-
● Rule out pauciarticular juvenile rheumatoid
sis of pauciarticular juvenile rheumatoid
arthritis versus Lyme disease. arthritis was made. Sophie was referred to an
ophthalmologist for a slit-lamp examination
STEP #12: Determine the appropriate to rule out the anterior uveitis that is often
patient disposition. associated with pauciarticular juvenile
● Refer the patient to the pediatrician by tele- rheumatoid arthritis. The slit-lamp examina-
phone for additional evaluation and treatment. tion was negative.
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CHAPTER 49
Shin Pain in a Child
■ Covey J. Lazouras, PT, DPT, NCS

Description of the Symptom ■ Muscle pain that has persisted for longer
than 2 weeks
This chapter describes possible causes of shin ■ Pain with repetitive activities, such as
pain in a child. Local causes are defined as oc- running
curring distal to the knee and proximal to the ■ Any neurological signs suggesting in-
ankle along the anterior portion of the lower creased compartmental pressure (ie, pares-
leg. Remote causes are defined as occurring thesia or weakness of muscles below the
outside this region. knee)
■ Pain, edema, and redness over soft tissue of
Special Concerns the calf or anterior compartment
■ Sudden onset of pain after mechanical in- ■ Any of the above with fever, nausea,
jury particularly with weight bearing malaise, or fatigue
and/or palpation
■ Complaint of bone pain without mechani-
cal injury that has persisted for longer than
2 weeks

CHAPTER PREVIEW: Conditions That May Lead to Shin Pain in a Child

T Trauma
REMOTE LOCAL
COMMON
Not applicable Bone bruise 972
Fracture of the tibia or fibula 974
Muscle contusion 974
Muscle strain/tear 975
Stress fracture of the tibia or fibula 977
Toddler’s fracture of the tibia 977
UNCOMMON
Lumbar radiculopathies: Epiphyseal fractures 973
• L4 radiculopathy 971 Nerve entrapments:
• L5 radiculopathy 971 • Common peroneal nerve 975
• S1 radiculopathy 971 • Superficial peroneal nerve 975
RARE
Not applicable Not applicable
(continued)

967
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968 Chapter 49 Shin Pain in a Child


SHIN PAIN IN A CHILD

I Inflammation
REMOTE LOCAL
COMMON
Not applicable Aseptic
Medial tibial stress syndrome (shin splints) 974
Septic
Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Aseptic Aseptic
Not applicable Not applicable
Septic Septic
Osteomyelitis of the lumbar Cellulitis 972
vertebra 972 Dengue fever (break bone fever) 973
Osteomyelitis of the tibia or fibula 976

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Va Vascular
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Anterior compartment syndrome 972
RARE
Not applicable Sickle cell anemia/crisis 976

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
1528_Ch49_967-981 07/05/12 2:16 PM Page 969

Chapter 49 Shin Pain in a Child 969

Degenerative (continued)

SHIN PAIN IN A CHILD


REMOTE LOCAL
RARE
Not applicable Not applicable

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Malignant Primary, such as:
• Ewing’s sarcoma 978
• Leukemia 978
• Osteosarcoma 979
• Rhabdomyosarcoma 979
Malignant Metastatic, such as:
• Metastatic disease 978
Benign, such as:
• Aneurysmal bone cyst 977
• Osteochondroma 978
• Osteoid osteoma 978
• Unicameral bone cyst 979

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Idiopathic leg pain (growing pains) 974
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.
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970 Chapter 49 Shin Pain in a Child


SHIN PAIN IN A CHILD

Common Ages at Which Shin Pain Presents in a Child


APPROXIMATE AGE CONDITION
Birth to 3 Years Bone bruise
Cellulitis
Dengue fever
Leukemia
Muscle contusion
Osteomyelitis
Sickle cell anemia/crisis
Toddler’s fracture
Preschool (3–5 Years) Bone bruise
Cellulitis
Dengue fever
Idiopathic leg pain (growing pains)
Muscle contusion
Osteomyelitis
Sickle cell anemia/crisis
Toddler’s fracture
Elementary School (6–11 Years) Bone bruise
Cellulitis
Dengue fever
Epiphyseal fractures
Fracture
Idiopathic leg pain (growing pains)
Malignant tumors
Muscle contusion
Osteoid osteoma
Sickle cell anemia/crisis
Unicameral bone cyst
Middle School (12–14 Years) Aneurysmal bone cyst
Bone bruise
Cellulitis
Dengue fever
Epiphyseal fractures
Fracture
Idiopathic leg pain (growing pains)
Malignant tumors
Medial tibial stress syndrome
Muscle contusion
Muscle strain/tear
Osteochondroma
Osteoid osteoma
Sickle cell anemia/crisis
Unicameral bone cyst
High School (15–18 Years) Aneurysmal bone cyst
Anterior compartment syndrome
Bone bruise
Cellulitis
Common peroneal nerve entrapment
Contusion
Fracture
Malignant tumors
Medial tibial stress syndrome
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Chapter 49 S1 Radiculopathy 971

SHIN PAIN IN A CHILD


Common Ages at Which Shin Pain Presents in a Child—cont’d
APPROXIMATE AGE CONDITION
Muscle contusion
Muscle strain/tear
Osteoid osteoma
Sickle cell anemia/crisis
Stress fracture
Superficial peroneal nerve entrapment

Overview of Shin Pain in a Child is confirmed with magnetic resonance imag-


ing. Surgical intervention may be indicated
Shin pain is a common symptom reported in in severe cases of lower extremity pain
the pediatric population. Shin pain can occur accompanied by neurological signs.
as a result of a clearly defined mechanical event
or be a sign and symptom of the normal grow- ■ L5 Radiculopathy
ing process, that is, “growing pains.” The for- Chief Clinical Characteristics
mer usually occurs at night. Shin pain from a This presentation includes pain in the lum-
nonmechanical event should be explored fur- bar spine and paresthesias radiating from the
ther. Remote causes of shin pain are rare but lateral aspect of the hip and buttock to the lat-
the theoretical possibility exists. One must con- eral aspect of the knee, extending anterolat-
sider pathology at L4, such as an epidural erally down to the foot. Depending on the
abscess, osteomyelitis, or a tumor, as a potential severity, the presentation may also include
source of nerve root compression or central motor loss in the muscles innervated by the
canal stenosis at the L4–L5 level. Impingement L5 nerve root.
on the roots of L4–L5 of the lumbar plexus as Background Information
they traverse the pelvis could theoretically be A lumbar disk herniation is the most com-
caused by an intrapelvic tumor or infection. mon cause for this condition. The diagnosis
is confirmed with magnetic resonance imag-
Description of Conditions That ing. Surgical intervention may be indicated
May Lead to Shin Pain in a Child in severe cases of lower extremity pain
accompanied by neurological signs.
Remote
■ S1 Radiculopathy
LUMBAR RADICULOPATHIES
Chief Clinical Characteristics
■ L4 Radiculopathy
This presentation typically includes pain in the
Chief Clinical Characteristics lumbar spine and paresthesias radiating from
This presentation can be characterized by the buttock to the posterior aspect of the knee
pain in the lumbar spine and paresthesias and extending posterolaterally from the knee
radiating from the anterior aspect of the hip, to the foot. Depending on the severity, the
thigh, and knee, sometimes ending antero- presentation may also include a decreased or
medially from the knee to the foot. Depend- absent Achilles tendon reflex and motor loss
ing on the severity, this presentation may also in the muscles innervated by the S1 nerve.
include a decreased or absent patellar ten-
don reflex and motor loss in the muscles Background Information
innervated by the L4 nerve. Prone knee bend A lumbar disk herniation is a common cause
may reproduce symptoms. for this condition. The diagnosis is con-
firmed with magnetic resonance imaging.
Background Information Surgical intervention may be indicated in
A lumbar disk herniation is the most com- severe cases of lower extremity pain accom-
mon cause for this condition. The diagnosis panied by neurological signs.
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972 Chapter 49 Osteomyelitis of the Lumbar Vertebra

■ Osteomyelitis of the Lumbar ■ Bone Bruise


SHIN PAIN IN A CHILD

Vertebra Chief Clinical Characteristics


Chief Clinical Characteristics This presentation includes swelling, skin discol-
This presentation may include an insidious on- oration, and pain to touch and possibly with
set of localized pain over the infected vertebral weight-bearing activity (eg, during peak anterior
body, fever, chills, and malaise.1 tibialis muscle activity during gait). Stretching
or contraction of affected muscle also reproduces
Background Information the symptom of pain. The mechanism of injury
As the infection progresses pain can refer in a is typically blunt trauma via a fall or projectile.
dermatomal pattern and neurological com-
promise may occur.1 Treatment is with antibi- Background Information
otic medications.1 Conventional radiographic techniques are lim-
ited in providing accurate bone marrow charac-
Local terization and therefore the diagnosis of bone
bruising is essentially based on magnetic reso-
■ Anterior Compartment nance imaging findings.4 Radiographs, how-
Syndrome ever, are able to identify a fracture that may have
Chief Clinical Characteristics occurred as a result of trauma. A complication
This presentation includes pain out of pro- of bruising of the shin bone includes compart-
portion to the injury or increasing pain over ment syndrome and the possibility of a frac-
time. ture.4 Treatment typically involves relative rest,
nonsteroidal anti-inflammatory medications,
Background Information
and rehabilitation interventions intended to
Compartment syndrome is due primarily to
reduce pain.
increased intracompartmental pressure. The
mechanism involved in the development of ■ Cellulitis
increased pressure depends on the precipi-
Chief Clinical Characteristics
tating event.2 Two distinct types of compart-
This presentation includes redness of the skin,
ment syndrome have been recognized. The
red streaking of the skin or broad areas of red-
first type is associated trauma to the affected
ness, swelling, warmth, pain or tenderness, and
compartment, as seen in fractures or muscle
drainage or leaking of yellow clear fluid or pus
injuries contained in a compartment of the
from the skin. If the condition spreads to the body
limb. The second type is associated with
via the blood, then fevers and chills can result.
repetitive loading or microtrauma associ-
ated with physical activity. This second type Background Information
is what is described as exertional compart- This condition can occur in almost any part of
ment syndrome. Thus, this syndrome may the body. Most commonly it occurs in areas that
be acute or chronic in nature. The most seri- have been damaged or are inflamed for other
ous complication of a contusion is compart- reasons, such as inflamed lesions, contaminated
ment syndrome. Contused tissue within a cuts or abrasions, foreign objects in the skin,
confined compartment can rapidly reach and areas with poor circulation.5 This condi-
elevated or critical pressure levels.2 Clini- tion has no predilection for any racial or ethnic
cians should have a low threshold for testing group or gender. For the vast majority of cases,
compartments for increased pressure if there is no age-group predilection. Perianal cel-
compartment syndrome is suspected. Surgi- lulitis usually occurs in children younger than
cal intervention to release compartment 3 years. Group B Streptococcus cellulitis occurs
pressure may be indicated because irre- in infants younger than 6 months.6 Pain dispro-
versible damage such as nerve entrapment, portional to the physical examination or severe
vascular compromise, and tissue necrosis pain on passive movement of the extremities
may occur with prolonged elevated pres- requires prompt evaluation by a surgeon.
sures. In general, however, early diagnosis, Uncomplicated forms of this condition have an
with institution of the appropriate treat- excellent prognosis with standard antibiotic
ment, results in a good outcome.3 treatment.6
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Chapter 49 Epiphyseal Fractures 973

■ Dengue Fever (Break Bone Fever) acute phase of injury. Magnetic resonance im-

SHIN PAIN IN A CHILD


Chief Clinical Characteristics aging can depict altered arrest lines and
This presentation includes shin pain, headache, transphyseal bridging abnormalities prior to
retro-orbital pain, myalgia, arthralgia, rash, their being evident on plain radiographs.
and hemorrhagic manifestations. Fever and Background Information
other symptoms may subside after 3 to 4 days. The age of the patient at the time of injury
An individual with this condition may recover is of paramount importance in helping
completely, or the fever may return with a rash predict clinical outcomes because more cor-
within 1 to 3 days. The clinical symptoms are rection, or loss of growth, can be anticipated in
often persisting fatigue and exhaustion, even af- younger patients. Injuries to the physes of
ter the fever has subsided. 14- to 15-year-old girls or 17- to 18-year-old
boys are of little consequence due to their lim-
Background Information ited growth potential.10 As a result, any growth
This condition is an acute benign febrile syn- plate injury is unlikely to be clinically signifi-
drome that occurs in tropical regions. It is cant. However, injuries in younger children
caused by a virus transmitted to humans with full growth potential can cause significant
through the bite of an infected Aedes aegypti problems and a wide range of clinical effects.
mosquito. Most cases in the United States oc- Long-term follow-up is essential to determine
cur in returning travelers, but the incidence of whether or not complications will occur. Most
dengue is increasing along the Texas–Mexico physeal injuries should be reevaluated in the
border and in other parts of the southern short term to ensure maintenance of reduction
United States. A serious complication of this and proper anatomical relationships. After ini-
condition is dengue hemorrhagic fever, which tial fracture healing has occurred, physeal frac-
can result in internal bleeding and death. tures require additional follow-up radiographs
Ninety percent of cases of dengue hemor- 6 months and 12 months following injury to
rhagic fever occur in individuals less than assess for growth disturbance. Management of
15 years of age.7 Course of treatment for such physeal fractures can thus be divided into
dengue fever without complications includes two phases. The first phase involves ensuring
analgesics, fluid replacement, and bed rest.8 bone healing, and the second phase is moni-
toring growth.11 Ligamentous laxity tests of
■ Epiphyseal Fractures the joints of the injured side may elicit pain
Chief Clinical Characteristics and positive findings similar to those indica-
This presentation typically includes complaints tive of joint injury. Do not dismiss positive
of localized joint pain, usually following a trau- joint laxity test findings as only involving the
matic event (eg, fall, collision). Swelling near a related joint. Complete growth retardation or
joint with focal tenderness over the physis is partial growth arrest may result in progressive
usually present. Injuries to the physes are more limb-length discrepancies. Complete growth
likely to occur in an active pediatric population, arrest is uncommon and depends on when the
especially boys, in part due to the greater struc- injury to the physis occurs in relation to the
tural strength and integrity of the ligaments remaining skeletal growth potential.11 The
and joint capsules than of the growth plates. younger the patient, the greater the potential
These binding filamentous structures are two to for problems associated with growth. While
five times stronger than the growth plates at fractures involving the tibia and fibula are the
either end of a long bone and, therefore, are less most common lower extremity pediatric frac-
often injured in children sustaining excessive ex- tures, those involving the proximal tibial epi-
ternal loads to the joints.9 Plain radiographs may physis are among the most uncommon but
depict physeal widening as the only sign of dis- have the highest rate of complications. When
placement. Radiographic stress views (varus displacement occurs, the popliteal artery is
and valgus) may be indicated in certain vulnerable. At the tibial metaphysis, the artery
patients. Magnetic resonance imaging has is just posterior to the popliteus muscle. Com-
proved to be the most accurate evaluation tool plications of these injuries may include vascu-
for the fracture anatomy when performed in the lar insufficiency, peroneal nerve palsy, and
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974 Chapter 49 Fracture of the Tibia or Fibula

anterior physis closure, which may cause sig- Background Information


SHIN PAIN IN A CHILD

nificant genu recurvatum.12 This condition is a common pediatric disorder


with an incidence of about 15%. Two peak
■ Fracture of the Tibia or Fibula incidences exist from 3 to 5 years of age, and
Chief Clinical Characteristics later on, in 8- to 12-year-olds. There is a
This presentation includes pain, swelling, greater incidence in girls.17 Physical examina-
and possible lower leg angulation from either tion is normal without any evidence of
direct trauma or indirect trauma (rotational trauma, joint swelling, erythema, decreased
forces). Fractures of the legs, especially the range of motion, limp, or tenderness. Evalua-
lower legs, often come from simple falls dur- tions, including radiographs and erythrocyte
ing active play. There is an inability to sedimentation rate, are normal.16 Typical
ambulate with tibia fracture; however, treatment involves watchful waiting.
ambulation is possible with isolated fibula
fracture. ■ Medial Tibial Stress Syndrome
(Shin Splints)
Background Information
Fractures of the tibia generally are associated Chief Clinical Characteristics
with fibula fracture, because the force is This presentation includes pain and swelling
transmitted along the interosseous mem- located at the anteromedial aspect of the lower
brane to the fibula. Significant numbers of leg (ie, insertion of the posterior tibialis tendon
these injuries are open, because the skin and in the leg). Overuse is the most common etiol-
subcutaneous tissue are very thin over the ogy including repetitive, sustained exertion or
anterior tibia.13 Open fractures require insufficient recovery time between activities.18
immediate orthopedic referral for irrigation, Background Information
debridement, and possible open reduction Runners running on hard surfaces without
and internal fixation. These variations require proper footwear are predisposed to this condi-
long leg posterior splints. Isolated midshaft tion. Improper stretching and neglecting to
or proximal fibula fractures do not require warm up before exercising also may contribute
immobilization in a long leg cast. A few days to shin injury. Conservative treatment includes
without weight-bearing activity until swelling nonsteroidal anti-inflammatory medications,
resolves, followed by weight-bearing activity splinting, and/or immobilization. A change in
as tolerated, is recommended.14 Prognosis is training routine and/or equipment may be
generally good yet is dependent on degree of indicated.19 Corticosteroid injections are
soft tissue injury and bony comminution.15 considered for individuals with this condition
Complications of this injury may include in whom rest, immobilization, and anti-
neurovascular compromise, compartment inflammatory medications have failed. In gen-
syndrome, peroneal nerve injury, infection, eral, the prognosis is very good with rest and
gangrene, osteomyelitis, delayed union, conservative management. Possibility of full
nonunion, or malunion.14 tendon rupture or stress fracture exists if con-
■ Idiopathic Leg Pain (Growing dition is ignored.3
Pains) ■ Muscle Contusion
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation includes pain late in the day This presentation is characterized by direct
or that awakens a child at night. The pain is not trauma to the muscle group with subsequent
specifically related to joints. Pain is intermittent swelling, tenderness to palpation, and pain with
with symptom-free intervals of days, weeks, or passive and active range of motion resulting
months. The specific etiology of growing pains from bleeding within the muscle.
is unknown and it is often a diagnosis of exclu-
sion.16 The most likely causes are the aches and Background Information
discomforts resulting from the jumping, climb- This condition accounts for one-third of all
ing, and running that active children do dur- sports injuries.20 This condition is caused by
ing the day. blunt trauma to the outer aspect of the muscle,
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Chapter 49 Superficial Peroneal Nerve 975

resulting in tissue and cellular damage and NERVE ENTRAPMENTS

SHIN PAIN IN A CHILD


bleeding deep within the muscle and between ■ Common Peroneal Nerve
the muscle planes. The resultant tissue necro-
sis and hematoma lead to inflammation. A Chief Clinical Characteristics
contusion usually can be distinguished from a This presentation can be characterized by
muscle rupture because residual function exercise-related leg pain with or without
remains after a contusion.20 Depending on the associated dermatomal numbness. Pain is not
size of the lesion, a hematoma also may be universal, and if present, it is often related to
appreciated. A complete examination of the the specific cause of the nerve compromise.25
injured area and surrounding areas must be For example, a nerve compromise secondary
emphasized to identify other possible injuries. to traumatic injury from blunt trauma will
Typical treatment involves relative rest and likely result in pain secondary to soft tissue
rehabilitative interventions. The most serious swelling and inflammation.
complication is compartment syndrome. Signs Background Information
and symptoms of compartment syndrome Traumatic causes can include wounds and
include pain out of proportion to the injury or contusions, direct fractures involving the
increasing pain over time. Contused tissue lateral knee, and direct lacerations.26 This
within a confined compartment can rapidly condition may be caused by ankle sprains
reach elevated or critical pressure levels. Surgi- with associated proximal fibula fractures,
cal intervention should not be necessary unless knee dislocations, or compression from in-
the diagnosis of compartment syndrome is traneural or extraneural tumors. Compres-
considered and confirmed. Heterotopic ossifi- sion of the nerve against the fibrous or fascial
cation may also be a complication of muscle layers of the well-developed muscles of the
contusions.21 legs of athletes has also been seen as a causal
factor.27 Treatment for this condition is typi-
■ Muscle Strain/Tear cally nonsurgical, with surgical intervention
Chief Clinical Characteristics reserved for cases in which the anatomical
This presentation includes swelling, bruising site of entrapment is well characterized.
or redness, muscle spasm, pain at rest, pain
when the specific muscle or the joint in relation ■ Superficial Peroneal Nerve
to that muscle is used, and weakness of the mus- Chief Clinical Characteristics
cle or tendons and inability to use the muscle.22 This presentation includes numbness or pares-
Symptoms can vary, depending on how severe thesia in the distribution of the nerve, and
the strain. occasionally vague pain about the lateral leg
Background Information extending to the dorsum of the foot. The pain
The mechanism of injury is typically caused by can be chronic and present for several years
twisting or pulling a muscle or tendon. This and may be associated with other foot and
condition can be acute or chronic. An acute ankle symptoms, or the pain can be acute and
strain is caused by trauma or an injury such as associated with recent trauma or surgery
a blow to the body; it can also be caused by about the ankle.28 Infrequently, weakness of
improperly lifting heavy objects or overstress- the dorsiflexors and everters of the foot may
ing the muscle tissue.23 Chronic strains are be seen with associated foot drop in more
usually the result of overuse—prolonged, proximal entrapments of the superficial per-
repetitive movement of the muscles and ten- oneal nerve. Typically, symptoms increase
dons. Minor or moderate strain often heals with activity such as running, walking, and
well with home treatment and rehabilitation. squatting.29 Rest or avoiding the precipitat-
Severe strains may require surgery to repair ing activity often relieves the symptoms.
the torn muscles or tendons. Complications of Background Information
an acute severe muscle strain if left untreated Percussion along the superficial course of the
include long-term pain, limited movement, nerve over the proximal fibula, lateral leg, or
and deformity resulting from muscle imbal- the anterior ankle may cause a positive result
ances and chronic compensation.24 on Tinel test, with reproduction of radiating
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976 Chapter 49 Osteomyelitis of the Tibia or Fibula

pain.30 Direct palpation with pressure on the and laboratory data are necessary. Three-phase
SHIN PAIN IN A CHILD

site of entrapment may also induce or exac- technetium radionuclide bone scanning is
erbate symptoms. Repeating the examina- used for imaging studies; however, magnetic
tion after a particular activity that exacer- resonance imaging is increasingly being used
bates symptoms may produce findings not to define bone involvement in patients with a
present on the initial examination at rest. In negative bone scan.34 Blood, bone, and joint
some cases of superficial peroneal nerve aspirate cultures are obtained before any
entrapment associated with direct or indi- antibiotics are given to identify the pathogen.
rect trauma, patients may present with Treatment includes aspiration, antibiotics,
symptoms of complex regional pain syn- immobilization of the affected area, and surgi-
drome, which creates a diagnostic and thera- cal drainage if necessary.33
peutic challenge.31 Although rare, plain
radiographs of the leg may reveal bony ab- ■ Sickle Cell Anemia/Crisis
normalities that may contribute or be the Chief Clinical Characteristics
cause of entrapment. In cases of suspected This presentation involves four patterns of acute
proximal entrapment, knee radiographs may presentation: sudden acute chest pain where
show abnormalities of the proximal fibula, coughing up of blood can occur, abdominal cri-
such as exostoses, osteochondromas, and frac- sis, joint crisis, and bone crisis. Bone crisis is an
ture callus.28 If necessary, computed tomogra- acute or sudden pain in a bone usually in an
phy or sonography can provide more detailed arm or leg. The affected area may be tender to
information on the bony anatomy of the palpation. Common bones involved include the
area, and a sonogram can help localize cystic large bones in the arm or leg: the humerus, tibia,
masses that cause impingement of the nerve. and femur. The same bone may be affected
Surgical decompression may be indicated in repeatedly in future episodes of bone crisis.35
cases when symptoms are not relieved by This presentation includes severe pain, which is
nonoperative options. This can include the most common sign of sickle cell disease emer-
release of the superficial peroneal nerve at gencies (acute sickle cell crises).36 Commonly no
the lateral leg for surgical decompression mechanical event precedes a crisis, but one or more
with partial or full fasciotomy.31 of the following situations may have contributed
to the start of the painful sickle crisis: dehydra-
■ Osteomyelitis of the Tibia tion, infection, fever, hypoxia (decrease in oxy-
or Fibula gen to body tissue), bleeding, cold exposure, drug
Chief Clinical Characteristics and alcohol use, pregnancy, or stress.35
This presentation includes a rapid onset of Background Information
symptoms including general malaise, high fever, This condition is a hereditary condition of the
chills, severe constant pain, swelling, and ten- blood where the red blood cells become dis-
derness over the metaphysis of the involved torted (into a sickle shape) under conditions of
bone. The limb is painful with any movement, hypoxia and clog the vessels, resulting in vascu-
which leads to an appearance of paralysis in lar occlusion. This disease is present mostly in
infants.32 individuals of African descent. It also is found,
Background Information with much less frequency, in eastern Mediter-
Approximately 50% of cases occur in pre- ranean and Middle Eastern populations. The
school-aged children. Preponderance in males male-to-female ratio is 1:1. This disease process
is observed in all age groups. In some cases the is a lifelong condition. It first manifests in the
infection starts in the bony metaphysis and second half of the first year of life and persists
spreads to an adjacent joint, creating a septic for the entire life span.37 Further complicating
arthritis. It is caused by an infection in the this presentation is that children with sickle cell
bone secondary to a minor injury with the disease have weakened immune systems and
most common causative bacterium being are at increased risk for developing secondary
Staphylococcus aureus followed by Streptococ- infection, especially in the lungs, kidneys,
cus pneumoniae and Streptococcus pyogenes.33 bones, and central nervous system. There is a
To confirm the diagnosis, adequate radiologic frequent occurrence of Salmonella osteomyelitis
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Chapter 49 Aneurysmal Bone Cyst 977

in areas of bone weakened by infarction. Tenderness, swelling, warmth, and pain with

SHIN PAIN IN A CHILD


Repeated infarction of joints, bones, and palpation of the lower third of the tibia also may
growth plates leads to aseptic necrosis, espe- be noted.41
cially in weight-bearing areas such as the femur.
Background Information
This complication is associated with chronic
This is a common fracture of toddlers and
pain and disability and may require changes in
preschoolers. The term refers to an undis-
employment and lifestyle.38 A sickle cell crisis
placed oblique or spiral fracture of the tibial
can often be managed efficiently and quickly in
diaphysis with an intact fibula.42 Because the
a hospital’s emergency department with intra-
periosteum in the toddler is sufficiently tough,
venous fluids and pain medicines. A person sus-
the child may be able to walk on the leg, even
pected of having this condition should not
though fractured, albeit with pain and a limp.
delay going to the hospital for emergent med-
Typically there is an acute onset without
ical evaluation and treatment.35–37
known history of trauma. It occurs when the
■ Stress Fracture of the Tibia child is learning to walk, running, or steps on
or Fibula something and loses his or her footing. A sud-
Chief Clinical Characteristics den twisting of the tibia (shin bone), perhaps
This presentation is characterized by insidious from getting the foot caught in a crib slat,
onset of activity-related pain. Pain is repro- causes a fracture in a spiral pattern. The frac-
duced with palpation or percussion of the affected ture may be very subtle on the initial radi-
area. Inspection of the site may reveal localized ograph and may only be recognized on a
swelling and possible erythema. Loading the follow-up film when callus is evident. Treat-
affected bone using specific maneuvers may ment consists of a below-knee walking cast for
reproduce pain.39 Early on, the pain typically is approximately 3 weeks. A long-leg cast is
mild and occurs toward the end of the inciting applied to relieve the symptoms. Healing is
activity. The proximal tibial shaft is the most rapid, within 3 or 4 weeks.43
common site of stress fractures. TUMORS
Background Information ■ Aneurysmal Bone Cyst
Stress fractures are overuse injuries of bone. Chief Clinical Characteristics
Girls have a higher incidence of stress fractures This presentation may include pain of rela-
than boys. Muscle fatigue and structural tively acute onset that rapidly increases in
malalignments may contribute to stress frac- severity over 6 to 12 weeks. Local skin temper-
tures.40 Treatment consists of activity restric- ature may increase and a palpable bony
tion to minimize symptoms (ie, a period of swelling may be present.
non–weight-bearing ambulation may be nec-
essary) before engaging in a program of Background Information
increasingly demanding strengthening and This condition is a benign tumor; therefore
conditioning exercise leading to eventual re- it is not the type of disease that will spread to
turn to play in 8 to 12 weeks.39 Treat cortical other areas. However, a bone cyst can cause
stress fractures of the anterior tibial midshaft destruction of bone and isolated symptoms
with care since they tend to heal more slowly at the site of the bone cyst.44 Compared with
(average of 6 months) and are prone to males, females have an increased incidence.
delayed union or nonunion. In most cases, This condition may occur in patients ages 10
stress fractures can be managed successfully to 30 years, with a peak incidence in those
with conservative measures. High-risk dis- 16 years of age. About 75% of patients are
placed stress fractures, however, require surgi- younger than 20 years. The most common
cal intervention to ensure proper healing. site is the metaphyseal region of the knee;
however, any bone may be affected with the
■ Toddler’s Fracture of the Tibia lower leg being affected in 24% of the
Chief Clinical Characteristics cases.45 The most common problem caused
This presentation may include pain, limping, by this condition is weakening of the bone.
or refusal to bear weight on the affected side. This may lead to increased susceptibility to
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978 Chapter 49 Ewing’s Sarcoma

fracture at that location. Because of this, the ■ Metastatic Disease


SHIN PAIN IN A CHILD

bone cyst may need to be treated in the Chief Clinical Characteristics


operating room to prevent a fracture. Some- This presentation typically involves bone or
times the bone cyst will not be found until soft tissue pain in combination with pallor,
after a fracture has happened. In this case, fatigue, tachycardia, and headache and fever.
treatment of the fracture will also include Pain may be the only local presenting symptom.
treatment of the bone cyst to prevent a
recurrence of this problem.44 Documented Background Information
cases of malignant conversion exist; how- If a mechanical source of injury is not easily
ever, this scenario is rare.46 identified or if the child presents with the
above systemic symptoms, the diagnosis of
■ Ewing’s Sarcoma malignancy of the blood (leukemia), bone
Chief Clinical Characteristics (osteosarcoma), or soft tissue (rhabdomyosar-
This presentation includes pain and local coma) should be considered. Immediate refer-
swelling at the site of the tumor. Constitu- ral to a primary care physician is indicated.
tional symptoms such as fever and malaise may Treatment options range from surgical resec-
be present specifically when metastatic disease tion and reconstruction of host tissues to
is present.47 chemotherapy and radiation treatment.

Background Information ■ Osteochondroma


Ewing’s sarcoma is the second most com- Chief Clinical Characteristics
mon malignant bone tumor in children and This presentation can include pain around the
adolescents. These tumors most commonly area of the bony protrusion and may cause
develop in the axial skeleton, but may arise secondary nerve and tendon inflammation.
in any bone; particularly the diaphyses of the
humerus and femur. Approximately 25% of Background Information
patients with this condition may present This condition involves cartilage-covered
with overt metastasis, typically in the lungs, bony excrescence (exostosis) that arises from
bone marrow, or bone.48 Ewing’s tumors are the surface of a bone. This condition is the
typically sensitive to both chemotherapy and most common bone tumor in children,
radiotherapy; surgical excision is utilized as accounting for 50% of all benign bone
well.49 tumors in children. Its peak incidence is in
the second decade of life with a male-to-
■ Leukemia female ratio of approximately 2:1.52 This
Chief Clinical Characteristics condition can occur at any time from birth
This presentation typically includes diffuse, to the cessation of growth. Complications of
nonspecific bone pain with fever and ele- osteochondromas include fractures, bony
vated white blood cell and sedimentation deformities, neurological and vascular in-
rates. Lesions to the mouth are also often juries, bursa formation, and malignant
seen later.50 transformation.53 The treatment of sympto-
matic osteochondromas is surgical excision.
Background Information Hereditary multiple exostosis is a hereditary
This condition is a cancer of the white blood autosomal dominant disorder where many ex-
cells and is the most common form of child- ostoses are noted. Growth disturbances may
hood cancer.51 Changes on x-ray may show occur as one result of this complication.54
focal lesions of bone resorption seen in the
long bones of the body. Occasionally, chil- ■ Osteoid Osteoma
dren with this condition can have patholog- Chief Clinical Characteristics
ical fractures associated with osteopenia/ This presentation consists of focal bone pain
osteoporosis of the bones, such as the tibia.51 at the site of the tumor. The condition wors-
Treatment consists of the primary interven- ens at night and increases with activity, and
tions of chemotherapy, radiation, and bone it is dramatically relieved with small doses of
marrow transplant. aspirin.
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Chapter 49 Unicameral Bone Cyst 979

Background Information Background Information

SHIN PAIN IN A CHILD


The most common skeletal sites are the Rhabdomyosarcoma is the most common
metaphysis or diaphysis of long bones, which pediatric soft tissue sarcoma, and two-thirds
are affected in 73% of patients. This condi- of affected patients are less than 10 years of
tion more commonly affects males than fe- age.61,63 They can develop anywhere in the
males. The male-to-female ratio is 2:1. The body but are often found in the extremities.
age range in patients is 5 to 56 years. The le- Approximately half the tumors of the limbs
sion initially appears as a small sclerotic are alveolar in nature and are therefore
bone island within a circular lucent defect. highly metastatic.64 Treatment typically in-
The tumors may regress spontaneously. This cludes presurgical neoadjuvant chemother-
condition is classified as cortical, cancellous, apy, surgical excision, and postsurgical
or subperiosteal.55,56 The tumor usually does chemotherapy; radiation therapy is used if
not grow, and it occasionally regresses spon- needed.
taneously or becomes dormant, leaving
residual sclerosis. The tumor has no malig- ■ Unicameral Bone Cyst
nant potential.57 Complications include Chief Clinical Characteristics
pathological fractures secondary to de- This presentation usually includes local pain
creased structural integrity of the affected over one bone in an individual who is skele-
bone.58 Several techniques are available for tally immature. Most individuals with this
ablation of osteoid osteoma. The tumor can condition present with symptoms and signs of
be percutaneously ablated by using radio- a pathological fracture.
frequency, ethanol, laser, or thermocoagula- Background Information
tion therapy under computed tomographic This condition is a common benign fluid-
guidance.59 filled lesion found almost exclusively in chil-
■ Osteosarcoma dren. It occurs most frequently in children
ages 5 to 15 years, with an average age of ap-
Chief Clinical Characteristics proximately 9 years.65,66 Much has been writ-
This presentation may be characterized by ten about the diagnosis and management of
deep achy pain that is usually mechanical in these lesions, and evidence of a variety of
nature (exacerbated by activity of the extrem- successful treatment strategies can be found
ity),47 local swelling, and decreased range of in the literature. In general, treatment may
motion.60 Symptoms are often caused by mi- be summarized as watchful waiting more
crofractures through the involved area, and in than trying to promote natural healing.
severe cases secondary to stretching and com- When such cysts are immediately adjacent to
pression of the surrounding structures.61 a growth plate, they are referred to as active
Background Information cysts; when they have achieved some dis-
Osteosarcoma is the most common primary tance from the growth plate, they are consid-
malignancy of bone in children. It typically ered to be latent cysts. This distinction has
occurs between 10 and 20 years of age, with been used in the past, because it was believed
the peak age incidence during the adolescent to have prognostic significance. Injury to the
growth spurt.62 The onset of symptoms is in- growth plate (physis) may occur secondary
sidious in nature, often related to a minor to direct cyst expansion, pathological frac-
trauma such as a sports-related injury.61 ture, or unintended mechanical disturbance
Treatment of the tumor requires surgical during surgical intervention. Growth arrest
ablation and adjuvant chemotherapy. secondary to unicameral bone cysts is related
to direct cyst expansion across the growth
■ Rhabdomyosarcoma plate and into the epiphysis of the proximal
Chief Clinical Characteristics humerus.67 Growth arrest also has been re-
This presentation includes a mass in an ex- ported following treatment of the cyst either
tremity that may or may not be painful. Pain by local injection of steroids or by curettage
or tenderness may be present if the tumor is and bone grating.68 Growth disturbance
displacing a peripheral nerve. leading to angular deformity or disturbed
1528_Ch49_967-981 07/05/12 2:16 PM Page 980

980 Chapter 49 References

longitudinal growth has been estimated to 19. Kortebein PM, Kaufman KR, Basford JR, Stuart MJ.
SHIN PAIN IN A CHILD

possibly occur in approximately 14% of Medial tibial stress syndrome. Med Sci Sports Exerc.
Mar 2000;32(3 suppl):S27–33.
cases.66 Steroid injection has been a success- 20. Best TM. Soft-tissue injuries and muscle tears. Clin
ful treatment, even in the setting of cyst Sports Med. Jul 1997;16(3):419–434.
extension into the epiphysis.69 The overall 21. Jackson DW, Feagin JA. Quadriceps contusions in
outcome and prognosis is good. The lesion is young athletes. Relation of severity of injury to treat-
ment and prognosis. J Bone Joint Surg Am. Jan 1973;55
believed to resolve spontaneously in most (1):95–105.
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3. Detmer DE, Sharpe K, Sufit RL, Girdley FM. Chronic ropathy: a clinical and electrophysiologic study of 116
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Dis J. Feb 1997;16(2):227–240. ropathy of the superficial peroneal nerve. A bilateral
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43. Halsey MF, Finzel KC, Carrion WV, Haralabatos SS, The diagnostic accuracy of MRI versus CT imaging
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44. Clough JR, Price CH. Aneurysmal bone cyst: pathogen- tures in children. Clin Orthop Relat Res. Mar 2005(432):
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45. Cottalorda J, Kohler R, Sales de Gauzy J, et al. Epidemi- M. Percutaneous CT guided resection of osteoid
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Genet. Jan 2 1995;55(1):43–46. physeal involvement: confirmation with magnetic
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Semin Musculoskelet Radiol. 1997;1(2):281–284. Orthop. Mar 1982;2(1):71–79.
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CHAPTER50
Ankle and Foot Pain in a Child
■ Robert C. Sieh, PT, DPT

Description of the Symptom ■ Skin breakdown, rash, or infection of any


area around the foot
This chapter describes possible causes of ankle ■ Unexplained mass(es)
and foot pain in a child. Local causes are de- ■ Swelling of one limb for longer than
fined as occurring between the distal one-third 24 hours
of the tibia and the toes, inclusive of muscu- ■ Pain that increases with exercise
loskeletal and neurovascular structures that ■ Any numbness, tingling, or other loss of
cross the ankle joint, rearfoot, midfoot, fore- sensation
foot, and toes. Remote causes are defined as ■ Sudden inability to walk or loss of muscle
occurring outside this region. strength

Special Concerns
■ Pain of sudden onset without known cause
■ Pain that continues with rest or elevation of
the feet

CHAPTER PREVIEW: Conditions That May Lead to Ankle and Foot Pain in a
Child

T Trauma
REMOTE LOCAL
COMMON
Not applicable Fractures:
• Calcaneal 993
• Epiphyseal 993
• Pathological 994
• Stress 994
• Talar 994
• Toddler’s fracture of the tibia 994
Ill-fitting shoes 996
Ligament sprains:
• Anterior talofibular ligament 997
• Calcaneofibular ligament 997
• Metatarsophalangeal joint (turf toe) 998
Muscle strain 998
Puncture wounds 1000

982
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Chapter 50 Ankle and Foot Pain in a Child 983

Trauma (continued)

ANKLE AND FOOT PAIN IN A CHILD


REMOTE LOCAL
UNCOMMON
Not applicable Ankle impingement syndromes:
• Anterior 989
• Posterior 990
Apophysitis:
• Achilles tendon insertion (Sever’s disease) 990
• Fifth metatarsal (Iselin’s disease) 990
Nerve entrapments:
• Common peroneal nerve entrapment 998
• Lateral plantar nerve 999
• Tarsal tunnel syndrome 999
RARE
Lumbar radiculopathies: Not applicable
• L5 radiculopathy 988
• S1 radiculopathy 988
Popliteal entrapment syndrome 988

I Inflammation
REMOTE LOCAL
COMMON
Not applicable Aseptic
Bursitis:
• Retrocalcaneal bursitis 991
• Subcutaneous Achilles bursitis 991
Tendinitis:
• Achilles tendinitis 1001
• Flexor hallucis longus tenosynovitis 1001
• Tibialis posterior tendinitis 1002
Septic
Ingrown toenails 996
UNCOMMON
Not applicable Aseptic
Juvenile rheumatoid arthritis 997
Plantar fasciitis 1000
Retrocalcaneal bursitis 1000
Septic
Cellulitis 991
Fungal disease 995
Hand-foot-mouth disease 996
Joint sepsis 996
Osteomyelitis 999
(continued)
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984 Chapter 50 Ankle and Foot Pain in a Child

Inflammation (continued)
ANKLE AND FOOT PAIN IN A CHILD

REMOTE LOCAL
RARE
Not applicable Aseptic
Rheumatic fever 1000
Seronegative spondyloarthropathies 1000
Septic
Necrotizing fasciitis 998
Tuberculosis 1002

M Metabolic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Gout 995
RARE
Not applicable Ehlers-Danlos syndrome 992
Fabry’s disease 992
Gaucher’s disease 995
Myositis ossificans 998

Va Vascular
REMOTE LOCAL
COMMON
Not applicable Hemophilia 996
UNCOMMON
Not applicable Osteochondritis dissecans of the talus 999
Sickle cell anemia 1001
RARE
Not applicable Avascular necrosis:
• Navicular (Köhler’s disease) 990
• Second metatarsal head (Freiberg’s infarction) 990
Compartment syndrome 992
Peripheral arterial disease 999
Thalassemia (familial Mediterranean fever) 1002

De Degenerative
REMOTE LOCAL
COMMON
Not applicable Bunions 991
Heel spurs 996
UNCOMMON
Not applicable Not applicable
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Chapter 50 Ankle and Foot Pain in a Child 985

Degenerative (continued)

ANKLE AND FOOT PAIN IN A CHILD


REMOTE LOCAL
RARE
Not applicable Hallux rigidus 995
Peroneal tendon subluxation 999

Tu Tumor
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Not applicable
RARE
Not applicable Malignant Primary, such as:
• Ewing’s sarcoma 1003
• Leukemia 1003
• Osteosarcoma 1004
• Rhabdomyosarcoma 1004
• Synovial sarcoma 1004
Malignant Metastatic:
Not applicable
Benign, such as:
• Calcaneal cysts 1002
• Ganglion cyst 1003
• Giant cell tumor of the tendon sheath 1003
• Glomangioma 1003
• Osteoblastoma 1003
• Osteochondroma 1004
• Osteoid osteoma 1004

Co Congenital
REMOTE LOCAL
COMMON
Not applicable Accessory navicular 989
Flat-foot deformities:
• Flexible 993
UNCOMMON
Not applicable Flat-foot deformities:
• Rigid 993
Tarsal coalitions:
• Calcaneonavicular coalition 1001
• Talocalcaneal coalition 1001
(continued)
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986 Chapter 50 Ankle and Foot Pain in a Child

Congenital (continued)
ANKLE AND FOOT PAIN IN A CHILD

REMOTE LOCAL
RARE
Not applicable Claw toes 991
Congenital oblique talus 992
Congenital vertical talus 992
Hallux varus 995
Hammer toes 995
Polydactyly 1000
Trevor’s disease (dysplasia epiphysealis
hemimelica) 1002

Ne Neurogenic/Psychogenic
REMOTE LOCAL
COMMON
Not applicable Not applicable
UNCOMMON
Not applicable Complex regional pain syndrome (reflex
sympathetic dystrophy) 992
RARE
Not applicable Not applicable

Note: These are estimates of relative incidence because few data are available for the less common conditions.

Common Ages at Which Ankle/Foot Pain Presents in a Child


APPROXIMATE AGE CONDITION
Birth to 3 Years Congenital vertical and oblique talus
Hand-foot-mouth disease
Hemophilia
Juvenile rheumatoid arthritis
Leukemia
Sickle cell anemia
Preschool (3–5 Years) Cellulitis
Ewing’s sarcoma
Fractures
Hand-foot-mouth disease
Hemophilia
Ill-fitting shoes
Juvenile rheumatoid arthritis
Leukemia
Osteoid osteoma
Puncture wounds
Rheumatic fever
Sickle cell anemia
Tuberculosis
Elementary School (6–11 Years) Accessory navicular
Avascular necrosis of the metatarsal head
Avascular necrosis of the navicular
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Chapter 50 Ankle and Foot Pain in a Child 987

ANKLE AND FOOT PAIN IN A CHILD


Common Ages at Which Ankle/Foot Pain Presents in a Child—cont’d
APPROXIMATE AGE CONDITION
Complex regional pain syndrome
Ewing’s sarcoma
Fabry’s disease
Familial Mediterranean fever
Fungal infection
Gaucher’s disease
Giant cell tumor of the tendon sheath
Hemophilia
Juvenile rheumatoid arthritis
Osteochondroma
Osteoid osteoma
Rheumatic fever
Sickle cell anemia
Tarsal coalitions
Middle School (12–14 Years) Accessory navicular
Ankle impingement syndromes
Ankle sprain
Avascular necrosis of the metatarsal head
Avascular necrosis of the navicular
Bursitis
Calcaneal apophysitis
Complex regional pain syndrome
Dysplasia epiphysealis hemimelica
Ewing’s sarcoma
Fabry’s disease
Familial Mediterranean fever
Fracture (calcaneus)
Fungal infection
Gaucher’s disease
Giant cell tumor of the tendon sheath
Glomangioma
Hallux rigidus
Heel spurs
Hemophilia
Ingrown toenails
Juvenile rheumatoid arthritis
Muscle strain
Necrotizing fasciitis
Osteochondritis dissecans of the talus
Osteochondroma
Osteoid osteoma
Osteomyelitis
Peroneal tendon subluxation
Popliteal entrapment syndrome
Puncture wounds
Rheumatic fever
Rigid flat foot
Seronegative spondyloarthropathies
Sickle cell anemia
Tarsal coalitions
Tendinitis
(continued)
1528_Ch50_982-1006 07/05/12 2:17 PM Page 988

988 Chapter 50 L5 Radiculopathy


ANKLE AND FOOT PAIN IN A CHILD

Common Ages at Which Ankle/Foot Pain Presents in a Child—cont’d


APPROXIMATE AGE CONDITION
High School (15–18 Years) Avascular necrosis of the metatarsal head
Compartment syndrome
Ehlers-Danlos syndrome
Fabry’s disease
Gout
Hemophilia
Myositis ossificans
Necrotizing fasciitis
Osteochondritis dissecans of the talus
Osteomyelitis
Popliteal entrapment syndrome
Rheumatic fever
Seronegative spondyloarthropathies
Sickle cell anemia

Overview of Ankle and Foot Pain Background Information


in a Child A lumbar disk herniation is the most com-
mon cause for this condition. The diagnosis
Ankle and foot pain is relatively rare in young is confirmed with magnetic resonance imag-
children. In many cases, the ankle or foot pain ing. Surgical intervention may be indicated
will have simple causes that can be treated easily in severe cases of lower extremity pain ac-
by the therapist. However, it is very important to companied by neurological signs.
rule out other causes of the pain due to the
harmful effects of some causes if overlooked. ■ S1 Radiculopathy
These include many forms of inflammation, Chief Clinical Characteristics
vascular compromise, systemic diseases, and This presentation typically includes pain in
other such problems that would require a refer- the lumbar spine and paresthesias radiat-
ral to the appropriate physician. The following ing from the buttock to the posterior aspect
section describes the thought process used when of the knee and extending posterolaterally
considering such a referral. from the knee to the foot. Depending on the
severity, the presentation may also include
Description of Conditions That a decreased or absent Achilles tendon reflex
May Lead to Ankle and Foot Pain and motor loss in the muscles innervated by
in a Child the S1 nerve.
Remote Background Information
A lumbar disk herniation is a common cause
LUMBAR RADICULOPATHIES for this condition. The diagnosis is confirmed
■ L5 Radiculopathy with magnetic resonance imaging. Surgical
Chief Clinical Characteristics intervention may be indicated in severe cases
This presentation includes pain in the lum- of lower extremity pain accompanied by neu-
bar spine and paresthesias radiating from rological signs.
the lateral aspect of the hip and buttock to
the lateral aspect of the knee, extending an- ■ Popliteal Entrapment Syndrome
terolaterally down to the foot. Depending Chief Clinical Characteristics
on the severity, the presentation may also This presentation may be characterized by
include motor loss in the muscles innervated symptoms and signs consistent with claudica-
by the L5 nerve root. tion and lower limb ischemia with exercise.1
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Chapter 50 Anterior 989

Background Information symptoms due to increased pressure in the

ANKLE AND FOOT PAIN IN A CHILD


This is a rare condition in which the popliteal area.4 This condition is more common in girls,
artery is compressed by various muscle ten- with pain and tenderness medial to the nav-
dons in the region. History, physical examina- icular as the most common sign.2,3
tion with auscultating and palpating the
Background Information
peripheral pulses, and use of Doppler sonog-
An external oblique x-ray is needed to prop-
raphy if available before and after exercise can
erly visualize this condition.2 Physical testing
help identify this condition.1 Use of magnetic
may include pain with testing the posterior
resonance imaging can also identify possible
tibialis tendon.5 This is due to the tendon in-
compression sites due to individual differences
serting on the accessory bone instead of the
in anatomy. Treatment should be a referral for
true navicular, causing too much stress to the
surgical treatment.1
area.5 Treatment includes use of a walking cast,
with occasional need for surgical resection if
Local
symptoms persist.2–5
■ Accessory Navicular
ANKLE IMPINGEMENT
Chief Clinical Characteristics SYNDROMES
This presentation includes pain with weight
bearing and palpation to the navicular re- ■ Anterior
gion (Fig. 50-1).2,3 Tight shoes may increase Chief Clinical Characteristics
This presentation involves restriction with
pain when the foot is dorsiflexed, and palpa-
tion tenderness to the anterior ankle joint.
Often osteophyte formation is present in the
tibiotalar region and is the cause of the im-
pingement and resulting pain (Fig. 50-2).6
Background Information
Os trigonum
An oblique anteromedial impingement radi-
ograph is usually the best method of looking
for these osteophytes.6 Treatment consists of

Osteomyelitis
Accessory navicular

Medial Kohler disease


Lateral
malleolar malleolar Stress fracture
ossicle ossicle Freiberg
disease

Accessory
navicular Apophysitis
Tarsal Tumors Ingrown
condition (osteoid toenail
Plantar osteoma)
fasciitis Hammer
toe
FIGURE 50-2 Pain localization points about the
FIGURE 50-1 Common accessory ossification centers. ankle and foot.
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990 Chapter 50 Posterior

surgical removal of the osteophytes that to nonsurgical treatment and return to full
ANKLE AND FOOT PAIN IN A CHILD

are causing the trauma to the surrounding activity in 4 to 8 weeks.3,4,10


tissues.6
■ Fifth Metatarsal (Iselin’s
■ Posterior Disease)
Chief Clinical Characteristics Chief Clinical Characteristics
This presentation involves restriction with This presentation usually involves pain,
pain when the foot is plantarflexed, with swelling, and tenderness under the proximal
palpation tenderness to the posterior ankle region of the fifth metatarsal (especially with
joint. weight bearing near the attachment of the
peroneus brevis; see Fig. 50-1).3,12
Background Information
This syndrome is very common in dancers Background Information
and soccer players, especially those with a This is an overuse injury to the region
history of flexor hallucis tenosynovitis.7,8 while performing running, jumping, and
Many times an ankle sprain injury with a cutting type activities. The most common
congenital abnormality such as large talus, group affected is 10- to 12-year-olds.12
os trigonum (see Fig. 50-2), or loose bodies Due to the ligaments and tendons being
will result in an impingement of the poste- stronger than bone at this age, the bone
rior capsule of the ankle.9 This results in can avulse or cause a traction apophysitis
pain and limited plantarflexion. Physical similar to that in the calcaneus in Sever’s
testing including pain with passive plan- disease. Physical testing elicits pain with re-
tarflexion reproducing the child’s pain is sisted eversion with end-range plantarflex-
positive for posterior impingement. Non- ion or dorsiflexion.12 The usual course of
surgical management consisting of anti- treatment is immobilization and then
inflammatory modalities, taping, stretching, strength and proprioceptive training.3,12
and/or steroid injections is the treatment for
this condition.7 If unsuccessful, surgical in- AVASCULAR NECROSIS
tervention may be warranted.7 ■ Navicular (Köhler’s Disease)

APOPHYSITIS Chief Clinical Characteristics


This presentation involves pain and limping
■ Achilles Tendon Insertion with forefoot supination in young children
(Sever’s Disease) (ie, 3 to 7 years old).3 The pain is often local-
Chief Clinical Characteristics ized to the medial border of the midfoot
This presentation commonly involves often (see Fig. 50-1). Swelling in the talonavicular
bilateral focal pain and swelling near the in- joint and pain with motion (supination/
sertion on the calcaneous (see Fig. 50-1), tight pronation) can help with diagnosis if the
heel cords leading to decreased dorsiflexion of child is too young for conclusive x-rays.3,5
the ankle, and walking on toes secondary to
Background Information
pain with weight bearing. The child usually
This condition is avascular necrosis of the
has just increased the workload or intensity
navicular (see Fig. 50-2).3 The treatment
of competitive sports.
consists of rest, a walking cast, and medial
Background Information longitudinal arch supports until healing is
This condition is a traction apophysitis of complete, which can take up to a full year.3,5
the Achilles tendon insertion at the calca-
neus (see Fig. 50-2). The most commonly af- ■ Second Metatarsal Head
fected age range is 8 to 12 years.3,10,11 X-rays (Freiberg’s Infarction)
can be helpful to confirm the diagnosis by Chief Clinical Characteristics
ruling out other causes of pain.3,4,10 Treat- This presentation commonly includes a fe-
ment consists of stretching, strengthening, male adolescent who reports pain on the ball
activity limitation, and use of heel cups/ of the foot with increased levels of lower ex-
change in footwear. Most children respond tremity repetitive activities (see Fig. 50-1).3,5
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Chapter 50 Claw Toes 991

Background Information ■ Subcutaneous Achilles Bursitis

ANKLE AND FOOT PAIN IN A CHILD


This is a condition of avascular necrosis of the Chief Clinical Characteristics
second metatarsal head (see Fig. 50-2).3,5,13 This presentation includes local pain, tender-
Physical examination demonstrates pain and ness to palpation, and swelling just posterior
stiffness near the region.13 X-rays show an to the Achilles tendon insertion at the calcaneal
enlarged and flattened metatarsal head and tuberosity. Palpable warmth and observable
decreased joint space.13 Treatment consists redness may be present in the same area.
of non–weight-bearing activities, and use of
various orthoses to decrease stress in this Background Information
region.3,5 If symptoms persist, surgical inter- Subcutaneous Achilles bursitis is a result of
vention may be necessary.13 inflammation and swelling of the bursa
between the Achilles tendon and the skin.
■ Bunions This may occur from mechanical trauma to
Chief Clinical Characteristics the tendon or it may be associated with in-
This presentation commonly includes pain or sertional Achilles tendinopathy (tendinitis/
tightness with shoe wear. A bunion is an abnor- tendinosis) or retrocalcaneal bursitis. Con-
mality involving an increased angle between tinued irritation of the inflamed and
the first and second metatarsal with the big toe swollen bursa may lead to thickening of the
in a valgus position.5,14 bursal walls and fibrosis. Clinical examina-
tion confirms the diagnosis. Treatment is
Background Information
nonsurgical, and typically involves footwear
This condition is most commonly seen in
modification.
young girls who are approximately 9 years old
with pressure over the medial aspect of the ■ Cellulitis
foot near the first metatarsal head.14 This
condition is commonly found in children Chief Clinical Characteristics
with neurological disorders and those with This presentation typically involves warmth,
connective tissue disorders.14 Treatment con- tenderness to palpation, swelling, fever, malaise,
sists of wider width shoes and then surgery if and radiation of the symptoms toward the
necessary.5 extremities can be seen.15
Background Information
BURSITIS This condition is an inflammation of the skin
■ Retrocalcaneal Bursitis cells. It is usually differentiated from os-
Chief Clinical Characteristics teomyelitis based on appearance, with this
This presentation can involve pain, palpable condition demonstrating more swelling and
warmth, tenderness to palpation, and some redness.16 Medical management is needed be-
swelling at the medial and lateral aspects fore treatment with physical therapy.15 If not
of the Achilles tendon. Pain is reproduced treated medically, the inflammation can
with firm palpation of the tissue anterior to spread rapidly.15
the tendon, as well as with dorsiflexion of the
ankle. ■ Claw Toes
Chief Clinical Characteristics
Background Information This presentation can be characterized by
This condition is the result of inflammation anatomical deformity and pain in the toes with
within the bursa that lies between the Achilles weight bearing.13
tendon and the calcaneus. Predisposing
factors include trauma, systemic disease, Background Information
and biomechanical or structural factors such This condition is a deformity in which the
as prominent posterosuperior calcaneal metatarsophalangeal joint is in extension,
tuberosity (Haglund’s deformity) and rear- while the proximal and distal interphalangeal
foot varus. Clinical examination confirms joints are in flexion. It has been associated with
the diagnosis. Treatment is nonsurgical and some underlying neurological diseases such as
typically involves footwear modification. hereditary sensory motor disorders or spinal
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992 Chapter 50 Compartment Syndrome

cord abnormalities.13 If pain occurs, surgical Background Information


ANKLE AND FOOT

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