Esclerosis

Lateral Amiotrófica

Klgo. Mg. Jorge Molina Blamey

Magíster de Gestión en Salud
Especialista en Kinesiología Respiratoria.(Denake)
Especialista en Terapia Respiratoria (CLCPTR)
Director Kinesiología Clínica Alemana-Universidad del Desarrollo
Director Diplomado Kinesiterapia UPC CAS-UDD
Director Denake
Past-President División de Kinesiología Sociedad Chilena de Medicina Intensiva

Corporación ELA Septiembre 2016

 Definición
• Enfermedad Degenerativa Progresiva del
Sistema Nervioso Central.

• Escasa Prevalencia.

• Esperanza de Vida: 3-5 años desde que se Dg.

• 10% sobrevive más años.

Qué es
https://beyondthedish.files.wordpress.com/2015/12/myelin_sheath.jpg
 Incidencia y prevalencia
• Incidencia: 1–2 casos nuevos 100.000 háb/año

• Prevalencia: 4-6 casos 100.000 háb.

 Int. J. Epidemiol. Advance Access published July 27, 2016

International Journal of Epidemiology, 2016, 1–18

doi: 10.1093/ije/dyw061
Original article

Original article

Variation in worldwide incidence of

amyotrophic lateral sclerosis: a meta-analysis
Benoı̂t Marin,1,2,3,4,5,6 Farid Boumédiene,1,2,3 Giancarlo Logroscino,5,6
Philippe Couratier,1,2,7 Marie-Claude Babron,8,9
Anne Louise Leutenegger,8,9 Massimilano Copetti,10
Pierre-Marie Preux1,2,3 and Ettore Beghi4,*

Downloaded from http://ije.oxfordjournals.org/ by guest on

1
INSERM, U1094, Tropical Neuroepidemiology, Limoges, France, 2Univ. Limoges, UMR_S 1094, Tropical
Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, CNRS FR 3503 GEIST, F-
87000 Limoges, France, 3CHU Limoges, Centre d’Epidémiologie de Biostatistique et de Méthodologie de

Distribución heterogénea en el mundo

la Recherche, Limoges, France, 4Laboratorio di Malattie Neurologiche, IRCCS Istituto di Ricerche
Farmacologiche Mario Negri, Milano, Italy, 5Department of Basic Medical Sciences, Neuroscience
and Sense Organs, University of Bari ‘Aldo Moro’, Bari, Italy, 6Unit of Neurodegenerative Diseases,
University of Bari ‘Aldo Moro’, at ‘Pia Fondazione Cardinale G. Panico’, Lecce, Italy, 7CHU Limoges,
Service de Neurologie, Limoges, France, 8INSERM UMR 946, Genetic Variability and Human Diseases,
Paris, France, 9University Paris Diderot, UMR 946, Paris, France and 10Unit of Biostatistics, IRCCS
‘Casa Sollievo della Sofferenza’, San Giovanni Rotondo, Italy
*Corresponding author. Mario Negri IRCCS Institute for Pharmacological Research, Via La Masa 19, 20156 Milano, Italy.
E-mail: [email protected]
Accepted 4 March 2016
International Journal of Epidemiology, 2016, Vol. 00, No. 00 7
 Clinical Neurophysiology 127 (2016) 2643–2660

Contents lists available at ScienceDirect

Clinical Neurophysiology
journal homepage: www.elsevier.com/locate/clinph

Review

Assessment of the upper motor neuron in amyotrophic lateral sclerosis

William Huynh a,b,⇑, Neil G. Simon c, Julian Grosskreutz d, Martin R. Turner e, Steve Vucic f,
Matthew C. Kiernan a W. Huynh et al. / Clinical Neurophysiology 127 (2016) 2643–2660 2655
a
Brain and Mind Centre, University of Sydney, NSW, Australia
b
Prince of Wales Clinical School, University of New South Wales, NSW, Australia
c
Department of Neurology, St Vincent’s Hospital, Darlinghurst, Australia
d
Hans-Berger Department of Neurology, University Hospital Jena, Jena, Germany
e
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
f
Western Clinical School, University of Sydney, Sydney, Australia

a r t i c l e i n f o h i g h l i g h t s

Article history:
! Clinical signs of UMN involvement are an important component in diagnosis of ALS.
Accepted 27 April 2016
! Novel neuroimaging and electrophysiology may facilitate demonstration of UMN degeneration in ALS.
Available online 5 May 2016
! Improving early ALS diagnosis can facilitate the development of effective therapies.

Keywords:
Amyotrophic lateral sclerosis
Motor neuron disease a b s t r a c t
Upper motor neuron
Imaging
Clinical signs of upper motor neuron (UMN) involvement are an important component in supporting the
Transcranial magnetic stimulation
diagnosis of amyotrophic lateral sclerosis (ALS), but are often not easily appreciated in a limb that is con-
currently affected by muscle wasting and lower motor neuron degeneration, particularly in the early
symptomatic stages of ALS. Whilst recent criteria have been proposed to facilitate improved detection
of lower motor neuron impairment through electrophysiological features that have improved diagnostic
Fig. 11. 18F-fluorodeoxyglucose PET analysis in ALS patients
sensitivity, assessment
demonstrating of upper The
hypometabolism. motor neuron
images showinvolvement remains
three-dimensional essentially
rendering of the clinical. As a surface
brain cortical result, of
there
the clusters of voxels in which patients with ALS show hypometabolism compareddiagnostic
is often a significant with healthydelay
controls.
thatUptake is substantially
in turn may impactimpaired mainly
institution in the frontal and anterior
of disease-modifying therapy
cingulate cortex. Reproduced with permission (Chio et al.,and
2014).
access to other optimal patient management. Biomarkers of pathological UMN involvement are also
required to ensure patients with suspected ALS have timely access to appropriate therapeutic trials. The
present review provides an analysis of current and recently developed assessment techniques, including
corticomotoneuronal involvement as well as PMA being partand
of the more likelyapproaches
increases used
the likelihood of objectively demonstrating
Biomarcadores, técnicas de difusión, tomografía, espectroscopía
novel imaging electrophysiological to study corticomotoneuronal pathology in ALS.
MND/ALS clinical spectrum. In addition, patients ! 2016with MND/ALSFederation
International UMNofdysfunction in MND thereby
Clinical Neurophysiology. facilitating
Published by Elsevierearlier
Irelanddiagnosis
Ltd. This is an
also showed clusters of relative hypermetabolism within
open accessthe cere-
article andCCinstitution
under the of potentially
BY-NC-ND license disease-modifying agents (Pohl
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
bellum, occipital cortex, upper brain stem, and medial temporal et al., 2001b; Kaufmann et al., 2004; Turner et al., 2005a; Furtula
cortex (Van Laere et al., 2014). Patients with PLS demonstrated a et al., 2013; Grieve et al., 2015; Bae et al., 2016). In a study that
more widespread abnormality with symmetrically reduced combined structural imaging and electrophysiological approaches
Contents bilaterally in the prefrontal cortex, anterior cingulate,
metabolism revealed that the presence of precentral gyrus cortical thinning
Documento descargado de http://www.elsevier.es el 06/09/2016. Copia para uso personal, se prohíbe la transmisión de este documento por cualquier medio o formato.

n e u r o l a r g . 2 0 1 4;6(2):91–95

Neurología Argentina
www.elsevier.es/neurolarg

Revisión

Esclerosis lateral amiotrófica (ELA): seguimiento

y tratamiento

Cecilia Quarracino a,∗ , Raúl Carlos Rey b y Gabriel Eduardo Rodríguez c

a Residente de Neurología, División Neurología, Hospital Ramos Mejía, Centro Universitario de Neurología José María Ramos Mejía,
Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina
b Jefe de División de Neurología, Hospital Ramos Mejía, Centro Universitario de Neurología José María Ramos Mejía,

Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina

c Sector Enfermedades de motoneurona, División de Neurología, Hospital Ramos Mejía, Centro Universitario de Neurología

José María Ramos Mejía, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina

información del artículo r e s u m e n

Historia del artículo: La esclerosis lateral amiotrófica es una enfermedad neurológica degenerativa que afecta a
National Clinical Guideline Centre

Motor neurone disease

Motor neurone disease: assessment and management

NICE guideline NG42

Methods, evidence and recommendations
February 2016
 antibodies
Monotherapy Monotherapy Add-on therapy Add-on therapy Monotherapy Add-on therapy
Follow-up 2 years 12 weeks 5 years 1 year 27–39 months 1 year
Mean increase in Clinical IVIg dosing interval IVIg dose reduction Sustained clinical No significant improvement
strength of 22%, deterioration increased from Rituximab
of 43% and regimenimprovement
Monotherapy
in all Monotherapy
in group mean scores; Add-on therapy Add-on therapy Monotherapy Add-on therapy
versus 0% in after 2 months 7 days to 12 days, increase in strength
Clinical outcome patients
Mean increase in subjective
Clinical and objective IVIg dosing interval IVIg dose reduction Sustained clinical No significant improvemen

Multifocal motor neuropathy: diagnosis,

control group and subjective in one patient, but strength of 22%, improvements
deteriorationin strength
increased from of 43% and improvement in all in group mean scores;
clinical improvement IVIg dose increased versus 0% in andafter
disability in individual
2 months 7 days to 12 days, increase in strength patients subjective and objective
by 24% in control group patients; IVIg dosing and subjective in one patient, but improvements in strength

pathogenesis and treatment strategies

other patient interval increased fromclinical improvement IVIg dose increased and disability in individual
4 weeks to 5 weeks in by 24% in patients; IVIg dosing
one patient other patient interval increased from
4 weeks to 5 weeks in
At least 11* 0 1 1 3 4
one patient
Lotte Vlam, W.-Ludo van der Pol, Elisabeth A. Cats, Dirk
Number of patients At least 11* 0
C. Straver,
1
Sanneke Piepers,
1
Hessel
3
Franssen 4

WS and ofLeonard
or MMN patients (improvement H.patients).
strength in 18 of 21 vanAbbreviations:
den Berg experiencing
IVIg, intravenous immunoglobulin; MMN, multifocal motor neuropathy.
improvement
*Not mentioned solely for MMN patients (improvement of strength in 18 of 21 patients). Abbreviations: IVIg, intravenous immunoglobulin; MMN, multifocal motor neuropathy.
Abstract | Multifocal motor neuropathy (MMN) is a rare inflammatory neuropathy characterized by slowly
ong-term effects of immunoglobulin complement factor C5, did not allow dose reduction of
progressive, asymmetric distal limb weakness without sensory loss. The clinical presentation of MMN
tment have been confirmed in several IVIg in most patients, although
Although thesome improvements
long-term effects ofinimmunoglobulin complement factor C5, did not allow dose reduction of
VIg treatment doesTable
may 1 |
notmimic Differential
mild diagnosis
prevent aamyotrophic
objectivelateral of multifocal
motor sclerosis,
performance motor other
measures neuropathy
variants
and conduction of motor neuron disease, or chronic inflammatory
maintenance treatment have been confirmed in several IVIg in most patients, although some improvements in
n muscle strength—probably
demyelinating
Feature due to block were
polyneuropathy observed after
Multifocal
studies, treatment
with asymmetric in an
motor IVIgAmyotrophic
17,18,20,91,126 open-label
onset.does
treatment study
Differentiation
notLower a mildis important,
preventmotor Chronicmotor
objective asperformance
these diseases
inflammatory Lewis– and
measures differ in
conduction
generation—over time.17,18,59 of 13 patients. 134
Several case
neuropathy
gradual declinereports and
in muscle small
lateral uncon- neuron due
strength—probably disease
to demyelinating
block were observed after treatmentSumner
in an open-label study
prognosis and treatment. The electrophysiological
trolled studies have described treatment
finding of
experiences with
conduction block in the absence of abnormalities
ongoing axonal degeneration—over
sclerosis time.17,18,59 ofpolyneuropathy
13 patients.134 Several case reports
syndromeand small uncon-
in sensory nerves ritixumab,
is the hallmarka monoclonal of antibody
MMN, but directed canagainst
be difficult
the to detect. Intravenous
trolled studies haveimmunoglobulin is
described treatment experiences with
Distribution
tory therapies other than of have
IVIg weaknessB-cell-specific Asymmetric
antigen CD20. 135–140
The Asymmetric
results from these Asymmetric Symmetric
ritixumab, a monoclonal Asymmetric
antibody directed
Other therapies
efficacious in most patients, but long-term maintenance therapy does not prevent slowly progressive axonal the
against
ed in open-labelProminent
trials andsensory
in one symptoms
studies are inconsistent (Table 2). A randomized
No Immunomodulatory No controlled
therapies other than
No IVIg have B-cell-specific
Yes antigen CD20.135–140
YesThe results from these
degeneration.
d trial. Prednisolone and plasmapher-Moreover, cyclophosphamide,
trial is, therefore, been investigated
needed to further establish althoughthe safetyeffective,
in open-label andtrials and has
in one substantial adverse (Table 2).
studies are inconsistent effects, and the controlled
A randomized
Tendon
e in most patientsefficacy
with MMNofreflexes
andother
may immunosuppressive Normal or
placebo-controlled Increased
trial. Prednisolone Decreased
and plasmapher- General hyporeflexia
trial is, therefore,The
needed Decreased
to further establish the safety and
efficacy of rituximab treatment drugs,
in patients including
with MMN. rituximab, is not established. underlying pathological
ymptoms.20,127–129 Cyclophosphamide decreased in
esis are ineffective in weakened
in most patients with in weakened
MMN and may or areflexia
efficacy of rituximab treatment in in patients
weakened with MMN.
mechanisms of MMN
used to treat patients with MMN, and
are unclear,
Conclusions weakened but IgM symptoms.
muscles*
even exacerbate autoantibodies
muscles 20,127–129 against
muscles the ganglioside GM1 may cause
Cyclophosphamide muscleschanges
in
d to be effective in nodalcase
several
Disease and perinodal
series.
course MMNstructures
is a treatable
Slowly that
was the firstcompromise
disorder
progressive drug
and usedshould, to treat
Rapidly nerve
patients
as such, be conduction.
with MMN, and Further
Slowly or rapidly Conclusions elucidation
Progressive of theProgressive
or relapsing disease
w of these studies,mechanisms
130
28 patients (68%) differentiatedlead has
from tobeen
MND. reported
Conduction to be effective in
block is 130 several
the case series. MMN is a treatable disorder and should, as such, be
may ultimately In a
improved
recent review of
treatment
progressive
these studies,
strategies.
28
progressive In this Review, we discuss or
patients (68%) differen tiated from MND.
the diagnostic
relapsing
Conduction block is the
significant improvement, but the sub- electrophysiological hallmark of MMN but may be dif-
criteria for
Cerebrospinal MMN,
fluid
fects of cyclophosphamide restrict its and provide No an update
reported some on
or the
significant
No
ficult to identify. Nerve conduction studies should, there- current understanding
improvement, No but the sub- of MMN
electro
Yes pathogenesis.
physiological hallmark We
of also
MMN
Rare butdescribe
may be dif-
protein >1 g/l stantial adverse effects of cyclophosphamide restrict its ficult to identify. Nerve conduction studies should, there-
available treatments fore,and
be aspromising new therapeutic
extensive as required—including
long-term use.60
strategies.
proximal
fore, be as extensive as required—including proximal
tudies28,130–132 have suggestedtiters
Increased benefi-
of GM1-nerve segments—in
Common patients with asymmetric,
Rare progres- Rare Raresegments—in patients with Rare
Uncontrolled studies 28,130–132
have suggested benefi- nerve asymmetric, progres-
tment with interferon-β,
Vlam, cyclosporine,
L. et al.
specific sive limb weakness
Nat. Rev. Neurol. 8, 48–58cial
IgM antibodies without
(2012); evident
publishedsensory disturbance
online 22 November 2011; doi:10.1038/nrneurol.2011.175
effects of treatment with interferon-β, cyclosporine, sive limb weakness without evident sensory disturbance
thioprine and mycophenolate mofetil or upper motor neuron signs. Although treatment
methotrexate, azathioprine
with
and mycophenolate mofetil orSymmetric
upper motor neuron signs. Although treatment with
with MMN. However, Abnormal
Introduction MRI signal inIVIg
a randomized the is efficacious
Asymmetric
in most patients with No
MMN, a slowly No Asymmetric
brachial plexus in some patients with MMN. However, a randomized IVIg is efficacious in most patients with MMN, a slowly
ed trial showed that adjunctive motor
Multifocal treat- neuropathy
progressive decline in musclewhich
(MMN),
placebo-controlled strengthtrial
during
was showedlong-term
first deficits despite
that adjunctive treat- increasing IVIg in
progressive decline doses.
muscle17,18 Alternative
strength treat-
during long-term
henolate mofetil did not alter the dis-
Response to intravenous IVIg treatment usually occurs. Determinants
Yes ment with mycophenolate of perma-
No mofetil did not Noalter the dis- Yestreatment usually occurs. Yes
tients with MMN, described
and did not
immunoglobulin
inallow
1985, isnent
a rare disorder,
weakness are with
thecourse
number a prevalence
of yearswith
without of IVIg ment strategies that prevent permanent weaknessperma-
IVIg Determinants of are,
ease of patients MMN, and did not allow nent weakness are the number of years without IVIg
tion of IVIg doses. around
133 0.6 per
Treatment with100,000 individuals.
treatment and the amount
signi
1
It ofisreduction
ficant a purely
axon ofmotor
loss. Future doses.133therefore,
IVIgtreatment needed.
Treatment with Although
treatment and theclinical,
amount of immunological and
axon loss. Future treatment
onoclonal antibody Response toagainst
corticosteroids No‡ aim to prevent axon loss. No No Yes Yes
directed strategies should
neuropathy with slowly progressive, asymmetric, predomi-
eculi zumab, a monoclonal antibody electrophysiological studies have improved our under-
directed against strategies should aim to prevent axon loss.
*In some patients, reflexes are brisk. May aggravate symptoms.
‡
nantly distal weakness of limbs. Conduction block was standing of MMN, the underlying pathogenic mechanisms
| NEUROLOGY identified as the electrophysiological NATUREcharacteristic that dis-
REVIEWS | NEUROLOGY VOLUMEneed further
8 | JANUARY 2012 dissection.
| 55 VOLUME 8 | JANUARY 2012 |
tinguished MMN
compatible from motor
with neuron disease
© 2011 Macmillan Publishers Limited. All rights reserved
with LSS than MMN are (MND). muscle
a relapsing– 2–4
In action
this Review, we discuss
(CMAP)the clinical characteristics,
© 2011 Macmillan Publishers Limited. All rights reserved
potential following proximal
SPECIAL ARTICLE
Practice Parameter update: The care of the patient with
amyotrophic lateral sclerosis: Drug, nutritional, and
respiratory therapies (an evidence-based review)
Report of the Quality Standards Subcommittee of the American Academy of Neurology

R.G. Miller, MD, FAAN ABSTRACT

C.E. Jackson, MD, FAAN Objective: To systematically review evidence bearing on the management of patients with amyo-
E.J. Kasarskis, MD, trophic lateral sclerosis (ALS).
PhD, FAAN
Methods: The authors analyzed studies from 1998 to 2007 to update the 1999 practice param-
J.D. England, MD,
eter. Topics covered in this section include slowing disease progression, nutrition, and respiratory
FAAN
management for patients with ALS.
D. Forshew, RN
W. Johnston, MD Results: The authors identified 8 Class I studies, 5 Class II studies, and 43 Class III studies in ALS.
S. Kalra, MD Important treatments are available for patients with ALS that are underutilized. Noninvasive ven-
J.S. Katz, MD tilation (NIV), percutaneous endoscopic gastrostomy (PEG), and riluzole are particularly important
H. Mitsumoto, and have the best evidence. More studies are needed to examine the best tests of respiratory
MD, FAAN function in ALS, as well as the optimal time for starting PEG, the impact of PEG on quality of life
J. Rosenfeld, MD, PhD, and survival, and the effect of vitamins and supplements on ALS.
FAAN Recommendations: Riluzole should be offered to slow disease progression (Level A). PEG should
C. Shoesmith, MD, BSc be considered to stabilize weight and to prolong survival in patients with ALS (Level B). NIV should
M.J. Strong, MD be considered to treat respiratory insufficiency in order to lengthen survival (Level B), and may be
S.C. Woolley, PhD considered to slow the decline of forced vital capacity (Level C) and improve quality of life (Level
C). Early initiation of NIV may increase compliance (Level C), and insufflation/exsufflation may be
considered to help clear secretions (Level C). Neurology® 2009;73:1218 –1226
Address correspondence and
reprint requests to the American
GLOSSARY
Academy of Neurology, 1080
Montreal Avenue, St. Paul, MN AAN ! American Academy of Neurology; ALS ! amyotrophic lateral sclerosis; FVC ! forced vital capacity; HFCWO ! high
55116 frequency chest wall oscillation; MIE ! mechanical insufflation/exsufflation; MIP ! maximal inspiratory pressure; NIV !
[email protected] noninvasive ventilation; PCEF ! peak cough expiratory flow; Pdi ! transdiaphragmatic pressure; PEG ! percutaneous endo-
scopic gastrostomy; QOL ! quality of life; RIG ! radiologically inserted device; SNP ! sniff nasal pressure; TIV ! tracheos-
tomy invasive ventilation.

Amyotrophic lateral sclerosis (ALS) is a neurodegenera- efit and received US Food and Drug Administration
tive disease characterized by loss of motor neurons in approval (see below), there have been advances in symp-
Neurology
the spinal cord, brainstem, 73,Octuber
and motor 13 2009
cortex. The cause tomatic treatment for patients with this disease. In this
of the disease is still not known. ALS is not curable, but revision, we update the riluzole practice advisory
a number of important therapies are available. In 1999, and address other management issues for care of
the American Academy of Neurology (AAN) published patients with ALS. This article addresses riluzole,
 Figure 1 Nutrition management algorithm

*e.g., Bulbar questions in the Amyotrophic Lateral Sclerosis Functional Rating Scale, or other instrument. †Prolonged meal
time; ending meal prematurely because of fatigue; accelerated weight loss due to poor caloric intake; family concern about
feeding difficulties. ‡Percutaneous endoscopic gastrostomy: rule out contraindications.
 Figure 2 Respiratory management algorithm

PFT " pulmonary function tests; PCEF " peak cough expiratory flow; NIV " noninvasive ventilation; SNP " sniff nasal pressure;
MIP " maximal inspiratory pressure; FVC " forced vital capacity (supine or erect); Abnl.nocturnal oximetry " pO2 !4% from
 Historia
• Estudios de Charcot. (1865)

• Análisis clínicos de los pacientes y su

evolución en cada paciente.

• Hallazgos anatómicos en autopsia.

 Historia
• Estudios de Charcot. (1865)

• “Informe de una mujer joven con una debilidad

progresiva y que paralelamente desarrolla
aumento del tono muscular con contracturas, con
intelecto preservado al igual que el esfínter
urinario”
• Lo correlaciona en autopsia con lesión
degenerativa en cordón lateral de la médula
espinal.
 Historia
• Estudios de Charcot. (1865)

• Describe posteriormente la debilidad gloso-

faríngea relacionada a la atrofia de los núcleos
motores de la parte baja del tronco cerebral.
• Esto se conoce como la parálisis bulbar
progresiva.
 ELA: Características
• Microscopio:
• Pérdida de neuronas en el asta anterior
medular y en los núcleos motores del
troncoencéfalo, respetando los responsables
de los movimientos oculares.
 ELA: Características
• Daño en neuronas motoras:

– Adelgazamiento de raíces anteriores medulares.

– Pérdida de mielina en nervios motores.
– Atrofia muscular por denervación.
 Epidemiología
• 5-10% de los casos son familiares. (autosómica
dominante o recesiva).

• Mas de 100 mutaciones en gen causante

pueden estar presentes.
 Signos y síntomas
• Afección del sistema motor que dirige, regula y
mantiene la musculatura esquelética responsable
del movimiento y de la relación con el entorno.

• No se afectan:
• Neuronas motoras oculares y esfinterianas.
• Sensibilidad superficial ni profunda.
• Función de la musculatura lisa.
• Conciencia.
• Función sexual.
 Signos y síntomas
• Inicio es localizado en alguna extremidad.
• Parálisis total entre 2-4 años.

• Insuficiencia respiratoria es progresiva.

• Principal causa de morbimortalidad.
 1. Síntomas y signos de neurona motora inferior

Signos y síntomas (neurona motora

A.- Debilidad muscular: Es el síntoma más relevante de la ELA y se debe a
la muerte progresiva de neuronas motoras. Se manifiesta cuando se ha per-
dido el 50% de la población de neuronas motoras. Inicialmente afecta a un
inferior)
grupo de músculos y se va difundiendo hacia otros a medida que avanza la
enfermedad.
La debilidad muscular (paresia) se valora mediante el Test Muscular
• A. Debilidad muscular.
Manual (TMM) que la gradúa de 0 a 5 (Tabla 3). El explorador ejerce fuer-
za contra resistencia a la contracción voluntaria de un determinado múscu-
• Dinamómetro.
lo o grupo muscular.

Tabla 3. Escala de valoración del Test Muscular Manual (TMM).

5: Fuerza muscular normal.

4: Disminución de la fuerza contra resistencia.

3: Imposibilidad de realizar fuerza contra resistencia, pero se mantiene el movimiento

completo contra gravedad.

2: Movimiento incompleto contra gravedad.

1: Contracción muscular sin movimiento.

0: Ausencia completa de contracción muscular.

Normalmente es suficiente valorar los siguientes grupos musculares:

 Signos y síntomas
• B. Atrofia Muscular.
– Por la debilidad muscular.

– Inspección de manos.

– Se generaliza.
 Signos y síntomas
• C. Fasciculaciones.

• D. Calambres musculares.

• Hipotonía y arreflexia.
 Signos y síntomas (neurona motora
superior)
• A. Debilidad muscular.
– Torpeza y pérdida de destreza.

– Pérdida del control inhibitorio que ejerce la vía

corticoespinal sobre neuronas motoras inferiores
que inervan los músculos antagonistas.
 Signos y síntomas
• B. Espasticidad.
• C. Hiperreflexia.
• D. Reflejos patológicos.
• E. Labilidad emocional.

• Reflejos patológicos en los músculos de la vía

aérea.
 Formas de Inicio
• ELA Clásica.
• Esclerosis lateral primaria.
• Amiotrofia espinal progresiva.
• Parálisis bulbar progresiva.
 Complicaciones bulbares
• Disfagia.

• Disartria.

• Alteraciones de la tos.

• Anartria (sin voz)

 Diagnóstico
• Clínico.

• Electrofisiológico.

• Neuroradiológico.

• Genético.
 Tratamiento
• Farmacológico:
– FDA.
– Riluzol. 6 meses a 1 año.
– Menos de 5 años de evolución.
– Sin TQT.
 Tratamiento
• Disfagia: reducción líquidos
• Uso de sonda gástrica.

• Disartria: Fonoaudiología.
• Sistemas de comunicación.
• “Tobii”
 Sistemas de comunicación
• Algunas de las herramientas que permiten realizar seguimiento de
ojos, son:
• iMotions1 : plataforma biométrica que
ofrece software y hardware (gafas y monitores de seguimiento de
ojos, entre otros) para realizar el seguimiento de ojos y análisis de
la expresión facial. iMotions se caracteriza por inferir la implicación
emocional con la que se está observando mediante datos relativos
al parpadeo, dilatación de la pupila o velocidad de movimiento 2 .
• SMI Redn Professional3 : es un conjunto de software y
herramientas de hardware que permiten hacer seguimiento de ojos
Mejora la arquitectura de la información y el diseño de interfaces
en sitios web estáticos y dinámicos, software y videojuegos.
Proporciona informes de rendimiento de comportamiento y
datos biométricos, analizando las acciones de los usuarios de forma
no intrusiva.
• Algunas de las herramientas que permiten realizar
seguimiento de ojos, son:
• EyeWorks4 : es una empresa que ofrece servicios de
hardware (cámaras, monitores, sensores, y otras
tecnologías) y software de seguimiento de ojos que se
puede aplicar a distintos aspectos.
• PyGaze:5 es un paquete de software libre de
seguimiento ocular que engloba varias aplicaciones y
que está destinado a personas con pocas nociones de
programación. El código se puede compartir con
cualquier tipo de ordenador y es compatible con
Windows, Linux y Mac OSX.
• Algunas de las herramientas que permiten realizar seguimiento de ojos, son:
• OptiKey6 : es un software de seguimiento de ojos de código abierto y gratuito para
Windows, cuya función es servir de teclado de ayuda virtual y llevar el control del
teclado y el ratón para personas con limitaciones motoras y del habla que sufren
enfermedades como el ELA (Esclerosis Lateral Amiotrófica). Si el usuario no
dispone de dispositivo de seguimiento ocular, OptiKey se puede utilizar con un
ratón o cámara web. También es posible convertir en voz aquellos que hemos
escrito con el teclado virtual.
• Tobii EyeX7 : software que permite "tocar donde se mire", para una interacción
con el ordenador portátil mucho más intuitiva y natural, mejorando la experiencia
del usuario, y sin la necesidad de usar el ratón o panel táctil para dar clic en algún
punto de la pantalla. Su tecnología avanzada es capaz de atenuar el brillo de la
pantalla si el usuario no está mirándola para ahorrar así energía y dar privacidad.
Igualmente, el ordenador permanecerá activo cuando el usuario se encuentre
enfrente de la pantalla del ordenador hasta que éste se aleje de la misma.
• Gazepoint8 : empresa dedicada al seguimiento de ojos para investigadores,
personas con discapacidad, diseñadores web, empresas de marketing, entre otros.
Su software, Analysis UX Edition, permite realizar tests de usabilidad,
visualizaciones de mapas de calor, secuencias de clics, etc.
 Musculo esquelético
• Tratamiento completo de todas las
articulaciones y tejidos blandos.
• Incluir ATM (separadores bucales).

• Uso de ayudas técnicas.

• Sistemas antiescaras.
• Transporte.
 Sialorrea
• Uso de amitriptilina.
• 25-50 mg/día.
• Atropina 0.4 mg cada 4 o 6 hrs.

• Glicopirrolato 2-8 mg/día. (anticolinérgico)

• Antihistamínicos.
• Toxina botulínica.

• Radioterapia local o cirugía.

 Estreñimiento
• Dieta rica en fibra.

• Líquido.

• Estimulantes del peristaltismo.

 Prevención TVP
• Uso sistemas mecánicos.

• Anticoagulación.

• Filtro en vena cava.

 Insuficiencia respiratoria
• VMNI.
• Traqueostomía (tipo)
• VMI.
• Elección de los equipos.

• Respaldo, baterías, grupo electrógeno.

• Sistemas de succión.
• “ambú”.
 VMNI
n 1980: Sullivan, aplica
CPAP en pacientes con
Apnea Obstructiva del
Sueño

n 1990. Uso masivo de la

Ventilación con Dos
Niveles de Presión
Positiva: BiPAP® de
Respironics
VENTILADORES
1970s 1980s 1990s 2000
IMV PSV PCV Closed Loop
 Open Loop

Operador
Presión
Input RESPIRADOR Volumen
(parametros)
Paciente
Flujo

H. Abona
 Closed Loop

Operador Alteraciones

Input RESPIRADOR
+ E
(Controlador / Efector) Paciente
(parametros)
-
Flujo o Presión
Volumen

Feedback

H. Abona
 Objetivos Fisiológicos
• Manejo apoyo del intercambio gaseoso
pulmonar.
• Ventilación Alveolar.
• Oxigenación arterial.

• Aumentar el volumen pulmonar.

• Mejoría de la CRF.

• Disminución del trabajo respiratorio.

Int care med 1994;20: 64-79

 Objetivos Clínicos
• Revertir la hipoxemia.
• Revertir acidosis.
• Manejo del distress.
• Prevenir y revertir atelectasias.
• Revertir la fatiga muscular.

Int care med 1994;20: 64-79

 PROGRAMACIÓN DE LA VM

• Modalidad de ventilación
• Frecuencia respiratoria
• Volumen corriente
• Fracción inspirada de oxígeno
• PEEP
• Relación I:E
• Patrón de flujo
 CRITERIOS CONEXIÓN VENTILACIÓN MECÁNICA

• Patología con pulmón • Patología obstructiva

normal: crónica:
– Depresión del SNC – Asma refractaria
– Enf. Neuromusculares – EPOC agudizada
– Trauma craneano
– Cirugía cardíaca • Patología restrictiva:
– Fracturas costales múltiples – SDRA
– Sepsis – Edema agudo del pulmón
– Tétanos
– Cirugia
 MONITOREO DEL PACIENTE VENTILADO

• Oximetría de pulso
• Gasometría arterial
• Rayos X de Tórax
• Glicemia y electrolitos
• Cultivo de secreciones seriados
• Monitoreo de V, P, fr, FiO2
• Control de capnografía
• Monitoreo de sedación
Desde el punto de lo competente
que debemos ser?
 Competencia: definiciones
• Pero… en el trabajo.

• Iniciativas.
• Tomar decisiones.
• Enfrentar eventos aleatorios..

• “uso sonda Nº12 o Nº14?

 Competencia: definiciones
• Le Boterf.
• Actuar con competencia:
• “Seleccionar, combinar, movilizar recursos”
• Recursos propios y del entorno.
• “Pertinente” (adecuada y oportuna)

• Logro del resultado a conseguir.

 Competencia: definiciones
• Existen competencias colectivas.
• Diversidad de ámbitos o áreas (clínicos,
docentes, investigación, gestión).
• Comunicación.
• Aprenden de su historia.
• Reflexivos.

• Esto genera mística e identidad.

GRACIAS