JUNE 5, 2008 | VOLUME 8 | ISSUE 11

Neurology Today
AN OFFICIAL PUBLICATION OF THE AMERICAN ACADEMY OF NEUROLOGY

www.neurotodayonline.com

INSIDE

6/5/08

MYSTERY ILLNESS: Antibody pattern discovered for pork plant-related neuropathy . . . . . . . . . . . . . . . . . . 3 EPILEPSY: Phenytoin associated with greater bone loss in young women . . . . . . . . . . . . . . . . . . . . . 15

After Vaccine-Autism Case Settlement, MDs Urged to Continue I Recommending Vaccines

By Dawn Fallik

NEW AAN GUIDELINE ON VERTIGO

By Kurt Samson

IN THE LAB: Botulinum spreads to CNS tissue in mice . . . . . . . . . . . . . . . . . . . .

AP Photo/Atlanta Journal-Constitution, John Spink.

GENETICS: Association found between Gaucher mutation and Lewy Body disorders . . . . . . . . . . . . . . . . . . . 18

n the May 27 Neurology, the AAN Quality Standards Subcommittee published guidelines for the diagnosis and treatment of benign paroxysmal positional vertigo. We asked Subcommittee member Timothy C. Hain, MD, professor of neuroscience at Northwestern Universitys Feinberg School of Medicine in Chicago, to help explain the new guidelines. Dr. Hain is an expert on dizziness and hearing loss. In a sidebar, Subcommittee member Joseph M. Furman, MD, PhD, professor of otolaryngology and neurology and director of the Division of Balance Disorders at the University of Pittsburgh Medical Center, provides a case example to demonstrate how a physician might apply the recommendations.

22

At the center of the latest autism-vaccine controversy: Dr. Jon S. Poling and his wife Terry Poling with 9-year-old Hannah.

PUBLISHED BY:

arents are worried about the rising rates of autism, and doctors are unsure of the cause or how to treat it. But a federal court decision has doctors

fearing that parents will stop vaccinating their children altogether for fear of inducing autism-like symptoms which could Continued on page 8

Hypertonic Saline Found to Reverse Transtentorial Herniation

By Stephanie Cajigal

WHAT IS BENIGN PAROXYSMAL POSITIONAL VERTIGO (BPPV)? BPPV is a clinical syndrome characterized by brief recurrent episodes of vertigo triggered by changes in head position with respect to gravity. BPPV is the most common cause of recurrent vertigo, with a lifetime prevalence of 2.4 percent. Typical signs of BPPV are evoked when the head is positioned so that the plane of the affected semicircular canal is spatially vertical and aligned with gravity. This produces a paroxysm of vertigo and nystagmus, or involuntary eye movements, after a brief latency. The duration, frequency, and intensity of symptoms of BPPV vary, and spontaneous recovery occurs frequently.

T
PERIODICALS

he administration of 23.4 percent saline was associated with reversed transtentorial herniation (TTH) of the brain and reduced intracranial pressure, investigators at Johns Hopkins University reported in a retrospective study published in the March 25 Neurology. Study author Robert D. Continued on page 9

Cross section of a normal brain (left) and a brain with intracranial shifts from supratentorial lesions (right). (1) Herniation of the cingulate gyrus under the falx. (2) Herniation of the temporal lobe into the tentorial notch. (3) Downward displacement of the brain stem through the notch.

WHAT CAUSES IT? BPPV results from abnormal stimulation of the cupula within any of the three semicircular canals in the inner ear, although most cases affect the posterior canal. As fluid in the inner ear rushes by the cupula, hair cells sense rotational acceleration and transmit this information to the brain through the vestibulocochlear nerve. The brain then makes appropriate physical adjustment to maintain balance and equilibrium. Abnormal Continued on page 10

DAMS.

10| | NEUROLOGY TODAY | JUNE 5, 2008

Vertigo

Continued from page 1

stimulation of these hair cells is often caused by canaliths. The most common cause of BPPV in people younger than age 50 is head injury. In older people, the most common cause is degeneration of the vestibular system of the inner ear. BPPV becomes much more common with advancing age. In half of all cases, BPPV are idiopathic.

WHAT ARE CANALITHS? Canaliths are tiny particles of calcium carbonate, or limestone, attached to tiny hairs in the inner ear. They are found in all normal ears, but sometimes become detached from the hair cells and can cause dizziness when they move about within the inner ear canals.

DESCRIBE THE THREE BASIC TYPES OF BPPV: POSTERIOR, ANTERIOR, AND HORIZONTAL CANAL BPPV. Within the inner ear there are three semicircular canals, and a specific type of BPPV is associated with each one. The most common type is posterior canal (PC) BPPV. It is the most common because it is the lowest canal in the inner ear and therefore detached canaliths tend to remain there. In PC BPPV, dizziness

and nystagmus are triggered when the head rotates about the axis of the PC, usually, but not always, when a patient lies down on one side. PC BPPV is only triggered on one side. The nystagmus direction a mixture of upbeating and torsion is unusual in other conditions, so PC BPPV can be easily diagnosed. [Nystagmus can be horizontal (on lateral gaze) or vertical (upbeating or downbeating, determined by the faster component up or down).] The second most common type of BPPV is horizontal canal (HC) BPPV. Canaliths that fall into it also tend to easily move out again. In HC BPPV dizziness and nystagmus is triggered when the head is moved to either side. HC BPPV nystagmus is horizontal rather than upbeating, and it is generally stronger than the nystagmus of PC BPPV. Horizontal nystagmus similar to that of HC BPPV can occur in other conditions, such as cerebellar disorders, but HC BPPV is nevertheless the most common source of this nystagmus pattern. The least common type of BPPV is anterior canal (AC) BPPV. In order to get into the anterior canal, canaliths must migrate upward with respect to the upright head, and this is unusual. In AC BPPV, symptoms are mainly triggered with the positioned head straight but turned backwards, and nystagmus is down beating, although this can also occur in other central conditions.

CANALITH REPOSITIONING TREATMENT FOR RIGHT-SIDED BENIGN PAROXYSMAL POSITIONAL VERTIGO

A Hypothetical Case Workup for Vertigo

By Joseph M. Furman, MD, PhD

57 year-old man presents to a neurologist complaining of episodes during the past week of a spinning sensation that is sometimes accompanied by mild nausea. The duration of the episodes is less than one minute. The episodes are all triggered by certain types of head movements including tilting the head back and turning toward the right side while lying in bed. He denies any other neurologic symptoms, tinnitus, or hearing loss. Past medical history is unremarkable. On physical examination, the patient is in no acute distress. His vital signs are normal. The cranial nerve examination is normal, including normal ocular motor function. The remainder of the neurological examination is also normal. Gait is intact.

Steps 1 and 2 are identical to the Dix-Hallpike maneuver. The patient is held in the right head hanging position (step 2) for 20 to 30 seconds, and then (step 3) the head is turned 90 degrees toward the unaffected side; the head is held for 20 to 30 seconds before turning the head another 90 degrees (step 4) so the head is nearly in the face-down position. The movement of the otolith material within the labyrinth is depicted with each step, showing how otoliths are moved from the semicircular canal to the utricle. It is the patients head position that is the key to a successful treatment.

WHAT IS THE RECURRENCE OR RELAPSE RATE FOR BPPV? Short-term relapses rates range from 7 percent to nearly 23 percent within a year of treatment. Over about five years, long-term recurrences may approach 50 percent.

of the inner ear. There is strong evidence for the effectiveness of the Epley maneuver. The Semont maneuver does not have as much evidence for effectiveness, but the head positioning is similar to the Epley maneuver.

POSITIONAL TESTING
A Dix-Hallpike maneuver was performed into the head-hanging left position, which did not trigger any symptoms or nystagmus. The patient was then brought back up to the sitting position. Next, a Dix-Hallpike maneuver was performed into the head-hanging right position. After approximately five seconds in this position, the patient reported the onset of his typical symptoms of vertigo. A burst of upbeat-torsional nystagmus was seen. The torsional component was such that the upper poles of the eyes beat toward the right shoulder. The intensity of the vertigo and nystagmus gradually decreased over 20 seconds until they were no longer present. A canalith repositioning treatment was performed. The diagnosis in this case was BPPV involving the right posterior canal. The nystagmus was triggered in the right Dix-Hallpike position. With BPPV of the posterior canal, upbeat-torsional nystagmus is triggered by the Dix-Hallpike test toward the affected side, as in this case toward the right. According to the AAN BPPV Guidelines, treatment in this case should include a canalith repositioning treatment.The Epley maneuver is an effective and safe therapy, while weaker evidence supports the Semont maneuver. There is insufficient evidence to indicate any benefit of mastoid vibration during a canalith repositioning maneuver, post-treatment restrictions such as keeping the head more or less erect for one to two days, or prescription of medications. Note that if a patient is not treated with a canalith repositioning maneuver, that transitory downbeat torsional nystagmus may be triggered after the patient is brought from the Dix-Hallpike position back up to the sitting position.

WHAT TECHNIQUES CAN BE USED TO TREAT POSTERIOR CANAL BPPV? Canalith repositioning maneuvers, originated by John Epley, MD, and Alain Semont, MD, are used for treating PC BPPV. Both maneuvers take about 10 minutes to

WHAT TECHNIQUES CAN BE USED FOR ANTERIOR AND HORIZONTAL CANAL BPPV? Similar canalith repositioning maneuvers for treatment of anterior and horizontal canal BPPV have been proposed, but convincing evidence for their effectiveness is

There is strong evidence for the effectiveness of the Epley maneuver.

perform, and involve a series of positions of the head in which canaliths are allowed to move within the inner ear. At the end of a successful maneuver, the canaliths have been moved into an insensitive part not yet available. Maneuvers for PC BPPV do not work for HC BPPV. The head positions of the proposed specific maneuvers are modified in order to move canaliths within these canals which are

JUNE 5, 2008| | NEUROLOGY TODAY | 11

perpendicular to the posterior canal. The HC maneuvers are moderately effective for HC BPPV. There is no current recommendation of a maneuver for AC BPPV.

The Brandt-Daroff exercises are an early form of canalith repositioning exercises. They are less effective than the later maneuvers developed by Epley and Semont.

treatment that inactivates the part of the ear that causes most BPPV. Singular neurectomy is another surgical treatment in which the nerve to the posterior canal is cut. While all available studies suggest

WHAT RESEARCH IS NEEDED TO BETTER UNDERSTAND AND TREAT THIS DISORDER? Class I (prospective, blinded) studies are still needed to clarify the best treatments for horizontal and anterior canal BPPV.

ARE ANY RESTRICTIONS NECESSARY FOR PATIENTS AFTER UNDERGOING THESE POSITIONING TECHNIQUES? Most studies indicate that restrictions are not necessary.

WHAT IS MASTOID VIBRATION AND IS IT IMPORTANT FOR THE EFFICACY OF THESE MANEUVERS? Mastoid vibration is the use of a massagerlike device to oscillate the affected ear during the canalith repositioning maneuvers. Most studies indicate that mastoid vibration is of no benefit.

The most common cause of BPPV in people younger than age 50 is head injury. In older people, the most common cause is degeneration of the vestibular system of the inner ear.

There are self-administered variants of both the Epley and Semont maneuvers for PC BPPV. There is presently insufficient evidence regarding the effectiveness of either of these maneuvers.

that surgery is effective, the studies were not of high enough quality to provide strong evidence of efficacy. Surgical treatments also are associated with a small risk of deafness.

WHAT ARE HABITUATION EXERCISES, AND HOW ARE THEY USED TO TREAT PATIENTS? Habituation exercises are procedures designed to elicit symptoms over and over, in the hopes that this will provoke a CNS reaction that will diminish the response. These exercises are ineffective.

ARE ANY MEDICATIONS EFFECTIVE FOR BPPV? Medications are not recommended for routine treatment of BPPV.

ARE THERE ANY EFFECTIVE SURGICAL TREATMENTS? Posterior canal occlusion is a surgical

WHAT RESOURCES ARE AVAILABLE FOR CLINICIANS TO LEARN MORE ABOUT THESE TECHNIQUES? Courses are given at the AAN on these maneuvers. Descriptions of all the maneuvers are available at this URL: www.dizziness-and-balance.com/ disorders/bppv/bppv.html.

REFERENCES: Fife TD, Iverson DJ, Gronseth GS, et al. Practice parameter: Therapies for benign paroxysmal positional vertigo (and evidence-based review) Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2008;70:2067-2074. Brandt T, Daroff RB. Physical therapy for benign paroxysmal positional vertigo. Arch Otolaryngol 1980;106:484-485. Brandt T, Steddin S, Daroff RB. Therapy for benign paroxysmal positioning vertigo, revisited. Neurology 1994;44:796-800. Epley JM. The canalith repositioning procedure: For treatment of benign paroxysmal positional vertigo. Otolaryngol Head Neck Surg 1992; 107:399-404. Semont A, Freyss G, Vitte E. Curing the BPPV with a liberatory maneuver. Advances in Otorhinolaryngology 1988;42:290-293.