Movement Disorders (revue)

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Facial action myoclonus in patients with olivopontocerebellar atrophy

Identifieur interne : 005B77 ( Main/Exploration ); précédent : 005B76; suivant : 005B78

Facial action myoclonus in patients with olivopontocerebellar atrophy

Auteurs : Jau-Shin Lou ; Josep Valls-Solé ; Camilo Toro ; Hallett

Source :

RBID : ISTEX:47D56128AD1CF981D9DDC5B4D2FAFA17D73D2E5C

English descriptors

Abstract

We studied four patients with familial olivopontocerebellar atrophy (OPCA) who had abnormal twitching of the cheeks and perioral muscles induced by facial movements. With the muscles at rest, electromyographic (EMG) recordings of the orbicularis oris and risorius muscles revealed myokymic discharges in the absence of visible movements. With voluntary contraction, the EMG showed synchronous discharges in the orbicularis oris and risorius muscles ipsilaterally associated with visible twitching. The duration of the EMG bursts was 10 to 75 ms with a frequency of 8 to 25 Hz, which suggested that the abnormal twitching was most consistent with a myoclonic disorder. Because it was induced by activation of the facial muscles, this movement disorder represents a form of action myoclonus.

Url:
DOI: 10.1002/mds.870090218


Affiliations:


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Le document en format XML

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<term>Adult</term>
<term>Atrophy</term>
<term>Atrophy, olivopontocerebellar (OPCA)</term>
<term>Brain Stem (pathology)</term>
<term>Brain Stem (physiopathology)</term>
<term>Cerebellum (pathology)</term>
<term>Cerebellum (physiopathology)</term>
<term>Electromyography</term>
<term>Electromyography (EMG)</term>
<term>Facial Expression</term>
<term>Facial Muscles (innervation)</term>
<term>Fasciculation (diagnosis)</term>
<term>Fasciculation (physiopathology)</term>
<term>Female</term>
<term>Functional Laterality (physiology)</term>
<term>Humans</term>
<term>Magnetic Resonance Imaging</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Motor Neurons (physiology)</term>
<term>Muscle Contraction (physiology)</term>
<term>Myoclonus (diagnosis)</term>
<term>Myoclonus (physiopathology)</term>
<term>Myokymia, facial</term>
<term>Neurologic Examination</term>
<term>Olivopontocerebellar Atrophies (diagnosis)</term>
<term>Olivopontocerebellar Atrophies (physiopathology)</term>
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<term>Fasciculation</term>
<term>Myoclonus</term>
<term>Olivopontocerebellar Atrophies</term>
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<term>Facial Muscles</term>
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<term>Cerebellum</term>
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<div type="abstract" xml:lang="en">We studied four patients with familial olivopontocerebellar atrophy (OPCA) who had abnormal twitching of the cheeks and perioral muscles induced by facial movements. With the muscles at rest, electromyographic (EMG) recordings of the orbicularis oris and risorius muscles revealed myokymic discharges in the absence of visible movements. With voluntary contraction, the EMG showed synchronous discharges in the orbicularis oris and risorius muscles ipsilaterally associated with visible twitching. The duration of the EMG bursts was 10 to 75 ms with a frequency of 8 to 25 Hz, which suggested that the abnormal twitching was most consistent with a myoclonic disorder. Because it was induced by activation of the facial muscles, this movement disorder represents a form of action myoclonus.</div>
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