Facial action myoclonus in patients with olivopontocerebellar atrophy
Identifieur interne : 005B77 ( Main/Exploration ); précédent : 005B76; suivant : 005B78Facial action myoclonus in patients with olivopontocerebellar atrophy
Auteurs : Jau-Shin Lou ; Josep Valls-Solé ; Camilo Toro ; HallettSource :
- Movement Disorders [ 0885-3185 ] ; 1994.
English descriptors
- KwdEn :
- Action myoclonus, Adult, Atrophy, Atrophy, olivopontocerebellar (OPCA), Brain Stem (pathology), Brain Stem (physiopathology), Cerebellum (pathology), Cerebellum (physiopathology), Electromyography, Electromyography (EMG), Facial Expression, Facial Muscles (innervation), Fasciculation (diagnosis), Fasciculation (physiopathology), Female, Functional Laterality (physiology), Humans, Magnetic Resonance Imaging, Male, Middle Aged, Motor Neurons (physiology), Muscle Contraction (physiology), Myoclonus (diagnosis), Myoclonus (physiopathology), Myokymia, facial, Neurologic Examination, Olivopontocerebellar Atrophies (diagnosis), Olivopontocerebellar Atrophies (physiopathology).
- MESH :
- diagnosis : Fasciculation, Myoclonus, Olivopontocerebellar Atrophies.
- innervation : Facial Muscles.
- pathology : Brain Stem, Cerebellum.
- physiology : Functional Laterality, Motor Neurons, Muscle Contraction.
- physiopathology : Brain Stem, Cerebellum, Fasciculation, Myoclonus, Olivopontocerebellar Atrophies.
- Adult, Atrophy, Electromyography, Facial Expression, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Neurologic Examination.
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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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Action myoclonus</term>
<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>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Fasciculation</term>
<term>Myoclonus</term>
<term>Olivopontocerebellar Atrophies</term>
</keywords>
<keywords scheme="MESH" qualifier="innervation" xml:lang="en"><term>Facial Muscles</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain Stem</term>
<term>Cerebellum</term>
</keywords>
<keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Functional Laterality</term>
<term>Motor Neurons</term>
<term>Muscle Contraction</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Brain Stem</term>
<term>Cerebellum</term>
<term>Fasciculation</term>
<term>Myoclonus</term>
<term>Olivopontocerebellar Atrophies</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Atrophy</term>
<term>Electromyography</term>
<term>Facial Expression</term>
<term>Female</term>
<term>Humans</term>
<term>Magnetic Resonance Imaging</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Neurologic Examination</term>
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<front><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>
</front>
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<name sortKey="Lou, Jau Hin" sort="Lou, Jau Hin" uniqKey="Lou J" first="Jau-Shin" last="Lou">Jau-Shin Lou</name>
<name sortKey="Toro, Camilo" sort="Toro, Camilo" uniqKey="Toro C" first="Camilo" last="Toro">Camilo Toro</name>
<name sortKey="Valls Ole, Josep" sort="Valls Ole, Josep" uniqKey="Valls Ole J" first="Josep" last="Valls-Solé">Josep Valls-Solé</name>
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