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KMID : 0359719950130040970
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Journal of the Korean Neurological Association
1995 Volume.13 No. 4 p.970 ~ p.978
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Neurophysiological Studies in a Patient with Hyperekplexia
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이명식
이상복/김용덕/김원찬/최영철
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Abstract
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The results of neurophysiological studies in a 20 year-old man who had pathological jumps and falls following unexpected auditory stimuli is presented. He also had action induced hemidystonia and polymyoclonus involving fingers of the both hands.
Somatosensory evoked potential study showed no giant cortical wave and jerk-locked back averaging electroencephalogram showed no jerk related cortical event. Polymyographic study after auditory stimuli showed electromyographic (EMG0 bursts
recruitment
pattern consistent with hyprekplexia and reticular reflex myoclonus. EMG bursts spread rostrally and caudally from the sternocleidomastoid musle via a slowly conducting neuronal pathways. These findings are compatibie with hyperekplexia.
Pathologically
increased gain of the brainstem reticular formation seems to cause hyperekplexia.
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KEYWORD
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