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Cortical Myoclonus in Huntington's Disease Movement Disorders 9:633-641, Thompson,P.D.,et al, 1994 See this aricle in Pubmed Article Abstract We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalized and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease. which may lead to substantial diagnostic difficulties.

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(click to filter results - removes previous filter) clonazepam Huntington's chorea movement disorder,treatment of myoclonus myoclonus,action myoclonus,cortical myoclonus,stimulus sensitive piracetam sodium valproate treatment of neurologic disorder

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