Rinsho Shinkeigaku. 2004 Sep;44(9):639-42.

[Cortical reflex myoclonus in adult onset Huntington's disease].

[Article in Japanese]

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Department of Neurology, National Hospital Organization Hyogo Chuo National Hospital.

Abstract

We report a case of cortical reflex myoclonus in adult onset Huntington's disease (HD). The patient is a 51-year-old woman. Chorea and myoclonus were observed on her face and extremities. Neurophysiological tests showed C reflex and abnormal waves preceding myoclonus by jerk-locked back averaging method but no giant somatosensory evoked potential. Gene analysis revealed the prolongation of CAG repeats (13/44) in IT15 gene. Oral administration of clonazepam was transiently effective for myoclonus. We should inscribe that the cortical reflex myoclonus may exceptionally manifest in HD.

PMID:: 15515712; [PubMed - indexed for MEDLINE]

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