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• Vol.44 No.9
• CLINICA NEUROL, 44: 639−642, 2004
• Vol.44 No.9 contents

Brief Clinical Note

Cortical reflex myoclonus in adult onset Huntington's disease

Itaru Funakawa, M.D., Kenichi Kimura, M.D. and Kenji Jinnai, M.D.

Department of Neurology, National Hospital Organization Hyogo Chuo National Hospital

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.

(CLINICA NEUROL, 44: 639−642, 2004)
key words: Huntington's disease, cortical reflex myoclonus, jerk-locked back averaging, c reflex

(Received: 12-Dec-03)