[Cortical reflex myoclonus in adult onset Huntington's disease].
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.
Funakawa
Itaru
I
Department of Neurology, National Hospital Organization Hyogo Chuo National Hospital.
Kimura
Kenichi
K
Jinnai
Kenji
K
D002540:Cerebral Cortex / Q000503:physiopathology; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications*; D008875:Middle Aged; D009207:Myoclonus / Q000209:etiology* / Q000503:physiopathology