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  • Title: [Cortical reflex myoclonus in adult onset Huntington's disease].
    Author: Funakawa I, Kimura K, Jinnai K.
    Journal: Rinsho Shinkeigaku; 2004 Sep; 44(9):639-42. PubMed ID: 15515712.
    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.
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