<Case Reports>A case of chronic and progressive choreiform movemetnt disorder with evident familial History

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A case of chronic and progressive choreiform movement disorder with evident familial history was reported. The patient, a 68-year-old housewife, developed typical involuntary movement and psychotic symptoms concomitant with Huntington's chroea. Familial history genetically indicates autosomal dominant penetrance. Her elder sister was diagnosed with Huntington's chorea at a local public hospital. Her son was diagnosed with chronic schizophrenia and has been treated medically. Choreiform movement, however, was not indicated in the son. Thus familial history of movement disorder is significant. However, certain factors made the diagnosis of Huntington's chorea questionable in this case. Atrophy of the caduate nucleus was not evident in plain brain CT films, although hypodensity of the cerebral whitematter was not negligible. Cerebellar signs such as dysmetria or ataxic gait were prominent though in the later stage. These results strongly suggest that this was a case of pseudo-Huntington form (Hirayama) of dentatoruburopallidoluysian atrophy rather than Huntington's chorea.