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Familial chorea and myoclonus epilepsy

Department of Neurology and Psychiatry, Hokkaido University School of Medicine, Sapporo (Drs. Takahata and Suzuki), the Kuchan Kosei-Hospital, Kuchan (Dr. Ito), the Ishiyama Hospital, Sapporo (Dr. Yoshimura), and the Midorigaoka Hospital, Obihiro (Dr. Nishihori).

A familial disorder was characterized by chorea, ataxia, myoclonus, convulsions, dementia, and mental retardation. In five cases, the main lesion affected cerebellar dentate nuclei, with nerve cell loss, gliosis, chromatolysis, and grumose degeneration. Fibrous glial cell proliferation was detected in the globus pallidus.

Dr. Takahata's address is North 14, West 5, Sapporo, Japan.

This study was supported in part by a research grant far specific disease from the Ministry of Health and Welfare, Japan.

Accepted for publication March 6, 1978.