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Department of Psychiatry (Dr. Naito) School of Medicine, Niigata University, Niigata, Japan, and the Psychiatric Research Institute of Tokyo (Dr. Oyanagi), Tokyo, Japan.
We studied five families with a syndrome of myoclonus, epilepsy, dementia, ataxia, and choreoathetosis. At autopsy, the major neuropathologic changes consisted of combined degeneration of the dentatorubral and pallidoluysian systems. The pattern of inheritance implied an autosomal-dominant trait. We have proposed a diagnostic nomenclature for this disease, "hereditary dentatorubral-pallidoluysian atrophy."
Address correspondence and reprint requests to Dr. Naito, Department of Psychiatry, School of Medicine, Niigata University, Asahi-machi 1, Niigata-shi 951, Japan.
Accepted for publication January 29, 1982.
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