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NEUROLOGY 1989;39:1527
© 1989 American Academy of Neurology

Olivopontocerebellar atrophy in a large Iakut kinship in Eastern Siberia

L. G. Goldfarb, MD, M. P. Chumakov, MD, P. A. Petrov, MD, N. I. Fedorova, MD and D. C. Gajdusek, MD

From the National Institute of Neurological Disorders and Stroke (Drs. Goldfarb and Gajdusek), National Institutes of Health, Bethesda, MD; the Institute of Poliomyelitis and Virus Encephalitides (Drs. Chumakov and Fedorova), Moscow, USSR; and Iakutsk State University (Dr. Petrov), Iakutsk, USSR.

One-hundred cases of olivopontocerebellar atrophy, type 1, were found and studied in the Iakut population of Eastern Siberia. The disease followed a slowly progressive course of cerebellar insufficiency caused by degeneration in the cerebellar cortex, nuclei pontis, and inferior oliva. The disorder shows an autosomal dominant pattern of inheritance with a lower penetrance in females. The disease spread from a small region in the Aldan valley 200 to 300 years ago.

Address correspondence and reprint requests to Dr. Goldfarb, NINDS, National Institutes of Health, Bldg. 36, Rm. 5B21, Bethesda, MD 20892.

Received March 28,1989. Accepted for publication in final form May 5, 1989.




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