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  • Title: Olivopontocerebellar atrophy in a large Iakut kinship in eastern Siberia.
    Author: Goldfarb LG, Chumakov MP, Petrov PA, Fedorova NI, Gajdusek DC.
    Journal: Neurology; 1989 Nov; 39(11):1527-30. PubMed ID: 2812335.
    Abstract:
    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.
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