Olivopontocerebellar atrophy in a large Iakut kinship in Eastern Siberia
- 1 November 1989
- journal article
- Published by Wolters Kluwer Health in Neurology
- Vol. 39 (11) , 1527
- https://doi.org/10.1212/wnl.39.11.1527
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.Keywords
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