title: Olivopontocerebellar atrophy in a large Iakut kinship in eastern Siberia.
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.
authors: National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892.
authors: L G
authors: LG
authors: Goldfarb
authors: M P
authors: MP
authors: Chumakov
authors: P A
authors: PA
authors: Petrov
authors: N I
authors: NI
authors: Fedorova
authors: D C
authors: DC
authors: Gajdusek
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005006:Ethnicity; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008875:Middle Aged; D009420:Nervous System / Q000473:pathology; D009849:Olivopontocerebellar Atrophies / Q000208:ethnology / Q000235:genetics* / Q000473:pathology; D010375:Pedigree; D012800:Siberia; D013132:Spinocerebellar Degenerations / Q000235:genetics*