The clinical picture of SCA1 in the Khabarovsk Region is characterized, along with cerebellar symptoms, by more frequent manifestations of pyramidal, extrapyramidal, cognitive, autonomic and psychiatric disorders. The age of patients was 34,1 ± 4,2 years, and the age of disease onset was 31,4 ± 2,3 years. The number of CAG-repeats in the ATXN1 gene varied from 43 to 63. The non-mutated ATXN1 gene in the Khabarovsk Region population contained 29,0 ± 0,4 CAG-repeats. The prevalence of SCA1 in the Khabarovsk Region was 1:100 000 of the population which corresponds to the world prevalence of 1–3:100 000 of the population.
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