A rare disorder characterized by a slowly progressive pure cerebellar ataxia associated with dysarthria. It has been described in 53 individuals from 26 families of Canadian origin. The mode of transmission is autosomal recessive. Positional cloning has led to the identification of several gene mutations. The disease is MONDO_0012549 (autosomal recessive ataxia, Beauce type). Also known as: ARCA1, SCAR8, autosomal recessive cerebellar ataxia type 1, spinocerebellar ataxia, autosomal recessive type 8.