,2 Variants in SYNE1 are associated with slowly progressive cerebellar syndromes with an adult onset, including autosomal recessive cerebellar ataxia type 1 or spinocerebellar ataxia type 8 (ARCA1/ SCAR8, [OMIM: 610743]), also known as ataxia of Beauce, named after the region in Quebec, Canada, with high prevalence of the disease.1 The gene discussed is SYNE1; the disease is cerebellar ataxia.