A potential role of sacsin has been implicated in an early onset neurodegenerative disorder, autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), which occurs with a high prevalence in the Charlevoix-Saguenay-Lac-Saint-Jean (CSLSJ) region of Quebec [32]. The gene discussed is SACS; the disease is autosomal recessive spastic ataxia.