Autosomal-recessive ataxia type 2 (ARCA2—OMIM #612016), also identified as COQ8A-ataxia, is a rare form of multisystemic ataxia characterized by slowly progressive early onset ataxia, combined with variable symptoms including exercise intolerance (25% of patients), dystonia (28%), epilepsy (32%), and intellectual disability (49%) [151,152,153]. This evidence concerns the gene COQ8A and cerebellar ataxia.