Spinocerebellar ataxia type 34 (SCA34 or ATX-ELOVL4; OMIM #133190) is a subtype of SCA [1] first described in 1972 by Giroux and Barbeau, who reported autosomal dominant segregation of both ataxia and erythrokeratoderma variabilis (EKV) in a French-Canadian family [2]. This evidence concerns the gene ELOVL4 and autosomal dominant cerebellar ataxia.