WM damage is known to occur in SCA, as histopathological studies show myeline loss in SCA3 patients [8, 9, 10], while animal models of SCA1 [11] and SCA3 [12, 13, 14] show impaired oligodendrocyte maturation with reduced myeline thickness in regions containing fewer mature oligodendrocytes [14]. This evidence concerns the gene ATXN3 and autosomal dominant cerebellar ataxia.