A prominent prevalence of SWJ is also identified in spinocerebellar ataxia (SCA) type 6 (80–100%), SCA3 (43–64%), ataxia-telangiectasia (31–85%) [11], SCAR4 (formerly SCASI and SCA24) [48], pointing to relevant differential diagnoses. This evidence concerns the gene VPS13D and Ataxia-telangiectasia.