Statistically significant enrichment of rare (minor allele frequency < 1 × 10–5) probably damaging SPTAN1 variants was identified in families with hereditary ataxia (HA) or hereditary spastic paraplegia (HSP) (12/1142 cases vs 52/23,847 controls, p = 2.8 × 10–5). Here, SPTAN1 is linked to hereditary ataxia.