Mutations in the gene encoding spastin (SPAST/SPG4; MIM:604277) are the most common cause of autosomal dominant uncomplicated “pure” HSP (MIM:182601) in North America and northern Europe, accounting for ~40% of cases (Hazan et al., 1999; Blackstone et al., 2011). Here, SPAST is linked to hereditary spastic paraplegia.