Mutations in SPTBN2 (encoding the membrane scaffold protein β-III SPECTRIN) are associated with Spinocerebellar Ataxia Type 5 (SCA5) (Cho and Fogel, 2013; Ikeda et al., 2006; Nicita et al., 2019; Ranum et al., 1994; Wang et al., 2014), Spinocerebellar Ataxia Autosomal Recessive 14 (SCAR14) (Elsayed et al., 2014; Lise et al., 2012; Yıldız Bölükbaşı et al., 2017), and ataxic cerebral palsy (Parolin Schnekenberg et al., 2015). Here, SPTBN2 is linked to Spectrin-associated autosomal recessive cerebellar ataxia.