To date, all individuals identified with TDP2 mutations exhibit a similar pathology of intellectual disability, seizures, and ataxia; a disease now denoted spinocerebellar ataxia autosomal recessive 23 (SCAR23) (Gómez-Herreros et al. 2014; Zagnoli-Vieira et al. 2018; Ciaccio et al. 2019; Errichiello et al. 2020; Zoghi et al. 2021). Here, TDP2 is linked to spinocerebellar ataxia, autosomal recessive 23.