PDYN and Ataxia: The frequency of <i>PDYN</i> variants is reportedly very low (~ 0.1%) in several ataxia cohorts screened to date.<h4>Case presentations</h4>We found five cases of SCA23 in two families (mean age at onset: 37.8 ± 5.5 years; mean age at examination: 64.2 ± 12.3 years) with a novel <i>PDYN</i> variant (c.644G > A:p.R215H).