Our data suggest that the majority of cases have a disease onset in late adulthood with a combination of dysarthria, ataxia, parkinsonism, and cognitive decline consistent with the phenotypes previously reported in MYORG mutations5,6,16, –, 19 and other autosomal dominant PFBC-causing genes.20 However, parkinsonism with supranuclear gaze palsy was frequently observed (37.8% of cases) in our cohort and has not been previously described in MYORG mutation carriers. Here, MYORG is linked to Ataxia.