These include POLG-related diseases (i.e., ataxia-myopathy syndrome [6]; “mitochondrial recessive ataxia syndrome”, or MIRAS [7]; ataxia with ocular myopathy and, more rarely, with psychiatric comorbidities or epilepsy [8]) and some C10orf2 (Twinkle) mutations, which can cause infantile-onset spinocerebellar ataxia (IOSCA), a severe neurodegenerative disorder characterized by progressive atrophy of the brainstem, cerebellum, and spinal cord and sensory axonal neuropathy [9]. Here, POLG is linked to cerebellar ataxia.