Mitochondrial recessive ataxia syndrome (MIRAS) is typically found in patients with homozygous or compound heterozygous p.W748S POLG1 mutations who present with adult or juvenile-onset ataxia combined with dysarthria, sensory neuropathy, late cognitive impairment, oculomotor defects, myoclonus, tremor, psychiatric symptoms and seizures [18,29]. This evidence concerns the gene POLG and recessive mitochondrial ataxia syndrome.