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]. The gene discussed is POLG; the disease is sensory peripheral neuropathy.