Recently, Deschauer et al. described a patient showing stroke-like episodes and a right occipital lesion, headache, seizures, elevated CSF lactate, ragged–red fibers and carrying heterozygous mutations in mtDNA polymerase gene (POLG1) arguing that MELAS could be included in POLG1 spectrum phenotype [7]. The gene discussed is POLG; the disease is stroke disorder.