Stroke-like episodes have been sporadically described as additional neuroradiological and clinical features in other mitochondrial disorders such as Alpers disease (37), coenzyme Q10 deficiency (38), 3-methylcrotonyl-CoA carboxylase 1 deficiency (39) and Leigh Syndrome French Canadian syndrome due to LRPRRC gene defect (40). This evidence concerns the gene MCCC1 and Stroke.