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We report on a case with adult-onset LS related to a  mutation. Two years after the onset of symptoms of LS, the proband developed a parkinson-like disease. The c.626C>T mutation is the most common pathogenic mutation found in 22 patients reported earlier in the literature with a defect in . The age of the previously reported cases varied between 14 months and 24 years. Our report expands the phenotypical spectrum of -related neurologic disease and provides clinical evidence for involvement of  in extrapyramidal syndromes.

Leigh syndrome followed by parkinsonism in an adult with homozygous c.626C>T mutation in MTFMT