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We report an unusual case of Leigh syndrome due to the m.10191T&gt;C mutation in the complex I gene MT-ND3. This mutation has been associated with a spectrum of clinical phenotypes ranging from infant lethality to adult onset. Despite infantile onset and severe symptoms, our patient has survived to early adulthood because of a strict dietary regimen and parental care. This patient is an extreme example of the frequently prolonged course of Leigh syndrome due to this particular mutation.

Long Survival in Patients With Leigh Syndrome and the m.10191T>C Mutation in MT-ND3