Leigh Syndrome, also termed subacute necrotizing encephalomyelopathy, presents as a progressive neurometabolic disorder and often results from mitochondrial DNA mutation. Such mutations disrupt the mitochondria’s ability to produce the cellular energy. Furthermore, the central nervous system appears to be most directly affected by mitochondrial dysfunction. The onset of Leigh syndrome is usually prior to 2 years of age, and although onset in adolescence and early adulthood is possible, it is rare (National Institute of Neurological Disorders and Stroke [NINDS], 2001).