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Medical Definition of Mitochondrial encephalomyopathies
1. Brain diseases associated with disease of the muscles, accompanied by morphological changes in the muscle mitochondria and their metabolism. The mitochondrial encephalopathies are often multisystemic and vary considerably in age at onset, distribution of weakness, severity, and course. Mitochondrial cytochrome c oxidase deficiency appears to be implicated and often several mitochondrial enzymes are affected in the same patient. The brain lesions can be said to be clinical and morphological expressions of a mitochondrial defect. (12 Dec 1998)