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Journal of Child Neurology
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Topical Review: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Strokelike Episodes (MELAS): Current Concepts

Michio Hirano, MD

Department of Neurology Columbia-Presbyterian Medical Center

Steven G. Pavlakis, MD

Departments of Neurology and Pediatrics Cornell University Medical College, New York, NY

Mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome is one of many mitochondrially inherited multisystem diseases. The features of 110 reported mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes patients are reviewed to define the clinical spectrum of this disease. The clinical disorder, in addition to emerging concepts of genetic etiology, is promoting our understanding of mitochondrial functions. New knowledge may lead to more rational therapies. Finally, the recent revolution in the study of mitochondrial diseases may further our understanding of other degenerative disorders and even aging. (J Child Neurol 1994;9:04-13).

Journal of Child Neurology, Vol. 9, No. 1, 4-13 (1994)
DOI: 10.1177/088307389400900102
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