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NEUROLOGY 1987;37:58
© 1987 American Academy of Neurology

Mitochondria1 encephalomyopathy and partial cytochrome c oxidase deficiency

S. Servidei, MD, R. P. Lazaro, MD, E. Bonilla, MD, K. D. Barron, MD, M. Zeviani, MD and S. DiMauro, MD

Department of Neurology (Drs. Servidei, Bonilla, Zeviani, and DiMauro), H. Houston Merritt Clinical Research Center for Muscular Dystrophy and Related Diseases, Columbia-Presbyterian Medical Center, New York. NY and the Department of Neurology (Drs. Lazaro and Barron), Albany Medical College, Albany, NY.

A 52-year-old man had slowly progressive weakness and wasting of limb-muscles, sensorineural hearing loss, and complex partial seizures. CT showed cerebral atrophy, but he was not demented. Muscle biopsy showed ragged-red fibers and decreased histochemical stain for cytochrome c oxidase. Biochemical studies showed decreased cytochrome c oxidase activity in crude muscle extracts and in isolated mitochondria (44 and 30% of normal), while other mitochondrial enzymes were normal. A comparable decrease of immunologically reactive enzyme protein was shown by immunotitration with antibodies against human heart cytochrome c oxidase. Partial defects of cytochrome c oxidase may cause adult-onset, slowly progressive mitochondrial encephalomyopathies.

Address correspondence and reprint requests to Dr. DiMauro, 4–420 College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032.

Supported by National Institute of Neurological and Communicative Disorders and Stroke center grant NS-11766 and a Muscular Dystrophy Association center grant. Dr. Servidei was supported by a Fellowship from the Unione, Italian Lotta Contro La Distrofia Muscolare (UILDM), Sezione Laziale "Giulia Testore," and Dr. Zeviani by a fellowship from the Muscular Dystrophy Association.

Received March 21, 1986. Accepted for publication May 14, 1986.




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