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Chronic progressive external ophthalmoplegia (CPEO)

Clinical, morphologic, and biochemical studies

From the Department of Neurology (Drs. Mitsumoto, Nemni, and Bradley), Tufts-New England Medical Center, Boston, the Department of Biology (Dr. Aprille), Tufts University, Medford and the Unit of Neurovisual Disorders (Dr. Wray), Department of Neurology, Massachusetts General Hospital, Boston, MA.

we studied skeletal muscles from eight chronic progressive external ophthalmoplegia patients with ragged-red fibers (group A), five CPEO patients without ragged-red fibers (group B), and five controls. The EM morphometric fraction of structurally abnormal mitochondria was increased in group A, and there was a similarly increased fraction of normal-appearing mitochondria in group B. State 3 respiration and uncoupled respiration were severely decreased in both groups. The morphometric mitochondrial fraction and respiratory functions were inversely related in all three groups. The cytochrome content in group A was normal; cytochromes b and cc₁ were decreased in group B. These studies point to a central role of mitochondrial dysfunction in all types of CPEO, but the basic abnormalities remain to be elucidated.

Address correspondence and reprint requests to Dr. Mitsumoto, Neurology Service, VA Medical Center, Case Western Reserve University, Cleveland, OH 44106.

Part of this study was supported by the Muscular Dystrophy Association of America Research Fellowship (HM) and a grant from the National ALS Foundation.

Presented in part at the thirty-third annual meeting of the American Academy of Neurology in Toronto, Canada, May 1981.

Accepted for publication August 12, 1982

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