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Long-term coenzyme Q₁₀ therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency

A ³¹P NMR study

Second Department of Internal Medicine (Drs. Nishikawa, Takahashi, Yorifuji, Nakamura, Ueno, and Tarui)
Department of Radiology, Osaka University Medical School, National Cardiovascular Center, Osaka, Japan. (Dr. Kozuka)
Department of Radiology, National Cardiovascular Center, Osaka, Japan. (Dr. Nishimura)

For 2 years we administered high doses of coenzyme Q₁₀ (CoQ) to a patient having mitochondrial encephalomyopathy with cytochrome c oxidase deficiency. Abnormal elevation of the serum lactate per pyruvate ratio and the increased concentration of serum lactate plus pyruvate induced by exercise decreased with CoQ treatment. This therapeutic effect continued for 2 years. ³¹P nuclear magnetic resonance spectroscopy showed acceleration of the postexercise recovery of the ratio of phosphocreatine to inorganic phosphate in muscle during CoQ treatment. These observations support the beneficial effect of CoQ on the impaired mitochondrial oxidative metabolism in muscle. Also, impaired central and peripheral nerve conductivities consistently improved during CoQ treatment. These results indicate that CoQ has clinical value in the long-term management of patients with mitochondrial encephalomyopathies, even though there are clinical limitations to the effects of this therapy.

Address correspondence and reprint requests to Dr. Nishikawa, The Second Department of Internal Medicine, Osaka University Medical School, 1-1-50 Fukushima, Fukushima-ku, Osaka 553, Japan.

Supported by a grant (62-2) from the National Center of Neurology and Psychiatry (NCNP) of the Ministry of Health and Welfare, Japan, and grant-in-aid (60117007) for Special Project Research of Selected Intractable Neurological Disorders from the Ministry of Education, Science and Culture, Japan.

Received March 11, 1988. Accepted for publication in final form September 27, 1988.

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