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NEUROLOGY 1986;36:45
© 1986 American Academy of Neurology

Treatment of Kearns-Sayre syndrome with coenzyme Q10

Saburo Ogasahara, MD, Yoshiro Nishikawa, MD, Shiro Yorifuji, MD, Fumihisa Soga, MD, Yusaku Nakamura, MD, Mitsuo Takahashi, MD, Shigeo Hashimoto, MD, Norio Kono, MD and Seiichiro Tarui, MD

Second Department of Internal Medicine, Osaka University Medical School, Fukushima 1–1–50, Fukushima-ku, Osaka 553, Japan, and The Second Department of Pathology, Kinki University Medical School, Sayama, Osaka 553, Japan (Dr. S. Hashimoto).

We studied the metabolism of coenzyme Q10 (CoQ) and the effects of CoQ therapy in five patients with Kearns-Sayre syndrome (KSS). Although the mitochondrial fraction was increased in muscles from KSS patients, CoQ content was slightly low. CoQ synthesis was normal in fibroblasts from KSS patients. Administration of 120 to 150 mg/d of CoQ improved abnormal metabolism of pyruvate and NADH oxidation in skeletal muscle. CoQ therapy decreased CSF protein concentration and CSF lactate/pyruvate ratio. ECG abnormalities and neurologic symptoms also improved.

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

Supported by part of Grant No. 84–08 from NCMMD of the Ministry of Health and Welfare of Japan, and a Basic Research Grant from the Muscular Dystrophy Association of the United States.

Accepted for publication May 15, 1985.




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