RT Journal Article
SR Electronic
T1 Treatment of Kearns‐Sayre syndrome with coenzyme Q<sub>10</sub>
JF Neurology
JO Neurology
FD Lippincott Williams &amp; Wilkins
SP 45
OP 45
DO 10.1212/WNL.36.1.45
VO 36
IS 1
A1 Ogasahara, Saburo
A1 Nishikawa, Yoshiro
A1 Yorifuji, Shiro
A1 Soga, Fumihisa
A1 Nakamura, Yusaku
A1 Takahashi, Mitsuo
A1 Hashimoto, Shigeo
A1 Kono, Norio
A1 Tarui, Seiichiro
YR 1986
UL http://n.neurology.org/content/36/1/45.abstract
AB 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.