Improvement of abnormal pyruvate metabolism and cardiac conduction defect with coenzyme Ql0 in Kearns‐Sayre syndrome

摘要: In a patient with Kearns-Sayre syndrome, concentration of coenzyme Q l0 , component the mito-chondrial electron transport system, was decreased in serum and mitochondrial fraction skeletal muscle. Serum concentrations lactate pyruvate were abnormally high, especially after exercise or oral glucose loading. Levels folic acid plasma CSF decreased. ECG showed first-degree atrioventricular block. After administration 60 to 120 mg daily for 3 months, levels became normal, improvement block ocular movements.