Effect of coenzyme Q10 in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): Evaluation by noninvasive tissue oximetry

We evaluated the effect of coenzyme Q10 supplementation to two patients wit h mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) by using noninvasive tissue oximetry with near-infrared sp ectra of hemoglobin from the quadriceps muscle during bicycle ergometer exe rcise. Patients showed distinct oxygen consumption patterns reflecting the defect in oxidative phosphorylation and the impairment in oxygen utilizatio n during exercise. Based on the oxygen consumption pattern, we considered o ne patient as having severe mitochondrial disorder and another patient as h aving mild one. After coenzyme Q10 supplementation, the oxygen consumption pattern of the patient with the severe form shifted to the mild one, while that of the patient with mild form remained unchanged. The shift of the pat tern to the mild form correlated well with reduction of the sum of the seru m lactate and pyruvate content during exercise. Noninvasive tissue oximetry may be useful to evaluate the effect of coenzyme Q10 supplementation to pa tients with mitochondrial encephalomyopathy including MELAS. (C) 1999 Elsev ier Science B.V. All rights reserved.