Coenzyme Q10 treatment in mitochondrial encephalomyopathies. Short-term double-blind, crossover study.
We report a short-term double-blind, crossover study of CoQ10 in 8 patients with mitochondrial encephalomyopathies. Four patients had myoclonus epilepsy with ragged-red fibers syndrome, 3 had mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome, and 1 had chronic progressive external ophthalmoplegia with myopathy. A trend of effectiveness of CoQ10 in several parameters was noted. Fatigability of daily activities was alleviated. The endurance to muscle exercise was augmented. Global muscle strength scored by Medical Research Council scale was increased. The extent of elevation in serum lactate and pyruvate levels after exercise was decreased. However, only the global MRC index score had a statistical significance (p < 0.05). There were no side effects during therapy. The serum CoQ10 levels were significantly lower in patients than in normal controls before CoQ10 treatment and increased significantly after treatment.
Chen
R S
RS
Department of Neurology, Chang Gung Medical College and Memorial Hospital, Taipei, Taiwan.
Huang
C C
CC
Chu
N S
NS
D000203:Activities of Daily Living / Q000145:classification; D000293:Adolescent; D000328:Adult; D000368:Aged; D003067:Coenzymes; D018592:Cross-Over Studies; D004311:Double-Blind Method; D004334:Drug Administration Schedule; D005080:Exercise Test / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019344:Lactic Acid / Q000097:blood; D008297:Male; D008875:Middle Aged; D017237:Mitochondrial Encephalomyopathies / Q000097:blood / Q000188:drug therapy*; D009460:Neurologic Examination; D010807:Physical Endurance / Q000187:drug effects; D019289:Pyruvic Acid / Q000097:blood; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000493:pharmacokinetics / Q000627:therapeutic use
D003067:Coenzymes; D014451:Ubiquinone; D019344:Lactic Acid; D019289:Pyruvic Acid; C024989:coenzyme Q10