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Coenzyme Q₁₀ with multiple vitamins is generally ineffective in treatment of mitochondrial disease

Montreal Neurological Institute and Hospital (Drs. Matthews, Ford, Sherwin, Karpati, Andermann, and Arnold, and D. O'Connor) and Meakins-Christie Laboratory (Drs. Dandurand and Eidelman), McGill University, Montreal, PQ, Canada; and the Genetics Laboratory (Dr. Matthews), Oxford University, Oxford, UK.

We followed 16 patients with a variety of mitochondrial diseases over one to four periods of treatment (2 months each) with coenzyme Q₁₀ plus vitamins K₃ and C, riboflavin, thiamine, and niacin, using independent measures of oxidative metabolism to assess efficacy. There were large (<threefold) increases in serum coenzyme Q₁₀ concentrations with treatment, but no measure of oxidative metabolism showed significant improvement with treatment for the group, nor did any individual patient show significant, reproducible, objective clinical improvement. The results suggest that coenzyme Q₁₀ plus vitamin therapy does not significantly improve mitochondrial oxidative metabolism in patients with mitochondrial disease in general. Any clinical benefit that may follow from short-term administration appears slight.

Presented in part at the 44th annual meeting of the American Academy of Neurology, San Diego, CA, May 1992.

Address correspondence and reprint requests to Dr. D.L. Arnold, Montreal Neurological Institute, 3801 University Street, Montreal, PQ, Canada H3A 2B4.

Supported by the Muscular Dystrophy Association of Canada (D.L.A.), the Medical Research Council of Canada, and NIH (P.M.M.).

Received May 8, 1992. Accepted for publication in final form September 24, 1992.

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