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Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q₁₀

From the Istituto di Clinica Neurologica (Drs. Bresolin, Bet, Moggio, Comi, Ferrante, and Scarlato), Centra Dino Ferrari, Università degli Studi di Milano; the Department of Cardiology (Drs. Binda and Nador), Ospedale Maggiore-Policlinico, Milano; and the Research Laboratory (Dr. Carenzi), Zambon Pharmaceutical Company, Bresso, Milano, Italy.

We tested the efficacy of coenzyme Q₁₀ (ubidecarenone, CoQ₁₀) therapy in patients with Kearns-Sayre syndrome and other mitochondrial myopathies with chronic progressive external ophthalmoplegia (CPEO). We treated seven patients for 1 year with daily oral administration of 120 mg of CoQ₁₀. Throughout the treatment most of our patients showed a progressive reduction of serum lactate and pyruvate levels following standard muscle exercise and generally improved neurologic functions. The ECG and echocardiogram showed no significant changes in our patients. None of our patients showed any improvement in ptosis and CPEO.

Address correspondence and reprint requests to Dr. Bresolin, Istituto di Clinica Neurologica, Centro Dino Ferrari, Università degli Studi di Milano, Via Francesco Sforza 35, 20122 Milano, Italy.

Received December 22, 1986. Accepted for publication in final form November 3, 1987.

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