Re: Coenzyme Q10 and CMT - 1985 research

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Proc Natl Acad Sci U S A. 1985 Jul;82(13):4513-6.

Biochemical rationale and the cardiac response of patients with muscle disease

to therapy with coenzyme Q10.

Folkers K, Wolaniuk J, Simonsen R, Morishita M, Vadhanavikit S.

Cardiac disease is commonly associated with virtually every form of muscular

dystrophy and myopathy. A double-blind and open crossover trial on the oral

administration of coenzyme Q10 (CoQ10) to 12 patients with progressive muscular

dystrophies and neurogenic atrophies was conducted.

These diseases included the Duchenne, Becker, and limb-girdle dystrophies,

myotonic dystrophy, Charcot-Marie-Tooth disease, and Welander disease.

The impaired cardiac function was noninvasively and extensively monitored by

impedance cardiography. Solely by significant change or no change in stroke

volume and cardiac output, all 8 patients on blind CoQ10 and all 4 on blind

placebo were correctly assigned (P less than 0.003).

After the limited 3-month trial, improved physical well-being was observed for

4/8 treated patients and for 0/4 placebo patients; of the latter, 3/4 improved

on CoQ10; 2/8 patients resigned before crossover; 5/6 on CoQ10 in crossover

maintained improved cardiac function; 1/6 crossed over from CoQ10 to placebo

relapsed. The rationale of this trial was based on known mitochondrial

myopathies, which involve respiratory enzymes, the known presence of CoQ10 in

respiration, and prior clinical data on CoQ10 and dystrophy.

These results indicate that the impaired myocardial function of such patients

with muscular disease may have some association with impaired function of

skeletal muscle, both of which may be improved by CoQ10 therapy. The cardiac

improvement was definitely positive. The improvement in well-being was

subjective, but probably real.

Likely, CoQ10 does not alter genetic defects but can benefit the sequelae of

mitochondrial impairment from such defects. CoQ10 is the only known substance

that offers a safe and improved quality of life for such patients having muscle

disease, and it is based on intrinsic bioenergetics.