Waiting to have rods lengthened June 2011  Claudie's son Pierrot and Caregiver Caroline

Coenzyme Q10 May Help in Friedreich's Ataxia, Mitochondrial Disease Return to Index

Coenzyme Q10, a dietary supplement that's available over the counter, has "come of age" with respect to its ability to alleviate symptoms in people with Friedreich's ataxia, some other forms of ataxia that are apparently directly related to a lack of coenzyme Q10, and perhaps in some mitochondrial disorders of muscle, says Salvatore DiMauro, a neurologist and MDA grantee who specializes in mitochondrial diseases at Columbia-Presbyterian Medical Center in New York.

Coenzyme Q10 — or CoQ10 — is part of the energy-producing machinery of the mitochondria (subunits in cells) and also acts as an antioxidant, combating dangerous free radicals that can destroy cell membranes and other structures.

DiMauro attended the Fifth European Meeting on Mitochondrial Pathology in Venice, Italy, in September, and was particularly impressed with a presentation by Raffaele Lodi of the University of Bologna (Italy) and Oxford University (England). Lodi, DiMauro says, "presented a study using CoQ10 that showed a remarkably good result in patients with Friedreich's ataxia that may connect with our own research." Lodi's studies, using nuclear magnetic resonance images of the brain and muscle, showed a decline in lactic acid [a metabolic byproduct that's undesirable in excess] and an improvement in ATP stores [a measure of stored molecular energy] in skeletal muscle and in the brain with CoQ10.

DiMauro reports that clinical measures of functional improvement in Friedreich's ataxia were also found with CoQ10 therapy.

He notes that he and other researchers have seen several patients with a genetic deficiency of CoQ10benefit from using the substance. Testing for such a deficiency is complex and requires a specialized medical center laboratory, he says.

Another group for which CoQ10 supplementation might be helpful is those with mitochondrial disorders, DiMauro says. In particular, he says, patients with MELAS, MERRF and Kearns-Sayre mitochondrial disorders have been studied by Japanese researchers and found to have some (though not major) deficiencies in their CoQ10 levels. "Whether that's enough to justify using CoQ10, I'm not certain," DiMauro says, but he believes it's probably worth trying.

DiMauro's group prescribes 600 to 1,000 milligrams a day of coenzyme Q10 divided into three doses and recommends that people take the pills with meals. He also endorses using a liquid form of CoQ10.

DiMauro recommends that people with neuromuscular disorders consult with their own doctors before taking a supplement, but he also says that CoQ10, at least in the recommended doses, hasn't caused any problems. "The good thing is, so far there have been absolutely no side effects. It seems to be a totally harmless compound, even in patients who went up to three grams [3,000 milligrams] a day on their own," he says.

Reprinted from Quest Magazine (MDA), 2001, No. 6, pg. 55-56

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