What Is CoQ10 (coenzyme Q10)?

From NNPDF Newsletter articles and family interviews.
This page is not intended as medical advice.
Consult with your physician before undertaking any treatment or therapy.

CoQ10 or coenzyme 10 is a nutritional supplement. The enzyme acts as an anti-oxidant to neutralize free radicals which can damage cells. It has been shown to help prevent heart disease (and is widely prescribed in Japan for this) and there is evidence that it improves neurological functions. CoQ10 also acts to lower blood cholesterol levels.

Only a limited number of research studies have been done on Coenzyme Q10 and many of these are related to heart disease. Dr. Salvatore DiMauro, of Columbia University, conducted one study on hereditary ataxia and CoQ10. Hereditary ataxia affects coordination, balance, and speech. Test subjects showed improvement, with some being able to leave wheelchairs and begin using walkers. However, the number of patients in the study was so small that no definite conclusions were possible. No controlled studies have been done on the effect of Coenzyme Q10 on Niemann-Pick Type C individuals.

Blood samples from several NPC individuals were taken. These samples were analyzed by Dr. DiMauro and Dr. Marc Patterson to determine if a deficiency of CoQ10 might exist with NP Type C. See a report of those studies in the Spring 2003 NNPDF Newsletter.

What do families say?

A number of families have begun using CoQ10 in recent months. As with many things related to NPC, the results have varied with individual children. In one case, a child who was tube fed and did not speak began to eat by mouth, speak, and have improved arm and leg movements after taking CoQ10. Most families reported improvements such as eating better, increased alertness, and more energy. Other families have reported little change and a few families have reported negative effects, including increased spastic movement and listlessness.

In the past, similar results have been reported with other nutritional supplements and diets, including amanatose, the Ketogenic diet, vitamin B12, and feeding tubes for increased caloric intake. When improvement occured, families have reported that the effect was not permanent.

Is CoQ10 safe?

There are no side effects reported in medical literature from using CoQ10 by itself. However, the number of studies is limited. Some families have reported diarrhea as a problem when using the powder form. It has been reported that there can be negative effects if CoQ10 is stopped quickly.

Nutritional supplements are not prescribed or regulated in the United States even when they have medicinal qualities. Some supplements can interact with other medications and some have maximum safe dosages. Certain formulations of CoQ10 have other supplements added, which may have their own side effects. You should consult with your physician before starting your child on CoQ10 or any other supplement or therapy.

How do I use CoQ10?

Families often start with 100 mg per day for school age children. The amount is then often gradually increased if no side effects are noted. 300 mg per day is the most common amount reported by families.

Gel caps containing the oil based form of CoQ10 are most widely used and can be found at GNC, Walmart, Whole Foods, and other stores. The gel cap can be taken by mouth or opened and mixed with food.

For individuals with feeding tubes, it has been found most effective to open the gel cap and mix the contents with hot tap water. Use the oil based form and do not heat on the stove, as this may lower the potency. The mixture can be given using a large syringe.

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