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Huntington's Disease (HD)

Huntington's Disease (HD)

Huntington's disease (HD) is an inherited genetic disease that destroys motor neurons that are located in the areas of the brain that govern movement. HD affects 15,000 to 30,000 people in the U.S. Symptoms usually begin to appear in early to mid-adulthood and include involuntary movements of the limbs or facial muscles, problems with coordination, cognitive difficulties, and depression. The disease progresses and usually leads to death over 15 or 20 years.

While certain drugs are now used to treat some of the symptoms of Huntington's, there is no drug treatment for the underlying disease. CoQ10 is a nutritional supplement that plays a role in the function of mitochondria, the energy factories of human cells. CoQ10 is also an anti-oxidant, helping soak up compounds known as free radicals that do damage to DNA and other proteins in our body.

Research shows that both damage from free radicals and a reduced supply of cellular energy may contribute to Huntington's. A pilot study conducted by Beal and associates showed that after two or more months of CoQ10 supplementation (360 mg per day), 83% of patients showed significant improvements in biochemical markers of energy production.

In 1997, a multi-center clinical trial began comparing CoQ10 and the drug Remacemide, each at 600 mg per day, in early stage Huntington's disease. Preliminary media reports indicate that the drug Remacemide (a glutamate blocker) had no effect on the decline in Total Functional Capacity of Huntington's Disease patients, and was found to confer no clinical benefit. However, CoQ10 slowed the decline by 13%, and also slowed decline on the HD Independence Scale by 17%. Reports indicate that these results showed a trend toward significance but are regarded as inconclusive. The Huntington Study Group, which organized the study, hopes to conduct a larger trial in order to determine whether CoQ10 therapy does significantly reduce the rate of decline in the early stages of the disease.

What is CoQ10?
CoQ10 is an antioxidant (vitamin), manufactured in the human body, and also present in small amounts in some foods -- unsaturated oils, fish, meats and nuts. As we age, we produce less CoQ10; and it would be difficult to get a significant amount from foods. It plays a role in the function of mitochondria, the energy factories of human cells. CoQ10 helps "soak up" compounds known as free radicals that do damage to DNA and other proteins in our body.

Medications and CoQ10 - Interactions
Some medications may interfere with the action of CoQ10 or perhaps decrease its production in the body. Such medications include "statins", some diabetes drugs, chemotherapy drugs and other medications. Many doctors now routinely prescribe supplements of CoQ10 for their patients who are taking cholesterol-lowering statins including lovastatin (Mevacor), simvastatin (Zocor), and pravastatin (Pravachol), and the dietary supplement Cholestin.

Persons using some oral diabetes drugs (glyburide [Diabeta, Glynase, Micronase], phenformin, and tolazamide) should speak with their doctors regarding CoQ10, which may lower blood sugar. It's possible that beta-blockers (propranolol [Inderal], metoprolol [Lopressor, Toprol], and alprenolol), phenothiazines, tricyclic antidepressants, methyldopa, hydrochlorothiazide, clonidine, and hydralazine may create a need for CoQ10, but this is not firmly established.

And there is a single report that CoQ10 interfered with the medication warfarin (Coumadin), an anticoagulant. People using warfarin should not take CoQ10 without first consulting their physician.

How much CoQ10 is safe to take?
The typical recommended dosage of CoQ10 based on various past studies is 30 to 300 mg daily; however, recent studies have used amounts of up to 1200 mg per day. Do not take such a large amount without consulting your doctor. If you do take supplements, it's best to take these in divided doses, two or three times a day, rather than all at once.

What's the best form to use?
CoQ10 comes in hard capsule or soft-gel form. Which ever form you take it is important to remember that CoQ10 is fat soluble--that means it should be taken with a small amount of fat to help it get absorbed into the body and be most effective. Vitamin E may also enhance the absorption of CoQ10 (30 IU vitamin E is a good amount to take). It appears that CoQ10 and vitamin E work synergistically (better together than separately).

Side effects
There appears to be none to few adverse effects associated with the usual amounts used in studies -- 30 to 300 mg per day. A very small percentage have some rare reported side effects including minor stomach upset, minimal loss of appetite, nausea, diarrhea. People taking CoQ10 late at night have reported insomnia.

Studies on the safety of CoQ10 have not been conducted on women who are pregnant or breastfeeding, or on children, and these individuals should not use CoQ10 prior to consulting with their physician.

**Although CoQ10 is definitely not a cure, studies such as these suggest how it may be beneficial to individuals with CHF and other heart ailments. However, as always, please consult your physician prior to starting any treatment plan.