Huntington’s Disease (HD) is an inherited disorder that causes the progressive breakdown of nerve cells in the brain. Symptoms develop gradually, usually starting between the ages of 30 and 50, and slowly impair a person’s ability to walk, talk, think and reason. If you have not interacted with a sufferer you may be totally oblivious to the trauma experienced by those who suffer, and those who live with sufferers.
Researchers at the Bruce Lefroy Centre for Genetic Health Resarch found that an active lifestyle might delay the onset of Huntington’s- Kathryn Borg
At the moment, medicine has no way to cure the disease, only to manage the symptoms. However, a number of natural agents, including a red wine compound and a popular nutritional supplement, are being studied for their potential against HD, some with promising results.
Firstly let’s look at the symptoms of HD. They can include involuntary jerking or writhing movements, slow uncoordinated movements, difficulty with speech and swallowing, problems with spatial perception, difficulty with walking, posture and balance. In addition there can be short-term memory loss, inability to initiate a task or conversation, problems with learning new information, tiredness and loss of energy and finally depression.
Researchers at the Bruce Lefroy Centre for Genetic Health Research in Victoria, Australia, recently made the discovery that an active lifestyle might delay the onset of HD. In mouse models of HD, environmental enrichment was found to significantly delay the first signs of the disease, so the Australian researchers decided to investigate whether there was any connection between the type of lifestyle people led, i.e. active or passive, and the age of disease onset in 154 adults with HD.
The study revealed that those whose daily lives included a high level of passive activities (i.e. lacking physical and intellectual challenges such as watching television, talking on the phone or working in an unchallenging job) had a disease onset that was, on average, four years earlier than those who had the least passive lifestyles.
According to the lead researcher Professor Martin Delatycki: “People with genetic pre-disposition to the disease could significantly delay its onset by minimising passive activities, and ensuring that they incorporate physical and intellectual activity in their daily lives.” Once again, we see evidence that some physical activity during the week, plus some type of mental stimulation is vital to keeping the body healthy and free from disease.
One of the supplements referred to above is Coenzyme Q10 (CoQ10). This vitamin-like compound, widely available as a nutritional supplement, is being investigated for HD because of its ability to support the function of mitochondria (the tiny ‘power plants’ that provide every cell in the body with energy). Mitochondria dysfunction is known to contribute to the neuro-degenerative process in HD, so, in theory, CoQ10 could provide therapeutic benefit. (Journal of Neural Transmission Supplementa 2004).
Clinical trials with HD patients are currently underway. So far the results of the CARE-HD trial by the Huntington Study Group (HSG), based at the University of Rochester Medical Centre in New York, have been encouraging. Using a control group over two-and-a-half years, the group taking CoQ10 showed a trend towards less functional decline. These patients were able to handle everyday responsibilities such as finances and domestic chores and to retain cognitive skills better, for longer. They were also less irritable and less depressed than those not taking thesupplement.
Vitamin E, the powerful antioxidant, has also shown potential for HD treatment. A number of trials have had mixed results but are ongoing. Reservatrol, better known for its heart-healthy effects, is also being investigated as a therapeutic agent for HD. This is a plant compound found in red grapes, peanuts and pomegranates. Reservatrol has been found to activate the sirtuin 1 gene. This is also known as the longevity gene which appears to have a positive effect in cases of HD. There has been some success in laboratories with animals, but clinical trials are now needed to see what effects this compound will have when used withpeople.
Another compound that has been used in studies is creatine. This is popular among body builders and athletes. It is an amino acid and has been gaining interest as a potential treatment for HD. Studies in Massachusetts, at the Bedford VA Medical Centre, found that mice improved motor performance and significantly extended survival rates (Journal ofNeurochemistry, 2003). Trials are currently under way to judge the correct dose to administer to patients to create a similar success.
The few promising results that I have touched on here (as well as others I have not included) suggest that there is hope on the horizon for HD sufferers. Although it is a genetic disorder, the findings indicate that it may still be possible to influence the disease to some extent, particularly if treatment is begun during the early stages.
It may take some time before the treatments are medically recommended, however ease of use, availability and good safety profiles mean that sufferers could try them, with the help of a qualified practitioner.
Kathryn@maltanet.net