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Huntington's Disease Information

Huntington's disease (HD), formerly known as Huntington's chorea, is a rare inherited genetic disorder characterized by abnormal body movements called chorea, and a reduction of various mental abilities. It takes its name from the Ohio physician George Huntington who described it precisely in 1872.

The symptoms of Huntington’s disease occur gradually over time - there is no sudden loss of abilities and it is hard to determine when symptoms initially occur. Symptoms of the disorder are both mental and physical.

Mental: Includes loss of cognitive ability (thinking, speaking), changes in personality (such as irritability, moodiness, or antisocial behavior). These symptoms may develop into dementia.

Physical: General lack of coordination and involuntary movements causing an unsteady gait. Most people with HD eventually exhibit chorea, which is jerky, random, uncontrollable, rapid movements, although some exhibit very slow movement and stiffness (bradykinesia, dystonia). Typically, the abnormal movements begin at the extremities and then later progress.

Although there is no treatment to fully stop the progression of the disease, there are treatments available to help control the chorea, although these may have the side effect of aggravating bradykinesia or dystonia.

Other standard treatments to alleviate emotional symptoms, like antidepressants, sedatives, and tranquilizers.

Nutrition is an important part of treatment, most HD sufferers need two to three times the calories than the average person to maintain body weight, so a nutritionist's advice is needed.

Speech therapy can help by improving speech and swallowing methods. This advice should be sought early on, as the ability to learn new things is reduced as the disease progresses.

To aid swallowing thickener can be added to drinks. When swallowing becomes hazardous the option of using a stomach PEG for intake of nutrients is often chosen, this reduces the chances of pnuemonia due to aspiration of food.

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