Huntington's Disease - An Overview

Huntington's Disease - An Overview

Huntington's Disease is a devastating and progressive neurological disorder that resu lts primarily from degeneration of nerve cells deep in the center of the brain. The condition was first described by George Huntington, a physician in New York, in 1872. Even then, the physician recognized the all-encompassing factors of the disorder when describing it as, "coming on gradually but surely, increasing by degrees, and often occupying years in its development until the hapless sufferer is but a quivering wreck of his former self".

The three most profound behavioral problems in Huntington 's disease come from the uncontrollable movements called "chorea," dementia, and the altered perception of the world. The course of Huntington's Disease can last anywhere from ten to 30 years from onset to death. The symptoms of HD usually develop when th e person is between the ages of 30 - 50 years, although they have been known to occur as young as two and as old as 70. It affects all races and ethnic groups and occurs with the same frequency in both men and women.

As the disease progresses, the se verity of the symptoms increase and can be divided into three stages. The first stage is characterized by subtle and slight uncontrollable muscular movements, including stumbling and clumsiness, lack of concentration and short-term memory lapses, and depr ession and changes of mood. In the middle of the disease's course, patients may need assistance with some daily activities because walking, speech and swallowing abilities deteriorate. Involuntary movements become more pronounced and may be noticeable to the casual observer. In the latter stages, patients with HD require full-time care, and often families o...

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...people afflicted with this disease in the United States. An additional 150,000 are at risk. Although very few cases of HD are due to fresh mu tation, everyone who has the HD gene will eventually develop the disorder unless he or she dies from some other cause before the signs appear. Furthermore, there is often little or no choice of residential care for those with HD, especially in the later s tages of the illness. However, the hope for a cure continues to mount every year and families and communities are joining together to promote better treatment and understanding for those with the disease. Hopefully, a cure will be found in my lifetime.

Internet Sources:

"About Huntington's Disease," Internet, available: http://www.med.jhu.edu/bhde/hdinfo.html.

http://www.kumc.edu/hospital/huntingtons/genetics.html

http://www.lib.uchicago.edu/~rd13/hd/review.html