Huntington's Disease (HD)

Basic Information:

  • Nowadays, Huntington's Chorea is commonly called Huntington's Disease.

  • People who have Huntington's disease do not die from the disease itself, but rather, they die from complications such as heart failure, pneumonia, and physical injuries from falling.

  • It was first recognized as a genetic disorder by an American doctor called George Huntington.

  • The defective Huntington's gene slowly causes the death of nerve cells.

What causes it?

Although the exact cause of Huntington's Disease is not known, it is known that Huntington's is hereditary. If a child has even one parent that has Huntington's Disease, they already have a 50% chance of getting Huntington's Disease. However, Huntington's Disease cannot skip a generation, for example, if a man had a father who had Huntington's, but he himself does not have it. His child will not get Huntington's.

How Common is Huntington's Disease?

  • There are around 30,000 people in the United States who have HD.(1)

  • 150,000 Americans have the potential to get HD.(2)

  • In Western countries it's estimated that about five to seven people per 100,000 are affected by HD. (3)

  • A very high concentration of HD has also been found in the Lake Maracaibo region of Venezuela where the prevalence of HD is about 700 per 100,000. (4)

  • In Australia, 1200 people have HD.(5)

  • Around 6000 people in Australia have the potential to get HD. (6)



What are the symptoms? How long do they it last? Is it deadly?

The early symptoms of HD include:

  • Excessive Restlessness.

  • Forms of nervous activities.

  • Handwriting changes.

  • Some clumsiness.

  • Some difficulty with everyday tasks.

There are also physiological symptoms such as:

  • Decreased ability to organize routine tasks.

  • Decreased ability to cope with new situations.

  • Short term memory loss.

  • Periods of depression, irritability and apathy.

  • Impulsiveness.

In the advanced stages, those symptoms become more and more obvious. As the disease becomes more and more serious, and the person will need full time support. A person with HD usually die in 15 to 20 years after the first symptoms appear. Since HD is not curable, an infected person will have the disease until they depart.



Can it be cured? If so, how? If not, is there any way that we can at least treat it?

Huntington's Disease cannot be cured or controlled, however, there have been drug trials for HD. There are people such as Dr. LaVonne Veatch Goodman (who along with her husband founded the Huntington's Disease Drug Works) that tries to find a cure for HD, or at least a way to slow down the course of the disease.


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References:

(1) Unknown. (2010, February 8). Huntington's disease. Retrieved from http://www.healthtree.com/articles/huntingtons-disease/causes.php

(2) Unknown. (2010, February 8). Huntington's disease. Retrieved from http://www.healthtree.com/articles/huntingtons-disease/causes.php

(3) S. E. Folstein Huntington's Disease. A Disorder of Families Baltimore: Johns Hopkins University Press, 1989

(4) R. Avila-Giron Medical and Social Aspects of Huntington's Chorea in the State of Zulia, Venezuela in: Advances in Neurology, Vol 1 (eds A. Barbeau, T.N. Chase and G.W. Paulson) New York: Raven Press, 1973, pp. 261-266

(5) P.M. Conneally Huntington's Disease: Genetics and Epidemiology American Journal of Human Genetics

(6) P.M. Conneally Huntington's Disease: Genetics and Epidemiology American Journal of Human Genetics 1984: 36, pp.506-526 1984: 36, pp.506-526