Huntington's disease

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In 1872 George Huntington described the disorder in his first paper "On Chorea" at the age of 22.[1]
A microscope image of medium spiny neurons (yellow) with nuclear inclusions (orange): these are caused by the disease

Huntington's disease or chorea is a genetic disorder. It affects the brain and gets worse over time. This can lead to movement coordination problems, loss of memory and thinking skills and behaviour changes. On average, it leads to death about fifteen years after the first appearance of signs of illness. It is found in about five to seven in 100,000 people.

History[change | change source]

The disease is named after George Huntington. In 1872, he was the first to describe the clinical condition he was seeing in patients in a scientific paper. In a 1908 review, an important physician William Osler said about this paper: "In the history of medicine, there are few instances in which a disease has been more accurately, more graphically or more briefly described."

According to Huntington, the disease is characterized by three things: it is hereditary; people who have it become cases for psychiatry ("insanity and suicide"), and it only occurs in adults. That was the clinical understanding at that time. Of these, the last one proved to be wrong (some children also show symptoms of the disease).

Symptoms[change | change source]

Symptoms of Huntington's disease usually begin between the ages of 35 and 44 years. They can, however, start in any age group from babies to old age.[2] In fact, children with Huntington's disease are often much sicker. They get worse faster. Instead of having chorea, they usually have rigidity (or stiffness) of their muscles and it is painful. Seizures are also a common symptom in children.[3]

Treatment and care[change | change source]

In 2015, there is still no treatment for the disease itself. There are medicines available to help with some of the symptoms. Tetrabenazine was approved in 2008 by the FDA. This medicine helps slow some of the restless movements of chorea.[4] Other medicines that can help with psychiatric symptoms and movements are benzodiazepines and anticonvulsants usually taken for seizures.[5]

References[change | change source]

  1. Huntington G (1872). "On Chorea". Medical and Surgical Reporter of Philadelphia (The Hague: Nijhoff) 26 (15): 317–321. ISBN 9061860113. Retrieved 2009-04-01.
  2. Walker FO (2007). "Huntington's disease". Lancet 369 (9557): 218–28. doi:10.1016/S0140-6736(07)60111-1. PMID 17240289.
  3. Walker FO (2007). "Huntington's disease". Lancet 369 (9557): 218–28 [219]. doi:10.1016/S0140-6736(07)60111-1. PMID 17240289.
  4. "FDA approves first drug for treatment of chorea in Huntington's disease". FDA Approves First Drug for Treatment of Chorea in Huntington's Disease. U.S. Food and Drug Administration. 15 August 2008. Retrieved 10 August 2008.
  5. "Management: treatment of manifestations". The National Center for Biotechnology Information. 11 December 2014. Retrieved 4 February 2015.