by Rick Alan
Huntington's disease (HD) is an inherited disorder that affects the brain. HD causes slow, progressive degeneration of nerve cells in certain areas of the brain. Eventually, HD results in:
A faulty gene on chromosome #4 causes HD. All people who inherit the faulty gene may eventually develop HD.
Having family members with HD increases your chance of developing HD. Each person whose parent has HD has a 50% chance of inheriting the disorder.
Symptoms most often develop between the ages of 30-50 years. Symptoms are mild at first and are often barely noticeable, but usually worsen over 15-20 years.
Abnormal body movements that worsen over time, may include:
Gradual deterioration or loss of intellectual abilities may include:
Loss of intellectual and behavior problems may include:
Ultimately, HD can:
The doctor will ask about your symptoms and medical history (including family medical history). A physical exam will be done. Blood tests can rule out other conditions. You may need to have imaging tests, which take pictures of internal body structures. Imaging tests include:
CT Scan of the Head
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There is a test that can determine if a person has inherited the gene for HD. This test may help to make the diagnosis of HD. It may also help to determine if a person has inherited the HD gene before symptoms appear. Genetic counseling is recommended before taking this test to review risks and benefits.
There is no cure for HD. Treatment aims to help control symptoms.
Drugs can help control abnormal movements and emotional symptoms of HD. These include:
Staying physically active helps people with HD to function better and longer. Often physical and occupational therapy may be of some benefit.
There is no way to prevent the onset of HD if a person has inherited the gene for the disorder. If you have a family history of HD, talk with a genetic counselor.
Hereditary Disease Foundation
Huntington Disease Society of America
Huntington Society of Canada
A physician's guide to the management of Huntington's disease. Huntington's Disease Society of America website. Available at: http://www.hdsa.org/images/content/1/1/11682.pdf. Accessed July 19, 2013.
Fast facts about HD. Huntington's Disease Society of America website. Available at: http://www.hdsa.org/images/content/1/3/13699.pdf. Accessed July 19, 2013.
Frank S, Jankovic J. Advances in the pharmacological management of Huntington's disease. Drugs. 2010;70(5):561-571.
Huntington disease. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated May 31, 2013. Accessed July 19, 2013.
Mestre T, Ferreira J, Coelho MM, et al. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database Syst Rev. 2009;8(3):CD006456.
NINDS Huntington's Disease information page. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/huntington/huntington.htm. Updated April 24, 2013. Accessed July 19, 2013
Paulsen JS, Hoth KF, et al. Critical periods of suicide risk in Huntington's disease. Am J Psychiatry. 2005;163(4):725-731.
Last reviewed July 2013 by Rimas Lukas, MD; Brian Randall, MD
Last Updated: 07/19/2013