Huntington 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:
Mental function and behavior problems may include:
Ultimately, HD can:
You will be asked about your symptoms and medical history (including family medical history). A physical exam will be done.
Your bodily fluids may be tested. This can be done with blood tests.
Images may be taken of your bodily structures. This can be done with:
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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 advised 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.
Consider joining a support group for people with HD. It will help to learn how others are learning to live with the challenges of HD.
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://hdsa.org/wp-content/uploads/2015/03/PhysiciansGuide_3rd-Edition.pdf. Accessed October 2, 2017.
Fast facts about HD. Huntington's Disease Society of America website. Available at: http://hdsa.org/wp-content/uploads/2015/03/HDSA_Fast-Facts.pdf?23ef42. Accessed October 2, 2017.
Frank S, Jankovic J. Advances in the pharmacological management of Huntington's disease. Drugs. 2010;70(5):561-571.
Huntington disease. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T113724/Huntington-disease. Updated July 25, 2016. Accessed October 2, 2017.
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. Accessed October 2, 2017.
Paulsen JS, Hoth KF, Nehl C, Stierman L. Critical periods of suicide risk in Huntington's disease. Am J Psychiatry. 2005;163(4):725-731.
9/3/2014 DynaMed Plus Systematic Literature Surveillance. Available at:http://www.dynamed.com/topics/dmp~AN~T113724/Huntington-disease: Wippold FJ, Brown DC, Broderick DF, et al. American College of Radiology (ACR) Appropriateness Criteria for dementia and movement disorders. Available at: http://www.acr.org/~/media/ACR/Documents/AppCriteria/Diagnostic/DementiaAndMovementDisorders.pdf. Updated 2014. Accessed September 30, 2014.
Last reviewed September 2018 by EBSCO Medical Review Board Rimas Lukas, MD Last Updated: 9/30/2014