Huntington's Disease

(Huntington Chorea; HD)
  • Definition

    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:
    • Abnormal body movements
    • Gradual deterioration or loss of intellectual abilities
    • Behavior problems
  • Causes

    A faulty gene on chromosome #4 causes HD. All people who inherit the faulty gene may eventually develop HD.
  • Risk Factors

    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

    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:
    • Sudden jerks or uncontrolled movements of the limbs or trunk
    • Facial grimacing
    • Continuous need to turn head and shift gaze
    • Walking that is unsteady or dance-like
    Gradual deterioration or loss of intellectual abilities may include:
    • Difficulty with eating and swallowing, which may result in weight loss
    • Difficulty dressing, sitting, and caring for oneself
    • Grunting or poor articulation of speech
    Loss of intellectual and behavior problems may include:
    • Trouble with attention and awareness
    • Confusion or disorientation
    • Loss of memory
    • Loss of judgment
    • Loss of ability to think rationally
    • Irritability and moodiness
    • Depression (common)
    • Anxiety
    • Social withdrawal or antisocial behavior
    • Irresponsible behavior
    • Obsessive-compulsive behavior
    • Personality changes
    • Psychosis—a severe emotional and behavioral disorder that often interferes with a person's ability to relate to others and to function in daily life
    • Paranoia—a mental disorder that involves feelings of being watched, followed, or harmed by others
    • Hallucinations—the perception of a thing or person that is not present
    Ultimately, HD can:
    • Cause the loss of the physical and mental ability to care for oneself
    • Cause severe disability, making full-time or nursing home care necessary
    • Result in death, often due to a fall or pneumonia
  • Diagnosis

    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
    CT Scan of the Head
    Copyright © Nucleus Medical Media, Inc.
    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.
  • Treatment

    There is no cure for HD. Treatment aims to help control symptoms.
    Medications
    Drugs can help control abnormal movements and emotional symptoms of HD. These include:
    • Tetrabenazine
    • Antipsychotics
    • Antidepressants
    Physical Fitness
    Staying physically active helps people with HD to function better and longer. Often physical and occupational therapy may be of some benefit.
  • Prevention

    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.
  • RESOURCES

    Hereditary Disease Foundation http://www.hdfoundation.org

    Huntington Disease Society of America http://www.hdsa.org

    CANADIAN RESOURCES

    Health Canada http://www.hc-sc.gc.ca

    Huntington Society of Canada http://www.huntingtonsociety.ca

    References

    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.

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