Huntington Disease

(Huntington Chorea; HD)

Definition

Huntington disease (HD) is a genetic problem of the brain. It causes the body to move in ways that the person cannot control. It also causes thinking problems that get worse over time.

Causes

HD is caused by a faulty gene. All people who inherit the faulty gene may get HD.

Risk Factors

The chance of getting HD is higher in those who have people in their family who have it.

Symptoms

Problems often start between 30 to 50 years of age. They are mild at first before they get worse.

Problems may be:

  • Changes in eye movements
  • Moving without control
  • Jerking movements of the arms, legs, face, or belly
  • Problems making decisions
  • Irritability
  • Lack of emotion
  • Mood swings
  • Problems walking and swallowing

Diagnosis

The doctor will ask about your symptoms and health history. You will be asked whether you have other people in your family with HD. A physical exam will be done. Cognitive tests may also be done.

Blood tests will be done to look for the faulty gene.

Images may be taken to support the diagnosis. This can be done with:

MRI Scan of the Brain

MRI of the Brain
Copyright © Nucleus Medical Media, Inc.

Treatment

There is no cure for HD. Symptoms can only be managed.

Medicine

Medicine may be given to ease body movements. These may be:

  • Antipsychotics
  • Tetrabenazine

Support

Counseling and support groups can help a person learn how to cope with HD.

Prevention

HD cannot be prevented in a person who has the faulty gene.

RESOURCES:

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

CANADIAN RESOURCES:

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

References:

Armstrong MJ, Miyasaki JM. Evidence-based guideline: pharmacologic treatment of chorea in Huntington disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology. 2012 Aug 7;79(6):597-603.
Fast facts about HD. Huntington's Disease Society of America website. Available at:
...(Click grey area to select URL)
Accessed October 18, 2019.
Huntington disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/huntington-disease . Updated July 24, 2018. Accessed October 18, 2019.
Huntington's disease information page. National Institute of Neurological Disorders and Stroke website. Available at:
...(Click grey area to select URL)
Updated March 27, 2019. Accessed October 18, 2019.
Kringlen G, Kinsley L, et al. The Impact of Family History on the Clinical Features of Huntington's Disease. J Huntingtons Dis. 2017;6(4):327-335.
Last reviewed September 2019 by EBSCO Medical Review Board Rimas Lukas, MD
Last Updated: 10/18/2019

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