Learn About Huntington Disease

What is the definition of Huntington Disease?

Huntington disease (HD) is a genetic disorder in which nerve cells in certain parts of the brain waste away, or degenerate. The disease is passed down through families.

Save information for later
Sign Up
What are the alternative names for Huntington Disease?

Huntington chorea

What are the causes of Huntington Disease?

HD is caused by a genetic defect on chromosome 4. The defect causes a part of DNA to occur many more times than it is supposed to. This defect is called a CAG repeat. Normally, this section of DNA is repeated 10 to 28 times. But in persons with HD, it is repeated 36 to 120 times.

As the gene is passed down through families, the number of repeats tends to get larger. The larger the number of repeats, the higher a person's chance of developing symptoms at an earlier age. Therefore, as the disease is passed along in families, symptoms develop at younger and younger ages.

There are two forms of HD:

  • Adult-onset is the most common. Persons with this form usually develop symptoms in their mid-30s or 40s.
  • Early-onset affects a small number of people and begins in childhood or the teens.

If one of your parents has HD, you have a 50% chance of getting the gene. If you get the gene from your parents, you can pass it on to your children, who will also have a 50% chance of getting the gene. If you do not get the gene from your parents, you cannot pass the gene on to your children.

What are the symptoms of Huntington Disease?

Abnormal behaviors may occur before movement problems develop, and can include:

  • Behavioral disturbances
  • Hallucinations
  • Irritability
  • Moodiness
  • Restlessness or fidgeting
  • Paranoia
  • Psychosis

Abnormal and unusual movements include:

  • Facial movements, including grimaces
  • Head turning to shift eye position
  • Quick, sudden, sometimes wild jerking movements of the arms, legs, face, and other body parts
  • Slow, uncontrolled movements
  • Unsteady gait, including "prancing" and wide walk

Abnormal movements can lead to falls.

Dementia that slowly gets worse, including:

  • Disorientation or confusion
  • Loss of judgment
  • Loss of memory
  • Personality changes
  • Speech changes, such as pauses while talking

Additional symptoms that may be associated with this disease include:

  • Anxiety, stress, and tension
  • Difficulty swallowing
  • Speech impairment

Symptoms in children:

  • Rigidity
  • Slow movements
  • Tremor
Not sure about your diagnosis?
Check Your Symptoms
What are the current treatments for Huntington Disease?

There is no cure for HD. There is no known way to stop the disease from getting worse. The goal of treatment is to slow the symptoms and help the person function for as long as possible.

Medicines can be prescribed, depending on the symptoms.

  • Dopamine blockers may help reduce abnormal behaviors and movements.
  • Drugs such as amantadine and tetrabenazine are used to try to control extra movements.

Depression and suicide are common among persons with HD. It is important for caregivers to monitor for symptoms and seek medical help for the person right away.

As the disease progresses, the person will need assistance and supervision, and may eventually need 24-hour care.

Who are the top Huntington Disease Local Doctors?
Elite
Elite
 
 
 
 
Learn about our expert tiers
Learn more
Elite
What are the support groups for Huntington Disease?

More information and support for people with HD and their families can be found at:

  • Huntington's Disease Society of America -- hdsa.org/
  • Genetics and Rare Diseases Information Center website -- rarediseases.info.nih.gov/diseases/6677/huntington-disease
What is the outlook (prognosis) for Huntington Disease?

HD causes disability that gets worse over time. People with HD usually die within 15 to 20 years. The cause of death is often infection. Suicide is also common.

It is important to realize that HD affects people differently. The number of CAG repeats may determine the severity of symptoms. People with few repeats may have mild abnormal movements later in life and slow disease progression. Those with a large number of repeats may be severely affected at a young age.

When should I contact a medical professional for Huntington Disease?

Call your provider if you or a family member develops symptoms of HD.

If you or someone you know is thinking about suicide, call or text 988 or chat 988lifeline.org. You can also call 1-800-273-8255 (1-800-273-TALK). The 988 Suicide and Crisis Lifeline provides free and confidential support 24/7, anytime day or night.

You can also call 911 or the local emergency number or go to the hospital emergency room. DO NOT delay.

If someone you know has attempted suicide, call 911 or the local emergency number right away. DO NOT leave the person alone, even after you have called for help.

How do I prevent Huntington Disease?

Genetic counseling is advised if there is a family history of HD. Experts also recommend genetic counseling for couples with a family history of this disease who are considering having children.

What are the latest Huntington Disease Clinical Trials?
A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Parallel Arm, Multicenter Study Evaluating the Efficacy and Safety of Pridopidine in Patients With Early Stage of Huntington Disease

Summary: This study will evaluate the efficacy and safety of pridopidine 45mg twice daily (BID) in patients with early stage manifest Huntington Disease (HD).

Match to trials
Find the right clinical trials for you in under a minute
Get started
An Open-Label Extension Study to Evaluate the Long-Term Safety and Tolerability of Intrathecally Administered RO7234292 (RG6042) in Patients With Huntington's Disease

Summary: This study will evaluate the long-term safety and tolerability of RO7234292 (RG6042) in participants who have completed other F. Hoffmann-La Roche, Ltd.-sponsored and/or Genentech-sponsored studies in the Huntington's disease (HD) in the development program for RG6042.

What are the Latest Advances for Huntington Disease?
Home-Based Exercise to Improve Motor Functions, Cognitive Functions, and Quality of Life in People with Huntington's Disease: A Systematic Review and Meta-Analysis.
The Safety of Deutetrabenazine for Chorea in Huntington Disease: An Open-Label Extension Study.
Tired of the same old research?
Check Latest Advances
Randomized controlled trials on the use of cannabis-based medicines in movement disorders: a systematic review.
Who are the sources who wrote this article ?

Published Date: May 04, 2021
Published By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Caron NS, Wright GEB, Hayden MR. Huntington disease. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews. Seattle, WA: University of Washington. www.ncbi.nlm.nih.gov/books/NBK1305/. Updated June 11, 2020. Accessed June 22, 2021.

Jankovic J, Lang AE. Diagnosis and assessment of Parkinson disease and other movement disorders. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley's and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 24.