Huntington's Disease (Chorea)

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Harvard Medical School
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Huntington's Disease (Chorea)
Reviewed by the Faculty of Harvard Medical School

Huntington's Disease (Chorea)

Diseases and Conditions
9339
Huntington's disease
Huntington's Disease (Chorea)
Huntington's Disease (Chorea)
htmHuntingtonsDisease
Huntington's disease causes nerve cells in the brain to stop working properly.
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InteliHealth
2010-06-27
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InteliHealth Medical Content
2012-11-07

What Is It?

Huntington's disease causes nerve cells in the brain to stop working properly. It leads to mental deterioration and loss of control over major muscle movements.

Huntington's disease worsens over time. It is an inherited (genetic) disease. Each child of a parent with Huntington's disease has a 50% chance of inheriting the illness.

Huntington's disease is relatively uncommon. It affects people from all ethnic groups. The disease affects males and females equally.

Symptoms

Huntington's disease can affect:

  • Intellectual ability

    • Memory loss

    • Inattention

    • Dementia

  • Uncontrollable movements

    • Twitching

    • Clumsiness

    • Dance-like movements

    • Slurred speech

    • Difficulty walking

    • Difficulty swallowing

  • Uncontrollable emotions

    • Personality changes

    • Depression

    • Irritability

    • Anxiety or lack of emotions (apathy)

Symptoms vary from person to person. They also can change over time. They usually start as subtle differences and progress to profound disability.

Symptoms typically begin between ages 35 and 50. However, they can appear at any age.

Huntington's disease is rare in children. In children, behavioral problems and mental deterioration are prominent. Rigidity and seizures are common. Huntington's disease progresses more rapidly in children than in adults.

Diagnosis

The symptoms of Huntington's disease begin subtly. As a result, diagnosis may not be made until the disease has started to worsen.

Someone with a family history of Huntington's disease who has symptoms often is diagnosed based on a physical and neurological exam.

A blood test can detect the gene that causes Huntington's disease. Everyone who carries the Huntington's gene eventually will develop the disease. The test cannot predict when symptoms will begin.

Genetic testing carries enormous emotional and practical consequences. You should discuss these issues with your doctor. Some people choose to be tested so they can make informed decisions about the future.

Many experts recommend that children under 18 should not get a genetic test. At 18, they can decide for themselves if they want to get tested.

Some hospitals offer prenatal testing. If you have a family history of Huntington's disease, you can determine your risk of passing the gene to your children.

Expected Duration

The gene that causes Huntington's disease is present from birth. Symptoms typically begin in mid-life. They last until the end of life.

Prevention

There is no way to prevent this disease in people who have inherited the genetic abnormality.

If you have a family history of Huntington's disease, you may have a genetic test. This helps determine your chances of passing the gene on to your children.

Treatment

There is no treatment to cure Huntington's disease or slow its progression.

Therapies can lessen the severity of symptoms. These include speech therapy and physical therapy.

Medications can help to control mood and involuntary movements.

When to Call a Professional

Discuss a family history of Huntington's disease with your doctor. Consider genetic testing to determine whether you carry the Huntington's gene. You also may want to understand the risks involved before having children.

Call your doctor if you experience any problems with emotional control, intellectual ability or movement. Keep in mind that these symptoms are not specific to Huntington's disease.

Prognosis

There is no treatment to halt the progression of Huntington's disease.

The course of the disease varies from person to person. Some people have mild symptoms that progress slowly. Others have severe symptoms at a young age.

People who exercise and remain active tend to have milder symptoms. Their disease may also progress more slowly than those who do not remain active.

However, everyone with Huntington's disease ultimately becomes severely debilitated. They have difficulty eating and swallowing. They become susceptible to infection. These complications usually cause death.

For adults, the time from the first symptoms until death is usually 10 to 20 years. In juvenile Huntington's disease, the disease progresses more rapidly. Death usually occurs within 8 to 10 years of disease onset.

Additional Info

Huntington's Disease Society of America (HDSA)
158 West 29th St.
7th Floor
New York, NY 10001-5300
Toll-Free: 1-800-345-4372
Fax: 212-239-3430
http://www.hdsa.org/

9713, 11030, 11073, 11080,
huntington's disease,gene
11080
dmtHealthAZ
Last updated December 03, 2012


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