Huntington's Disease (HD)

What is Huntington's disease?

Huntington's disease (HD), also called Huntington's chorea, is a rare, fatal disease that causes certain nerve cells in the brain to slowly atrophy, or waste away. This results in chorea (jerky movements of the face, arms, neck, and trunk that you cannot control) and dementia (a gradual loss of mental function).

The symptoms usually begin between the ages of 35 and 50 and get worse over time. Most people live for about 15 years after symptoms begin, but some live twice that long.

How does it occur?

The disease is passed from parent to child by a faulty gene. If one parent has this gene, his or her children have a 50% chance of having the disease. People with the disease often have children before symptoms appear and before they know they have the disease.

What are the symptoms?

The disease develops slowly and is sometimes difficult to recognize. Symptoms usually affect movement, personality, and mental function.

Symptoms related to movement may include:

clumsiness
repeated, irregular movement of eyebrows and forehead
facial grimaces
rigid muscles
loss of balance
trouble walking
uncontrolled jerking movements of arms, legs and trunk
halting speech.

Symptoms related to personality and mental function may include:

having hallucinations (seeing or hearing things that aren't really there)
having delusions (false beliefs or ideas)
being suspicious without cause
neglecting personal appearance (failing to change clothes, for example) and hygiene (failing to bathe or brush teeth)
neglecting duties (such as not paying bills or going to work)
being depressed
being irritable
behaving irresponsibly, impulsively, or violently (such as driving recklessly, getting drunk, or starting fights)
losing the ability to remember, think logically, or exercise judgment
becoming unaware of person, place, and time (you don't know who or where you are or what time, date, or day it is).

None of the symptoms are unique to Huntington's, but jerky movements (chorea) along with dementia is suggestive of the disease.

As time goes on the symptoms become worse. If symptoms begin when the person is younger, the disease may progress more rapidly.

How is it diagnosed?

Your health care provider will perform a physical exam and ask about symptoms and medical and family history. A special blood test is used to determine the presence of the defective gene.

How is it treated?

There is no cure. Drugs may help lessen the movements of chorea and, to some extent, can help control behavior. You may be cared for at home until symptoms become severe. At that time, placement in a care facility may be recommended.

For more information about Huntington's disease, contact:

The Huntington's Disease Society of America
158 West 29th Street, 7th Floor
New York, NY 10001-5300
1-800-345-4372
Web site: http://www.hdsa.org

Disclaimer: This content is reviewed periodically and is subject to change as new health information becomes available. The information provided is intended to be informative and educational and is not a replacement for professional medical evaluation, advice, diagnosis or treatment by a healthcare professional.

HIA File BRN3414F.HTM Release 9.0/2006