Huntington's Disease

What is Huntington's disease?

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

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.

What are the symptoms?

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

Symptoms related to movement and speech 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 are not really there)
• having delusions (false beliefs or ideas)
• being suspicious without cause
• neglecting personal appearance and hygiene (failing to change clothes or bathe, for example)
• 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 (not knowing who or where you are or what time, date, or day it is).

How is it diagnosed?

Your healthcare provider will examine you and ask about your symptoms and medical and family history. A blood test may be done to check for the gene that causes the disease.

How is it treated?

Medicines can help control the different types of symptoms. Tetrabenazine is used to help control the writhing and flailing movements that HD causes.

Other medicine can be used to help control behavior symptoms, which can sometimes be violent outbursts. Some people have mood swings or depression. These symptoms can often be lessened or controlled with medicine.

There is no medicine at this time that can stop the symptoms or prevent HD from getting worse.

How long will the effects last?

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. If symptoms start at a young age, the disease may worsen more quickly.

In addition to losing balance and the ability to walk without help, as the disease gets worse you will likely need help with what are called the activities of daily living, or ADLs. Examples of ADLs are eating, dressing, and using the bathroom. Family members can try to do as much as they can, but it can be helpful to have personal attendants to help with your daily tasks. There may come a time when you may need 24-hour care in a nursing facility.

How can I help take care of myself?

It's important to try to maintain as much independence as possible. Occupational therapists are very helpful in finding ways you can stay physically independent. Your healthcare provider and therapists will also tell you that it's very helpful to stay as strong as you can, so exercise is an important part of taking care of yourself. Your therapists will help you create an exercise program.

Also important is finding a counselor or mental health therapist who can help you sort out the usual ups and downs of life from symptoms of Huntington's disease. They can also help you with the depression that can come from the limitations of HD.

Work closely with your healthcare provider to find the medicines that help the most with movement problems and emotional problems.

Join a Huntington's support group and encourage your family members to join also. In addition to the supportive personal relationships that can develop in these groups, they are perhaps the best way to learn of helpful tips for making everyday life easier as well as learn about community resources for people with Huntington's and their families.

How can I prevent Huntington's disease?

Because Huntington's is a genetic disease, genetic counseling is highly recommended for all family members. The counselor will discuss the pros and cons of being tested to see if you carry the gene for HD. This is a very personal decision. The genetic counselor may also be able to tell you ways that are being investigated to see how the illness might be prevented in future generations.

For more information, contact:

The Huntington's Disease Society of America
Phone: 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 neur3414.htm Release 13/2010