Huntington's Disease

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KEY POINTS

• Huntington's disease is a rare nervous system disease that causes nerve cells in the brain to slowly waste away. It causes uncontrolled jerky movements of the face, arms, neck, and trunk. Huntington's disease also causes a gradual loss of the ability to think, remember, and plan.
• There is no cure for Huntington’s disease, but medicines and physical therapy may help control symptoms.

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What is Huntington's disease?

Huntington's disease (HD), also called Huntington's chorea, is a rare nervous system disease that causes nerve cells in the brain to slowly waste away. The loss of nerve cells causes uncontrolled jerky movements of the face, arms, neck, and trunk. Huntington's disease also causes a gradual loss of the ability to think, remember, and plan.

What is the cause?

Inside each cell of your body are genes. Genes contain the information that tells your body how to develop and work. The disease is passed from parent to child by one faulty gene. If one parent has this gene, a child has a 50% chance of inheriting the gene and having the disease. As a result of the faulty gene, your body makes an abnormal protein that eventually affects nerve cells in some parts of the brain.

What are the symptoms?

The disease develops slowly. First symptoms can range from problems with coordination to depression or learning difficulties.

Symptoms related to personality, behavior, and thinking may include:

• Hearing, seeing, smelling, or feeling things that others do not
• Believing things that are not true, such as believing that others are trying to hurt you
• Not taking care of yourself, such as not bathing or combing your hair
• Not doing things like paying bills or going to work
• Being depressed or irritable
• Doing things on impulse, such as driving recklessly, getting drunk, or starting fights
• Not being able to remember, think things through, or make decisions
• Not knowing who or where you are or what time, date, or day it is

Symptoms related to movement and speech may include:

• Clumsiness
• Repeated, irregular movements of your eyebrows and the muscles in your forehead
• Rigid muscles
• Loss of balance or trouble walking
• Uncontrolled jerking movements of face, arms, legs, and trunk that you can’t control
• Trouble speaking

The symptoms usually start between the ages of 35 and 50 and get worse over time. Most people live for about 15 to 20 years after they start having symptoms, but some live longer. If symptoms start at a young age, the disease usually gets worse more quickly.

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical and family history, and examine you. You will have a blood test to check for the gene that causes the disease. You may have tests such as:

• CT scan, which uses X-rays and a computer to show detailed pictures of the brain
• MRI, which uses a strong magnetic field and radio waves to show detailed pictures of the brain tissue

How is it treated?

There are no medicines that can cure Huntington’s disease. Some medicines can help control symptoms. For example, medicine may be prescribed to help control jerking movements. Other medicines can be used to help lessen or control behavior symptoms, such as violent outbursts, mood swings, or depression.

Physical therapy and exercise can help keep your muscles strong. Your therapists will help you create an exercise program tailored for you. Occupational therapy may help if you have problems doing things like eating and getting dressed.

It is also helpful to see a mental health professional to help you cope with stress and depression.

How can I help take care of myself?

As the disease gets worse you will likely need help with eating, dressing, and using the bathroom. Family members can give care, but it can be helpful to have home health aides to help with your daily tasks. There may come a time when you need 24-hour care, possibly in a nursing facility.

Work closely with your healthcare provider to find the medicines and therapies that are most helpful for you. Ask your provider:

• How and when you will get your test results
• If there are activities you should avoid
• How to take care of yourself at home
• What symptoms or problems you should watch for and what to do if you have them

Make sure you know when you should come back for a checkup. Keep all appointments for provider visits or tests.

Join a Huntington's support group. Support groups can help by sharing common concerns and solutions to problems with others in the same situation. You can find these services through your healthcare provider, therapy programs, and local and national support organizations.

How can I help prevent HD?

People who have the gene may pass the disease on to their children. Couples can get tested for the gene that causes HD. If one of you has the gene, you will need to decide whether to take the chance of passing the disease on to your children.

If you have the gene, it means that you will develop HD over time. Research is being done to find new ways to treat the disease. It is important to talk with a genetic counselor if anyone in your family has HD.

You can get more information from:

• The Huntington's Disease Society of America
  800-345-4372
  http://hdsa.org/