Huntington Disease

What causes Huntington disease?

Huntington disease is a genetic disorder. It is passed on from parents to children. If a parent has Huntington disease, the child has a 1 in 2 chance of getting it. If the child doesn’t get the disease, they won’t pass it on to their children. For 1 to 3 in 100 people with the disease, no family history of the disorder is ever found. The gene defect causes a change in a protein called huntingtin, which makes the protein harmful to brain cells.

What are the symptoms of Huntington disease?

The early symptoms of Huntington disease are often general:

• Irritability
• Depression
• Mood swings
• Trouble driving
• Trouble learning new things
• Forgetting facts
• Trouble making decisions

As it gets worse, these symptoms become more common:

• Trouble feeding oneself
• Trouble swallowing
• Strange and uncontrolled movements that are either slow or wild and jerking (chorea)
• Loss of memory and judgment
• Changes in speech
• Personality changes
• Disorientation and confusion
• Hallucinations, paranoia, and psychosis
• Poor judgment. This can cause injury or problems with the law.

In children, the symptoms often include Parkinson disease‒like features, such as:

• Slow movements
• Rigidity
• Tremors

How is Huntington disease diagnosed?

Many of these symptoms can be caused by other diseases, so your healthcare provider will do a detailed physical and nervous system exam. A family history of the disorder is often the biggest clue that you may have Huntington disease.

Special blood tests can help your healthcare provider find out how likely you are to get Huntington disease. A CT scan of the head can help your provider assess the scope and scale of brain cell damage and loss of brain tissue. An MRI or a PET (positron emission tomography) scan may also be used.

Who is at risk for Huntington disease?

If you have the Huntington disease gene, you will get the disease at some point during your life. The age of onset varies greatly from person to person, but most people get it in their 30s or 40s.

Huntington disease is rare. More than 15,000 Americans currently have it, but many more are at risk for it.

How is Huntington disease treated?

You can’t cure or slow the progression of Huntington disease. But healthcare providers can prescribe medicines to help with certain symptoms.

Medicines, such as haloperidol, tetrabenazine, and amantadine, can help control the unusual movements caused by Huntington disease. Haloperidol and tetrabenazine can also help prevent hallucinations and delusional thoughts. Depression and suicide are common among people with this disease. Antidepressants and anti-anxiety medicines may be prescribed to treat these symptoms. You may need psychotherapy to help treat behavioral problems.

Living with Huntington disease

As Huntington disease gets worse, you will eventually need constant help and supervision because of the debilitating nature of the disease. People usually die from the disease within 15 to 20 years of the start of symptoms.

If you have been diagnosed with or are at risk for Huntington disease, it is critical to stay physically fit as best you can. People who exercise regularly and stay active tend to do better than those who don’t.

A number of studies are underway to find new treatments for Huntington disease. Talk with your healthcare provider about whether any of these may be helpful to you or a loved one with the disorder.

When should I call my healthcare provider?

If you have Huntington disease, it is important to closely keep track of your condition. Call your healthcare provider if you notice any of the following symptoms:

• Increasing trouble with motor tasks, such as walking
• Abnormal movements that can't be controlled
• Trouble swallowing
• Mood changes, such as increasing symptoms of depression or feelings of suicide

Key points about Huntington disease

• Huntington disease is a genetic brain disorder.
• There is no cure at this time, but a variety of treatment approaches are currently being looked at.
• The goal of treatment is to manage your symptoms so that you can function as long as possible.
• If you have Huntington disease, your child has a 1 in 2 chance of getting it.
• Huntington disease affects your emotional, physical, and thinking abilities.
• As the disease gets worse, you will need more help and supervision. Eventually, you will need help 24 hours a day.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your healthcare provider if you have questions, especially after office hours or on weekends.

Medical Reviewer: Joseph Campellone MD

Medical Reviewer: Anne Fetterman RN BSN

Medical Reviewer: Raymond Kent Turley BSN MSN RN

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