Huntington disease is a brain disorder in which brain cells, or neurons, in certain areas of your brain start to break down. As the neurons degenerate, the disease can lead to emotional disturbances, loss of intellectual abilities, and uncontrolled movements.
Huntington disease has 2 subtypes:
Huntington disease is a genetic disorder. It is passed on from parents to children. If a parent has Huntington disease, the child has a 50% chance of developing it. If the child doesn’t develop the disease, he or she won’t pass it along to his or her children. For 1% to 3% of people with Huntington disease, no family history of the disorder is ever identified.
As the disease progresses, the following symptoms become more common:
In children, the symptoms often include Parkinson disease‒like features such as:
Because many of these symptoms can be caused by other diseases, a detailed physical and neurological exam is usually needed. Not surprisingly, 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 determine your likelihood of developing Huntington disease. A computed tomography (CT) scan of the head can evaluate the scope and scale of brain cell damage and loss of brain tissue. A magnetic resonance imaging (MRI) scan or a positron emission tomography (PET) scan may also be used.
If you have the Huntington disease gene, you will develop the disease at some point during your life. The age of onset of Huntington disease varies greatly from person to person, but most people develop it in their 30s or 40s.
Huntington disease is a rare disorder. More than 15,000 Americans currently have the disease, but many more are at risk of developing it.
You can’t cure or slow the progression of Huntington disease, but health care providers can offer medications to help with certain symptoms.
Drugs like haloperidol, tetrabenazine, and amantadine are especially helpful for controlling the unusual movements caused by Huntington disease. Haloperidol and tetrabenazine can also help offset hallucinations and delusional thoughts. Depression and suicide are common among those with Huntington disease. Antidepressants and antianxiety medications may be prescribed to treat these symptoms.
As Huntington disease progresses, you will need constant assistance and supervision because of the debilitating nature of the disease. People usually die from the disease within 15 to 20 years of developing symptoms.
If you have been diagnosed with, or are at risk for Huntington disease, it is critical to maintain your physical fitness 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 currently under way to examine possible therapies for Huntington disease. Talk with your health care provider about whether any of these therapies may be helpful to you or a loved one with the disorder.
If you have Huntington disease, it is important to closely monitor you condition, and contact your healthcare provider if you notice any of the following symptoms:
Tips to help you get the most from a visit to your healthcare provider:
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