Huntington's Disease

Huntington's disease is a brain disorder in which brain cells, or neurons, in certain areas of the brain start to break down. As the neurons degenerate, the disease can lead to emotional disturbances, loss of intellectual abilities, and uncontrolled movements.

Facts about Huntington's disease

Huntington's disease is a genetic disorder: It is passed on from parents to children. If a parent has Huntington's disease, the child has a 50 percent chance of developing it. If the child doesn’t develop the disease, he or she won’t pass it along to children. For 1 to 3 percent of people with Huntington's disease, no family history of the disorder is ever identified.

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

Huntington's disease is a rare disorder. Over 15,000 Americans currently have Huntington's disease, although many more are at risk for developing it.

Symptoms of Huntington's Disease

At first, people who are beginning to show the signs of Huntington's disease will exhibit nonspecific symptoms:

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

As the disease progresses further, the following symptoms become more common:

• Trouble feeding oneself
• Difficulty swallowing
• Strange and uncontrolled movements that are either slow or wild and jerking
• Loss of memory and judgment
• Changes in speech
• Personality changes
• Disorientation and confusion
• Hallucinations, paranoia, and psychosis

In children, the symptoms often include Parkinson's disease-like features such as:

• Slow movements
• Rigidity
• Tremors

Types of Huntington's disease

• Huntington’s disease has two subtypes:
• Adult-onset Huntington’s disease. This is the most common form of Huntington’s disease. People typically develop the symptoms in their mid-30s and 40s.
• Early-onset Huntington’s disease. In rare instances, children or adolescents will develop the disease. Children with the disease tend to experience abrupt difficulties with schoolwork and often have symptoms that are quite similar to Parkinson’s disease.

Diagnosing Huntington's disease

• Because many of these symptoms can be caused by other diseases, a detailed physical and neurological examination is usually needed. Not surprisingly, a family history of the disorder is often the biggest clue that you may have Huntington's disease.
• Special blood tests can help doctors determine your likelihood of developing Huntington's disease. A CT scan of the head can evaluate the scope and scale of brain cell damage and loss of brain tissue. An MRI scan or a PET scan may also be used.

Treatments for Huntington's disease

• You can't cure or slow the progression of Huntington's disease, but doctors 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's disease. Haloperidol and tetrabenazine can also help offset hallucinations and delusional thoughts. Depression and suicide are common among those with Huntington's disease, and antidepressants and antianxiety medications may be prescribed to treat these symptoms.

Managing Huntington’s disease

As Huntington's disease progresses, constant assistance and supervision is often necessary 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's disease, it is critical to maintain 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 underway to examine possible therapies for Huntington's disease. Talk with your doctor about whether any of these therapies may be helpful to you or a loved one with the disorder.

Prevention of Huntington's disease

Because Huntington's is a genetic disease, you can't do anything to prevent it if you have inherited it. If you have a history of Huntington's disease in your family, you may wish to have genetic counseling before having children of your own.