Huntington's Disease

Huntington disease is a brain problem in which brain cells (neurons) in parts of your brain start to break down. As the neurons break down, it can lead to emotional problems, thinking problems, and uncontrolled movements.  

Huntington disease has 2 subtypes:

• Adult-onset - This is the most common form. People typically develop the symptoms in their mid-30s and 40s.
• Early-onset - In rare instances, children or teens will get the disease. Children with the disease are less likely to have involuntary movements and can have slowness and stiffness, symptoms that are similar to Parkinson's disease.

Huntington disease is a genetic disorder. It is passed on from parents to children. If a parent has Huntington's disease, the child has a 1 in 2 chance of getting it. If the child doesn’t get the disease, he or she won’t pass it on to his or her children. For 1 to 3 in 100 people with the disease, no family history of the disorder is ever found.

The early symptoms of Huntington's 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.
• Involuntary 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.

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's disease.

Special genetic blood tests can help your healthcare provider find out how likely you are to get Huntington's disease. Genetic testing is only done in the setting of a comprehensive evaluation, with genetic counseling and a neurologic assessment.  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 positron emission tomography (PET) scan may also be used.

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

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

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

Medicines can help control the involuntary movements caused by Huntington's disease. Antipsychotics can also help prevent hallucinations and delusional thoughts. Depression and suicide are common among people with this disease. Antidepressants and antianxiety medicines may be prescribed to treat these symptoms. You may need psychotherapy to help treat behavioral problems. Research is ongoing to test whether Huntington’s can be treated at the genetic level, stopping the progression of the disease.

As Huntington’s 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's 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 under way to find new treatments for Huntington's disease. Talk with your healthcare provider about whether any of these may be helpful to you or a loved one with the disorder.

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

• Increasing trouble with motor tasks such as walking.
• Trouble swallowing.
• Mood changes, such as increasing symptoms of depression or feelings of suicide.