Chorea and Huntington's Disease

Huntington’s disease is a degenerative disease of the brain which is inherited as an autosomal dominant trait. This means that only one parent needs to have the abnormal gene to pass the disease on and there is about a 50% chance that a child will develop the disease.

Symptoms usually start in the 3rd or 4th decade of life but can begin in childhood or as late as retirement age. The main symptoms are involuntary movements, dementia and psychiatric disturbance. All symptoms worsen over time and ultimately the disease is fatal. Although there is no cure there are useful treatments for many of the symptoms.

As a result of the recent discovery of the abnormal Huntington’s disease gene, a blood test is now available for making a definitive diagnosis in individuals suspected of having this disease. At risk family members who do not have the symptoms can also be tested. However, because of the profound impact of such a test result it is recommended that this testing be done in the setting of an integrated gene testing program. The program offers individuals at risk genetic counseling, neurological and psychiatric evaluation to help ensure the safety of the patient throughout the testing process.

The most common movement disorder seen in Huntington’s disease is chorea. In mild cases the movements may make the patient look restless or fidgety. In more severe cases the movements can cause dramatic jerking or “dancing” movements of the body. Chorea can be seen in other neurological diseases and can also be caused by certain medications. Making an accurate diagnosis is extremely important since the prognosis and appropriate treatment can be quite different for these various causes of chorea.

At the Parkinson’s Disease and Movement Disorders Center of Albany Medical Center we specialize in the diagnosis and treatment of Huntington’s disease and other choreas. We participate in a national organization of investigators, called the Huntington Study Group, which is evaluating new experimental approaches to the treatment of Huntington’s disease. The clinical research team at the Movement Disorders Center is proud to be participating in several important clinical trials that are the first of their kind and are specifically evaluating potential disease slowing treatments for Huntington's disease.