Huntington's chorea

Huntington's Disease (Huntington's chorea) is a rare genetic disease involving wasting or degeneration of nerve cells of the brain. This degeneration causes chorea movements, called Huntington's chorea, which are uncontrollable and irregular muscle movements, especially of the arms, legs, and face.

Symptoms of Huntington's disease include:

• mental confusion that gets worse over time
• abnormal posture and
• involuntary movements

Symptoms usually develop between 35 and 50 years of age. Men and women are both as likely to develop the disease. Huntington's disease causes the brain to slowly decay. This leads to increasing chorea and loss of mental abilities. Eventually, abnormal movements and dementia become so severe that the person can no longer care for him or her self.

At present, there is no cure. Treatment centers on providing support and protecting the patient. Drugs can control the chorea somewhat, but the benefits are often outweighed by the side effects. Caretakers must see to the person's basic needs. These include hygiene, skin care, bowel and bladder care, and feeding. One must stay alert for suicide attempts. The person's family often needs emotional support. Caring for the person at home is often beyond the family's capacity.

A defective gene causes Huntington's disease. Only a single defective gene, inherited from either parent, is necessary to produce the disease. If one parent has a faulty gene, there is a 50 percent chance of the gene being passed on to children. Because symptoms do not appear until middle age, some parents may not even know that they are gene carriers until after they have children. Anyone inheriting the gene will eventually develop the disease. There is also a 50 percent chance that a child will not inherit the gene or develop the disease. Tests may now detect the gene for the disease before symptoms begin. This provides an opportunity for affected families to have informed genetic counseling.

Last Reviewed 2005

Disclaimer: This content is reviewed periodically and is subject to change as new health information becomes available. The information provided is intended to be informative and educational and is not a replacement for professional medical evaluation, advice, diagnosis or treatment by a healthcare professional.

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