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Huntington disease is an inherited, neurodegenerative and progressive brain disease that currently affects approximately 15,000 Americans. Most frequently the symptoms of HD occur between the ages of 30 and 50.
A major breakthrough was made in 1993 when the gene associated with Huntington disease was discovered. It is a familial disease. Each child of a parent with the huntingtin gene has a 50-50 chance of inheriting the gene. At least 150,000 Americans have a 50% risk of carrying the gene and developing the disease. This defect ultimately causes the death of nerve cells in the brain resulting in the following symptoms:
The abnormal facial and body movements are often described as uncontrollable dance-like, quick jerking movements. These movements are sometimes referred to as Huntington's chorea because "chorea" means dance. It is important to realize that there is wide variation in symptoms and affected individuals may experience only a few of the symptoms listed here.
Over the past 25 years, through the efforts of voluntary organizations, the scientific community, and affected families, this disease has become more widely known and understood. Although treatment for Huntington disease is limited, there are medicines which have been shown to be helpful in reducing individual symptoms, such as chorea. However, none as yet can change the course of the disease.
Institute for Neurodegenerative Disorders
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