Huntington Disease Dementia
Idan Sharon, MD
Nestor Galvez-Jimenez, MD
Francisco Talavera, PharmD, PhD
Helmi L Lutsep, MD
Huntington Disease Dementia Overview
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Huntington disease (HD) is a devastating disorder that destroys nerve cells (neurons) in parts of the brain. It also lowers levels of chemicals called neurotransmitters that carry signals in the brain. Loss of these cells and neurotransmitters causes uncontrolled movements, loss of mental abilities, and changes in personality, emotional state, or behavior. Mental, emotional, or behavioral decline severe enough to interfere with the ability to carry out everyday activities is known as dementia.
Huntington disease has been known for hundreds of years. In 1872, Dr. George Huntington became the first to publish a detailed description of the disease that has become linked with his name. Even at that time, scientists realized that the disease is hereditary (passed down in families).
Huntington disease is one of the most common genetic disorders. About 30,000 people in the United States have the disease. About 250,000 people are at risk for it because one of their parents has the disease. The disease affects all races and ethnic groups but is most common among white people of European descent. The disease usually strikes people in their 30s and 40s, although it can start at almost any age.
Huntington disease was first described as a movement disorder. It used to be called Huntington chorea. Chorea is a type of abnormal movement. The word “chorea” comes from the same root as “choreography.” It refers to the writhing, twisting, dance-like movements typical of the disease. While the movement disorder of HD causes significant disability, the mental and behavioral changes are actually much more debilitating. These changes worsen over time into severe dementia.
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