Huntington's disease (also known as Huntington's chorea) is a hereditary disease that is marked by chronic, progressive chorea and marked mental deterioration in middle life, usually in the fourth decade.
The disease has a prevalence of about 1 in 20,000 in the UK. It is inherited as an autosomal dominant trait with full penetrance (meaning that individuals born to a parent with Huntington disease have a 50% chance of developing the disease). The family history of the disease is often concealed.
The gene responsible for this condition resides on chromosome 4. Predictive DNA testing of at-risk relatives of sufferers reduces anxiety, whatever the test outcome.
Symptoms of Huntington's disease typically first appear between ages 30 and 50 years and include
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