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Huntington's Disease

Huntington's disease or Huntington's chorea is an inherited disorder characterized by abnormal body movements called chorea, and loss of memory. We also have evidence that doctors as far back as the Middle Ages knew of this devastating disease. The incidence is 5 to 8 per 100,000. It takes its name from the New York physician George Huntington who first described it precisely in 1872.

Huntington's disease is inherited in the autosomal dominant fashion, meaning that it is on a dominant allele and offspring of carriers have a 50% chance of inheriting the disease. (Crossing of a heterozygote with a homozygous recessive partner.) Symptoms of the disorder include loss of cognitive ability (thinking, speaking), changes in personality, jerking movements of the face and body in general and unsteady walking. These symptoms develop into dementia and cognitive decline (not mental retardation which is an older term referring to the lack of development of mental ability rather than loss of it.) and an advanced form of jerking called chorea, the Greek word for dance. It usually takes between 10-25 years for the disease to kill someone, and it is fatal 100% of the time. The disease is onset in the 30s and 40s in most cases; however there is an early childhood form that starts at the age of 2. Victims of this form rarely reach adulthood. One interesting fact about the disease is that it contributes to a chemical imbalance that leads many victims to commit suicide. This is also believed in part to be a result of the position sufferers find themselves in. Another interesting fact about the disease is that its origin is being traced back to a woman in the small Venezuelan fishing village of Barranquitas. Families there have a high presence of the disease and geneticists and doctors view this community as invaluable in the research of the disease.

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