fullcortex.net

(1999)

Do you have any information on Huntington’s disease?

Huntington’s disease is a degenerative disease of the brain that was recognized over 100 years ago by an American physician, George Huntington.

It also is known as Huntington’s chorea, as the disease causes an involuntary, complex disorder of movement called chorea (from the Greek word for dance). The other major manifestation of the disease is progressive mental deterioration.

Early symptoms of Huntington’s disease include subtle personality changes and moodiness that may progress to paranoia, and mild facial movement disorders such as grimacing. Unfortunately, the disease progresses so that ultimately most affected people become physically incapacitated and demented, and many require institutional care.
This disease usually has its onset between ages 35 and 50 and affects 1 or 2 persons in 20,000 people. The underlying cause is an autosomal, dominant genetic defect, meaning that it occurs equally in both sexes and can be inherited from one parent.
Because of the relatively late onset of its signs and symptoms, many persons who carry the gene that causes Huntington’s disease do not find out until they already have had children. If there is a family history of Huntington’s, genetic counseling in early adult life is very important, as there is a 1-in-2 chance that each offspring of an affected individual will inherit the causative gene. The psychological and ethical issues associated with genetic testing are complex, and warrant consultation and careful consideration by experts in the field.
Although the gene for Huntington’s chorea has been identified, there is no treatment for the underlying, progressive degenerative process in the brain. Medications may be of benefit to lessen the symptoms of chorea.