Aphasia -What is Huntington’s Disease? -Huntington’s Disease, also known as Huntington’s Chorea, is a severe, degenerative, hereditary disorder of the nervous system. -Who or what is Huntington’s Disease named for? -It is named after George Huntington, an American physician who first described the disease in 1872. -Who does Huntington’s Disease affect? -Anyone who inherits an abnormal gene on one of a pair of chromosomes designated as chromosome 4. One of the patient’s parents has to have had Huntington’s Disease in order for it to be passed on to their offspring. If one of the parents has the distorted chromosome, there is a 50 percent chance it will be passed on to their offspring(s). Anyone who inherits the chromosome will inevitably fall victim to Huntington’s Chorea.
-At what age or ages are most patients affected? -The symptoms usually begin when the patient is 35-40 years of age. However, the disease can strike anytime; children and people in their old age can also begin to show symptoms. Patients who develop Huntington’s Disease as children rarely live till adulthood. -What are some early symptoms of Huntington’s Disease? -Early symptoms include: Clumsiness, restlessness, dullness, irritability, carelessness about one’s personal grooming, depression, forgetfulness, mood swings, and involuntary twitching and/or lack of coordination. -What are some later symptoms of the disease? -Later symptoms include: Loss of memory, loss of concentration, pouting of lips, irregular raising of eyelids, pouting on cheeks. Soon, the head, shoulders, arms, and legs spasm uncontrollably. The disease gradually destroys the patient’s memory and intellect, and the patient loses all control over muscles and over mental ability.
-Is Huntington’s Disease fatal? -Yes, anyone who inherits it will inevitably die from it. At the present, there are no medications and no known cure. -Is there anyway to determine whether or not someone has the disease? -Yes. In 1993 the HD gene was isolated and a direct genetic test was developed that accurately determines whether or not someone carries the gene. However, since there is no known cure, many unsure patients decide not to take the test.
-Why is Huntington’s Disease often called Huntington’s Chorea? -It is called Huntington’s Chorea because often the patient’s spasms, combined with their voluntary walking, somewhat resemble a dance. Chorea means dance. It is derived from the same root as choreography. -Are there any celebrity casualties? -Woody Guthrie, the legendary depression-era folk singer and songwriter, was diagnosed with the disease in 1954, and lived with it for 13 years, until his death in 1967. Guthrie’s mother, Nora Guthrie, had also developed the disease, but as the rural Oklahoma doctors could not diagnose it, Mrs. Guthrie was placed in the state insane asylum, where she died. Medicine.