Huntington's disease

background

Huntington's disease (HD) is a neurological (nervous system) condition resulting from genetically programmed degeneration of neurons (brain cells) in certain areas of the brain. HD, previously known as Huntington's chorea and chorea maior, affects up to eight individuals per 100,000. It affects one out of 20,000 people of Western European descent and one out of one million people of Asian and African descent.
HD causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. HD is a familial disease, passed from parent to child through a mutation in the normal gene. HD causes cells in certain areas of the brain to slowly destruct. It usually starts in middle adulthood. HD gradually gets worse over time. In one to three percent of individuals with HD, no family history of HD can be found.
Affected people may be unable to take care of themselves and may need to be put in an institution. Death occurs in most affected people between 10 and 30 years after the disease is diagnosed. HD is not contagious. Death is due to infection (usually pneumonia), fall-related injuries, or suicide. The suicide rate for HD sufferers is much greater than the national average.
Some early symptoms of HD are mood swings, depression, irritability, trouble driving, and trouble learning new things, remembering a fact, or making a decision. When the condition progresses, difficulty in concentrating on intellectual tasks may occur, and the patient may have difficulty eating and swallowing. The rate of disease progression (worsening of the disease) and the age of onset vary from person to person.
There is no cure and there are no treatments that slow the progression of HD. Medication may be used to treat psychosis, depression, or movement.
Brain cells in HD patients slowly die, so scientists are paying close attention to the process of genetically programmed cell death that occurs deep within the brains of individuals with HD.
Juvenile HD has an age of onset anywhere between infancy and 20 years of age. The symptoms of juvenile HD are different from those of adult-onset HD in that they generally progress faster and are more likely to exhibit rigidity and bradykinesia (very slow movement) instead of chorea (involuntary movements).

Related Terms

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