Reviewed by Dr. Clement J. Cheng
Huntington’s Disease (HD) is a hereditary degenerative brain disease that affects one in every 10,000 Americans. HD causes cells in the caudate nucleus and brain cortex to die, resulting in deterioration of intellectual ability, emotional control, balance, and speech. Chorea, or uncontrollable shaking, is a trademark symptom of HD. No treatment reverses or stops the disease.
HD occurs as a single, autosomal, dominant gene. Because it is not a sex-linked condition, men and women share an equal risk of inheriting the disease. HD’s dominance presents a serious threat to future progeny; offspring of an HD parent stand a 50 percent chance of receiving HD.
A small number of cases of HD are sporadic, meaning that they have not been traced back to a known family history. It is possible that these cases are the result of a genetic mutation. It is also possible that the family history of the patient may be incomplete, so they are unaware of their genetic link to HD.
HD progression varies. Although HD onset typically occurs in middle age, there are some cases of early-onset HD. HD usually spans from 10 to 30 years. Patients with HD usually die of infection, or fall-related injuries. Early-onset HD progresses more rapidly than adult onset HD.