Abstract

HUNTINGTON’S DISEASE: A REVIEW

Arti Sharma* and Vinita Sharma

ABSTRACT

Huntington’s disease (HD) is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person’s physical and mental abilities during their prime working years and has no cure. The disease is caused by an autosomal dominant mutation in either of an individual's two copies of a gene called Huntingtin. HD is known as the quintessential family disease because every child of a parent with HD has a 50/50 chance of carrying the faulty gene. Symptoms usually appear between the ages of 30 to 50, and worsen over a 10 to 25 year period. Ultimately, the weakened individual succumbs to pneumonia, heart failure or other complications. Over time, HD affects the individual’s ability to reason, walk and speak. There is no cure for HD. Full-time care is required in the later stages of the disease. Treatments can relieve some symptoms and in some improve quality of life. The best evidence for treatment of the movement problems is with tetrabenazine.

Keywords: .