Huntington's disease (HD) is a fatal condition typically characterized by involuntary movements and dementia. The disease is caused by genetically programmed degeneration of brain cells, called neurons, in certain areas of the brain. This causes uncontrolled movements, loss of intellectual faculties and emotional disturbance.
The disease is a hereditary disorder passed on by a parent to child through a mutation in a gene. In North America, Huntington's disease occurs in about one of every 10,000 to 20,000 persons, affects males and females equally and crosses all ethnic and racial boundaries. Typically, symptoms begin between age 30 and 55. Symptoms appear in children in about 10 percent of families and children who develop the juvenile form of the disease rarely live to adulthood.
Each child of a parent with Huntington's disease has a 50 percent chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it on to subsequent generations. A person who inherits the HD gene will eventually develop the disease. Whether one child inherits the gene has no bearing on whether others will or will not inherit the gene. In 1 percent to 3 percent of individuals with HD, there is no family history of HD.
Early symptoms of Huntington's disease include:
In addition, patients may notice problems in their daily activities such as driving, learning new things, remembering a fact or making a decision.
As the disease progresses, concentration and short-term memory decline and involuntary movements increase. The ability to walk, speak and swallow deteriorates. Eventually, patients become unable to care for themselves. Death may occur from complications such as choking, infection or heart failure.
At this time, there is no known cure or treatment to reverse the course of the disease. There are treatments available to reduce the severity of some of its symptoms. As the disease progresses and a person's ability to tend to their own needs reduces, carefully managed multidisciplinary care giving becomes increasingly necessary.