Huntington's disease a fatal progressive form of dementia
Huntington's disease is an inherited progressive form of dementia in which personality, memory and moods change as the disease advances. First described by Dr. George Huntington in 1872,
Huntington's disease affects an estimated 30,000 Americans, a prevalence of about 1 in every 10,000 people. The prevalence of Huntington disease is between three and seven per 100,000 in populations of Western European descent.
Huntington’s is a degenerative disease characterized by jerking uncontrollable movement of the limbs, trunk, and face, progressive psychiatric difficulties and loss of mental abilities.
Cause of Huntington's disease
Huntington's disease is inherited. The gene that causes Huntington's disease was discovered in 1993. It is an autosomal dominant disease that can be inherited from either the father or the mother.
Any child, male or female, has a fifty percent chance of getting the disease themselves. Everyone who carries the gene will develop the disease. Huntington's does not skip generations.
Age of onset of Huntington's Disease
Huntington's disease typically reveals itself between the ages of 30 and 45. It is this fact that has allowed the disease to be passed on through the generations as people with the disease have often already had children before symptoms become apparent.
Juvenile Huntington’s occurs in approximately 16% of all cases of Huntington's. The juvenile form of Huntington's disease (also known as Westphal variant or akinetic-rigid Huntington's), develops before the age of 20. The disease progresses rapidly and can produce such severe muscle rigidity that the affected person may not be able to move at all.
Signs and Symptoms of Huntington's disease
Early symptoms of Huntington's disease include:
Progressive problems of lack of coordination, mood and muscular control,irritability, anxiety, depression, mood swings, forgetfulness, clumsiness, involuntary twitching. Problems with thinking, concentration and short term memory.
Later Symptoms of Huntington's disease
Later Symptoms of Huntington's disease include;
Deteriorating ability to concentrate and with short-term memory
An increase in involuntary movements of the head, trunk and limbs. Walking, speaking and swallowing abilities deteriorate.
Behavioral problems include irritability, aggression and other anti social behaviors. People unaware of their family history may come to the attention of the police and prison services because of their behavior.
Eventually the person is unable to care for themselves and requires total nursing care.
Treatment of Huntington's disease
At the present time there is no cure for Huntington's disease. There are medications than can help make some of the symptoms more tolerable and the person affected a bit more comfortable.
Prognosis in Huntington's disease The disease progresses without remission over 10-20 years. Death for most people with Huntington's disease is usually a result of infection, pneumonia, heart failure or choking.
Genetic Testing for Huntington's disease
The HD gene was isolated in 1993. A test is available that can accurately determine whether a person
carries the affected gene. The test is simple to do and involves giving a small amount of blood for analysis.
The test cannot predict when symptoms of Huntington's disease will begin.
Because there is no cure some people choose not to take the test.
Testing can help an adult decide whether or not to have children.
Famous person with Huntington's disease folk singer Woody Guthrie.
Huntington's disease is also known as Huntington's Chorea.
