Huntington's Disease

What is Huntington's Disease ?

In Huntington's disease (also called Huntington's chorea, hereditary chorea, chronic progressive chorea, or adult chorea), degeneration in the cerebral cortex and basal ganglia causes chronic progressive chorea (dance like movements) and mental deterioration, ending in dementia.

Huntington disease is an inherited disorder. Many people with Huntington's have already had children by the time they are diagnosed. These children have a 50-50 chance of inheriting the gene for the disorder.

Huntington's disease usually strikes people between ages 25 and 55 (the average age is 35), but 2% of cases occur in children, and 5% occur as late as age 60. Death usually results 10 to 15 years after onset, from heart failure or pneumonia. Because the disease is hereditary, it's prevalent in areas where affected families have lived for several generations.

Causes of Huntington's Disease

The cause of Huntington's disease is unknown. Because it's transmitted as an autosomal dominant trait, either sex can transmit and inherit it. Each child of a parent with this disease has a 50% chance of inheriting it; a child who doesn't inherit it can't pass it on to his children's.

Signs & Symptoms of Huntington's Disease

Symptoms begin with memory loss (especially of recent events) or confusion, changes in personality and mood that may include aggressive and anti-social behavior, as well as clumsiness or uncontrolled muscle movements.

As the condition progresses, other symptoms of dementia appear, for example, loss of rational thought and poor concentration. Involuntary movements, difficulties with speaking and swallowing, weight loss, depression and anxiety may also occur.

Diagnostic Tests

Positron emission tomography and deoxyribonucleic acid analysis can detect Huntington's disease, but there is no reliable confirming test.

Treatment

Because there is no known cure for Huntington's disease, treatment is supportive, protective, and based on the patient's symptoms. Tranquilizers, as well as chlorpromazine, haloperidol, or imipramine, help control choreic movements, but they can't stop mental deterioration. They also alleviate discomfort and depression but increase patient rigidity. To control choreic movements without rigidity, choline may be prescribed.

Prevention Tips

The two main ways used to help reduce the effects of the disorder are for the person with Huntington's to keep active and physically fit through exercise, and the use of supportive care. Supportive care means using aids and services such as psychotherapy, physiotherapy, speech therapy, communicative and memory aids, social support services, and dietary advice.