Huntington's Disease (HD)

WHAT IS HUNTINGTON'S DISEASE?

Huntington's Disease is an inherited condition which affects people in middle age. It attacks nerve cells in the brain and as these cells slowly degenerate, a person with HD loses all control of all his/her mental and physical abilities.

HOW IS HUNTINGTON'S DISEASE PASSED ON?

The pattern of inheritance for Huntington's Disease is autosomal dominant Where one parent has Huntington's disease, there is a 50% (1 in 2) chance of each and every child being affected.

A PERSON WITH HUNTINGTON'S DISEASE MAY BE AFFECTED IN SOME, OR ALL, OF THE FOLLOWING WAYS:

The disease usually begins with mild symptoms spread over a number of years. These include forgetfulness, clumsiness, and personality changes (for example, the affected person might become irritable, moody or depressed, and might behave strangely at times). As the disease progresses, physical and mental control steadily deteriorates. The affected person develops jerky movements and walking, talking and eating become difficult. He/she loses the ability to think clearly, plan, solve problems and remember. People with HD can live for ten to 20 years from the onset of the disease. Death is not usually due to Huntington's Disease itself but to another physical cause such as heart failure or pneumonia.

• About 1 in every 10,000 babies born in the UK will have inherited the faulty
• gene which leads to Huntington's Disease.
• Some people talk about 'Huntington's Chorea' instead of 'Huntington'sDisease'. Chorea (pronounced ker-ree-er) comes from the Greek word for dancing and describes
• the jerky movements made by people with the condition. But the word disease' is used more often than 'chorea' these days because the jerky movements are only one symptom of the disorder.
• Huntington's Disease is named after George Huntington, an American doctor who was the first to describe the disorder in 1872.

Although people who have inherited the faulty HD gene can develop HD at any age, most only start to show signs of the disease between the ages of 30 and 50. This means that some adults only find out they are at risk of developing HD when a parent shows signs of the disease later in life. By this time, these adults may have a family of their own, in which case not only are they at risk of developing HD, but their children are too. In families affected by H D, it is possible for a family member who is at risk to have a test to see if they have inherited the faulty gene. There is as yet no cure for HD, so a person has to have counselling to help him/her prepare for the result. A positive result means that he/she has inherited the faulty gene and will develop the disease. A negative result means that neither he/she nor any children he/she may have are at risk of developing the disease.

If you are interested in finding out more about Huntington's Disease, you can write (enclosing an A5 stamped addressed envelope) to: The Huntington's Disease Association, 108 Battersea High Street, London SW113HP. www.hda.org.uk

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