Huntington's disease 

Introduction 

Huntington's disease

An expert explains the effects of Huntington's disease. Lee, 39, talks about his life with Huntington's and the importance of getting tested if you have a family history of it.

Current research

Research is underway to find disease-modifying drugs and new treatments for the symptoms of Huntington's disease.

Exciting progress has been made in identifying potential ways of slowing down or halting the disease. For example, 'switching off' the faulty gene that causes Huntington's disease.

To see what clinical trials on Huntington's disease are running right now, go to Huntington's disease - clinical trials or visit the European Huntington's Disease Research Network.

Huntington's disease is an inherited disease of the brain. There is no cure, but much can be done to help families.

The disease damages some of the nerve cells in the brain, causing deterioration and gradual loss of function of areas of the brain. This affects movement, cognition (perception, awareness, thinking, judgement) and behaviour.

Early symptoms, such as personality changes, mood swings and unusual behaviour, are often overlooked at first and attributed to something else. See Huntington's disease - symptoms for more information.

Huntington's disease was originally called Huntington's chorea, after the Greek word for dancing. This is because the associated involuntary movements of the condition can look like jerky dancing.

Who is affected?

Both men and women with a family history of Huntington's can inherit the disease. Symptoms usually start to show during adulthood. 

Juvenile (children's) Huntington's disease develops before age 20. Only 5-10% of people with Huntington's develop the condition at a very young age, and the pattern of features may be different.

How common is it?

UK studies have found that approximately 6-7 people per 100,000 are affected by Huntington's disease.

However, the true figure is likely to be much higher. About 6,700 people diagnosed with the disease are currently being cared for by the Huntington's Disease Association. This means that the UK prevalance must be at least 12.4 per 100,000. 

There are also people who have inherited the disease but have not yet developed symptoms. This group, who are unaware they carry the disease, may have had children and passed on the disease without realising.

It is thought that the number of people who have the Huntington's gene and are not yet affected is twice that of those who have symptoms.

Outlook

There is no cure for Huntington's disease, and its progress cannot be reversed or slowed down.  

Medication can manage some of the symptoms, such as irritability or excessive movement. Therapies (such as speech and language therapy and occupational therapy) can help with communication and day-to-day living (see Huntington's disease - treatment for more information).

Generally, the disease progresses and gets worse for around 10 to 20 years until the person eventually dies. In the later stages of Huntington's disease, the person will be totally dependent and will need full nursing care.

Death is usually from a secondary cause, such as heart failure, pneumonia or another infection.

Last reviewed: 20/01/2011

Next review due: 20/01/2013

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