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Huntington's disease

Huntington's disease

Huntington's disease is an inherited disease of the brain, for which there is no cure. The disease damages 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.

 

Huntington's disease was originally called Huntington's Chorea, after the Greek word for dancing, as the associated involuntary movements can look like jerky dancing.

 

Who is affected?

Both men and women with a family history of Huntington's can inherit the disease and symptoms usually start to show in adulthood. There are approximately 6,000 people with the disease in the UK.

 

Twice as many people may 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.

 

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

 

Treatment

A diagnostic blood test which looks for the specific genetic defect can be used to establish the diagnosis in an adult with symptoms of this disease. This test can also be used in unaffected healthy individuals who have a family history of Huntington's disease to establish whether or not they carry the faulty gene and will, therefore, at sometime develop the disease.

 

Regrettably, we do not yet have treatments which slow or prevent the onset of this condition, but there is an enormous collaborative international research endeavour directed at finding new effective therapies.

 

At the London Brain Centre we do however have medical, physical and supportive treatments and therapies available through the Wellington Hospital to alleviate and manage the symptoms of this disease.

 


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Brain and neurology guide: conditions and treatments