Introduction

Huntington's disease is an inherited condition that damages certain nerve cells in the brain.

This brain damage gets progressively worse over time and can affect movement, cognition (perception, awareness, thinking, judgement) and behaviour.

Early features can include personality changes, mood swings, fidgety movements, irritability and altered behaviour, although these are often overlooked and attributed to something else.

This is because the involuntary movements associated with the condition can look like jerky dancing.However, "disease" is now the preferred term, because the condition involves a lot more than just abnormal movements.

Huntington's disease is caused by an inherited faulty gene. However, in around 3% of cases, there's no family history of the condition, normally because the parents died at a young age.

They may also test a number of physical functions, such as your eye movements, balance, control, movement and walking. Your speech and cognition may also be tested. All of these can be affected by Huntington's disease.

Genetic testing can be usedto confirm the diagnosis.

Treatments for Huntington's diseaseaim to improve any mood disturbance; this is done tomaintain skills used in daily living that can deteriorate over time.

Medication can help 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.

Support is also available for thefamily of a person with Huntington's disease. This includes, for example, testing family members who don't have any of the condition's features (manifest) to see whether they carrythe gene, or help with choosing a suitable care home in advanced cases.

During the condition's later stages, the person will be totally dependent and need full nursing care.

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

Who's affected by Huntington's disease?

Both men and womenwith a family history of Huntington's diseasecan inheritthe condition. Symptoms usually start toappear during adulthood.

Juvenile (children's) Huntington's disease develops before the age of20. Only5-10% of people with Huntington's disease develop it at a very young age, and the pattern of features may be different.

It was previously thoughtthat 4-6people in a population of 100,000 wereaffected by Huntington's disease. However, UK research carried out in 2012 found the actual figure for those affected by the condition to be about 12people per 100,000.

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

Current research

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

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

See our page on clinical trials for Huntington's disease for details of the clinical trials that are currently running. You can find out more by visiting the European Huntington's Disease Network .

Information about you

If you haveHuntington's disease, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register .

Content supplied by the NHS Website

Medically Reviewed by a doctor on 24 Jun 2016