It is a hereditary condition that damages specific nerve cells in the brain.
This brain damage progressively worsens over time and can affect movement, perception, awareness, thinking, judgment and behavior.
Huntington’s disease was initially called Huntington’s chorea (“chorea” is the Greek word for dancing). This is because the involuntary movements associated with the condition may seem like abrupt dances.
However, “disease” is now the preferred term because the condition involves much more than abnormal movements.
An inherited defective gene causes Huntington’s disease. However, in about 3% of cases, there is no family history of the disease, usually because the parents died at a young age.
The first features may include:
- Changes of personality
- Humor changes.
- Restless movements.
- Altered behavior
Although they are often overlooked and attributed to something else.
If you have symptoms of Huntington’s disease, your GP will refer you to a clinical specialist (usually a neurologist) if you feel that your symptoms need further investigation.
The specialist will ask about your symptoms to evaluate how likely you have Huntington’s disease and rule out similar conditions.
They can also evaluate physical functions, such as eye movements, balance, control, movement, and gait. Speech and cognition can also be tested since Huntington’s disease can be affected.
Genetic tests can be used to confirm the diagnosis.
Treatment of Huntington’s disease
There is no cure for Huntington’s disease, and its progress can not be reversed or slowed down. As the condition progresses, it can put pressure on family and relationships.
The treatments for Huntington’s disease aim to improve mood alteration; this is done to maintain the skills used in daily life that may deteriorate over time.
The medication can help control some symptoms, such as irritability or excessive movement. Therapies such as speech and language therapy and occupational therapy can help with communication and daily life.
Support is also available for a person’s family with Huntington’s disease. This includes, for example, analyzing family members who do not have any of the characteristics (manifest) of the condition to see if they are gene carriers or helping to choose a suitable care home in advanced cases.
Huntington’s disease usually progresses and worsens in 10 to 25 years from when it first appears, before the person eventually dies.
During the later stages of the condition, the person will be dependent and will need complete nursing care.
Both men and women with a family history of Huntington’s disease can inherit the condition. Symptoms usually begin to appear during adulthood.
Juvenile (childhood) Huntington’s disease develops before the age of 20. Only 5-10% of people with Huntington’s disease develop it at a very young age, and the pattern of characteristics may be different.
Previously it was thought that 4-6 people in a population of 100,000 were affected by Huntington’s disease. However, UK research conducted in 2012 found that the number of people affected by the disease is approximately 12 people per 100,000.
It is believed that the number of people who have the Huntington gene and who are not yet affected is about twice as many as people who have symptoms.
Current research Research is being conducted to find disease-modifying drugs and new treatments for the characteristics of Huntington’s disease.