Lottie Stewart Hospital
What is Huntington's Disease?
Huntington Disease is an inherited disease of the nervous system. This causes changes including:
- movements (chorea) and unsteadiness
- swallowing and speech difficulties
- emotional swings, irritability and feelings of sadness
- some memory loss, and reduced ability to plan and organise and eventually a type of dementia
- some lack of awareness of the changes taking place
- weight loss and increased need for food
The genetic change , or mutation, is due to an increase (or expansion) in the genetic "building blocks" (known as CAG trinucleotides) in the HD gene. The gene responsible was isolated in 1993. It is dominant. This means that if the gene is present, the symptoms or changes of Huntingtons disease will eventually appear. Each child of an affected parent has a fifty per cent chance of inheriting the abnormal gene as has each brother or sister of an affected person. Men and women are equally at risk. Symptoms or changes of Huntingtons disease usually appear between 35-45 years of age but may come on earlier or later.
