Huntington’s disease, also known as Huntington’s disease or Huntington’s chorea, is a hereditary disease of the brain that is today incurable. The disease is typically characterized by involuntary and uncoordinated movements. However, at the same time, loose muscle tone. Older names of the disease are chorea major, large St. Vitus ‘dance or simply St. Vitus’ dance.
In the German-speaking area, the disease is also often abbreviated to HD. HD stands for the English Huntington’s disease.
Sufferers of the disease suffer from the progressive destruction of a region of the brain, which is very important for the control of the muscles and fundamental mental functions. This area is the striatum.
In this area, defective cells destroy brain cells. These proteins are formed by a defect of the Huntington’s gene.
Disorders of the emotional life and the control of the muscles including the facial expressions, as well as the brain function altogether belong to the external symptoms of illness. In the final stage, the disease can lead to dementia. The fact that the facial expressions are often affected, it often leads to the fact that the progress of the disease in the external disease appearance is significantly more advanced than is really the case.
Huntington’s disease is usually around the age of 40 leading to the first symptoms of the disease. These are movement disorders and mental symptoms. The disease is autosomal dominant and belongs to the neurodegenerative diseases.
After the first symptoms, Huntington’s disease is always severe and, on average, leads to death after 15 years. With a few exceptions, sooner or later all carriers of the disease get sick. The allele causing the disease has been detectable since 1993 on the short arm of the fourth chromosome. Even with the unborn child through amniocentesis or chorionic villus sampling.