Huntington’s disease is very uncommon: in Sweden, 5 out of every 100,000 people are estimated to have the disease. The symptoms tend to arise at the age of 40–50, though in rare cases Huntington’s can begin before the age of 20 and later in life. The disease, which is hereditary, progresses for 15–20 years and leads to death.
Huntington’s disease is a condition that is shrouded in myths and prejudices. For a long time, it was colloquially referred to as the “dancing disease” because of the “throwing movements” that are typical of the disease. This lack of understanding, among other things, led to the condition being regarded as shameful, and it was therefore denied by many families of people affected. The grimaces, and some of the other physical symptoms, can also lead you to think – wrongly – that the person is severely under the influence of alcohol.
The person’s capacity for thought and short-term memory are affected at the very start of the disease. Increasing difficulties thinking through, planning and carrying out activities are also part of the early symptoms of Huntington’s disease. A range of other symptoms, which often occur early in Alzheimer’s disease and vascular dementia, tend to arise later. These may include difficulties finding the right word (anomia) and recognising objects (agnosia). In the late stage of the disease, all intellectual (cognitive) functions are severely impaired.
There is no curative treatment for Huntington’s disease. Medication can help with depression, worry and anxiety, which are common symptoms. In the early stages of the disease, low doses of neuroleptic drugs can be effective for the involuntary movements. In the end stage of the disease, balance problems and problems eating and swallowing mean that the person requires total care.
Huntington’s is a hereditary disease caused by damage to the genetic material. The damaged gene can be passed on to both girls and boys. As the condition is autosomal dominant, the child may develop Huntington’s even if only one parent has had the disease. It also means that children who do not inherit the gene will not develop the disease, either.
A blood test is now available that will reveal whether a person has inherited the gene that causes Huntington’s disease. The tests are voluntary and also include a psychosocial assessment.