Huntington's Disease

Huntington’s disease, also known as Huntington’s chorea, is a hereditary degenerative disorder of the central nervous system where the nerves become damaged and parts of the brain begin to deteriorate.

Huntington’s Disease affects movement, behavior and cognition of the individual and his or her ability to walk, think and reason. Symptoms do not usually appear until mid-life but there is a rare juvenile-onset form of Huntington’s, which affects people under the age of 20.

Causes of Huntington’s disease

Huntington’s disease is caused by a genetic defect on chromosome #4. The defect causes a section of the DNA to occur many more times than it is supposed to. The section is called a CAG repeat, and it is supposed to be repeated 10 to 35 times, but it will repeat 36 to 120 times in someone who has Huntington’s disease.

As the gene is passed on from one generation to the next, the number of repeats tends to get larger. The larger the number of repeats, you will have a greater chance of developing symptoms at an earlier age. This is why if the disease is passed along in families, it becomes evident at younger and younger ages.

Each child of an HD parent has a 50-50 chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it to subsequent generations.However, those who inherit the HD gene, will develop the disease at some point in their life, and can pass it on to their children.

Symptoms of Huntington’s disease

Behavior changes may occur before movement problems, and can include:

  • Antisocial behavior
  • Mood swings
  • Hallucinations
  • Irritability
  • Moodiness
  • Restlessness or fidgeting
  • Paranoia
  • Speech changes
  • Loss of memory


A doctor will perform a series of tests to determine whether or not a person has Huntington’s Disease. The doctor will ask for a family history of the patient due to the fact that Huntington’s is hereditary. Physical exams will be performed as well as neurological exams. A doctor will look for signs of dementia, abnormal movements and reflexes, and hesitant speech while performing varies tests on the patient. A head CT scan and MRI scan will be given, and may show loss of brain tissue.

Treatment for Huntington’s Disease

There is no current cure for Huntington’s Disease, and there is no known way to stop the disease from getting worse. The goal of treatment is to slow down the course of the disease and help the person function for as long and as comfortably as possible.

Medications vary depending on the symptoms. Dopamine blockers may help reduce abnormal behaviors and movements. Drugs such as haloperidol, tetrabenazine, and amantadine are used to try to control extra movements.

It is important for those who care for a person with the disease that he or she be closely monitored, as suicide and depression are common among people with the disease. There are, however, many support groups for those who have Huntington’s disease that will help those cope with the disease. Please visit Huntington’s Disease Society for America for more information about support groups.

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