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Huntington Disease

Overview :

Huntington disease is also called Huntington chorea or hereditary chorea. The word chorea comes from the Greek word for "dance" and refers to the involuntary movements of the patient's feet, lower arms, and face that develop as the disease progresses. It is occasionally referred to as "Woody Guthrie's disease" for the American folk singer who died from it. Huntington disease (HD) causes progressive loss of cells in areas of the brain responsible for certain aspects of movement control and mental abilities. A person with HD gradually develops abnormal movements and changes in cognition (thinking), behavior and personality.

The onset of symptoms of HD usually occurs between the ages of 30 and 50; although in 10% of cases, onset is in late childhood or early adolescence. Approximately 30,000 people in the United States are affected by HD, with another 150,000 at risk for developing this disorder. The frequency of HD is 4-7 cases per 100,000 persons.

Causes and symptoms

Huntington disease is caused by a defect in the HD gene (an inherited unit which contains a code for a protein), which is located on the short arm of chromosome 4. The gene codes for a protein called huntingtin, whose function is not known as of early 2005. The nucleotide codes (building blocks of genes arranged in a specific code which chemically forms into proteins), contain CAG repeats (40 or more of these repeat sequences). The extra building blocks in the huntingtin gene cause the protein that is made from it to contain an extra section as well. It is currently thought that this extra protein section, or portion, interacts with other proteins in brain cells where it occurs, and that this interaction ultimately leads to cell death.

The HD gene is a dominant gene, meaning that only one copy of it is needed to develop the disease. HD affects both males and females. The gene may be inherited from either parent, who will also be affected by the disease. A parent with the HD gene has a 50% chance of passing it on to each offspring. The chances of passing on the HD gene are not affected by the results of previous pregnancies.

The symptoms of HD fall into three categories: motor or movement symptoms; personality and behavioral changes; and cognitive decline. The severity and rate of progression of each type of symptom can vary from person to person.

Early motor symptoms include restlessness, twitching and a desire to move about. Handwriting may become less controlled, and coordination may decline. Later symptoms include:

  • dystonia, or sustained abnormal postures, including facial grimaces, a twisted neck, or an arched back
  • chorea, in which involuntary jerking, twisting or writhing motions become pronounced
  • slowness of voluntary movements, inability to regulate the speed or force of movements, inability to initiate movement and slowed reactions
  • difficulty speaking and swallowing due to involvement of the throat muscles
  • localized or generalized weakness and impaired balance ability
  • rigidity, especially in late-stage disease

Personality and behavioral changes include depression, irritability, anxiety and apathy. The person with HD may become impulsive, aggressive or socially withdrawn.

Cognitive changes include loss of ability to plan and execute routine tasks, slowed thought, and impaired or inappropriate judgment. Short-term memory loss usually occurs, although long-term memory is usually not affected. The person with late-stage HD usually retains knowledge of his environment and recognizes family members or other loved ones, despite severe cognitive decline.

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