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Huntington Disease

Huntington's disease (HD) results from genetically programmed degeneration of brain cells called neurons in certain areas of the brain. HD causes bizarre involuntary movements (chorea) and loss of intellectual abilities ( dementia ). HD is a familial disease, passed from parent to child through a mutation in the normal gene. The disorder was documented in 1872 by American physician George Huntington. The name "chorea" comes from the Greek word for "dance" and refers to the incessant quick jerky involuntary movements that are characteristic of this condition. Some early symptoms of HD are mood swings, depression, irritability or trouble driving, learning new things, remembering a fact, or making a decision. As the disease progresses, concentration on intellectual tasks becomes increasingly difficult and the patient may have difficulty feeding himself or herself and swallowing. An estimated one in 10,000 Americans has Huntington's disease, with about 30,000 known cases in the United States. About 150,000 Americans may be at risk of inheriting Huntington's disease from a parent. Medications are available to help manage the signs and symptoms of Huntington's disease, but treatments can't prevent the physical and mental decline associated with this condition.

Huntington's disease is an inherited condition characterized by abnormal body movements dementia and psychiatric problems. Each child of an HD parent has a 50-50 chance of inheriting the HD gene. Whether one child inherits the gene has no bearing on whether others will or will not inherit the gene. Signs and symptoms usually develop in middle age, and men and women are equally likely to develop the condition. As the disease progresses, concentration and short-term memory diminish and involuntary movements of the head, trunk and limbs increase. Eventually the person is unable to care for him or herself. Death follows from complications such as choking, infection or heart failure. Since the discovery of the gene that causes HD, scientific research has accelerated and much has been added to our understanding of Huntington's Disease and its effects upon different individuals.

Symptoms of Huntington Disease

Some Symptoms of Huntington Disease :

  • Mood changes, including depression
  • Behavior changes
  • Mild tremor
  • Facial movements, grimaces
  • Need to turn head to shift the gaze
  • Progressive dementia
  • Difficulty remembering things
  • Clumsiness
  • Lack of concentration

Treatment of Huntington Disease

  • There are treatments available to help control the chorea , although these may have the side effect of aggravating bradykinesia or dystonia .
  • Drugs such as tetrabenazine can be used to reduce the build up of the chemical dopamine in the brain and help control the involuntary movements. Medicines can be taken in liquid form if necessary.
  • Symptomatic treatment for the dementia is similar to that used for any organic brain syndrome .
  • In a lot of cases, medication such as anti-depressants should be considered as they can improve mood swings and treat depression.

 


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