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American Family Children's Hospital
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Huntington's Disease

UW Health's Movement Disorders Program, based at University Hospital in Madison, Wisconsin, treats patients with Huntington's disease.
 

What is Huntington's Disease?

 

Huntington's disease is a dominantly inherited disease that results in degeneration or loss of brain cells in certain areas of the brain. Huntington's disease often begins in the fourth or fifth decade of life but may begin earlier. The degeneration in the brain causes uncontrolled movements, loss of intellectual abilities and emotional disturbances.

 

What Causes Huntington's Disease?

 

Huntington’s disease is a genetic disease. A child of a person with Huntington’s disease has a 50 percent chance of inheriting the disease. The genetic abnormality results in degeneration of brain cells that control movement, intellectual ability and behavior.

 

Huntington's Disease Symptoms

  • Onset of abnormal, involuntary movements (chorea) is a hallmark in the clinical diagnosis. These movements may affect individuals at any stage of the disease and to different degrees of severity.
  • Early symptoms: mood swings, depression, difficulty learning new things, remembering facts or problems making decisions making decisions, clumsiness and slurred speech.
  • Progressed symptoms: severe motor disability affecting every aspect of motor function including balance, speech and swallowing, problems with concentration or intellectual tasks and difficulty controlling behavior.
  • The speed of disease progression is different from person to person.

Diagnosis

 

A definitive diagnosis of the abnormal gene defect leading to Huntington's disease can be obtained by DNA analysis (via a specialized blood test.) Genetic counseling is highly recommended prior to testing, especially in people that do not display obvious symptoms.

 

Treatment

 

Despite progress in the genetics and diagnosis of this disease, a cure is not yet available. The treatment consists of symptomatic and supportive assistance. Extensive research is ongoing to develop a cure as well as to see if medications may slow the progressive degeneration of the brain cells. Some medications including antidepressants, antipsychotics and others may help to relieve troublesome symptoms, but do not slow or stop the worsening process. The care of Huntington’s disease patients is best delivered by a movement disorder specialist.

 

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