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Definition  

Huntington's disease (HD) is an inherited disorder that affects the brain. HD causes slow, progressive degeneration of nerve cells in certain areas of the brain. Eventually, HD results in:

  • Abnormal body movements
  • Gradual deterioration or loss of intellectual abilities
  • Behavior problems
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Causes  

A faulty gene on chromosome #4 causes HD. All people who inherit the faulty gene may eventually develop HD.

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Risk Factors  

Having family members with HD increases your chance of developing HD. Each person whose parent has HD has a 50% chance of inheriting the disorder.

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Symptoms  

Symptoms most often develop between the ages of 30-50 years. Symptoms are mild at first and are often barely noticeable, but usually worsen over 15-20 years.

Abnormal body movements that worsen over time, may include:

  • Sudden jerks or uncontrolled movements of the limbs or trunk
  • Facial grimacing
  • Continuous need to turn head and shift gaze
  • Walking that is unsteady or dance-like

Gradual deterioration or loss of intellectual abilities may include:

  • Difficulty with eating and swallowing, which may result in weight loss
  • Difficulty dressing, sitting, and caring for oneself
  • Grunting or poor articulation of speech

Loss of intellectual and behavior problems may include:

  • Trouble with attention and awareness
  • Confusion or disorientation
  • Loss of memory
  • Loss of judgment
  • Loss of ability to think rationally
  • Irritability and moodiness
  • Depression (common)
  • Anxiety
  • Social withdrawal or antisocial behavior
  • Irresponsible behavior
  • Obsessive-compulsive behavior
  • Personality changes
  • Psychosis—a severe emotional and behavioral disorder that often interferes with a person's ability to relate to others and to function in daily life
  • Paranoia—a mental disorder that involves feelings of being watched, followed, or harmed by others
  • Hallucinations—the perception of a thing or person that is not present

Ultimately, HD can:

  • Cause the loss of the physical and mental ability to care for oneself
  • Cause severe disability, making full-time or nursing home care necessary
  • Result in death, often due to a fall or pneumonia
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Diagnosis  

The doctor will ask about your symptoms and medical history (including family medical history). A physical exam will be done. Blood tests can rule out other conditions. You may need to have imaging tests, which take pictures of internal body structures. Imaging tests include:

CT Scan of the Head  
CT Scan of the Head

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There is a test that can determine if a person has inherited the gene for HD. This test may help to make the diagnosis of HD. It may also help to determine if a person has inherited the HD gene before symptoms appear. Genetic counseling is recommended before taking this test to review risks and benefits.

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Treatment  

There is no cure for HD. Treatment aims to help control symptoms.

Medications  

Drugs can help control abnormal movements and emotional symptoms of HD. These include:

  • Tetrabenazine
  • Antipsychotics
  • Antidepressants

Physical Fitness  

Staying physically active helps people with HD to function better and longer. Often physical and occupational therapy may be of some benefit.

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Prevention  

There is no way to prevent the onset of HD if a person has inherited the gene for the disorder. If you have a family history of HD, talk with a genetic counselor.