Huntington's Disease

(Huntington Chorea; HD)

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

Causes

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

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.

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
Mental function 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
  • 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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