Huntington's Disease

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:

  • Causes

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

  • 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
    • MRI scan
    • PET scan

    CT Scan of the Head
    CT Scan of the Head
    Copyright © Nucleus Medical Media, Inc.

    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.

  • 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.

  • 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

    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

  • Treatment

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