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About Chorea

General Information

  • Two common types of chorea are Huntington's disease and Syndenham's chorea.
  • Huntington's disease is an inherited disorder of the nervous system. The disease is linked to a specific gene and characterized by gradual onset and progression of chorea and dementia. The children of an affected parent have a 50 percent chance of developing the disorder. About five people per 100,000 are affected.
  • The underlying cause is unknown, but the condition sometimes is associated with cell loss in certain portions of the brain.

Symptoms

  • Symptoms of Huntington’s disease usually do not begin until age 30 to 50. The initial symptoms are either abnormal movements or intellectual changes, and eventually both occur.
  • The earliest mental changes often consist of irritability, moodiness, and antisocial behavior, but a more obvious dementia eventually develops.
  • Abnormal movements initially appear as restlessness, but grossly abnormal movements eventually develop.

Treatment

  • Medication
    • Medical treatment to correct imbalances in dopamine and other brain chemicals and processes that help regulate movement and emotion can help control abnormal movements and aggressive behavior.
    • Side effects of these medications include sedation, depression, akathesia, apathy, slurred speech and trouble swallowing.
  • Surgery
    • Surgery may be an option if a patient is mentally fit after the onset of physical disabilities.
    • Stereotactic surgery allows doctors to pinpoint locations in the thalamus, a portion off the brain that relays sensory impulses, and create lesions that ease abnormal movements.

Outcome

  • Chorea is a progressive disease, and both movement and mental impairment eventually will progress.

The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the NeuroICU attending physician and team members direct your family member's care while in the ICU.  The NeuroICU team consists of the bedside nurses, nurse practitioners, physicians in specialty training (Fellows) and attending physicians. UCLA Neuro ICU Family Guide