Creutzfeldt-Jacob disease

Symptoms:

• personality changes
• hallucinations
• muscle twitching
• muscle stiffness
• nervous, jumpy feelings
• changes in gait (walking, locomotion)
• lack of coordination--stumbling, falls
• speech impairment
• poor enunciation (hard-to-understand speech or mumbling)
• sleepiness
• delirium or dementia develops rapidly
  • deterioration in all aspects of brain function
  • profound confusion, disorientation

Additional symptoms that may be associated with this disease:

• memory loss
• anxiety, stress, and tension

Signs and tests:

The history may be significant by the rapid onset and progression of symptoms. Characteristics of the disorder include rapidly progressive dementia, myoclonus (rapid and brief muscle contraction or "jerk"), and rigidity of the body.

A neurologic and motor system examination shows muscle twitching and spasm (myoclonus). There is a strong startle response. Muscle tone may be increased, or there may be weakness and muscle wasting (loss of muscle tissue). There may be abnormal reflexes or an increase in response of normal reflexes. Examination of visual fields shows areas of blindness that the person may not realize are present. There is loss of coordination related to visual-spatial perception changes and changes in the cerebellum, the area of the brain that controls coordination (cerebellar ataxia).

An EEG (electroencephalograph, a reading of electrical activity of the brain) shows characteristic changes indicating Creutzfeldt-Jacob disease.

Update Date: 02/09/00
Updated by: J. Gordon Lambert, MD, Associate Medical Director, Utah Health Informatics and adam.com editorial