Creutzfeldt-Jakob Disease - Signs and Symptoms

Creutzfeldt-Jakob disease (CJD) affects many areas of the brain. At UCSF, CJD is sometimes called the "great mimicker" because it causes symptoms that occur in many other neurological diseases. First symptoms vary widely and may include the following:

As the disease advances, patients may experience a rapidly progressive dementia and in most cases involuntary and irregular jerking movements called myoclonus. Patients also may appear startled and become rigid. In advanced stages of the disease, patients have difficulties with movement, swallowing and talking. In the final stage, patients lose all mental and physical function and may lapse into a coma. Many patients die from an infection such as pneumonia.

The average duration of disease -- from the onset of symptoms to death -- is four to six months. Ninety percent of patients die within a year. Some cases progress very rapidly, lasting only a few weeks before the patient's death, and others may last two or three years, especially if the disease occurs at an early age.