Memory Disorders | Creutzfeldt-Jakob Disease

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.

Although there is no cure for Creutzfeldt-Jakob disease (CJD), early diagnosis is important because the underlying cause of the dementia may be treatable.

Currently, there is no single diagnostic test. If CJD is suspected, you may undergo a series of tests. Your doctor will conduct a neurological examination and other tests such as a spinal tap to rule out more common and treatable forms of dementia and an electroencephalogram (EEG) to record the brain's electrical pattern, which can identify a specific abnormality that occurs in CJD.

Computerized tomography (CT) of the brain can help rule out the possibility that symptoms are caused by other problems such as a stroke or a brain tumor.

One of the most effective diagnostic tools is a magnetic resonance imaging (MRI) brain scan, which can reveal patterns of brain degeneration characteristic of CJD.

In cases where diagnosis is difficult even after an MRI, you may undergo a brain biopsy in which a neurosurgeon removes a small piece of tissue from the area of the brain that appears abnormal. The tissue is examined by a neuropathologist.

After death, a definitive diagnosis can be made with an autopsy that examines the whole brain.

The UCSF Memory and Aging Center consults with doctors from throughout the world who are treating patients with CJD. If you would like your doctor to consult with UCSF, ask your doctor to fax your records to the attention of Dr. Michael Geschwind at (415) 476-4800. Doctors at the Memory and Aging Center will review your records.

Copies of MRI films can be sent to:

Currently, there is no cure for Creutzfeldt-Jakob disease (CJD). Researchers have tested many drugs, including amantidine, steroids, interferon, acyclovir, antiviral agents and antibiotics. None has shown consistent benefit.

Treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Drugs can help relieve pain if it occurs. The drugs clonazepam and sodium valproate may help relieve myoclonus or irregular, jerking movements.

Researchers at the UCSF Memory and Aging Center and elsewhere are trying to identify compounds for a therapy or cure for CJD and other diseases caused by the infectious particles called prions.