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What is Creutzfeldt-Jakob Disease?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations
Related NINDS Publications

What is Creutzfeldt-Jakob Disease?
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. Typically, onset of symptoms occurs at about age 60.. There are three major categories of CJD: sporadic CJD, hereditary CJD, and acquired CJD. There is currently no single diagnostic test for CJD. The first concern is to rule out treatable forms of dementia such as encephalitis or chronic meningitis. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that is can be examined by a neurologist. Because a correct diagnosis of CJD does not help the patient, a brain biopsy is discouraged unless it is need to rule out a treatable disorder. While CJD can be transmitted to other people, the risk of this happening is extremely small.

Is there any treatment?
There is no treatment that can cure or control CJD. Current treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks.

What is the prognosis?
About 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

What research is being done?
The leading scientific theory at this time maintains that CJD is caused by a type of protein called a prion. The harmless and the infectious forms of the prion protein are nearly identical, but the infectious form takes a different folded shape than the normal protein. Researchers are examining whether the transmissible agent is, in fact, a prion and trying to discover factors that influence prion infectivity and how the disorder damages the brain. Using rodent models of the disease and brain tissue from autopsies, they are also trying to identify factors that influence the susceptibility to the disease and that govern when in life the disease appears.

919 N. Michigan Avenue
Suite 1100
Chicago IL 60611-1676
info@alz.org
www.alz.org
Tel: 312-335-8700 800-272-3900
Fax: 312-335-1110

Centers for Disease Control (CDC)

1600 Clifton Road, N.E.
Atlanta GA 30333
inquiry@cdc.gov
www.cdc.gov
Tel: 800-311-3435

Creutzfeldt-Jakob (CJD) Foundation Inc.

P.O. Box 611625
No. Miami FL 33261-1625
crjakob@aol.com
http://cjdfoundation.org/
Tel: 954-704-0519 305-891-7579
Fax: 954-436-7591 305-893-9050

National Organization for Rare Disorders (NORD)

P.O. Box 8923
(100 Route 37)
New Fairfield CT 06812-8923
orphan@rarediseases.org
www.rarediseases.org
Tel: 203-746-6518 800-999-NORD (-6673)
Fax: 203-746-6481