Articles » NINDS Creutzfeldt-Jakob Disease Information Page: NINDS

NINDS Creutzfeldt-Jakob Disease Information Page: NINDS

Article title: NINDS Creutzfeldt-Jakob Disease Information Page: NINDS

Conditions: Creutzfeldt-Jakob Disease

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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.

 Organizations

Alzheimer's Association
919 North Michigan Avenue
Suite 1100
Chicago, IL 60611-1676
info@alz.org
http://www.alz.org/
Tel: 312-335-8700 800-272-3900
Fax: 312-335-1110

Centers for Disease Control and Prevention (CDCP)
1600 Clifton Road, N.E.
Atlanta, GA 30333
inquiry@cdc.gov
http://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: 305-893-9050/954-436-7591 305-893-9050

National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel: 203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481

Related NINDS Publications and Information

Fact Sheet: Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob Disease (CJD) fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

La Enfermedad de Creutzfeldt-Jakob
Información de La Enfermedad de Creutzfeldt-Jakob/Spanish-language fact sheet on Creutzfeldt-Jakob disease compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Creutzfeldt-Jakob Disease Fact Sheet for Healthcare Workers and Morticians
Creutzfeldt-Jakob disease information for healthcare workers and morticians, compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Congressional Testimony on Transmissible Spongiform Encephalopathies
Congressional testimony on transmissible spongiform encephalopathies (TSEs), April 4, 2001.

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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