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Studies with patientsTable of Contents (click to jump to sections)
What is Creutzfeldt-Jakob Disease?
Is there any treatment?
What is the prognosis?
What research is being done?
Organizations
Related NINDS Publications and Information
Additional resources from MEDLINEplus
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.
Select this link to view a list of studies currently seeking patients.
OrganizationsAlzheimer'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 5312
Akron, OH
44334
crjakob@aol.com
http://cjdfoundation.org
Tel: 800-659-1991
Fax: 330-668-2474
305-893-9050
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT
06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100
Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291
Related NINDS Publications and InformationFact 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 sobre enfermedad del Creutzfeldt-Jakob del Instituto nacional de desórdenes y del movimiento neurológicos/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).
Transmissible Spongiform Encephalopathies
No Description Available at This Time
Congressional Testimony on Transmissible Spongiform Encephalopathies
Congressional testimony on transmissible spongiform encephalopathies (TSEs), April 4, 2001.
NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.
All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.
Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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