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NINDS Creutzfeldt-Jakob Disease Information Page

Condensed from Creutzfeldt-Jakob Disease Fact Sheet

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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 it 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.

NIH Patient Recruitment for Creutzfeldt-Jakob Disease Clinical Trials

Organizations

Column1 Column2
Alzheimer's Association
225 North Michigan Avenue
Floor 17
Chicago, IL   60601-7633
info@alz.org
http://www.alz.org
Tel: 312-335-8700 1-800-272-3900 (24-hour helpline) TDD: 312-335-5886
Fax: 866.699.1246

Centers for Disease Control and Prevention (CDC)
U.S. Department of Health and Human Services
1600 Clifton Road, N.E.
Atlanta, GA   30333
inquiry@cdc.gov
http://www.cdc.gov
Tel: 800-311-3435 404-639-3311/404-639-3543

Creutzfeldt-Jakob Disease (CJD) Foundation Inc.
341 W. 38th Street, Suite 501
New York, NY   10018
help@cjdfoundation.org
http://www.cjdfoundation.org
Tel: 800-659-1991
Fax: 330-668-2474

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

CJD Aware!
2527 South Carrollton Ave.
New Orleans, LA   70118-3013
cjdaware@iwon.com; info@cjdaware.com
http://www.cjdaware.com
Tel: 504-861-4627

Alzheimer's Disease Education and Referral Center (ADEAR)
National Institute on Aging
P.O. Box 8250
Silver Spring, MD   20907-8250
adear@nia.nih.gov
http://www.nia.nih.gov/alzheimers
Tel: 1-800-438-4380
Fax: 301-495-3334

Family Caregiver Alliance/ National Center on Caregiving
785 Market St.
Suite 750
San Francisco, CA   94103
info@caregiver.org
http://www.caregiver.org
Tel: 415-434-3388 800-445-8106
Fax: 415-434-3508

Department of Agriculture (USDA)
National Agricultural Library
10301 Baltimore Avenue
Beltsville, MD   20705-2351
http://www.nal.usda.gov
Tel: 301-504-5755/301-504-6856 (TDD/TTY)
Fax: 301-504-6927

Food and Drug Administration (FDA)
U.S. Department of Health and Human Services
5600 Fishers Lane, CDER-HFD-240
Rockville, MD   20857
http://www.fda.gov
Tel: 301-827-4573 888-INFO-FDA (463-6332)

World Health Organization
Avenue Appia 20
1211 Geneva 27
Switzerland,   info@who.int
http://www.who.int
Tel: (+ 41 22) 791 21 11
Fax: (+ 41 22) 791 3111

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Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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Last updated April 18, 2014