NINDS Creutzfeldt-Jakob Disease Information Page

Condensed from Creutzfeldt-Jakob Disease Fact Sheet

Table 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?
Clinical Trials
Organizations
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 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. P.O. Box 5312 Akron, OH 44334 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

Related NINDS Publications and Information

Creutzfeldt-Jakob Disease Fact Sheet
Creutzfeldt-Jakob Disease (CJD) fact sheet 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).
NINDS Transmissible Spongiform Encephalopathies Information Page
Transmissible Spongiform Encephalopathies (TSEs) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
The Dementias: Hope Through Research
Information booklet about Alzheimer's disease, vascular dementia, and other types of dementia compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Testimony on Transmissible Spongiform Encephalopathies, April 4, 2001.
Congressional testimony on transmissible spongiform encephalopathies (TSEs), April 4, 2001.

Publicaciones en Español

Enfermedad de Creutzfeldt-Jakob

Prepared by:
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 May 16, 2012