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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 (the most common form, in which people do not have any risk factors for the disease); hereditary (in which the person has a family member with the disease and tests positive for a genetic mutation), and acquired (in which the disease is transmitted by exposure to brain and nervous system tissue, usually through certain medical procedures. A form called variant CJD can be acquired by eating meat from cattle affected by a disease similar to CJD, called bovine spongiform encephalopathy (commonly called “mad cow” disease).  Symptoms of CJD include problems with muscular coordination, personality changes including progressive and severe mental impairment, impaired vision that may lead to blindness, and involuntary muscle jerks called myoclonus.  People eventually lose the ability to move and speak and enter a coma.  Tests that help in the diagnosis of CJD include electroencephalography (which measures brain waves), detection of certain proteins in the fluid that surrounds the brain and spinal cord, and magnetic resonance imaging.. 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 individual, a brain biopsy is discouraged unless it is need to rule out a treatable disorder. .

Is there any treatment?

There is no treatment that can cure or control CJD, although studies of a variety of drugs are now in progress. 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 70 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 National Institute of Neurological Disorders and Stroke (NINDS, a part of the National Institutes of Health, supports research on CJD through grants to major medical research organizations. 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


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Alzheimer's Association
225 North Michigan Avenue
Floor 17
Chicago, IL   60601-7633
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
Atlanta, GA   30333
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
Tel: 800-659-1991
Fax: 330-668-2474

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
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;
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
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
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
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
Tel: 301-827-4573 888-INFO-FDA (463-6332)

World Health Organization
Avenue Appia 20
1211 Geneva 27
Tel: (+ 41 22) 791 21 11
Fax: (+ 41 22) 791 3111

Related NINDS Publications and Information
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Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

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.

Last updated September 3, 2015