Creutzfeldt-Jakob Disease Fact Sheet for Healthcare Workers and Morticians

Description

Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that can be experimentally transmitted from one animal to another, as well as from human patients to other humans and animals. It affects about one person in every one million people each year worldwide. The low random incidence of CJD indicates that person-to-person transmission probably does not occur through normal contact. Spouses and other household members of people with sporadic CJD appear to be at no higher risk of contracting the disorder than the general population. CJD affects both men and women, and most often appears in people aged 50 to 75. The majority of cases occur on a sporadic basis via unknown mechanisms. In a few rare families (7-10% of all cases), the disease is hereditary. In a smaller number of cases (<1% of all cases) it is transmitted iatrogenically through contaminated surgical instruments (e.g. implanted EEG depth electrodes) and human material (e.g. human growth hormone, dura mater or corneal transplants).

Symptoms of CJD may be similar to those of other progressive dementias such as Alzheimer’s disease, but it tends to progress much more rapidly, usually culminating in death over the course of a few months to a year. In the early stages of CJD, individuals may have failing memory, changes in behavior, lack of coordination, or visual disturbances. As the disease progresses, mental deterioration becomes pronounced; involuntary movements, especially muscle jerks, appear; and the patient may become blind, develop weakness in the arms or legs, and ultimately become comatose. There is currently no effective treatment for CJD, but clinical trials of potential therapies may begin soon. Information about clinical trials may be available at http://clinicaltrials.gov.

NINDS scientists have developed a test for CJD that detects a protein in cerebrospinal fluid that can aid in the diagnosis of the disease. A press release about this test is available at the URL: http://www.ninds.nih.gov/news_and_events/pressrelease_transmissible_spongiform_encephalopathies_092596.htm. However, the only way to absolutely confirm a diagnosis of CJD is by brain biopsy or autopsy.

Scientists recently found evidence linking an outbreak of a variant form of CJD (vCJD), largely in Britain, to mad cow disease (bovine spongiform encephalopathy or BSE), a deadly brain disease- similar to CJD-that affects cattle. While there is still no definitive evidence for how this transmission took place, it is believed that transmission occurred through the ingestion of beef contaminated by the BSE agent.

Precautions

To protect themselves, health-care professionals should employ universal precautions when handling blood and spinal fluid samples from patients with CJD. When performing medical/surgical procedures and post-mortem examinations, the most important safety rule is to avoid self-induced injury from instruments used in the course of removing and processing tissues for pathological examination. In particular, avoid contact between contaminated material and skin with cuts or abrasions.

Normal sterilization procedures such as boiling or irradiating materials do not prevent transmission of CJD. Whenever possible, contaminated instruments and other materials should be discarded as medical pathological waste or destroyed by incineration. When this is not possible, special disinfection methods may be employed. Re-used instruments and materials should be kept moist until they can be appropriately decontaminated and cleaned. Detailed guidelines for infection control during patient care, for disinfection and disposal of CJD-contaminated material, and for performing an autopsy and embalming are available in the document, "WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies." The URL for this document is http://www.who.int/csr/resources/publications/bse/WHO_CDS_CSR_APH_2000_3/en/ . To receive a printed copy of this report, contact the World Health Organization at the following address:

Communicable Disease Surveillance and
Response Publications
World Health Organization
CH-1211 Geneva 27
Switzerland
Fax: +41 22 791 4198
Email: csr@who.int, Attention: Publications

Research

The NINDS conducts and supports research on neurodegenerative brain disorders such as CJD. This research is aimed at finding ways to prevent, treat, and cure these disorders.

Where can I get more information?

For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network (BRAIN) at:

BRAIN
P.O. Box 5801
Bethesda, MD 20824
(800) 352-9424
http://www.ninds.nih.gov

 Organizations:

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	Centers for Disease Control and Prevention (CDCP) 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
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)	National Institute of Allergy and Infectious Diseases (NIAID) National Institutes of Health, DHHS 6610 Rockledge Drive, MSC 6612 Bethesda, MD   20892-6612 http://www.niaid.nih.gov Tel: 301-496-5717
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	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
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) National Institutes of Health, DHHS 31 Center Drive, Rm. 9A06 MSC 2560 Bethesda, MD   20892-2560 http://www.niddk.nih.gov Tel: 301-496-3583 TTY: 866-569-1162

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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 August 10, 2005