About Prion Diseases
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a
family of rare progressive neurodegenerative disorders that affect both humans
and animals. They are distinguished by long incubation periods, characteristic
spongiform changes associated with neuronal loss, and a failure to induce
||Cattle at a trough, and a buck deer in the
wild. (Courtesy Ermias Belay)
The causative agent of TSEs is believed to be a prion. A prion is an abnormal,
transmissible agent that is able to induce abnormal folding of normal cellular
prion proteins in the brain, leading to brain damage and the characteristics signs
and symptoms of the disease. Prion diseases are usually rapidly progressive and
A List of Prion Diseases
Listed below are the prion diseases identified to date. Click the linked diseases
to go to their respective topic sites. CDC does not currently offer information
here on every prion disease listed.
Human Prion Diseases
Animal Prion Diseases
Links to Organizations Outside CDC
National Prion Disease
Pathology Surveillance Center
Division of Neuropathology, Case Western Reserve University. National CJD
surveillance system established in collaboration with CDC.)
BSE/TSE Action Plan of the Department of Health and Human Services (DHHS)
The BSE/TSE Action Plan of DHHS has four major
Surveillance for human disease is primarily the
responsibility of CDC.
Protection is primarily the responsibility of the Food
and Drug Administration (FDA).
Research is primarily the responsibility of the National
Institutes of Health (NIH).
Oversight is primarily the responsibility of the Office
of the Secretary of DHHS.
Press Release: HHS Launches Expanded Plan to Combat "Mad Cow Disease"
On DHHS site
The Public Health Impact of Prion Diseases
Belay E., Schonberger L. Annu. Rev. Public
PDF format (198 KB/25 pages)
Transmissible Spongiform Encephalopathies in Humans
Belay E. Annu. Rev. Microbiol.
format (208 KB/32 pages)
WHO Infection Control Guidelines for Transmissible Spongiform
Encephalopathies: Report of a WHO Consultation, Geneva, Switzerland,
23-26 March 1999
(From the World Health
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