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 inflammatory response.
The causative agents of TSEs are believed to be prions. The term “prions” refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain. The functions of these normal prion proteins are still not completely understood. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.
Identified Prion Diseases
Listed below are the prion diseases identified to date. CDC does not currently offer information on every prion disease listed below.
Classic CJD is a human prion disease. It is a neurodegenerative disorder with characteristic clinical and diagnostic features.
vCJD has a different clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile of the prion protein gene.
BSE also known as Mad Cow Disease is a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion.
- The Public Health Impact of Prion Diseases Cdc-pdf[PDF – 191KB]
Belay E., Schonberger L. Annu. Rev. Public Health2005;26:191-212
- Transmissible Spongiform Encephalopathies in Humans Cdc-pdf[PDF – 183KB]
Belay E. Annu. Rev. Microbiol. 1999;53:283-314
- WHO infection control guidelines for transmissible spongiform encephalopathies. Report of a WHO consultation, Geneva, Switzerland, 23-26 March 1999External
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BSE/TSE Action Plan of the Department of Health and Human Services (DHHS) has four major components:
- Surveillance for human disease is primarily the responsibility of CDC.
- Protection is primarily the responsibility of the Food and Drug Administration (FDA)External.
- Research is primarily the responsibility of the National Institutes of Health (NIH)External.
- Oversight is primarily the responsibility of the Office of the Secretary of DHHSExternal.