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 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.

Human Prion Diseases

Animal Prion Diseases

BSE/TSE Action Plan

BSE/TSE Action Plan of the Department of Health and Human Services (DHHS) has four major components: