About Prion Diseases

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 always fatal.

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

• Creutzfeldt-Jakob Disease (CJD)
• Variant Creutzfeldt-Jakob Disease (vCJD)
• Gerstmann-Straussler-Scheinker Syndrome
• Fatal Familial Insomnia
• Kuru

Animal Prion Diseases

• Bovine Spongiform Encephalopathy (BSE)
• Chronic Wasting Disease (CWD)
• Scrapie
• Transmissible mink encephalopathy
• Feline spongiform encephalopathy
• Ungulate spongiform encephalopathy

Links to Organizations Outside CDC

National Prion Disease Pathology Surveillance Center
(From the Division of Neuropathology, Case Western Reserve University. National CJD surveillance system established in collaboration with CDC.)

Resources

BSE/TSE Action Plan of the Department of Health and Human Services (DHHS)
The BSE/TSE Action Plan of 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).
• 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.