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Prion Diseases
 Prion Diseases Toolkit




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. Source: CDC


Regulations & Guidelines

(1) Prion Diseases

(2) Guidelines for Infection Control in Dental Health-Care Settings — 2003

NIH  Prion Diseases 

Prion Diseases 

National Institute of Neurological Disorders and Stroke  NINDS Creutzfeldt-Jakob Disease Information Page 
MedlinePlus  Creutzfeldt-Jakob disease (CJD) 

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Best Practices





Key Learnings as of September 2017

Prions exhibit unusual resistance to conventional chemical and physical decontamination procedures. Considering this resistance and the invariably fatal outcome of CJD, procedures for disinfecting and sterilizing instruments potentially contaminated with the CJD prion have been controversial for years. Scientific data indicate the risk, if any, of sporadic CJD transmission during dental and oral surgical procedures is low to nil. Until additional information exists regarding the transmissibility of CJD or vCJD, special precautions in addition to standard precautions might be indicated when treating known CJD or vCJD patients. Source: CDC

Related Articles MedlinePlus


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Instructional Resources
Image Library

Creutzfeldt-Jakob disease

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Patient Resources


  Risk for Travelers 

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