Prion diseases as transmissible zoonotic diseases

Area of Science:

• Neuroscience
• Infectious Diseases
• Veterinary Medicine

Background:

• Prion diseases, known as transmissible spongiform encephalopathies (TSEs), cause fatal neurological dysfunction in animals.
• Key examples include scrapie, chronic wasting disease, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD).
• BSE, or mad cow disease, is a zoonotic disease linked to variant CJD (vCJD) in humans, primarily through contaminated food products.

Purpose of the Study:

• To summarize current knowledge on prions, focusing on molecular mechanisms.
• To review bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) epidemiology and diagnostics.
• To analyze European Union policies on prion disease risk and communication, offering recommendations for Korea.

Main Methods:

• Literature review of studies on prion diseases.
• Analysis of epidemiological data for BSE and vCJD.
• Examination of diagnostic procedures and risk management policies.

Main Results:

• BSE epidemic peaked in 1992, with significant decline due to surveillance, but atypical forms and iatrogenic transmission persist.
• vCJD is linked to BSE, with human cases primarily from contaminated cattle products.
• Effective surveillance and screening programs have reduced BSE prevalence in Western countries.

Conclusions:

• Understanding prion molecular mechanisms is crucial for developing effective diagnostics and therapeutics.
• Continued vigilance is necessary to manage ongoing risks of prion diseases, including atypical forms and iatrogenic spread.
• International policy analysis provides a framework for national strategies to prevent and control prion diseases.