Abstract
The epidemics of classical bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) related to BSE-infected food are coming to an end. The decline in concern about these diseases may invite complacency and questions whether surveillance for human prion diseases is still necessary. This article reviews the main points of surveillance and why it is still needed: animal sources for human prion infection other than BSE cannot be excluded; the potentially increasing circulation of prions between humans by blood, blood products and medical procedures; the prevalence of vCJD prion carriers in the UK; and the scientific study of prion diseases as paradigm for other neurodegenerative diseases with "prion-like" spread of pathological proteins. We conclude that continuation of detailed surveillance of human prion disorders would be prudent in view of all these points that deserve clarification.
Highlights
It was a veritable news bombshell when, in March 1996, the UK Minister of Health announced the likely transmission of the epidemic of bovine spongiform encephalopathy (BSE) in cattle to humans, causing a new variant of Creutzfeldt-Jakob disease
National surveillance for human prion diseases was started in several countries prior to 1996, but after the advent of variant CreutzfeldtJakob disease (vCJD), surveillance systems were established in many EU and some other countries to determine the extent to which human exposure to BSE prions might affect national populations
The threat of circulation of prions between humans, from external sources or by iatrogenic procedures including blood/ blood product therapies, must be considered, an issue highlighted by the prevalence data on vCJD infection in the UK that suggest a significant fraction with a carrier state of the population
Summary
The epidemics of classical bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) related to BSE-infected food are coming to an end. The decline in concern about these diseases may invite complacency and questions whether surveillance for human prion diseases is still necessary. This article reviews the main points of surveillance and why it is still needed: animal sources for human prion infection other than BSE cannot be excluded; the potentially increasing circulation of prions between humans by blood, blood products and medical procedures; the prevalence of vCJD prion carriers in the UK; and the scientific study of prion diseases as paradigm for other neurodegenerative diseases with “prionlike” spread of pathological proteins. We conclude that continuation of detailed surveillance of human prion disorders would be prudent in view of all these points that deserve clarification
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