Periodic activity report 2005 - Year 2

NEUROPRION A EUROPEAN APPROACH WITH WORLDWIDE VISION

Mad Cow disease (BSE), Creutzfeldt-Jakob disease (CJD) and scrapie belong to an unique class of infectious diseases, which affect both human and animals. This group of Transmissible Spongiform Encephalopathies (TSEs), better known as prion diseases, is caused by a new type of infectious agents highly resistant to conventional methods of decontamination. Known to exist for more than two centuries, prion diseases have caused several crises throughout the years. With the appearance of the first Bovine Spongiform Encephalopathy (BSE) cases in the 1980s, these diseases have become a major problem for society with important health and economical consequences. The coordination of the research on prion diseases at the European level has played a significant role in the crisis management of BSE. Indeed scientists have identified risk factors, advised authorities and provided efficient diagnostic tests to ensure food safety and procedures to decontaminate prions. However, as the biology of prion diseases is not yet fully understood, they could still potentially pose a major threat to society.

Presently there are no reliable diagnostic tests available for living beings. The compelling evidence suggesting that variant CJD is transmissible through blood transfusion reinforces the need for preclinical, ante-mortem diagnostic tests for humans as well as for animals. New forms of prion diseases in sheep, denominated as atypical scrapie, have been described and raise further questions to the possible risk for humans and the presence of BSE in small ruminants. Although strong progress was achieved in understanding the replication and pathogenesis of the infectious agent, the development of a possible treatment is just in the early stages. Finally, the Canadian experiences show clearly the dramatic economic consequences of prion crises, which support the need for excellent and well coordinated research on prion diseases.

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