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Permanent Monitoring Panel -
Transmissible Spongiform Encephalopathy

Members of the Panel:

Robert Will (UK)

Herbert Budka (Austria); Carleton Gajdusek (France); Bernardino Ghetti (USA); Maura Ricketts (Canada/Switzerland)

Associate Members:
Paul Brown (USA); Colin Masters (Australia)

Summary of the Emergency

An outbreak of a 'new variant' of Creutzfeldt-Jakob disease (nvCJD) has occurred within the past few years in Great Britain. It has been traced to a similar disease in cattle called bovine spongiform encephalopathy (BSE, or 'mad cow disease'), and has probably resulted from the consumption of beef containing cattle brain tissue.

BSE has also occurred in other European countries as a result of the importation of live cattle or cattle feed, and it is feared that nvCJD may be incubating in people living outside as well as inside Great Britain. Precautionary measures to prevent further spread of the disease from cattle to humans or between humans are under consideration in countries that have imported products derived from cattle (e.g., gelatin) or humans (e.g.,gamma globulin) from countries in which BSE or nvCJD have occurred.


Priorities in dealing with the Emergency

The primary goal of this Panel for the coming year will be to create an up-to-date body of material on various aspects of TSE, including the new variant of Creutzfeldt-Jakob disease resulting from exposure to bovine spongiform encephalopathy, for inclusion in the WFS home page on the world wide web.

A second important goal will be an exploration of ways by which urgent issues can be brought to the attention of individuals who are in a position to do something about their prevention or amelioration. One approach would be to convene a session at a future Annual Planetary Emergencies meeting in Erice with the specific topic of translating ideas into action; speakers would include prominent individuals who have devoted their careers in the world arenas of politics, communications, and finance.

Papers giving further details on TSE


Background and
present status of BSE
and nvCJD


An overview of 
Transmissible Spongiform Encephalopathy


Mutations and normal polymorphisms in the prion protein gene (PRNP) open reading frame


Infection control Guidelines for TSEs in hospitals and home-care settings


Visualization of a battlefield: the pathology of human transmissible spongiform encephalopathies