Consultation on Public Health and Animal Transmissible Spongiform
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WHO/CDS/CSR/APH/2000.2 WHO Consultation on Public Health and Animal Transmissible Spongiform Encephalopathies: Epidemiology, Risk and Research Requirements with the participation of Office International des Epizooties Geneva, Swizterland 1-3 December 1999 World Health Organization Communicable Disease Surveillance and Control This document has been downloaded from the WHO/CSR Web site. The original cover pages and lists of participants are not included. See http://www.who.int/emc for more information. © World Health Organization This document is not a formal publication of the World Health Organization (WHO), and all rights are reserved by the Organization. The document may, however, be freely reviewed, abstracted, reproduced and translated, in part or in whole, but not for sale nor for use in conjunction with commercial purposes. The views expressed in documents by named authors are solely the responsibility of those authors. The mention of specific companies or specific manufacturers' products does no imply that they are endorsed or recommended by the World Health Organization in preference to others of a similar nature that are not mentioned. Contents Opening ..............................................................................................................................1 Section 1. Background ..........................................................................................................4 1.1 Trends in Bovine Spongiform Encephalopathy..........................................................4 1.2 Trends in variant Creutzfeldt Jakob Disease ..............................................................5 Section 2. Bovine Spongiform Encephalopathy in Sheep.....................................................6 2.1 Transmission of Bovine Spongiform Encephalopathy into sheep: Experimental evidence and ongoing work in the United Kingdom ...............................................6 2.2 Estimating the epidemiology of Bovine Spongiform Encephalopathy in sheep ........7 2.3 France: Status report on investigations of Bovine Spongiform Encephalopathy in sheep ........................................................................................................................8 2.4 Netherlands: Status report on investigations of Bovine Spongiform Encephalopathy in sheep ....................................................................................................................8 2.5 Spongiform Encephalopathy Advisory Committee - Risk assessment of Bovine Spongiform Encephalopathy in sheep......................................................................9 2.6 European Union - Risk assessment of Bovine Spongiform Encephalopathy in sheep ......................................................................................................................10 2.7 Office International des Epizooties - Measures relating to BSE ............................14 Section 3. Scrapie Elimination............................................................................................15 3.1 Principles, practice and experience of scrapie elimination.......................................15 3.2 Transmissible Spongiform Encephalopathies in sheep – Surveillance programmes ............................................................................................................16 3.3 Breeding scrapie resistant sheep: The genetics of Transmissible Spongiform Encephalopathies in sheep .....................................................................................16 3.4 Industry and scrapie elimination: Perspective of the Scrapie Information Group....17 3.5 Animal vaccine-related Transmissible Spongiform Encephalopathy risks: Scrapie outbreak in Italy .....................................................................................................18 Section 4. Chronic Wasting Disease ...................................................................................18 4.1 Epidemiology of Chronic Wasting Disease..............................................................18 4.2 Diagnosis and pathogenesis of Chronic Wasting Disease........................................20 4.3 Assessing interspecies Transmissible Spongiform Encephalopathy transmission using the cell-free conversion assay.......................................................................21 4.4 Centers for Disease and Control and Prevention investigations relating to Chronic Wasting Disease exposures....................................................................................22 Section 5. Feline Spongiform Encephalopathy ...................................................................22 5.1 Pathology and epidemiology of Feline Spongiform Encephalopathy ......................22 Section 6. Transmissible Spongiform Encephalopathies in Other Species.........................24 6.1 Transmissible Spongiform Encephalopathies in captive wild bovids and felids......24 6.2 The implications to humans of zoo primates infected with Bovine Spongiform Encephalopathy......................................................................................................25 6.3 Are there prion diseases of fish?...............................................................................25 6.4 The case for the zoonotic origins of Creutzfeldt-Jakob Disease ..............................26 Section 7. Evaluation of Animal Transmissible Spongiform Encephalopathy Zoonotic Potential..............................................................................................................27 7.1 Diagnostic tests for Transmissible Spongiform Encephalopathies: Status and update of the European Union initiative .............................................................................27 7.2 Potential of diagnostic tests to distinguish between Bovine Spongiform Encepathology and scrapie ......................................................................................27 7.3 Diagnostic protocols for Transmissible Spongiform Encephalopathies: Rationales and their applications...............................................................................................28 7.4 Diagnostic tests and standardized reagents: WHO initiative....................................29 7.5 The Rendering Industry – Current sourcing and current product uses in animals....29 7.6 Could vaccines prepared from animal brain tissue pose a risk of transmission of Transmissible Spongiform Encephalopathies to humans?..................................33 7.7 Surveillance and eradication of animal diseases: Principles from human diseases....................................................................................................................34 Section 8. Recommendations.............................................................................................36 Section 1: Background on new variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy .................................................................................36 Section 2: Bovine Spongiform Encephalopathy in small ruminants such as sheep and goats .......................................................................................................................36 Section 3: Scrapie control programmes ...................................................................................38 Section 4: Chronic Wasting Disease........................................................................................39 Sections 5 and 6:Feline Spongiform Encephalopathies and Transmissible Spongiform Encephalopathies in other species.............................................40 Section 7: Evaluation of animal Transmissible Spongiform Encephalopathy zoonotic potential .................................................................................................................41 Annex 1 Agenda ..............................................................................................43 Annex 2 List of Participants ............................................................................47 WHO/CDS/CSR/APH/2000.2 This report summarizes the presentations made to the World Health Organization on the occasion of a meeting held at its headquarters in Geneva from 1 to 3 December 1999, and provides the assessments and recommendations made by the consultation. Opening In 1986 the first case of a previously unrecognized neurologic disease of cattle was diagnosed in the United Kingdom (UK). Within a few years it was clear that there was an evolving epidemic outbreak of a new transmissible spongiform encephalopathy (TSE). To date over one hundred and seventy-five thousand cases of bovine spongiform encephalopathy in cattle (BSE) have been reported from the UK, and over eleven hundred in eight countries of continental Europe (ranging from one to over four hundred cases per country). The TSEs are degenerative brain diseases seen in humans and some domestic and wild (captive and free-ranging) animals. The family of diseases is characterized by an incubation period usually measured in months to years. There is no treatment and no cure. The nature of the agent has not been fully determined, although the predominant theory is that a previously unrecognized pathogenic agent called a prion, an abnormally folded protein, is responsible. There is no early diagnostic test or screening test available for use on an easily accessed biological tissue in live animals, and to date, the gold standard tests to differentiate the strains of TSEs within an animal species require inoculation of mice and the observation