The very first international working discussion on slow infections of the nervous system was entitled "Slow, Latent, and Temperate Virus Infec­ tions" and was held at the National Institutes of Health (NIH) in December 1964. The primary impetus was the discovery and investigation of kuru in New Guinea by D. Carleton Gajdusek, M. D. This working discussion brought together investigators in human and veterinary medicine, virolo­ gists, microbiologists, and neuropathologists actively engaged in laboratory work with viruses that illustrated properties of latency, masking, slowness, or temperateness, with emphasis on subacute and chronic neurologic dis­ eases of unknown etiology. In the Preface to the monograph of published papers presented at the working discussion, Gajdusek and Gibbs wrote the following: After microbiology had given solution to the etiology of most acute infections of the central nervous system and after fungi and bacteria had been incriminated in impor­ tant chronic disorders of the nervous system such as torula and tuberculosis men­ ingitis, we have been left, in neurology, with a wide range of subacute and chronic affections of the central nervous systems of unknown etiology. Some of these diseases, still listed as idiopathic, are among the most prevalent afflictions of the central nervous system. Many others with familial patterns of occurrence do not yet have their basic pathogenesis or underlying metabolic defect elucidated, although we tend to think of them as genetically mediated.

Inhalt

1. The Potential Risk to Humans of Amyloids in Animals.- I. Host Range and Pathogenesis.- 2. Bovine Spongiform Encephalopathy Distribution and Update on Some Transmission and Decontamination Studies.- 3. Preliminary Observations on the Pathogenesis of Experimental Bovine Spongiform Encephalopathy.- 4. Recent Observations on the Epidemiology of Bovine Spongiform Encephalopathy.- II. Transmission and Pathogenesis of Spongiform Encephalopathies.- 5. Scrapie: Studies on Vertical and Horizontal Transmission.- 6. Transmission of Sheep and Goat Strains of Scrapie from Experimentally Infected Cattle to Hamsters and Mice.- 7. Experimental Transmission of Scrapie to Cattle.- 8. An Assessment of Transmissible Mink Encephalopathy as an Indicator of Bovine Scrapie in U.S. Cattle.- 9. Experimental Infections of Cattle and Mink with the Agents of Transmissible Mink Encephalopathy, Scrapie, and Bovine Spongiform Encephalopathy.- 10. BSE-Free Status: What Does It Mean?.- 11. Differing Neurohistologic Images of Scrapie, Transmissible Mink Encephalopathy, and Chronic Wasting Disease of Mule Deer and Elk.- 12. Analysis of Risk Factors and Active Surveillance for BSE in Argentina.- 13. Speculations on the Origin of BSE and the Epidemiology of CJD.- III. Biochemistry: Protein Chemistry, Molecular Biology, and Molecular Genetics of the Spongiform Encephalopathies.- 14. Structure and Biologic Characteristics of Prion Protein (Scrapie Amyloid): Implications for the Safety of Naturally Derived Biologics.- 15. Prion Strains and Neuromuscular Disease in PrP Transgenic Mice.- 16. Deciphering Prion Diseases with Transgenic Mice.- 17. Posttranslational Modifications and Conformational Changes of PrPSc and Their Relationship to Infectivity.- 18. Amyloidosis: The Key to the Epidemiology and Pathogenesis of Transmissible Spongiform Encephalopathies.- 19. Effect of Amphotericin B on Different Experimental Strains of Spongiform Encephalopathy Agents.- IV. Pathogenesis, Molecular Biology and Genetics, and Immunohistochemistry.- 20. Genetics of Human Spongiform Encephalopathies: Current Status.- 21. PrP Allelic Variants Associated with Natural Scrapie.- 22. The Formation of Scrapie-Associated Prion Protein In Vitro.- 23. Proteinase K-Resistant Prion Protein Detection in Animal Tissues and In Vitro.- 24. Elimination of Scrapie-Agent Infectivity in Naturally Derived Biologics.- 25. Cellular and Scrapie Prion Protein Immunolocalization and In Vitro Amyloid Formation.- V. Public Health Considerations of Human and Animal Spongiform Encephalopathies.- 26. Real and Theoretical Threats to Human Health Posed by the Epidemic of Bovine Spongiform Encephalopathy.- 27. Incidence of Creutzfeldt-Jakob Disease in the European Community.- 28. Problems in the Evaluation of Theoretical Risks for Humans to Become Infected with BSE-Contaminated Bovine-Derived Pharmaceutical Products.- 29. Evaluation of BSE Risk Factors Among European Countries.- Author Index.