Note: Intro -- Contents -- Dedication in Remembrance of Andreas Scheid (1941-2001) -- Note of the Series Editor -- Preface to the Second Edition -- Preface to the First Edition -- Transmissible Spongiform Encephalopathies: The Prion Theory - Background and Basic Information -- Non-Virus Properties of the Scrapie Agent -- The Concept of Two Isoforms of the Prion Protein -- Basic Experiments Pro and Contra Prions -- The Search for a Nucleic Acid -- Structural Transitions of the Prion Protein as Basis for the Replication Mechanism -- Infectious, Sporadic and Familial Etiology of Prion Diseases Based on the Prion Model -- Outlook -- Acknowledgment -- References -- Structural Biology of Prions -- The Primary Structure of the Prion Protein -- The Neurotoxic Model Peptide, PrP106-126 -- Metal-Binding Domains of the Prion Protein -- Secondary Structure Analysis of the Prion Protein -- Tertiary Structure of the Normal Prion Protein (PrPC) -- Influence of Familial Mutations on the Structure of the Prion Protein -- Tertiary Structure of the Infectious Prion Protein (PrP TSE ) -- Deciphering the Structure of Infectious Prion Protein with Antibodies -- Prion Protein Folding Pathways and Their Conformational Properties -- Conclusion -- References -- Prion Strains and Species Barriers -- Prion Strains -- Molecular Basis of Prion Strains -- Species Barriers -- Subclinical Prion Infection -- Implications of Prion Disease vs. Prion Infection -- Re-Evaluating Prion Strains and Species Barriers -- Acknowledgement -- References -- Prions of Saccharomyces and Podospora -- Three Genetic Criteria for Prions -- [URE3] as a Prion of the Ure2 Protein -- Structure of Ure2p -- Aggregation of Ure2p in vivo and in vitro -- [PSI] as a Prion of the Sup35 Protein -- Interaction with Other Proteins in the Cell and Influence of Chaperones -- Prion Variants. , Other Prions in Saccharomyces cerevisiae -- [Het-s], a Prion with a Normal Cellular Function -- Are in vitro Produced Filaments Infectious? -- Acknowledgement -- References -- Human Prion Diseases: Cause, Clinical and Diagnostic Aspects -- Creutzfeldt-Jakob Disease -- Sporadic CJD (spCJD) -- Epidemiology and Cause -- Clinical Features -- Diagnosis -- Iatrogenic (iCJD) -- Epidemiology and Cause -- Clinical Features -- Diagnosis -- Variant (vCJD) -- Epidemiology and Cause -- Clinical Features -- Diagnosis -- Genetic (gCJD) -- Epidemiology and Cause -- Clinical Features -- Diagnosis -- Gerstmann-Sträussler-Scheinker Syndrome (GSS) -- Epidemiology and Cause -- Clinical Features -- Diagnosis -- Fatal Familial Insomnia -- Epidemiology and Cause -- Clinical -- Diagnosis -- Kuru -- Epidemiology and Cause -- Clinical Features -- Diagnosis -- References -- Epidemiology and Risk Factors of Transmissible Spongiform Encephalopathies in Man -- Sporadic CJD (spCJD) -- Descriptive Epidemiology -- Incidence -- Cluster -- CJD in Europe -- Role of the Codon 129 Genotype -- Risk Factors -- Iatrogenic Cases -- Variant CJD (vCJD) -- References -- Aspects of Risk Assessment and Risk Management of Nosocomial Transmission of Classical and Variant Creutzfeldt-Jakob Disease with Special Attention to German Regulations* -- Transmissible Spongiform Encephalopathies -- Characteristics of the Etiological Agent -- Risk Assessment -- Reported Transmissions of Human TSEs -- Iatrogenic and Accidental Transmission in Nosocomial Settings -- Stratification of Patients -- Stratification of Tissues -- Efficiency of Transmission via Various Routes of Exposure -- Retrospective Analysis of Iatrogenic CJD Cases -- Risk Management - Current Strategies to Minimize the Risk of Transmission -- Principles for the Prevention of Transmission -- Hospital Care. , Transmission via Surgical Instruments or Medical Products -- Patients with a Recognizable Risk of Classical CJD (Including GSS and FFI) -- Patients with a Recognizable Risk of vCJD -- Patients without a Recognizable Risk of CJD or vCJD -- Transmission through Blood and Blood Products -- Summary and Outlook -- Appendix 1 -- Destabilization and Inactivation of TSE Agents:Theoretical Approaches and Assay Systems (also see chapter by Taylor p 136 -145 in this volume.) -- Appendix 2 -- General Principles Underlying Current Guidelines for the Reprocessing of Surgical Instruments and Medical Devices in Various European Countries -- Acknowledgements -- References -- Resistance of Transmissible Spongiform Encephalopathy Agents to Decontamination -- Chemical Methods of Inactivation -- Heat Treatment -- Observations on the Thermostability of TSE Agents -- Combining Heat with Exposure to Sodium Hydroxide -- Current Concerns -- References -- Bovine Spongiform Encephalopathy and Its Relationship to the Variant Form of Creutzfeldt-Jakob Disease -- Cause of BSE -- Properties of Agents That Cause TSE -- The BSE Agent -- Clinical Signs of BSE -- Sensation -- Mental Status -- Posture and Locomotion -- Epidemiology of BSE -- Rendering of Animal Waste -- Origin of the Epidemic -- PrP Genetics and Influence of Breed on the Occurrence of BSE -- Descriptive Epidemiology -- Hypotheses for the Origin of BSE -- Factors Influencing the Occurrence of BSE in the UK -- Sheep Source Hypothesis -- Cattle Source Hypothesis -- The Case for a 'Sporadic 'BSE Origin -- The Case for a Subclinical Carrier Origin -- Conclusion -- Horizontal Maternal and Paternal Transmission -- Horizontal Transmission -- Maternal Transmission -- Paternal Transmission -- Cattle with BSE Born after the 1988 Feed Ban -- Variant Form of CJD and Its Consequences -- Geographical Distribution of BSE. , Transmissibility of Prion Diseases -- Natural Host Range for BSE -- TSE in Primates Resulting from Exposure to the BSE Agent -- Experimental Host Range of the BSE Agent -- Tissue Distribution of BSE Infectivity in Cattle with Natural and Experimental BSE -- Pathogenesis of TSE -- Pathogenesis of Experimental Scrapie in Rodents -- Pathogenesis of Natural Scrapie in Sheep -- Pathogenesis of Experimental BSE in Cattle following Oral Challenge -- Relationship of Variant CJD to BSE -- Origin of Infection in Humans -- Experimental Transmission -- Transmission to Conventional Mice and Biological Strain Typing -- Transmission to Transgenic Mice -- Molecular Strain Typing -- Comparative Neuropathology -- Tissue Distribution of Prion Protein-Scrapie Isoform -- Effect of the Occurrence of Variant Form of CJD -- Concluding Remarks -- Acknowledgments -- References -- Possibilities to Manage the BSE Epidemic: Cohort Culling versus Herd Culling - Experiences in Switzerland -- Objective of a Culling Strategy -- The Likelihood of Additional Cases of BSE in an Infected Herd -- Alternative Culling Programmes -- Experiences Gained in Switzerland -- Choosing a Culling Strategy -- Conclusion -- References -- Regulatory Aspects of BSE and CJD with Special Emphasis on Germany -- Special Regulations for the Prevention and Management of BSE -- Epidemiological Supervision -- Trading Limitations for Living Cattle -- Restrictions for the Trade with Food and Other Materials of Bovine Origin Including Animal Feed -- Requirements for the Keeping of Cattle Including the Ban of Feed Containing Material Derived from Ruminants -- Appropriate Eradication and Elimination of BSE-Infected and/or Endangered Animal Collectives Including the Decontamination of Agricultural Facilities. , Requirements Concerning the Production of Materials of Bovine Origin Including Slaughtering and Human Food Production -- Requirements for Processing of Rough Materials of Bovine Origin in Pharmacology, and Cosmetics and Toiletries -- Requirements for the Production of Animal Feed Containing Material of Animal Origin -- Safety Regulations in Agriculture and Meat Procession -- Special Regulations for the Prevention and Management of CJD -- Law-Enforced Obligations to Report Human CJD Cases to the Official Authorities -- Exclusion of a Defined Group of Individuals as Blood Donors -- Introduction of Higher Valanced Procedures for Processing Medical Equipment and/or Laundry in the Hospital Setting -- Preference of Disposable Instruments -- Safety Regulations for Medical Staff -- Appendix 1 -- The Challenge for the Public Health System -- Properties of Transmissible Spongiform Encephalopathies -- Types of TSEs of Concern -- Minimizing the Risks of Transmission of Spongiform Encephalopathies by Medicinal Products -- Implications of the Variant Form of Creutzfeldt-Jakob Disease: Blood and Blood Products -- Implications of the Variant Form of Creutzfeldt-Jakob Disease: Other Medical Interventions -- The Future -- References -- Author Index -- Subject Index -- A -- B -- C -- E -- F -- G -- H -- I -- K -- L -- M -- N -- P -- R -- S -- T -- U -- V -- Y.