Transmissible Spongiform Encephalopathies:: Scrapie, Bse and Related Human Disorders

This book provides a comprehensive summary of the current status of information on the agents of scrapie, Creutzfeldt-Jakob disease, bovine spongiform encephalopathy and related diseases. Topics covered include current concepts of the nature of the agent, possible genetic variability of the agent, the role of the prion protein gene and linked genes in influencing disease susceptibility and species tropism, biochemical, electron microscopic and molecular biological analyses of proteins and nucleic acids in infected tissues, in vitro approaches to study of the transmissible agents, epidemiological studies of bovine and human spongiform encephalopathies. Topics are presented as a series of review papers prepared by experts in the field. Readers will find here, in a single volume, the most current series of chapters covering many aspects of the field.

In spite of a long history of intense investigation the transmissible spongiform encephalopathies remain a poorly understood family of neurodegenerative diseases. This group of diseases has been described in a wide variety of animal species and includes kuru, Creutzfeldt-Jakob disease, and Gerstmann-Straussler syndrome in humans, and scrapie, bovine spongiform encephalopathy, and related syndromes in ruminants and rodents. In all cases spongiform degeneration and astrocytosis are seen in specimens of brain and a filterable transmissible agent is present in the brain and some other tissues of affected individuals. However, the precise nature of this agent remains unknown. Agent infectivity, which can so far only be assayed by serial transmission to new individuals, be remarkably resistant to inactivation has been shown to by heat, chemicals, and irradiation. These properties create significant biohazard possibilities during exposure to infected tissues. Transmission between humans was originally reco- gnized in the unique epidemiology of kuru in New Guinea tribesmen, and concern about transmission from animals to humans has re-emerged as a result of the current epidemic of bovine spongiform encephalopathy in dairy cattle in Great Britain. Although interspecies transmission has often been achieved experimentally, its efficiency is highly variable. There- fore, the possibility of spread of bovine spongiform encephalopathy from cattle to humans or various animal populations cannot be accurately predicted at this time. This volume presents a comprehensive update of know- ledge concerning the transmissible spongiform encephalo- pathies.

In spite of a long history of intense investigation, the transmissible spongiform encephalopathies are a poorly understood grouping of neurodegenerative diseases. This family of diseases includes kuru, Creutzfeldt-Jakob disease, and Gerstmann-Str ussler syndrome in humans and scrapie, bovine spongiform encephalopathy (mad-cow disease) and related syndromes in ruminants and rodents.