Mad Cow Disease And Related Spongiform Encephalopathies

Bovine spongiform encephalopathy (BSE) has become the most publicly recognizable example of a group of fatal neurodegenerative diseases caused by proteinaceous infectious particles called prions. The contributors to this volume, all internationally recognized experts in their fields, provide an introduction to prion biology, followed by reviews of the latest information on BSE, vCJD, and chronic wasting disease, an animal prion disease that has recently emerged in North America.

The alarm sounded by Canada's recently confirmed case of bovine spongiform encephalopathy (BSE) has reaffirmed the exigency of establishing improved safeguards and more aggressive surveillance protocols in North America and around the world. Research converging on the probable causative agent-prion proteins-calls for intensive assessment of the headway gained in tracing prions, testing for transmissible neurodegenerative diseases, and developing methods for cornering the epidemic. Administered by an illustrious panel of 36 international contributors, this timely book marshals techniques for prion protein assay and diagnosis of transmissible spongiform encephalopathies (TSEs).

The alarm sounded by Canada's recently confirmed case of bovine spongiform encephalopathy (BSE) has reaffirmed the exigency of establishing improved safeguards and more aggressive surveillance protocols in North America and around the world. Research converging on the probable causative agent-prion proteins-calls for intensive assessment of the headway gained in tracing prions, testing for transmissible neurodegenerative diseases, and developing methods for cornering the epidemic. Administered by an illustrious panel of 36 international contributors, this timely book marshals techniques for prion protein assay and diagnosis of transmissible spongiform encephalopathies (TSEs).

This book presents important analyses of current issues in BSE (bovine spongiform encephalopathy or "mad cow disease") as a fatal neurological disease of cattle, believed to be transmitted mainly by feeding infected cattle parts back to cattle. More than 187,000 cases have been reported world-wide, 183,000 of them in the United Kingdom (UK) where BSE was first identified in 1986. The annual number of new cases has declined steeply since 1992. Humans who eat contaminated beef are believed susceptible to a rare but fatal brain wasting disease, variant Creutzfeldt-Jakob Disease (vCJD). About 160 people have been diagnosed with vCJD since 1986, most in the UK and none linked to any Canadian or U.S. meat consumption.

Prions are an entirely new class of pathogens, and scientists are just beginning to understand them. Although they have plagued humans and animals for 3 centuries, only in the last 2 decades have researchers linked them to diseases like Mad Cow. This book tells the strange story of their discovery, and the medical controversies that swirl around them. The author, Philip Yam, is a well-respected and connected journalist who is now an editor at Scientific American.

"How the Cows Turned Mad tells the story of a disease that continues to elude on many levels. Yet science has come far in understanding its origins, incubation, and transmission. This book is a case history that illuminates the remarkable progression of science."--BOOK JACKET.

In Advancing Prion Science, the Institute of Medicine's Committee on Transmissible Spongiform Encephalopathies Assessment of Relevant Science recommends priorities for research and investment to the Department of Defense's National Prion Research Program (NPRP). Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are invariably fatal neurodegenerative infectious diseases that include bovine spongiform encephalopathy (commonly called mad cow disease), chronic wasting disease, scrapie, and Creutzfeldt-Jakob disease. To develop antemortem diagnostics or therapies for TSEs, the committee concludes that NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEsâ€"from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment.