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Prion diseases are among the most devastating, costly, and controversial emerging infections. The best known is bovine spongiform encephalopathy (BSE), or “mad cow disease,” which devastated the British meat industry when about 160 human fatalities occurred as a result of ingestion of prion-infected beef. BSE has now been reported in a few U.S.  animals. Other prion diseases are widespread in U.S.  wildlife. This updated summary describes the biology of the infectious protein, or prion, which causes these horrible diseases. It discusses how the prospects of safeguarding our food and blood supply are hampered by insensitive laboratory tests, critically reviews the claims made by government agencies about the safety of beef and other foods, and describes some promising new approaches to testing.

By George Smith, MPH, M(ASCP): George Smith, earned his BS and MPH at the School of Public Health , University of California at Berkeley . He took a traineeship in clinical microbiology at University of California Medical Center/San Francisco General Hospital . Semi-retired, Mr. Smith’s career encompassed ten years of research in infectious disease, including plague immunology, and about twenty years as a practicing clinical microbiologist/public health microbiologist with Children’s Hospital of Central California , and Fresno County Department of Community Health, Public Health Laboratory.