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University of California Press

About the Book

Fear of mad cow disease, a lethal illness transmitted from infected beef to humans, has spread from Europe to the United States and around the world. Originally published to much acclaim in France, this scientific thriller, available in English for the first time and updated with a new chapter on developments in 2001, tells of the hunt for the cause of an enigmatic class of fatal brain infections, of which mad cow disease is the latest incarnation. In gripping, nontechnical prose, Maxime Schwartz details the deadly manifestations of these diseases throughout history, describes the major players and events that led to discoveries about their true nature, and outlines our current state of knowledge. The book concludes by addressing the question we all want answered: should we be afraid?

The story begins in the eighteenth century with the identification of a mysterious illness called scrapie that was killing British sheep. It was not until the 1960s that scientists understood that several animal and human diseases, including scrapie, were identical, and together identified them as transmissible spongiform encephalopathy (TSE). The various guises assumed throughout history by TSE include an illness called kuru in a cannibalistic tribe in Papua New Guinea, an infectious disease that killed a group of children who had been treated for growth hormone deficiencies, and mad cow disease. Revealing the fascinating process of scientific discovery that led to our knowledge of TSE, Schwartz relates pivotal events in the history of biology, including the Pasteurian revolution, the birth of genetics, the emergence of molecular biology, and the latest developments in biotechnology. He also explains the Nobel Prize–winning prion hypothesis, which has rewritten the rules of biological heredity and is a key link between the distinctive diseases of TSE.

Up-to-date, informative, and thoroughly captivating, 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 authoritative book is a stunning case history that illuminates the remarkable progression of science.

About the Author

Maxime Schwartz, a molecular biologist, is Professor at the Institut Pasteur in Paris, which he headed from 1988 to 1999. He is also Director of Research at the Centre national de la recherche scientifique (CNRS) and is currently serving as Director of Laboratories of the French agency for food safety (AFSSA). He is the author of many scientific papers and Pasteur, des microbes au vaccin (1999, with Annick Perrot).

Table of Contents

Prologue

1: The Sheep Are Strangely Dizzy
2: Molecules and Microbes
3: Mad Dogs and Earthworms
4: Scrapie under the Microscope
5: Creutzfeldt, Jakob, and Others
6: Scrapie Is Inoculable
7: And Goats, and Mice
8: Scrapie Is Contagious
9: Kuru and the Fore People of Papua New Guinea
10: The Wall Comes Down
11: From Pearl Necklace to Double Helix
12: The Phantom Virus
13: A Tragedy in the Making
14: One Case per Million
15: Prions
16: April 1985
17: The "Kiss of Death"
18: The Return of the Spontaneists
19: To Grow—and to Die
20: Lessons Learned
21: Have the Cows Gone Mad?
22: From Cows to Humans
23: From Cows to Sheep? From Humans to Humans?
24: The Secret in the Closet
25: Unmasking "The Disease"
26: Have We Conquered "The Disease"?
27: 2001

Epilogue
Notes
Bibliography
Chronology
Acknowledgments
Index

Reviews

"If you would like an opportunity to learn some fascinating history and at the same time have a relaxing and interesting read of a scientific subject, this is the book for you.”
The Lancet
"An excellent overview. . . . In an easily understandable way [Schwartz] explains scientific findings.”
British Medical Journal (Bmj)
“[Schwartz] succeeds in formulating a history of research on TSEs that captivates the reader -- science and storytelling at their best.”
British Medical Journal (Bmj)
“Maxime Schwartz develops the history of TSE as a mystery novel. . . Schwartz poses questions, suggests possible answers, and then describes the scientific advances that produced the answers. The book . . . should appeal to a wide range of readers.”
Nature
“Schwartz recounts the history of the prion diseases . . . as if writing a mystery. One of the attractive features of this book is its historical perspective, beginning with . . . scrapie in 17th-century England.”
New England Journal Of Medicine
“Schwartz. . . has written a lucid and gripping account of these events in the context of the latest scientific research.”
Natural History
“The drama of Schwartz’s book... is in the slow coming together of several historical strands of scientific research, given the momentum of a detective story by his personification of the book’s malign protagonist: a rogue protein given to feints and counterattacks.”
Irish Times
“This is a concise and extremely readable account, which provides a good overview of the growth of knowledge about TSEs and renders accessible some extremely complex scientific information. “
Medical History
"Schwartz's fully engrossing, two-century-plus detective story provides a thoroughgoing history of the discovery of 'mad cow' and related diseases that also illuminates the ways in which science works. I could not put this book down."—Jon Beckwith, author of Making Genes, Making Waves: A Social Activist in Science

"Rarely have I read a book as scary, interesting, informative and enjoyable."—John E. Talbott, University of California, Santa Barbara

Praise for the French edition:

"Maxime Schwartz's book . . . constitutes an ode to science, to its rigor, to its perseverance, but also, as we shall see, to its modesty. How the Cows Turned Mad is a gothic historical novel: its author, molecular biologist and former director of the Pasteur Institute, leads us along a thread that unravels over almost three centuries, from Louis XV to Tony Blair."—Le Figaro

"But above all, and this is indeed remarkable in a work which treats such a scientific subject, How the Cows Turned Mad is not a scientific treatise for scientists, but rather a book. And as such, it reads easily and pleasurably."—Le Généraliste

"How the Cows Turned Mad: that's the title of this book, almost a detective novel, just published by the molecular biologist Maxime Schwartz. An indispensable tool that allows us to sort through the truths and untruths and finally assess the situation."—Panorama du médecin