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How the Cows Turned Mad [Hardcover]

Maxime Schwartz , Edward Schneider
2.0 out of 5 stars  See all reviews (1 customer review)
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Book Description

March 15 2003 0520235312 978-0520235311 First Edition
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.

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*Starred Review* Two and a half centuries ago, sheep in England started trying to scrape their wool off; in France, to shake uncontrollably. The Brits dubbed their phenomenon scrapie; the French called theirs tremblant. Between then and now, similar conditions in cows and humans were discovered and assigned the group name transmissible spongiform encephalopathies (TSEs): diseases that fill the brain with holes as in a sponge and spread from one organism to another. Their cause eluded researchers until quite recently. SOP since Pasteur sought an invasive bacterium or virus with increasingly powerful tools, which TSE agents eluded. Eventually, evidence pointed to a genetic cause involving transformation of a normal into a deviant gene by another deviant gene introduced orally into the affected organism. You had to eat something from a sick organism to become sick, and once that became popular knowledge after the concurrence of human and bovine TSE cases in England in the 1990s, there was a panic. That reaction seems unjustified; according to Schwartz, TSEs will continue to be a very minor cause of human death. Meanwhile, there may be much to learn from TSE research about such symptomatically similar illnesses as Alzheimer's disease. Writing with immense concentration and clarity, French molecular biologist Schwartz makes the long hunt for the unexpected culprit gene utterly engrossing. Ray Olson
Copyright © American Library Association. All rights reserved

Review

"An excellent overview. . . In an easily understandable way [Schwartz] explains scientific findings."--"British Medical Journal"

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2.0 out of 5 stars Boring & Dry May 19 2004
Format:Hardcover
Maxime Schwartz was a molecular biologist and is now a professor at the Pasteur Institute in Paris. Schwartz traces the history of medical research into spongiform encephalopathies, and how the scientific understanding of how they are spread has changed over time. If you know anything about Bovine Spongiform Encephalopathy (BSE) or Mad Cow disease, I don't think you'll learn anything new in this book. How the Cows Turned Mad is not a sensational book, nor even a good book. Quite simply it is too wordy and dull.
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Amazon.com: 4.3 out of 5 stars  7 reviews
3 of 3 people found the following review helpful
5.0 out of 5 stars Well Written, Scary as heck June 24 2006
By P. S. Matz - Published on Amazon.com
Format:Paperback
An amazing tour of the history of prion diseases. From start to finish, it's well written, beuatifully explained and frighrening. If this book hasn't scared you, read it again
2 of 2 people found the following review helpful
5.0 out of 5 stars Page Turner Aug. 9 2012
By Rodger Shepherd - Published on Amazon.com
Format:Paperback
I refer the reader to the other reviews for a description of the content of the book. I simply want to align myself with those reviewers who were enthusiastic about this book. The story of the transmissable subacute spongioform encephalopies is very engagingly and authoritatively told by the author (who is a "he" - not a "she"). If you like the sort of stories that Berton Roueche wrote, then you will enjoy this book. Among other things, this book is a wonderful reminder that medicine is a constant "work in progress" and that seemingly esoteric research can suddenly become very relevant.

I would note that the book takes the story to 2003. Subsequent developments are easily tracked down with Google. I found current introductary articles on protein folding very helpful.
1 of 1 people found the following review helpful
4.0 out of 5 stars Solid, if surface-level, overfiew of mad cow and other TSEs Aug. 9 2009
By S. J. Snyder - Published on Amazon.com
Format:Paperback
Schwartz does a good job with the history of scrapie, kuru, vCJD, etc. However, once we get to Prusiner and prion territory, while she does a good job of explaining his conclusions (along with those who generally agree), she doesn't fully look at the controversy over prions, or the controversy over whether or not Prusiner was making a "Nobel push."

This is a solid introduction, but read somebody like Richard Rhodes, "Deadly Feasts," for much more detail on the modern end. (Rhodes does a bit much ax-grinding on Prusinder, though.)
3 of 5 people found the following review helpful
5.0 out of 5 stars The molecular biology is astounding Aug. 6 2005
By Bruce P. Barten - Published on Amazon.com
Format:Hardcover
This is a very complicated matter, with highly specific vocabulary that attempts to describe a variety of forms of a disease which is capable of being distinguished by different incubation periods in the various inbred species of genetically pure or altered mice that have been inoculated with transmissible spongiform encephalopathies (TSEs) in the strains that have been isolated before the French edition of this book went to press near the end of the year 2000. A key word is prion, a protein that might form part of the membrane of a normal cell. Originally in this book, prion was defined by Stanley Prusiner, winner of the Nobel Prize in 1997, in 1982 as the carrier of the infection for TSEs. "Prions are small proteinaceous infectious particles which are resistant to inactivation by most procedures that modify nucleic acids." (p. 100). Forming rods in a polymer structure, ultimately doctors, "when examining brain tissue from kuru patients, had been able to recognize what they called amyloid plaques" (pp. 101-102).

Assuming that any cow in England which showed signs of bovine spongiform encephalopathy was an indication that the entire herd had been fed contaminated meat and bone meal, (from "forty-six British plants that until 1988 had converted a total of 1.3 million metric tons of meat and bones into animal feed" p. 147), "the total number of cattle affected by the disease from the beginning of the epidemic until the end of 2000 was nearly two hundred thousand in Great Britain," (p. 151). Since the cow form of the disease and the sheep form act differently in mice who are infected, a grand experimental test was performed to see if any sheep have picked up the BSE form:

"In the summer of 2001, rumors began to circulate to the effect that the BSE agent had been found in sheep; the official outcome was to be announced at the end of the year. Europe's health authorities were in a state of red alert. If the results were positive, drastic steps would have to be taken in the sheep-farming sector. Then, just two days before the outcome was made public, there was a dramatic announcement: The researchers had made a mistake. They had mingled samples of sheep brains with samples of cattle brains--and thus there are still no data on the possible transmission of BSE to sheep in natural conditions." (p. 188).

I have noticed that when people try to assign unique numbers to anything, there is always someone who fails to notice that two of those numbers are not the same. I have even worked with a computer that had so few consecutive numbers in a field that it was not able to tell the difference between numbers that had more than the number of digits in the field. There are forty million sheep in Britain, few of which look like cows, even in that night in which all cows are black, but worse than that: the brain samples might look a lot like brain samples from a cow. This experiment was more than double blind if no one kept tract of how samples were mingled.

I love the word epizootic: "Why was an epizootic--an animal epidemic--declared at one particular time, the early 1980s, and only in the United Kingdom?" (p. 189). It must be related to "the death of six white tigers from the Bristol zoo between 1970 and 1977; they died of what was then diagnosed as a transmissible spongiform encephalopathy, but no one knows what became of the corpses. . . . After all, it isn't often that a cow eats tiger in the way that we eat beef." (p. 190). There are so many things no one knows.
4.0 out of 5 stars Review for neurobiology May 20 2013
By riveralv - Published on Amazon.com
Format:Paperback
Over the past sixty years bovine spongiform encephalopathy (BSE), or "mad cow disease", has become a popular neurological topic in society due to the increasing fear and anxiety concerning it's association with variant Creutzfeldt-Jakob disease (vCJD). How the Cows Turned Mad expands upon the history behind the discovery of the fatal brain infection in sheep and follows through to its subsequent appearance in both cows and humans. The book presents the histology and physiology of transmissible spongiform encephalopathy (TSE) by examining the diseases from multiple scientific standpoints. With a background in molecular biology of bacteria, the author of How the Cows Turned mad - Maxime Schwartz is a professor at the Institute Pasteur in Paris as well as the Director of Laboratories for the French Food Safety Agency. While working for the French Food Safety Agency Schwartz chaired a committee that advised the French government on the licensing of genetically modified organisms for general consumption. While some topics of the book may be frightening, the main concept is to provide information on BSE and other associated brain infections so that people can have a understanding of the diseases origins and transmission. The book presents the current information on BSE with the intent to educate the general public while also detailing the disease process and histology of the infections in a format that scientists in the neurological disease field could appreciate. There were many good sections of the book that I found highly enjoyable, with lots of interesting cases involving the diseases which were presented in a educational way that could be easily understood. The book was an overall good read and provided many detailed examples of the progression of the diseases, but it was dry at times and could have used more figures or pictures to illustrate important physiological details, for these reasons I would give this book 4 out of 5 stars.
How the Cows Turned Mad details the scientific progressions that have led to our current understanding of prion diseases, more specifically "mad cow disease", in a detective novel format. Beginning with the first veterinary accounts of brain infection in sheep the novel explores the various ways in which the infection could have mutated and been passed to bovine stock. Pasteur's work with physical chemistry and the microscope began to focus the investigations of the brain infections from bacteria to newer, smaller microbes called viruses. Professor Charles Besnoit was the first to apply microscopic investigation to the nervous system of animals infected by scrapie, thereby allowing him to distinguish between the causes of death for the animals. This allowed scientists for the first time to begin to track which diseases were actually killing the animals, while also presenting a view of how the disease progressed to fatality. A combination of work by the scientists Hans Creutzfeldt and Alfons Jakob eventually resulted in the detection of transmissible spongiform encephalopathy in human cases where behavioral and motor activity symptoms were observed. The book explains some of the scientific procedures used to eventually confirm that the fatal brain infections found in sheep, bovine and humans were in fact both similar forms of transmissible spongiform encephalopathy. The disease that for so long had been considered scrapie in sheep, goats and bovine and Creutzfeldt-Jakob disease in humans was found to be one in the same. The cause of scrapie/CJD was finally established by a scientist named Stanley Prusiner, he identified a protein called a prion and found that the protein accumulated in the brains of infected humans and animals to form plaques. Prions were found to propagate by transmitting a misfolded protein state that guides further misfolding of healthy proteins. Since prion diseases have exponential growth rates the accumulation in infected tissue causes damage and eventual cell death. The stability of the folded prion structure also provides a resistance to denaturation via chemical and physical agents making disposal of contamination tissue extremely difficult. Several cases of CJD being contracted via infected bovine lead to wide-spread panic among European and American farmers, in conjunction with public mistrust of bovine meat products. Because of strict measures taken by the United States as well as the United Kingdom the BSE epidemic has largely come to and end, but due to the long incubation period of CJD it will be many years before we can confirm an end to the disease.
How the Cows Turned Mad details the history of the diseases known as transmissible spongiform encephalopathy in a clear manner, although some chapters of the book could be more concise. The writing style of the author is clearly understandable and presented in a format that the lay man could comprehend. Although some chapters, such as 5 and 10 seemed to be longer than necessary to relay the information, the book as a whole was clear and organized. There was not ever a point where the wordiness of the author became a problem with understanding the point of the chapter, but there were sections I felt could have been elaborated on more as well. The inconsistencies were more of the exception to the rule though as I found the book as a whole to be very enjoyable to read. One of the more major issues I had with the book was that it could have used more figures and photographs, there were very few and even as I write this I find it difficult to recall any one of them vividly. Pictures would probably help to solidify and visually represent the disease processes as well as reiterating their similarities. By visually allowing the reader to explore the disease I feel that it would help to audience to become more engrossed in the progression of the disease over time. Figures may also help to organize some of the data that is presented in the body of the book in order to understand trends that were explained. The general tone of the book is somber, which is to be expected when dealing with the neurological infections being discussed, but the final statement of the book is one of hope. A hope that the worst of the infection that has destroyed so many lives around the world is over. The story is a continuing one, but How the Cows Turned Mad will allow many a better understanding of infectious neurological infections.
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