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How the Cows Turned Mad Hardcover – March 15, 2003
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From The New England Journal of Medicine
In How the Cows Turned Mad, Maxime Schwartz recounts the history of the prion diseases, or subacute spongiform encephalopathies, as if writing a mystery. Schwartz refers to all the prion diseases under one rubric, "The Disease," as if a single criminal with many disguises were active. This story has truly been an exceptional one, with events that have dramatically and unexpectedly affected the economy, public health, and the political scene. We now know that there are a number of subacute spongiform encephalopathies of animals (e.g., scrapie and "mad cow disease," or bovine spongiform encephalopathy) and of humans (e.g., kuru and Creutzfeldt-Jakob disease) that are transmissible and that have similar pathologic features. The transmissible agent, a prion, is a misfolded protein (called PrP(sup Sc)) that has assumed a conformation different from that of the normal cellular form of the protein (called PrP(sup C)). In the presence of PrP(sup Sc), PrP(sup C) is converted into additional molecules of PrP(sup Sc), which then accumulates, resulting in the induction of disease. The prion is remarkable because it apparently contains no nucleic acid, triggers no immune response, causes a disease with an extraordinarily long incubation period, and is highly resistant to inactivation by physical and chemical agents (such as heat and formalin) that normally are expected to inactivate a pathogen. One of the attractive features of this book is its historical perspective, beginning with coverage of early records related to scrapie in 17th-century England and ending with a discussion of recent concerns in France about the spread of bovine spongiform encephalopathy and a new variant of Creutzfeldt-Jakob disease that has been strongly linked to bovine spongiform encephalopathy. The work of Pasteur is highlighted, presumably in part because Schwartz previously served as head of the Pasteur Institute. Schwartz reviews the many ways in which the prion diseases have surprised us and also covers recurrent themes that have frequently been overlooked. For example, in the 1930s scrapie was transmitted to sheep by means of inoculation with a formalin-treated vaccine against the virus that causes louping ill. The vaccine transmitted scrapie because it had been prepared from sheep that were presumed to have unrecognized scrapie and because scrapie resists formalin inactivation. This event presaged instances of iatrogenic spread of the subacute spongiform encephalopathies, such as one in which Creutzfeldt-Jakob disease was transmitted by the implantation of electrodes that had previously been used in an affected person and on which prions had been incompletely inactivated by formaldehyde vapor. The transmission of kuru after ritual cannibalistic ingestion of the tissues of relatives who had died of kuru reminds one of how bovine spongiform encephalopathy became endemic in cattle as a result of the ingestion of feed that contained contaminated cattle carcasses. Schwartz informs us that these events are reminiscent of a still earlier event recognized by Nocard, a student of Pasteur, in which anthrax was spread from fertilizer that had been prepared from carcasses of anthrax-infected animals. The subacute spongiform encephalopathies still have much to teach us, both about their pathogenesis as well as about the role of misfolded proteins in other neurodegenerative diseases. We will need to attend to the history of these diseases carefully and also be guided by current data in order to ensure that we respond appropriately in the future to the surprises of these diseases. How the Cows Turned Mad should be understandable to readers with a relatively limited scientific background; however, it would have been helpful for Schwartz to have provided some figures to show, for example, the conversion of PrP(sup C) into PrP(sup Sc). The information about these diseases is reasonably current, with only rare errors. In the interest of space, Schwartz unfortunately was selective in his discussions of prion disease and in listing references and crediting scientists who contributed to knowledge in this field. Pasteur wrote, as cited by Schwartz, "Might it not be permitted to believe . . . that the day will come when easily utilized preventive measures will end these scourges which abruptly afflict and terrify people . . . ?" In these times of outbreaks of West Nile virus infection and the severe acute respiratory syndrome, one cannot help but think of the more cautious and somber comment of Claudius in Shakespeare's Hamlet: "Diseases desperate grown / By desperate appliance are relieved, / Or not at all." Some stories that involve intrigue and human tragedy, such as Hamlet, will always challenge us and leave unanswered questions. Raymond P. Roos, M.D.
Copyright © 2003 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.
*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
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Top Customer Reviews
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.
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.
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.)
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.