Product Description

In 1996, British doctors were horrified to discover that mad cow disease (BSE), an affliction that had been plaguing British cattle for ten years, had jumped the species barrier and was appearing in humans as variant Creutzfeldt-Jakob disease (vCJD). Not unlike the mad cows, victims of vCJD suffer from a degenerative neurological disease that peppers the brain with microscopic holes, causing dementia, loss of motor control, and certain death. What alarms researchers and public health officials worldwide is that the incubation period for vCJD may be as long as 10 or even 15 years, and during this period those infected are symptom-free. And because the disease is so far undetectable except by autopsy, there is no way of knowing with certainty how many people have already been infected. In fact, even travelers who spent time in the U.K. from the early 1980s through the mid-1990s are still considered to be at some risk. What's more, although the U.S. has not detected any mad cows within its borders, there are plenty of "mad deer" running free in several states, and the disease afflicting them is a BSE-type neurological disorder. Called chronic wasting disease (CWD), the illness in these deer has yet to be linked with any human deaths. But given BSE's ability to jump species, there are no guarantees.

In The Pathological Protein, Philip Yam describes how, in this atmosphere of uncertainty, scientists have discovered that the agent of disease in vCJD and a host of other devastating neurological disorders is a bizarre, misshapen version of a protein called a prion. Once introduced into the human neurological system, malformed prions recruit the body's own normal prion proteins, giving them the same pathological ability to destroy brain tissue. Unlike the better-known pathogens that afflict humans -- bacteria, viruses, and parasites -- prions have so far proved resistant to drug therapies and even standard sterilization. No amount of cooking infected meat will prove effective against them.

In a medical detective story with an undercurrent of urgency, Yam describes how the mysterious prion was discovered, how it has been linked to a number of exotic and poorly understood illnesses, and how likely it is that scientists will soon find effective tools for controlling its spread, diagnosing its presence, and treating the devastating disorders it causes.

Philip Yam has been writing and editing for Scientific American since 1989 and is currently the magazine's News Editor. He lives in New York City. This is his first book.

Product Details

• Amazon Sales Rank: #636145 in Books
• Published on: 2003-06
• Original language: English
• Number of items: 1
• Binding: Hardcover
• 304 pages

Review
. . . a detailed yet readable history of the mad cow scare and other prion diseases. -- Library Journal, June 15, 2003

. . . good at explaining the complex science behind TSEs, as well as the prospects for better diagnosis and treatment. -- The Economist, August 14, 2003

. . . thorough, entertaining and honest. -- The New Republic Online, Summer 2003

An unforgettable overview, compelling readers to learn more about this unfolding, scientific saga. -- Choice, March 2004

From the Inside Flap
In the space of 12 months, Stephen Churchill lost his focus, his memory, then most of his speech, then even the ability to dress, feed, and clean himself. He developed an excessive fear of water and sharp objects and refused to bathe or shave. And before long, with his unsteady gait and his tendency to fall, he spent his days slumped in a wheelchair or confined to a bed. To the staff of the nursing home where Stephen lived, the relentless decline was depressingly familiar -- it had all the earmarks of Alzheimer's disease. But something in the picture did not fit. The patient, when he died, was only 19 years old.

Doctors later discovered that Stephen had succumbed to a new kind of killer, the prion, now known to be the cause of mad cow disease in cattle, chronic wasting disease in American deer and elk, and Creutzfeldt-Jakob disease and fatal insomnia in humans, among other exotic ailments. Doctors and researchers have been aware of some of these diseases for a century and more, but only in the last two decades have scientists even begun to understand just how the pathological protein spreads to new species and kills its victims.

In this timely and intriguing book, Philip Yam describes the history of the scientific effort to track down and understand the prion, and the medical effort, still underway, to devise treatments for those who suffer from its ravages.

About the Author
Philip Yam has been writing and editing for Scientific American since 1989 and is currently the magazine's News Editor. He lives in New York City. This is his first book.

Customer Reviews

Easily the best book of its kind
Philip Yam's book The Pathological Protein is easily the best book of its kind. Written in clear, simple language for the non-specialist audience, The Pathological Protein is a thoroughly comprehensive, concise and, above all, scientifically accurate review of BSE and related diseases. Yam has been writing and editing for Scientific American since 1989 and this, his first book, demonstrates the high standard to which all science writers ought to aspire.

The first chapter of The Pathological Protein describes, from a very human perspective, the effects of variant Creutzfedt-Jakob disease on one victim, 19 year-old Stephen Churchill, and his family. From this tragedy, Yam then goes on to review the history of CJD and the mysterious diease 'kuru', which reached epidemic proportions amongst the Fore people of Papua-New Guinea because of their cannibalistic funerary rites. After discussing the hereditary transmissible spongiform encephalopathies (TSEs) of humans, outlines what is known of the TSEs of animals. Philip Yam's reviews of scrapie, BSE, transmissible mink encephalopathy and chronic wasting disease are up-to-date, interesting, and extremely readable.

There is an interesting episode related in the book. Carlton Gajdusek had been searching, unsuccesfully, for the cause of kuru. William Hadlow, and American scrapie researcher on a secondment to the United Kingdom, visited the Wellcome Medical Museum in London to look at a display on kuru that Gajdusek had prepared. It was Hadlow who first noticed the very close resemblance between kuru and scrapie. The similarities in epidemiologic features, general clinical pattern and the neurohistologic changes led him to the realisation that these diseases were probably mmebers of the same family. As a result of Hadlow's insight transmission experiments were started which, eventually, led to our current understanding of the TSEs

This book covers the hypotheses for the origins of BSE, the evidence for the link between BSE and vCJD, current methods and problems of diagnosis of the TSEs, and the search for cures. Philip Yam clearly is thoroughly versed in the scientific literature of the TSEs, but also interviewed a broad range of scientists, consumers advocates and regulators. So, he knows what he is writing about, and this is made evident by the clarity and accuracy of his explanations. Although there is no 'dumbing down' of a difficult and complex subject, the author has written a book which makes his subject easily accessible to the non-specialist reader. The book is referenced, well indexed, has a useful glossary and also suggests sources for further information, including the more useful web sites and organisations providing suport and help for families of CJD victims. While the book is written for the interested lay person, I would have no hesitation in recommending Philip Yam's The Pathological Protein to veterinarians and colleagues who want an interesting, thorough and current review of these fascinating diseases.

An engaging and important read!
Particularly now that mad cow disease has been found in the US, "The Pathological Protein" is an important and engaging read. The book covers not only mad cow disease and its human form, variant Creutzfeldt-Jakob's disease, but it also covers the underlying cause: misfolded proteins called prions. Yam explains different types of prion diseases and discusses the researchers that have been striving to understand the prion and control the devistating neurological illnesses it causes. Yam discusses the safety of beef today and what could be done to stem the rise in mad cow disease. Well-researched, exceptionally well-written, and highly recommended!

This is a book you should read
Protecting the quality of U.S. beef is a concern for each of us individually and for the economy. The Pathological Protein is a good read, a great source of valuable information about prion diseases, and an important guide to some very questionable areas of meat producing.