The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases

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.

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.
March 26, 2004