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For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of sheep rub their fleeces raw before collapsing. In England, cows attack their owners in the milking parlors, while in the American West, thousands of deer starve to death in fields full of grass. What these strange conditions–including fatal familial insomnia, kuru, scrapie, and mad cow disease–share is their cause: prions. Prions are ordinary proteins that sometimes go wrong, resulting in neurological illnesses that are always fatal. Even more mysterious and frightening, prions are almost impossible to destroy because they are not alive and have no DNA–and the diseases they bring are now spreading around the world. In The Family That Couldn’t Sleep, essayist and journalist D. T. Max tells the spellbinding story of the prion’s hidden past and deadly future. Through exclusive interviews and original archival research, Max explains this story’s connection to human greed and ambition–from the Prussian chemist Justus von Liebig, who made cattle meatier by feeding them the flesh of other cows, to New Guinean natives whose custom of eating the brains of the dead nearly wiped them out. The biologists who have investigated these afflictions are just as extraordinary–for example, Daniel Carleton Gajdusek, a self-described “pedagogic pedophiliac pediatrician” who cracked kuru and won the Nobel Prize, and another Nobel winner, Stanley Prusiner, a driven, feared self-promoter who identified the key protein that revolutionized prion study. With remarkable precision, grace, and sympathy, Max–who himself suffers from an inherited neurological illness–explores maladies that have tormented humanity for centuries and gives reason to hope that someday cures will be found. And he eloquently demonstrates that in our relationship to nature and these ailments, we have been our own worst enemy. Advance praise “The Family that Couldn’t Sleep is a riveting detective story that plumbs one of the deepest mysteries of biology. The story takes the reader from the torments of an Italian family cursed with sleeplessness to the mad cows of England (and, now, America), following an unlikely trail of misfolded proteins. D. T. Max unfolds his absorbing narrative with rare grace and makes the science sing.” –Michael Pollan, author of The Omnivore’s Dilemma and The Botany of Desire “Much has been written about prions and Mad Cow Disease–nearly all of it is worthless. Thankfully, from the world of journalism comes D.T. Max to set things right. Throw all those other “Mad Cow” books in the trash: This is the book to read about prions–or whatever you want to call them. It’s a riveting tale, told by someone with a very special understanding, derived in part from his own strange ailment. Find a cozy spot, clear your schedule and dive in.” – Laurie Garrett, author of Betrayal of Trust and The Coming Plague “D. T. Max deftly unfolds the mysterious prion in all its villainous guises. Although scientists do not fully understand these proteins–how they replicate and wreak such havoc in their victims’ brains–The Family That Couldn’t Sleep reveals their historical, cultural, and scientific place in our world. Prepare to be enlightened, entertained, and frightened.” –Katrina Firlik, MD, author of Another Day in the Frontal Lobe “A great book. D.T. Max has drawn the curtain on a cabinet of folly and malady that will stagger your imagination.” – Philip Weiss, author of American Taboo “D.T. Max has combined the enthralling medical anthropology of Oliver Sacks with the gothic horror of Stephen King to produce a medical detective story that is as intelligent as it is spooky. The villain of The Family That Couldn’t Sleep is the prion, a tiny little protein that causes some of the most terrifying, brain-mangling, creepy diseases known to man. Always fascinating–how could it not be, given that its characters include cannibals, mad cows, madder sheep, a Nobel prize-winning pedophile, and, most poignantly, an Italian family cursed by fatal insomnia?–Max’s book is also a gripping account of scientific discovery, and a heartfelt meditation on what it means to be cursed with an incurable, and brutal, illness.” – David Plotz, author of The Genius Factory
Over 200 references to literature about the Anga people of New Guinea. Covers mostly journal articles and books published 1901-1972 in English, German, and French. Besides Introduction, entries arranged by authors under Ethnology, anthropology, and history; Linguistics; and Medicine and physical anthropology. Appendix consists of census units of various linguistic groups. 2 maps.
This volume marks the first time that a collection of contemporary facial scoring techniques and their utility, whether clinical, experimental, theoretical, or otherwise, follows an historical introduction of the area, thereby recording the developmental history of this science.
Perhaps the best-documented epidemic in the history of medicine, kuru has been studied for more than fifty years by international investigators from medicine and the human sciences. This significantly revised edition of the landmark anthropological classic Kuru Sorcery brings up to date the anthropological contribution to understanding disease, the medical research that resulted in two medical Nobel Prizes, and the views of the Fore people who endured the epidemic and who still believe that sorcerers, rather than cannibalism, caused kuru. The kuru epidemic serves as a prism through which to see how Fore notions of disease causation bring into single focus their views about the body, the world of social and spiritual relations, and changes in economic and political conditions-aspects of thought and behaviour that Western medicine keeps separate.
These eighty-two letters speak for themselves. They document the discovery and the origins and pursuance of the first year of study of kuru in great detail and should serve to dispel any misconceptions about who did what, and answer many hows, whens, and whys about the early field work and laboratory investigations on kuru. Above all, the publication of these letters is a tribute to the late Dr. Joseph E. Smadel who had so much to do with the first year of our kuru investigation, and through whom the laboratory at the National Institutes of Health became our base, where we were able to demonstrate the slow virus etiology of kuru, the first proved slow virus infection of man.