On April 25, 1985, a British veterinarian named Dr. Colin Whitaker was called to examine a sick Holstein in a herd of three hundred milk cows in Kent, in southeastern England. The miserable animal was aggressive, nervous, and awkward. After several weeks, the helpless cow was slaughtered, and her carcass was processed at a rendering plant. In the next eighteen months, seven more cows from the same herd also sickened and were put down, and three other herds in southeastern England each suffered three similar cases. By late 1987, 420 cases of bovine spongiform encephalopathy (BSE) had been confirmed in England and Wales, beginning a furor over British beef that may not subside for years since no one can predict how many more cases are continuing to incubate. It is the history of BSE and all of the related spongiform diseases that Richard Rhodes tells so well in Deadly Feasts: Tracking the Secrets of a Terrifying New Plague.
Rhodes begins his story with a dramatic account of a Fore cannibal feast in the eastern highlands of New Guinea in 1950. Although eating the dead had been a custom among the Fore for only about a century, the practice had become popular among Fore women and children. By the time Dr. D. Carleton Gajdusek arrived in New Guinea in 1957, Fore women, especially, were being devastated by kuru. (The term kuru, in the Fore language, means “shivering—with cold or with fear.”) Gajdusek had graduated summa cum laude in biophysics from the University of Rochester at nineteen and then studied physical chemistry under Linus Pauling at the California Institute of Technology and microbiology under John Enders at Harvard. Gajdusek had been studying child development in Australia, and he went to New Guinea when Dr. Vincent Zigas, the public health officer in the eastern highlands of New Guinea, requested help in studying the fatal kuru.
Kuru is a terrifying disease, with its victims staggering, starving, choking, immobilized in their own waste, but usually remaining mentally alert until their deaths in three to six months. The malady presented Gajdusek and Zigas with anomalous symptoms. It seemed to be a degenerative brain disease similar to Parkinson’s and Alzheimer’s; unlike them, however, it appeared capable of transmission: An epidemic spread to 5 to 10 percent of the population in the hardest-hit villages. A further puzzle was the complete lack of infection in any of the victims. The first clue came when Gajdusek sent a kuru brain to Dr. Igor Klatzo, a neuropathologist at the National Institutes of Health (NIH) in Bethesda, Maryland. Klatzo reported finding microscopic knots of a form of protein known as amyloid, and he reported to Gajdusek that the kuru brains resembled the damaged brains of victims of Creutzfeldt-Jakob disease.
Creutzfeldt-Jakob disease (CJD) was named for two German physicians. In 1913, Hans Gerhard Creutzfeldt treated a young woman in Breslau, Germany, for uncontrollable tremors and involuntary laughter. Creutzfeldt’s autopsy revealed millions of dead brain cells, partly replaced by glial repair cells that appeared as stars under his microscope. Seven years later, Dr. Alfons Jakob at the University of Hamburg read a paper describing similar autopsy findings on four of his patients. Eventually—but not for some years—the distinctive evidence of CJD came to be recognized as the holes that gave the brain a spongy appearance. Hence, the term “transmissible spongiform encephalopathy” (TSE) that now covers CJD, kuru, and the other diseases of humans and animals that manifest spongiform pathology and scrapie-associated fibrils (SAF). CJD apparently occurs spontaneously in one out of a million people spread evenly around the world. Unlike kuru, which usually damages only the cerebellum, CJD also attacks the cerebrum, which explains the dementia common among its victims.
William Hadlow was an American veterinarian working in England in 1959 studying scrapie, a disease of sheep that had been known in England for more than two hundred years. (It first appeared in the United States in 1947.) Hadlow chose to study scrapie sheep brains in detail and discovered the same spongiform structure with astrogliosis—star-shaped glial cells—that Creutzfeldt and Jakob found in humans. Then Hadlow had a stroke of luck. Gajdusek had been appointed to the NIH staff in 1958 and had quickly put together a traveling exhibit of color photographs of kuru brains. The exhibit was at the Wellcome Medical Museum in London in June of 1959, and Hadlow went to see it. The color photomicrographs of spongiform disease arrested his attention; when he read papers published by Gajdusek and Zigas on kuru, he called its resemblance to scrapie “uncanny.”
A letter from Hadlow to Gajdusek (who had never heard of scrapie) forced Gajdusek to admit that perhaps a slow virus, rather than heredity, was the cause of scrapie. Recognizing the need for detailed studies of scrapie transmission in other animals—the disease has a five-year incubation period in sheep—Gajdusek appointed an expert virologist, Clarence Joseph Gibbs, Jr., to experiment with scrapie in mice in 1961. Gajdusek then returned to New Guinea and discovered the changes...
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