Cannibalism: A Perfectly Natural History(76)
Ultimately, it’s impossible to know just how many sick cows were hurried off to the slaughterhouse, but the numbers are thought to have been significant, especially since examples of this sort of practice are not unheard of within the meat industry, even decades later. In August 2014, Federal prosecutors indicted three Northern California slaughterhouse workers. They were charged with cutting off “USDA Condemned” stamps from sick cows and then slaughtering them while inspectors were on their lunch breaks. It is suspected that significant numbers of diseased animals were processed and sold for human consumption.
Until this time, there hadn’t been much publicity about what was going on, and the British government made an effort to keep it that way. Their veil of secrecy might have remained in place far longer if several publications hadn’t broken the BSE story in April 1988. The industry standard, Farming News, ran a front-page headline that read “Spongiform Fear Grows,” while the Sunday Telegraph set the stage for the term “Mad Cow Disease” with a story entitled “Raging Cattle Attacks.” An earlier paper in Nature also demonstrated that scrapie had been experimentally transmitted from sheep to monkeys, supporting Wilesmith’s hypothesis that cows had gotten sick from eating scrapie-infected sheep rendered into meat and bone meal. Note: Most scientists now believe that it is more likely that BSE originated from a spontaneous mutation in cows and did not result from sheep scrapie jumping to a new species.
The Southwood Committee published its official report in February 1989. Their most important finding supported the government’s claim that they were dealing with scrapie, and so they reported that, “the risk of transmission of BSE to humans appears remote.” They also concluded that there was no evidence the disease was related to Creutzfeldt-Jakob disease (CJD), the rare but deadly form of human spongiform encephalopathy.
The authors of the Southwood report also painted a rosy picture for anyone concerned about the spread of BSE, predicting that it would begin to decline in the early 1990s and die out spontaneously sometime after 1996. No further effort was required. Beef was safe. Long Live the Queen!
I met Dr. Laura Manuelidis on a beautiful summer day in New York City’s Chelsea district. When she isn’t writing and publishing critically acclaimed poetry, Manuelidis heads up the Neuropathology section at Yale. She has become the foremost spokesperson for the relatively few scientists who believe that prions are not the self-replicating, seemingly immortal, proteinaceous pathogens we’ve been led to believe. In 2007, she and her coworkers identified a 25-nanometer viruslike particle found in both sheep scrapie and CJD.
No one has ever seen a prion protein (PrP), but according to Manuelidis the reason has nothing to do with size.
“They probably don’t exist,” she told me.
Manuelidis has been researching neurodegenerative diseases for more than 30 years, and she and her colleagues have performed a wide range of studies on transmissible spongiform encephalopathies (TSEs) like Creutzfeldt-Jakob disease. Their results support a very different, though far from new, conclusion—that viruses are the cause of these neurodegenerative diseases, which also include kuru and bovine spongiform encephalopathy (BSE). Since the name “prion” implies infectivity (the ability of a pathogen to establish infection), in Manuelidis’s book, clumps (or plaques) of misfolded proteins exist, but since they aren’t infective, prions do not exist. According to Manuelidis, then, what is being called a prion requires a new name.
She explained that proteinaceous plaques aren’t confined to neurological disorders like kuru, but are also seen in peripherally located viral diseases. “Conventional viruses also induce protein aggregates and amyloid fibers that are prionlike. The plaques are an end-stage product that doesn’t occur early in these infections.” I later learned that the abnormal protein masses were also characteristic of diseases like rheumatoid arthritis and diabetes—and in none of these instances were the clumps or the proteins that made them up transmissible.
“So what you’re saying is that the misfolded proteins aren’t the cause of the spongiform encephalopathy, they’re an effect—a late-stage result of a viral assault. Correct?”
“Yes,” she replied.
But if these plaques aren’t pathogens and they’re not infective, what exactly are they?
According to Manuelidis, they’re “a runaway defense mechanism against the infecting agent,” which she believes is viral in origin.
“When these misfolded proteins do show up, infectivity drops through the floor,” she told me. In other words, once the body’s defenses kick into gear (which ultimately leads to the production of amyloid plaques), the pathogen is less able to infect another host. The spread of the virus is curtailed.
On May 16, 1990, John Gummer, the head of the UK’s Ministry of Agriculture, infamously responded to the public concerns over potentially contaminated beef in the UK by feeding a hamburger to his four-year-old daughter on the BBC television show Newsnight.
In 1993, two British dairy farmers died of CJD, a disease that was supposed to strike one out of every 1 million people. The government response was that it was just a bizarre coincidence. In May of the same year, 15-year-old British schoolgirl Victoria Rimmer began having trouble keeping her balance and within weeks she was falling constantly. Admitted to the hospital, Rimmer underwent a battery of tests—all of which came back negative. Finally a brain biopsy was obtained and the doctor who examined the results was stunned. Her brain was riddled with holes and amyloid plaques identical to those seen in the brains of kuru victims. With hesitancy, the physician informed Victoria’s grandmother that the girl had spongiform encephalopathy—mad cow disease. What took place next was even more incredible. An investigator from the government’s CJD-surveillance unit in Edinburgh visited Mrs. Rimmer, warning her not to say anything about her granddaughter’s condition. “Think about the economy,” he told her, “think about the Common Market.”