BY JIM SCANLON
In mid-March a frightening announcement was made by a British Health Minister. Scientists had determined that "the most likely explanation" for 10 new, unusual cases of Creutzfeldt-Jacob Disease, a rare but inevitably fatal degenerative brain disease, was "exposure" to the agent that causes BSE, or "Mad Cow Disease." Bovine Spongiform Encephalopathy, as the disease is known, broke out among British cattle in the mid 1980s, peaked in 1992, and has been rapidly declining.
Overnight, a seven billion dollar industry was threatened with disaster, and thousands of rural communities—not to mention the European Economic Community—were suddenly presented with a bleak, contentious and ominous future.
The sale of British beef slumped and most European countries banned it. The British government reacted by calling the bans "hysterical" and "unfair." There was talk of slaughtering thousands of British cattle—mostly older dairy cows—and who would pay farmers how much for their losses. A relatively young Frenchman who died of CJ Disease was said to have liked to eat beef bone marrow and the price of beef fell all over Europe even though cattle herds there were free of BSE.
What got lost in the turmoil was just what were people talking about when they said "beef" and beef products?" Most people think of a steak or a hamburger or roast beef when they think of eating "beef" and not beef eyeballs, spleen, spinal chords, intestines, brains, testes, hooves and ovaries—or a mixture of some, or all of the above.
Furthermore, vaccines, drugs, glue, cosmetics, vitamin capsules, protein supplements and who knows what else are all made with rendered animal tissue, and one doesn't usually think of using or eating these as "eating beef."
To add insult to injury, the two foremost international scientific periodicals in the world, Science and Nature, both editorialized severe criticism, not only of the way in which the announcements were made, but the flimsiness of the evidence linking BSE, the disease of cattle, to Creutzfeldt-Jacob Disease, a rare disease of elderly humans very much like Altzheimer's Disease. There can be no doubt that the evidence is sparse. How could such a thing happen to provoke such a panic?
The infectious agent responsible for a number of fatal brain diseases of humans and a large number of domestic and wild animals is now called a "prion." These protein molecules used to be called "unconventional viruses" or "viral particles." Unlike viruses they are not easily destroyed by heating or by ultraviolet radiation. In some species (humans), the infection can be transmitted hereditarily, and in others (mink), not. The latency period can be relatively short, 18 months where there is direct injection of ground up brain tissue, or up to 30 years. Indeed, the disease is thought to occur spontaneously!
Disease occurs when a normal protein in the brain comes in contact with a mutant form of the same protein. The mutant causes the normal protein to fold abnormally and this spreads slowly and inevitably destroys the brain as a functional organ and with that the person.
What is different about the 10 recent cases of CJ Disease in Britain is the young age of the victims, which range from 16 to 39, six female and four male. None of them were farmers although one was a butcher and another vacationed on a farm which was free of Mad Cow Disease. One was even a strict vegetarian, although only since 1991.
CJ Disease is unusual in people under fifty except for its manifestation under the name of kuru (shaking sickness) among formerly stone age hill tribes of New Guinea which used to practice a morning ritual involving the preparation and eating of body parts of the deceased, including the brain. (This practice, usually called ritual cannibalism or endocannibalism, can be thought of as a holy communion with the dead family member, a sort of "last supper").
Transmission took place among the women who did the cooking and the male and female children who helped. The mutant protein presumably entered the body through cuts and abrasions or though the eyes, nose or mouth. Adult men got the disease at a much lower rate. Some 2,500 people died of the disease during the 17-year-period of official surveillance. When cannibalism was banned by Australian Colonial authorities in the late 1950s, the infectious particles were taken out of the food chain and the disease eventually died out.
In a bizarre aside, D. Carlton Gajdusek, the medical anthropologist who shared the Nobel Prize for his work linking kuru with CJ Disease, Fatal Familial Insomnia and Transmissible Mink Encephalopathy, was arrested by the FBI on April 4, 1996. He was charged with child sexual abuse of one of the 50 or so children he brought back from New Guinea, raised and educated. A spokeswoman at the Frederick County (Maryland) Jail said he was released on $350,000 bail. The alleged victim is now a 23 year old man.
Even though the British Government was actively looking for cases of Creutzfeldt Jacob-like diseases since 1989, no one can be sure if these ten represent the full number of cases. The new cases seemed more like kuru than classical CJ Disease. All of the victims presented symptoms which were psychiatric in nature and, since the infectious agent involved does not produce an immune response, the only sure way to tell is by autopsy after death. Since medical examiners themselves have contracted this disease doing post mortem examinations, it is sometimes difficult to get doctors to confirm suspected cases.
Two newspaper articles in the London Mail on Sunday (2/18/96 "Four Teenagers Killed By CJD," and 12/17/95, "Mad Cow Deceit") were posted on the Internet detailing the horror and agony of the young people affected and the great difficulty some family members experienced trying to alert medical doctors that the disease might indeed by BSE, or Mad Cow. They called for more specific detailed information on the victims and assurances that all cases will be properly recorded.
Since the presenting symptoms of the victims were personality changes, depression, withdrawal, difficulty sleeping, fearfulness, staggering incoordination and paranoia, it is easy to understand how cases among young adults might be misconstrued or misdiagnosed, and cases which resulted in suicide, overlooked. The outward manifestations of the disease in humans is similar to the way sheep and cows are affected. The French word for Scrapie, the disease of sheep, is "Tremblant de Moulton" (shaking, trembling). It has a meaning similar to the Fore word "kuru" (trembling, shaking).
Strangely coincidental, in 1987 (before "prion" was an accepted term) while doing research on "unconventional viruses" (which cause CJ Disease, BSE and others) which might be involved in AIDS dementia, the Coastal Post learned of a cluster of Creutzfeldt Jacob Disease in Tucson Arizona involving three young men who worked in the Hughes Missile Plant, and others. An anonymous local neurologist had observed so many cases of suspected CJ Disease (20) over a ten year period that he or she asked for an investigation. It was later reported by the Arizona Daily Star that the Center for Disease Control found the Tucson cluster to be a maximum of 7 cases and "a coincidence."
Considering that the population of England, Scotland, Wales and Northern Ireland is 56 million, and Arizona is 4 million (Tucson, 400,000), a cluster of even 7 cases in a small city might seem important. If not then, then now—keeping in mind that British scientists were actively looking for the 10 cases they found.
While Britain struggles to protect its $6 to $7 billion beef industry, the U.S. Department of Agriculture expressed confidence that the U.S. $160 billion beef industry is safe. Nevertheless, concern has been expressed that the lack of reports of BSE in the U.S might be the result of insufficient testing and monitoring. Unlike Britain, the U.S. has no laws against the use of sheep or cattle carcasses in nutritional supplements, although voluntary guidelines were hastily agreed to after the British beef catastrophe.
The conventional explanation of the epidemic of BSE in Britain, which so far has affected 158,882 cattle on 33,292 farms, is that it started in the 1980s when the production process for nutritional supplements was changed. This allowed sheep body parts infected with scrapie (the sheep version of BSE) to enter the cattle food chain. (Pigs, chickens and pets were also fed similar supplements.) When infected cattle themselves were slaughtered and their offal rendered and fed to other cattle, a particularly well adapted form of the infectious molecule was refined, adapted and passed on and on.
There are a number of alternative theories on the origin of BSE in cattle including the use of organic phosphates poured directly on the backs of animals to eliminate the larva parasitic warble fly and, the most recent and intriguing, that the Icelandic hay mite is a reservoir of molecules which causes scrapie, the brain disease in sheep.
Since the banning of cannibalistic cattle feed supplements in the UK, the number of new cases of BSE has plummeted among older dairy cows and has all but disappeared from beef cattle which don't live as long. Considering that the peak for BSE was 1992 and the incubation time for kuru is about 5 years, the 10 cases may be just the beginning of an enormous epidemic of an undetectable, incurable, untreatable, horrible infirmity which destroys the brain and cripples the body before killing. Or the 10 cases could be something else which has only now been noticed, and thousands of cows will be sacrificed out of irrational fear.
It remains to be seen what effect the episode will have on factory farming, but it will certainly give a boost to organic food production.