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Public Health Implications of Mad Cow Disease

May 5, 1996 by Michael Greger 
M.D.

Peter Hall showed the first signs of depression around Christmas, 1994. In five months he was in a wheelchair. He died at age 20 of Creutzfeldt-Jacob disease, a relentlessly progressive and invariably fatal dementia [95] which usually attacks people in their sixties[33]; cases under 30 are exceedingly rare[112]. Around the next Christmas, an uproar ensued when leading British neuropathologist Sir Bernard Tomlinson refused to feed his children burgers out of fear that they might contract this disease f rom infected beef[102]. His fears were realized on Wednesday, March 20, 1996, when the British government announced that the most likely explanation of Peter's death and 9 other recently diagnosed cases of Creutzfeldt-Jacob Disease among English young pe ople [41] was exposure to bovine spongiform encephalopathy(BSE)[71].

'Very unhappy' were the words British vet Colin Whitaker used to describe the dairy [12] cow who became the world's first documented case of BSE on a fine spring morning in 1985[123]. Dubbed Mad Cow Disease by the British press[53], BSE has by now a dec ade later stricken over 150,000 cattle [106]. The fear now is that through the consumption of infected beef Britain may be on the brink of the largest public health calamity since the Black Death[121] with worst case scenario estimates involving the deat hs of millions of people[101].

Creutzfeldt-Jacob disease (CJD) is a human spongiform encephalopathy[70] whose standard clinical picture involves weekly deterioration[67] into blindness and epilepsy[59] while one's brain perforates like swiss-cheese[105]. The World Health Organizatio n recently agreed with the British government's conclusion that there is a new variant of CJD whose appearence is best explained by the BSE epidemic in cows because of a number of consistent unusual features.[136]. Other than being extraordinarily young[ 41], the human victims also had atypical EEGs[96] and took twice as long to die[33]. The real clincher came, though, when their brains were autopsied[108]. Along with unusual psychiatric symptoms[80], the brain pathology was found to be vividly reminisce nt[69] of Kuru, a disease found in a New Guinea tribe of cannibals which ate the brains of their dead[46a]. In the words of Germany's leading expert on CJD "everything suggests that BSE is the cause..."[93].

The probable [107] link between BSE and CJD, viewed by Britain's Chief Medical Officer as "cause for serious concern", came as a complete reversal of the position the government held for a decade[58]. The next day 5 European countries banned the importa tion of British beef[37] and 10,000 British schools dropped beef from their menus[30]. That Friday McDonald's stopped serving British beef[29] and by Monday, Burger King[119] and Wendy's[125] stopped too. And Tuesday, less than a week after the announc ement was made, the European Union decided to quantantine the island, voting an immediate and indefinite worldwide ban on the export of British beef[87]. The same day in one of the biggest operations in decades, Ireland deployed extra police with troop s on standby to seal off its border to prevent cattle smuggling [94]. Finally, two days later, British beef was banned in Britain, but only from cattle considered to be higher at risk[117].

The debatedly [70] novel[3] infectious agents that cause spongiform encephalopathies like CJD and BSE evoke no immune response [50] and consequently may slowly accumulate[14] for an invisible latency period of up to 30 years.[60] No one knows how many people have already been infected. John Pattison, Dean of the University College of London Medical School and Chairman of the British government's Spongiform Encephalopathy Advisory Committee (SEAC), thinks there could be 500,000 people already incubatin g CJD[27]. "At this stage," he adds," we have to say it's totally unpredictable."[88] Needless to say, he does not feed beef to his grandson[82]. Microbiologist Steven Dealler, secretary of the Spongiform Encephalopathy Research Campaign, places the po ssible death count at 2 million people[118]. Professor Pattison reportedly agreed with this worst case scenario assessment.[118] This could mean up to a half million deaths a year as the epidemic peaks into the next century according to Richard Lacey[64] , a microbiology consultant for the World Health Organization and child health specialist.[68]

Called the "most intriguing, unsolved puzzle in modern biology"[141] it is now close to being generally accepted that the cause of both the disease in humans and cows isn't a virus or a bacteria, but a "prion", an infectious protein.[78] Not only is it not known how they replicate[6], the whole concept challenges the basic tenets of biology.[45] Because of their unique makeup, they are practically invulnerable. They are not adequately destroyed by cooking[13], canning[14], nor freezing.[40] Chemicals or enzymes which degrade nucleic acids[55], proteolytic enzymes of the digestive tract[49], and usable doses of UV or ionizing radiation[40] are all ineffective in destroying their infectivity. Even heat sterilization[40], domestic bleach[59a], and formal dehyde[14] sterilization have little or no effect. In fact, the only way to ensure that one's burger is safe is to marinate it in Drain-O (or other concentrated alkali).[59] They are the smallest[9], most lethal self-perpetuating biological entities in th e world.[74] In six years, BSE has gone from the most serious threat ever posed to British agriculture[44] to what the Prime Minister calls the worst crisis to confront the government in general, since the Falklands War.[72] Widespread fear first struck in 1989[19]. Only months after the government concluded that the disease probably wouldn't spread to other species[1], Max, someone's pet Siamese, died of a hitherto unknown feline spongiform encephalopathy[68]. BSE-infected pet food was "overwhelmingly the most li kely explanation."[36] And then zoo animals started dropping dead.[4] Together, this sparked a public uproar[19] with unprecedented media attention.[50] Fearing its spread into the human population, hospitals[34], nursing homes[34], and over 2000 schools[ 21], affecting over 750,000 school children[7], stopped serving beef or restricted its consumption. By May 1990, a quarter of the population reportedly refused to eat beef.[38] In six months beef prices dropped 10[5]-25%[3], devastating the cattle industry. The final blow came when Australia[43], Israel[43], and a dozen other countries[24] banned the importation of British beef because of the BSE epidemic.

After a $6.5 million advertising campaign touting red-meat consumption, from Britain's Meat and Livestock Commission[34], though, and the Minister of Agriculture munching burgers with Cordelia[100], his four-year-old daughter for the TV cameras[16], the schools put beef back on the menu[50] and beef consumption regained semi-normal levels.[38] Despite continued warnings from scientists like Lacy, the European community had lifted its ban and most other countries had greatly relaxed their trade restricti ons[7] until news of these first human deaths recently broke.[90]

Now with the worldwide ban in effect, Britain has been forced to agree to kill and incinerate[26] (or perhaps mince and bury)[103] millions of cattle[75] at a total cost of billions of dollars[11]. The decision is supported by the Minister of Agricultur e[119] and the National Farmer's Union[119], while admitting the mass slaughter would be 'too horrific to contemplate.'[120] In response to the proposed slaughter, an Hindi group in India offered the afflicted cows sanctuary while a Cambodian newspaper s uggested that the cows be used to detonate the country's buried landmines.[145]. The extermination may not have eliminate the epidemic, though, as evidence exists that prions can remain infectious for years in the soil[61] and/or may be harbored by insec ts.[76] A better solution would seem to be to finding out which cows were actually infected instead of just indiscriminate killing. Such a test for detecting BSE in live cattle was offered to the British government seven years ago by Harash Narang, a cl inical virologist with the public health service[85]. Dr. Narang was subsequently fired, and reportedly had his tires slashed five times, his home broken into, and his brakes tampered with[126]. Critics presume the government did not want the public to know how much infected beef was entering the food chain[126] and therefore discouraged such tests to protect the $4.6 billion beef industry[85] In fact Dr. Lacey claims that the British government has at all stages concealed facts and corrupted evidence beyond much reasonable doubt[68].

The Labor Party charged the government with a "reckless disregard for public health"[119], "seriously complacent decisions"[121] historically and a "pathetic"[90] response to the current crisis. Others echoed similar charges of procrastination and delay [85]. A New Scientist editorial explained how the British government "tried to push scientific advice aside when it did not suit them."[106] An editorial in The Lancet criticized the government for making the error of equating the absence of evidence of risk with evidence of little or no risk.[82] Statements like "There's no way of predicting..." seemed to transform in the halls of government into "There is no evidence..."[107]. British agriculture minister Angela Browning's January pronouncement that her government's stance was "ultra precautionary" [115] bears a certain resemblance to similar statements now coming from the United States government.[116] Six months ago Britain's Prime Minister was still asserting that there was absolutely no co nnection between BSE and CJD[115] and still attempts to reassure the public that beef is safe to eat to this day[82]. Likewise the USDA continues to adamantly parrot day after day that there is no BSE in the USA[130].

The United States has the highest per capita beef consumption in the world.[65] and 100,000,000 cattle[8] Traditional bovine spongiform encephalopathy hit North America in 1993.[15] The first cow discovered to be infected, one of many imported from the UK before both Canada and the US banned the importation of British cattle, was found on a ranch in Alberta, Canada.[8] Of the 499[116] British cattle imported into the US before the 1989 ban, 188 of them have been melted down into lard and protein[8] (pre sumably for other livestock to eat) and 35 remain unaccounted for.[116] One of the imported bulls slaughtered had a "central nervous system abnormality" of which the USDA reported, "There is no definitive evidence that [the bull] either had or did not hav e BSE."[8] Although the importation of British beef has been banned from the US for a decade due to an unrelated disease[7], over 13 tons of meat and bone meal, which has been implicated in the birth of the British epidemic, has come into the US from Engl and between 1982 and 1989.[2]

"BSE was 'almost certainly'[10] caused by feeding cattle ground up, dead, diseased sheep"[18] infected with an ovine spongiform encephalopathy known as scrapie.[20] In modern agribusiness, cows are no longer herbivores. "Protein concentrates" (or meat an d bone meal, both euphemisms for mashed- up bits of other animals left over from the slaughterhouse) are fed to dairy cows[22] to improve milk production,[65] for example. The real problem now, though, is not that we've made cows meat eaters but that we'v e turned them into cannibals as well; the recycling of the remains of infected cows into cattle feed[60] has probably led to the epidemic's explosive spread.[42] An editorial in the British Medical Journal described BSE as resulting "from an accidental e xperiment on the dietary transmissibility of prion disease between sheep and cows."[57] A subsequent experiment of this kind, with humans, probably occurred in England in the late 1980's when meat contaminated with BSE entered the food chain.[57] The res ult of this experiment is awaited "as we live through the incubation period" over the next decades.[57]

Indigenous conditions here conducive to a BSE outbreak include the presence of scrapie in 39 states.[54] The 40-year[52] USDA Scrapie Eradication Program has been deemed a "dismal failure"[63] and even implicated in the recent rise of scrapie-infected sh eep.[54] Admitting defeat, the USDA scrapped the Scrapie Eradication Program 2 years ago and replaced it with an "entirely voluntary" control program.[21] The proportion of sheep to cattle in the US is dramatically smaller than in the British Isles though , which helps minimize the risk of an outbreak.[65] This is a moot point, however, if BSE is already here. Since 1947 there have been 25 outbreaks of Mink Spongiform Encephalopathy (also called TME) on US fur farms.[22] This perplexed researchers who were unable to orally infect mink with scrapie-infected sheep brains.[49] A clue came in 1985 when TME wiped o ut a population of minks in Wisconsin who hadn't eaten any sheep.[35] Their diet consisted almost exclusively of dairy cattle called "downers,"[54] an industry term describing a syndrome in which cows mysteriously drop down and are too sick to get up.

The possibility, then, that US dairy herds were harboring some form of BSE intrigued University of Wisconsin veterinary scientist Richard Marsh.[65] To test this, Marsh inoculated US cattle with the infected mink brains.[49] As predicted, they died.[49] And when he fed the brains of these cows to healthy mink they too died of a spongiform encephalopathy[54] providing what he thought was the missing link.[66] Marsh hypothesized that the proposed BSE strain indigenous to the US manifests itself as more of a do wned cow disease than a mad cow disease.[49] With about 300,000 cows going down for unexplainable reasons every year in the US[65], this has frightening implications on a grand scale. The downer cow is then melted down in a process called rendering into feed and her bones are boiled[128] (along with her skin and cartilage)[77] to make gelatin, a main ingredient in Jell-O and marshmallows. The critical experiment came when Marsh inoculated scrapie infected sheep brain into US cattle.[63] If you do this i n England the cows go mad, twitching[16] and kicking into a rabid frenzy.[12] But in America, cows instead stagger to their deaths like downer cows do[65], supporting the belief that a form of BSE is already here in the United States.

By 1990 the USDA had 60 labs monitoring US cattle herds for BSE.[7] In 1991 APHIS, the governmental agency which ensures the health of the nation's livestock, concluded that the "possibility of BSE appearing in US cattle is extremely low."[21] The assump tion made by APHIS and others[2], however, was that "scrapie infected sheep were the only source of the BSE agent."[21] This is certainly questionable in light of the evidence for an indigenous BSE agent. Likewise, the USDA surveillance program (described as slow, clumsy, and ineffective)[66] began looking for the rabies-like symptoms of the traditional British strain of BSE[52], in effect ignoring Marsh's findings.[21] In June 1992 a USDA consultant group continued to disregard the available evidence, de ciding that changes in the research program to accommodate the possibility that BSE was already present in the US were "not appropriate at this time."[21] No surprise really, when one realizes that this panel included representatives of the National Milk Producers Federation, the National Renderers Association, The American Sheep industry Association and the National Cattleman's Association.[21] According to a newspaper report, though, in 1993 the USDA finally backed down and started testing downercows[6 5].

In all, 2,660 brains from 43 states have been examined by APHIS[116] with plans to increase the number[92]. They report that no evidence for BSE has been found[116]. Unfortunately they were evidently relying on standard techniques used by British offic ials[129]. As reported in the April 1994 issue of the Journal of Infectious Diseases not only does the suspected American version of BSE differ in outward appearance, the affected brain also looks different than the British variety. Reportedly only a sm all percentage of the few thousand brains are therefore even being properly tested.[131] Even, however, if the more sensitive techniques were utilized in all the cases, Marsh feels that this number would be insufficient to impart a sense of security[129] . There is also suspicion that the USDA might try to cover up any suspected cases in an attempt to protect the $36 billion[129] beef industry from collapse.[47]

In Germany, for example, scientists have admitted that there have been many cases of BSE that were not reported[124]. When the monetary compensation given to the British farmer whose infected cows were incinerated by the government rose from 50% of mark et value to full compensation, the number of reported cases shot up 73%.[59] Presumably, the earlier economic disincentive persuaded farmers to overlook a few mad cows. In general in fact British BSE cases have been severely underreported with as few as 60% of clinical cases reaching UK government statistics. It was reported in the Sunday Telegraph that "British officials believe that some European countries concealed or ignored evidence of 'mad cow disease' for fear of the consequences for their own farming industries."[124] The problem, as many English pundits saw it, is that the Ministry of Agriculture, Fisheries, and Food represents the interests of both consumers and the beef industry.[78] A similar conflict of interest exists in the United States.

The mandate of the USDA is to promote agricultural products but also to protect consumer health. In Britain, at least, it would seem that the government's attempt at protecting the beef industry by concentrating more on PR crisis management than on d oing anything substantial ended up not only hurting the industry, but consumers, farmers, and the government as well.[98] With scientists like Marsh saying "The exact same thing could happen over here as happened in Britain,"[35] and with beef consumption already at a thirty-year low,[34] the USDA is justifiably worried. There was even a complaint filed with the FDA concern ing a woman with CJD who had been taking a dietary supplement containing bovine tissue.[4] Like England, we have been feeding dead cows to living cows for decades.[7] In fact, here in the US a minimum of 14% of the remains of rendered cattle is fed to oth er cows[49] (another 50% goes on the pig and chicken menu).[2] In 1989 alone almost 800 million pounds of processed animals were fed to beef and dairy cattle.[115] Partly because of this, the USDA has conceded that "the potential risk of amplification of the BSE agent is much greater in the United States" than in Britain.[8]

To make things worse, there has been a dramatic increase in the use of animal protein in commercial dairy feed since 1987.[35,49] The recent introduction of bovine growth hormone will only increase the need for rendered animal proteins in the rations of dairy cattle--of whom we eat 2.6 billion pounds of annually[8]. According to top encephalopathy expert Joseph Gibbs, one out of every million cattle naturally develops BSE.[65] A single teaspoon of ingested high infectivity meat and bone meal is thought to be enough to cause BSE in a cow[80]. Between this and eviden ce that prions may be able to adapt to their hosts and become more virulent with time[22], it would seem absolutely necessary to enact the ban and stop recycling this disease through US cattle. In June 1993 the Foundation on Economic Trends, a Washington public interest group[191], petitioned the FDA to ban all feeding of ruminants (cows, sheep) to other ruminants as the European Commonwealth had done three years before[23]. The legal petition was largely ignored[115]. The next year the FDA did propose to at least stop feeding sheep offal to cows, but it was blocked by vehement protests from the rendering and livestock industries[83]. A week after the Britain's recent admission that people m ay be dying from eating burgers, another public interest group, the International Center for Technology Assessment, filed a similar legal petition[115]. Leading consumer group Public Voice for Food and Health Policy has since also called for a ban[[135].

Three days after the latest petition was filed the meat industry announced a "voluntary" ban on feeding cows to cows[115]. In Britain they tried a similar voluntary ban; it failed miserably[115]. In the US the same rendering industry promised to stop feeding sheep brains to cows years ago; the FDA confirmed that this failed also[84]. On April 3, 1996 the World Health Organization called for a worldwide ban on feeding animal tissues to livestock[134] The FDA has promised to "expedite" such regulation s.[132] This is expected to mean that putting a formal ban in place will take 12 to 18 months[132]. Even with the law in Britain, though, surprise random inspections last year showed half of the English slaughterhouses in violation of the cow to cow ban regulations[91], but it is better than no law at all[105].

US officials admit that it is "very difficult" to verify compliance with the current voluntary ban[32]. In fact weeks after the "voluntary" ban was announced by the industry the feeding of rumi nant protein was still continuing at rates of million of pounds a day[115], supporting the director[104] of the Center for Media and Democracy John Stauber's contention that the oxymoronic voluntary ban was just a "worthless PR sham".[132]

A spokesperson for the National Cattlemen's Beef Association (NCBA), admitted 3 years ago that his industry could indeed find economically feasible alternatives to feeding rendered animal protein, but that the NCBA did not want to set a precedent of be ing ruled by "activists"[Food Chemical News, July 5, 1993]. Another NCBA spokesperson, Gary Weber, appeared on the Oprah Winfrey Show this April[131]. Clearly alarmed and disturbed by the fact that cows in the US are forced to eat cattle remains, Oprah swore she would never eat another burger again[131]. Our government knew that the such feeding practices would be "vulnerable to media scrutiny" as portrayed in an internal PR crisis management document.[115] After Oprah tried to remind the audience tha t cows were supposed to be herbivores, Dr. Weber defended the practice by stating "Now keep in mind, before you--you view the ruminant animal, the cow, as simply a vegetarian--remember that they drink milk."[131]

Cattle prices plummeted after the show aired.[97] The president of the National Cattlemen's beef Association called Oprah "a cheerleader for...anti-beef propaganda."[203] "We're not going to sit back and let trash TV trash a vital industry..." said the Texas Agriculture Commissioner in a prepared statement.[97] Texas agriculture officials are planning to bring a lawsuit against the opposing guest on the show, Howard Lyman, a cattle rancher turned vegetarian under a 1995 state law that prohibits unfoun ded comments about perishable food items[97]. These are among the same Texas officials that responded to the tragic news of the human deaths in Britain by staging a mocking April 4 publicity stunt cook-out.[115] On the show was Beryl Rimmer, whose 16 yea r old granddaughter lay in a coma, blind and dying, one of the ten diagnosed with the new variant of CJD[131]. A doctor from the government's CJD surveillance unit reportedly told her not to make her granddaughter's plight public; she should "think of the economy and the Common Market."[68]

In the same show Weber asserted that no animal could ever enter a US packing plant displaying BSE symptoms[131]. Even if this is true, the majority of infected and infectious cattle become beef before clinical symptoms arise.[58] In fact, for every "mad " cow incinerated in the UK, there may be hundreds slaughtered and sold to butchers before any overt symptoms develop.[51] Narang estimates that a third of all English cattle going into the food chain are infected with BSE.[133] Because of this, it is estimated that every adult in the UK has eaten on average 50 meals containing tissue from infected cattle[126]. In the Journal of Public Health Medicine, Dealler, the microbiologist, wrote that in all probability most humans who have consumed British bee f since the start of the BSE epidemic will have been exposed to the infection.[58] In America it may be even harder to stop infected cattle from entering the food supply since it has been argued that dairy cattle are routinely culled earlier than in Britain.[135]

Within a week the National Cattlemen's Beef Association prepared a position statement which stated that "Scientific evidence indicates that beef (meat) and milk do not present a risk as there is no evidence that the agent that causes BSE is present in me at and milk"[114] First of all, even the USDA has stated that "the safety of British beef cannot be demonstrated for 20 or more years."[21] As retired professor of clinical neurology WB Matthews put it, "Claims that British beef is entirely safe to eat.. .are scarcely scientific when the question has not been tested and is, perhaps, untestable."[28] The NCBA is probably attempting to refer to a study based solely on mice in which no detectable infectivity was found in muscle, but beef is not just muscle; it's laced with peripheral nerves and lymphoid tissue[39] which have both been shown to be infectious.[39]

And it is possible that muscles do in fact harbor sufficiently deadly numbers of prions; spongiform encephalopathies of goats, minks, and hamsters can be transmitted through muscle alone.[55] No tissue, though, concentrates the pathogens more than brain or spinal cord.[46] In the slaughterhouse, beef is recovered by mechanically sawing down the corpse, which may imbed brains (which at this stage h ave the consistency of pudding)[59] throughout the meat.[55] In addition, the prions are thought by some to continuously accumulate in one's system, so that even small doses could build up with time.[14] Milk, however, is safe according to the World Heal th Organization[62]. So although BSE is more prevalent in dairy cows, the potential danger is probably in their quick retirement into hamburger, not their milk[48].

There is a cluster of CJD in eastern Pennsylvania, however, which was linked to cheese consumption and there is a case description published in the New England Journal of Medicine demonstrating that milk from a Creutzfeldt-Jacob diseased woman was indeed infectious[25].

Just because someone consumes an infectious dose of BSE, though, does not mean that they will necessarily get the disease. There is still no direct proof that BSE can even cause CJD in humans.[112] The recent deaths in Britain may turn out to be unrela ted to beef consumption. One experiment which is currently underway is to do further studies of the autopsied brains called strain typing which could provide strong empirical evidence of a BSE-CJD link[86] Results will not be forthcoming for at least 18 months, though, and may not even then provide definitive answers[112]. The preliminary results from another study involving mice genetically engineered to express human proteins seem to minimize the risk to humans, but are not yet conclusive[102]. Simi larly it will be a year and a half before firm conclusions can be drawn[112].

BSE has been able to infect and (therefore) kill cats, antelopes, and even ostriches from presumably eating infected protein, though.[1] Eighty cats have now since followed Max to the grave[101]. Experimentally, one can give BSE to monkeys[56], pigs, ch impanzees [8] and thirteen other species[58]. There really is no reason to believe that BSE will not similarly infect humans.[8] So far, in fact, BSE has proven more infectious than most other transmissible spongiform encephalopathies.[39] The latest dat a has come from the comparing of the cow and human prion proteins. Two striking similarities were found between gorillas, chimpanzees, humans and cows which might have "predisposed" higher primates to cattle prion infections[97], a finding that "can onl y be interpreted as worrying."[102]. This could explain why people could develop CJD from eating meat[97]. So what are the odds that humans are susceptible? No one knows. When Dealler was recently asked by a member of the British Parliament what the c hances of any particular person contracting CJD from BSE-infected meat were he replied "Between zero and 100 percent."

Incidentally, cows aren't the only possible source of prion disease. Paul Brown, medical director of the NIH neurological disorders institute with the US Public Health Service[17], believes that pigs and poultry could be harboring BSE and passing it on to humans, adding that pigs are especially sensitive to the disease.[103] "It's speculation", he says, "but I am perfectly serious."[103] On March 20, the British government banned farmers from feeding processed cattle blood, bones, fat, and offal to pigs and poultry.[31] A week and a half later they even halted the use on meat and bone meal as agricultural fertilizer[113] and we in the United States can't even get them to stop feeding cows to other cows.

If indeed a form of BSE is in the United States and causing disease among American meat eaters, one would expect to see an increase in Creutzfeldt-Jacob disease incidence. CJD, however, is not a reportable illness in this country[131]. Because the Cen ters for Disease Control (CDC) does not actively monitor the disease [32] a rise similar to the one in Britain could be missed[32]. Other than the cheese-associated five-fold expected rate in the Lehigh Valley[139], a striking increase in CJD was reporte d in Florida[143], and there is anecdotal report of an cluster in Oregon[144]. An analysis of death certificates in a number of states, though, showed a stable and typical CJD incidence rate from 1979 to 1993[81]. Just recently the CDC initiated an ext ensive study of death certificates in four states[138]. Death-certificate diagnoses are well known to be not always accurate, though.[140]

CJD is seriously underdiagnosed at present[70]. The most common misdiagnosis of CJD is Alzheimer's disease[70]. Both diseases show overlapping symptoms and pathology[70]. There can be spongy changes in Alzheimer's and amyloid plaques in CJD.[70] In f act the brains of the young people who died from the new CJD variant in Britain look like Alzheimer's brains[83]. Stanley Prusinger, the scientist who coined the term prion, believes Alzheimer's may even turn out to be a prion disease[115].The true prev alence of prion diseases in this or any other country remains a mystery[70]. An informal survey of neuropathologists registered a theoretical range of 2-12% of all dementias as actually CJD[70] whereas the official rate in this country is less than 1 cas e in a million per year. Compounding the situation, autopsies are rarely even performed on atypical dementias[70], because medical professionals are often unwilling out of fear of infection[68].

Four million Americans are affected by Alzheimer's[115]; it is the fourth leading cause of death among the elderly in the US.[142] Epidemiological evidence suggests that people eating meat more than four times a week for a prolonged period have a 3 time s higher chance of suffering a dementia than long-time vegetarians[79]. A preliminary 1989 study at the University of Pennsylvania showed that over 5% of patients diagnosed with Alzheimer's were actually dying from a human spongiform encephalopathy.[21] That means that as many as 200,000 people in the United States[21] may already be dying from mad cow disease each year.

In an examination of the risk of a major CJD epidemic in Britain, one of the government advisors wrote "I can't imagine it will just stop at ten cases..." It seems he was right. By the end of March the number was revised to an unconfirmed 12[27] Then came two women in their 30's dying in Germany[48]. A 26-year old man just died in France[109]; French authorities concluded it was "exactly identical" to the ten in Britain after consulting with health officials there[114]. The latest suspected victim i s a 24 year old woman in New York who may have contracted the disease years after receiving a Christmas gift of British beef cutlets[137]. And by the end of April another English girl was diagnosed with CJD[110]. At age 15 she is thought to be the world 's youngest victim of the disease[113].

After the diagnosis of BSE pattern CJD was confirmed, leading neurologist Peter Behan made the controversial announcement that she "picked it up through hamburgers."[113] Assuming a ten year incubation period, all of the young people dying of this disease in Britain over the last few months were presumably infected when there were but only a few hundred infected cows being turned into beef. In the year 1990 alone, it is estimated that 250,000 BSE-infected cows we re eaten, which of course begs the question how many people might be dying at the turn of the century[111].

In the United States we are left with an industry that continues to risk public safety and a government protecting that industry's interests over those of the consumer. A clue as to why the government has been so stubborn in enacting a ban on feeding co w and sheep remains to cows and sheep can be found in a 1991 internal USDA document entitled "BSE: Rendering policy" retrieved through the Freedom of Information Act.[21] It weighed the costs and benefits of a number of preventative measures including a t otal ban on feeding ruminants to ruminants.[21] The supporters of this option felt that this minimized the risk to public health.[21]

APHIS, however, goes on to explain that the "disadvantage" of this approach is "that the cost to the livestock and rendering industries would be substantial"[115] The lost revenue to cattle producers of such a ban is estimated at a dollar per cow[89]. The document continues, asserting that such a policy "could pose major problems for the US livestock and rendering indust ries."[21] After all, the rendering, feed, and cattle industries do rack up annual sales of only $1.2 billion, $20 billion, and $60 billion, respectively.[8] The Archbishop of York recently blamed the BSE epidemic on greed and profit.[127]. Other than simply corporate profiteering, I think this crisis shows to what length governments will go to prevent financial harm to powerful lobbies in general, and in doing so risk immeasurable harm to those they claim to represent.


May 5, 1996 Please feel free to use and/or distribute in any way -- Michael Greger M.D.
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30. Financial Times 3/22/96:1
31. Financial Times 3/23/96:5
32. New York Times 3/27/96:A12
33. Sunday Telegraph 3/31/96:1
34. US News and World Report (1990) Jul 2:44
35. Scientific American (1990) May:34
36. Science (1990) 249:1492-3
37. Guardian 3/26/96:13
38. British Food Journal (1992) 94(9):23-6
39. British Medical Journal (1993) 95(8):22-34
40. Veterinary Record (1992) Jul 4:2
41. Guardian 3/26/96:7
42. Veterinary Record (1992) Feb 15:146
43. New Scientist (1990) Jun 9:32-4
44. Veterinary Record (1990) Nov 3:440-1
45. Lancet (1988) Sep 10:607-8
46. Bulletin of the World Health Organization (1992) 70(2):183- 190
47. Guardian 3/27/96:T4
48. Dev Biol Stand (1993) 80:157-170
49. Dev Biol Stand (1993) 80:111-8
50. Prion Diseases of Humans and Animals , edited by Bradley, B. et al. 1992):285-299
51. Guardian 3/21/96:6
52. Dev Biol Stand (1993) 80:119-121
53. New Scientist (1993) Oct 9:50-1
54. Sub-acute Spongiform Encephalopathies , edited by R. Bradly (1991):41-6 55. Nutrition and Health (1991) 7(3):117-134
56. Veterinary Record (1993) Apr 17:403-5
57. British Medical Journal (1992) 304:929-930
58. Journal of Public Health Medicine (1995)17(3) 261-8
59. Food Microbiology (1990) 7:253-279
60. Lancet (1993) 342:790-3
61. Sub-acute Spongiform Encephalopathies , edited by R. Bradly (1991) 195-202
62. Reuter wire (4/3/96) Experts see minimal...
63. Sub-acute Spongiform Encephalopathies , edited by R. Bradly (1991):272-274
64. Reuter wire (4/3/96) British farmers eager...
65. Wisconsin State Journal (1993) Sep 26:1C
66. Capital Times (1993) Sep 11:6A
67. Mosby's Medical and Nursing Dictionary 2nd Edition (1986)
68. Mad Cow Disease:The History of BSE in Britain by RW Lacey (1994)
69. British Medical Journal (3/30/96) The link is...
70. British Journal of Psychiatry (1991) 158:457-70
71. Lancet (1996) 347:889
72. Weekly Telegraph (4/10-16/96) Britain will defy...
73. Lancet (1996) 247:945
74. Scrapie and Mad Cow Disease (1993) by G.D. Hunter
75. Chicago Tribune 4/11/96:N14
76. Henryk Wisniewski's findings published in Lancet
77. B Anne Wickham in the British Medical Journal, Potential transmission of...
78. Nature (1996) 380:273-4
79. Neuroepidemiology (1993) 12(1):28-36
80. Lancet (1996) 347:916-7
81. MMWR (1996) 45(14):303
82. Lancet (1996) 347(9006):915
83. Newsweek 4/8/96:58-59
84. Wall Street Journal (4/1/96) US groups move...
85. Wall Street Journal (3/22/96) Beef disaster in...
86. British Medical Journal (1995) 311:1415-6
87. British Medical Journal (1995) 311:1419-20
88. CNN (3/21/96) Countries ban British...
89. Reuters wire (3/29/96) US will ban...
90. Reuters wire (3/28/96) Britain temporarily bans...
91. New York Times, The Logic of the 'Mad Cow' Scare by John Darnton
92. AP wire (3/26/96) US inspectors to...
93. Reuters wire 3/26/96) German scientists suggest...
94. Reuters wire (3/26/96) Ireland guards border...
95. Oxford Textbook of Medicine (1996) 3:3981
96. British Journal of Medicine (3/30/96) BSE linked to...
97. Reuter wire (4/24/96) New link found...
98. British Journal of Medicine (3/30/96) Meltdown: media and...
99. Living Marxism (2/96) 87
100. Wall Street Journal (3/21/96) British suppressed beef...
101. Economist (3/30/96) Mad cows and...
102. www.nature.com, Nature science update (5/3/96) Genetic link between...
103. New Scientist 4/6/96:3-5
104. Toxic Sludge Is Good for You, by John Stauber, Sheldon Rampton (1995)
105. NIH expert CJ Gibbs speaking at Cornell University 12/1/94
106. New Scientist 3/30/96:3
107. New Scientist 3/30/96:4
108. New Scientist 3/30/96:6
109. PA wire 4/26/96 French CJD death...
110. PA wire 4/26/96 Girl, 15, got...
111. PA News wire 4/27/96 Expert warns of...
112. Lancet (1996) 347:945-48
113. Reuters World Report wire 4/27/96 British scientist warns...
114. London Times 4/15/96 Frenchman, 17, dies...
115. PR Watch (1996) 3(1):1-8
116. USDA APHIS (4/2/96) BSE Factsheet
117. Financial Times 3/29/96:16
118. London Times 4/18/96 BSE deaths 'could...
119. Financial Times 3/26/96:1
120. Financial Times 3/25/96:1
121. Financial Times 3/23/96:6
122. Independent 4/13/96 Slaughtered cattle to...
123. Sunday Telegraph 3/31/96:12
124. Sunday Telegraph 3/31/96:12
125. Guardian 3/26/96:1
126. Guardian 3/23/96:23
127. Sunday Times 4/7/96 Fear grows as...
128. Herald (Rock Hill, SC) 3/28/96:1A
129. New York Times 3/27/96:12A
130, Newsday3/27/96
131. Oprah Winfrey Show transcript 4/16/96:1-9
132. New York Times (3/20/96) Safeguards against mad-cow...
133. Guardian 4/1/96 Brain disease predated...
134. New York Times (4/4/96) U.N.'s WHO seeks...
135. Reuters North America wire (4/30/96) Conditions ripe for...
136. The Lancet (4/13/96) 347:1036
137. Watertown Daily Times 4/29/96:6
138. New Scientist (4/20/96):10
139. Neurology (4/93) 43:A316, Abstract 614P
140. Neurology (4/93) 43:A316, Abstract 615P
141. California Institue of Technology professor of biology Dr. Hood
142. American Journal of Medical Genetics (1995) 60:12-18
143. Neurology (4/94) 4a(Suppl 2):A260, Abstract 537S
144. (4/16/96) Margie Boule, columnist...
145. New Yorker (4/15/96):102


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