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 from 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 people [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 decade 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 deaths 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 Organization recently agreed with the British government's conclusion that there is a new variant of CJD whose appearance 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 reminiscent[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 importation 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 announcement was made, the European Union decided to quarantine 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 troops 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 incubating 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 possible 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 formaldehyde[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 the 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 Agriculture[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 suggested that the cows be used to detonate the country's buried landmines.[145]. The extermination may not have eliminated the epidemic, though, as evidence exists that prions can remain infectious for years in the soil[61] and/or may be harbored by insects.[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 clinical 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 absolu-tely no connection 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].                                       
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