Epidemic in waiting

September 22, 2001 The Guardian (London) by Kevin Toolis
There is no cure for it, no vaccine, no test. What we know about BSE is that most of Britain's 60m population have been exposed to it. We know that its human form, vCJD, has so far claimed 106 lives in Britain, five of them in one Leicestershire village. What we don't know is how humans contract the disease, how long the incubation period is, and how far the government is to blame. Kevin Toolis investigates a potential time bomb:

It began with a fall. Seventeen-year-old Stacey Robinson was crossing her mother's living room with three-month-old Josh in her arms when she walked straight into the wall. Stacey, born and brought up in the tiny village of Queniborough in Leicestershire, hit the floor hard, bruising her face and blackening her eye.

"We knew we had to do something then," says Stacey's 25-year-old brother, Simon Robinson. "She saved Josh but her laugh was funny, it went really deep. Her eyes were wide open and her pupils were dilated all the time. Even when she smiled - a really big grin - it was not her. She was acting out of character. The doctor put her on antidepressants because she had just had the baby. The doctor said postnatal depression and I went along with it. And then it got worse and worse. That's when the howling started and we had to send her away to hospital."

Stacey didn't want much out of life. She loved animals, horses, McDonald's, loud music, the band Oasis and hanging out with friends. She liked meat. She was a bit of a tearaway in a small English village tearaway sort of way; mischievous rather than mean. It was no big surprise when she got pregnant at 16. Or that her boyfriend wasn't much help with the baby. Stacey didn't care. She loved Josh - she had what she wanted. As it turned out, Stacey wasn't depressed. Her brain was being eaten away by the rogue prion agent responsible for mad cow disease. Stacey had variant Creutzfeldt-Jakob disease (vCJD), the infectious human form of bovine spongiform encephalopathy (BSE). Little clumps of indissoluble protein were building up in her cerebral cortex, destroying her neural pathways and giving her brain a sponge-like appearance. Stacey's brain was dying inside her skull and she was dying with it.

The howling went on for five months, night and day, from the autumn of 1997 to the spring of 1998, a low, growling, demonic yowl that escaped from her lips as if from deep within the earth; the cry of the damned. It could be heard halfway along the ward in Leicester's Royal Infirmary as Stacey plunged into madness. Soon she lost the power to walk, to talk, to eat, to clean herself, to use the bathroom. She turned aggressive, kicking, swearing and assaulting her nurses. She battered her forearm against the bed until it was black and blue. She held her hand under a scalding hot tap and felt no pain. In the end, the doctors turned her ordinary city hospital room into a padded cell.

In the same hospital ward, another young woman, Pamela Beyless, 24, was struggling to breathe. Pamela, once a bright, vivacious financial clerk, had developed the same mysterious illness a few months before. Now she was in a coma, incontinent, catatonic, her lungs fighting against pneumonia. Pamela had already, as Stacey soon would, become a living shell, a twisted human form on the bed, whose eyes stared vacantly into space. Pamela's last words to her parents before slipping into that mental darkness had been a terrifying plea for life. "Will I be saved?"

Stacey and Pamela were the first to be struck down in an unparalleled cluster of human cases of BSE centred on Queniborough. Within the space of two summers, five villagers - Glen Day, 35, Christopher Reeve, 24, a 19-year-old unidentified male and the two women - would die after contracting the disease.

The Queniborough cluster is unprecedented. Worldwide, there have been 110 cases of vCJD, 106 in Britain, three in France and one in Ireland. Queniborough has a population of 2,279, yet five of those 110 victims came from the village. Somehow, through some hidden pathway, the village's young people were attacked by one of the most terrifying diseases ever encountered.

Queniborough was soon nicknamed the "Village of the Damned", but this first cluster was also a potential breakthrough for medical researchers. Every previous victim of vCJD contracted the disease in isolation. There was no way of narrowing down how they became infected. For the first time, because of links between the Queniborough victims, it was potentially possible to trace the disease back and uncover the exact route of transmission. "There has got to be a connection, so many people, so close. It's not a coincidence," Simon Robinson says. "I was best mates with Christopher at school. I knew Glen to say hello to in the street, he lived a quarter of a mile away. There is something. Something to do with Queniborough. I need to know how my sister died and what killed her."

Mysteriously, the National CJD Surveillance Unit, charged with tracking the human form of BSE, at first denied the cluster's existence, but then reversed its position after local newspapers uncovered the existence of the fourth and fifth victims. In the end, the investigation of the Queniborough cluster was left to two doctors, Philip Monk and Gerry Bryant, from the poorly funded Leicester Health Authority. Monk and Bryant, both former GPs, had extensive experience investigating more common public health risks, such as food poisoning or tuberculosis outbreaks, but tracking down the source of the Queniborough vCJD infection across decades was an awesome undertaking.

It took hundreds of hours of interviewing the villagers of Queniborough, the victims' families, butchers, slaughtermen, grazers, auctioneers, animal health inspectors, farmers and prion disease specialists to build up a picture both of what the victims ate and the complex food chain that turned cows in a Leicester farmer's field into meat on a Queniborough dinner plate. Monk and Bryant had to wade through filing cabinets of esoteric scientific papers on prion theories before they could formulate what they describe as their "plausible hypothesis". They believe the cluster can be traced back quite simply to how two local butchers, John Smith and Ian Bramley, killed and butchered the beasts they sold to the villagers. "We owe it to the victims' families and to the villagers to find the answer to the outbreak," Monk says. "What has happened here has driven us on. There are a lot of questions that the whole world needs answering."

Finding out how the Queniborough victims became infected has become the most compelling medical detective mystery in the world today. The cluster holds the potential answer to two vital questions. How do humans actually contract mad cow disease? And how long is the incubation period?

Mad cow disease began in Britain, but not eating British beef does not necessarily lessen your risk of contracting the disease. Animal feed contaminated with the infectious BSE agent was exported to over 100 countries, whose livestock could now be harbouring mad cow disease. As a safety measure, the US, and a host of other countries, ban blood transfusions from anyone who has lived in Britain in the last 20 years for more than three months. But cow by-products end up in a bewildering range of everyday items, from the jelly in pastries to office furniture. Bovine serum is used in the manufacture of insulin and polio vaccines. Beef tallow, industrialised processed fat, finds its way into creams, ointments and even toothpaste. British pharmaceutical companies annually export pounds 5bn worth of products and hold a 12% share of the world market. For the past two decades, a significant percentage of those pharmaceuticals have used by-products, as commonplace as the gelatine in pill coatings, derived from BSE-infected cows.

In August, researchers from the University of California School of Medicine announced an apparent major breakthrough in the medical battle against vCJD. Rachel Forber, 20, from Merseyside, was apparently "cured" of vCJD after treatment with two previously licensed drugs, quinacrine, an anti-malarial, and chlorpromazine, an anti-psychotic. After 19 days of treatment, Forber, who had been forced to use a wheelchair, was able to walk unaided and complete a variety of coordination tests.

But claims of a "cure" were met with stony scepticism by the British scientific community. You cannot cure someone of a disease when you are not even sure they are infected in the first place. The only way definitively to ascertain whether Forber had vCJD would be through postmortem splicing of her brain. And in any case, another suspected vCJD patient treated alongside Forber continued to degenerate despite the drug treatment. Medical science still has no rule book to counter the disease. There is no cure, no proven treatment, no vaccine and no accepted test for infection.

Variant Creutzfeldt-Jakob disease is part of a family of transmissible spongiform encephalopathy (TSE) diseases that attack the central nervous systems of different species and fatally change the shape of key proteins in the brain. TSEs are biologically unique. The infective agent, a rogue proteinaceous infectious particle, has no DNA, the normal chemical alphabet of every living thing. In a poorly understood mechanism, rogue prions "infect" normal prions by changing their molecular shape from a helix to a flat sheet. The malformed prions, though still chemically identical to normal prions, are resistant to the brains' enzyme activity which removes no longer wanted proteins. The rogue prions accumulate and progressively destroy brain function.

Prions are among the most infectious agents known to man. In cattle, just one gram of BSE infected brain tissue is enough to infect a 500kg cow. Prions cannot be killed by heat, radiation, boiling or chemical destruction. Anyone who has had a surgical operation in Britain in the past 15 years has already potentially been exposed to vCJD through the use of surgical instruments - prions cannot be destroyed by the normal sterilisation techniques.

The clinical symptoms of vCJD are a catalogue of doom: muscle spasms (myoclonus), the involuntary loss of muscle movement including speech (akinetic mutism), cortical blindness and progressive dementia. "Your brain function level regresses back through the evolutionary stages. You become more primitive and then you die," Monk says. The median time span between the onset of symptoms and death is 14 months.

There is only one real previous model for TSE infection in humans. Up until the late 1950s, the Fore people of Papua New Guinea, who lived in Stone Age conditions, practised ritualistic cannibalism where the bodies of dead relatives were cut up and eaten at funeral feasts. Unfortunately for the Fore, their cannibalism was recycling a fatal prion disease that became known in their language as "kuru" - to tremble.

In 1981, Dr Richard Klitzman, then a young medical researcher, now a Columbia University professor, went to Papua New Guinea to investigate kuru clusters. The time of kukum na kaikai , cooking and eating relatives, was long over, but new cases of kuru were still appearing. Klitzman was able to identify three clusters where the incubation periods ranged from 21 to 28 years. In one instance, three boys as infants had been fed parts of their aunt at her funeral in 1955. One died six years later but the others lived for another 25 years before succumbing to kuru. It was the only funeral feast the boys had attended. Klitzman believes the maximum incubation period could be as long as 45 years. "The clusters in New Guinea show that one infected meal was enough to transmit the agent. Anyone in Britain in the late 1980s through to 1996, even on vacation, who ate one infected steak, could die from it."

Queniborough is an archetypal English village, complete with thatched cottages and roses, a church, two pubs, a scout hall, a school and one butcher's shop. The skyline is dominated by the soaring 14th century spire of St Mary's, whose churchyard is filled with the gravestones of villagers dating back to the 17th century. Off the main road, almost hidden away, are poorer, newer, working-class estates. Queniborough is a quiet place of untroubled lives, rigid class hierarchies and familiar patterns; a short walk in the countryside, a pint of beer at the Britannia public house at lunchtime then home for a traditional Sunday roast of beef, potatoes and two veg. Except in Queniborough, this weekly ritual, unknown to its participants, had a deadly portent. "You don't expect to die from eating a piece of meat," says Pamela's father, Arthur Beyless.

Scientists now believe that the disease that killed Pamela Beyless was born in England around the same time as she was, in the early 1970s. Somewhere in England's green and pleasant land, a single cow developed a mutation of the prion gene that turned the protein products of that gene into the BSE agent. Parts of that cow were probably eaten by human beings but other parts - guts, bones and the carcass - were rendered down and industrially processed into meat and bone meal (MBM). That was then used as a protein supplement feed for other cows. Some of those other cows became infected and bits of them, in turn, were fed to other cows in a spiralling chain of infection.

Feeding cows to cows was not a British invention. As agricultural production intensified in Europe and the US in the 20th century, finding cheap protein supplements to boost the yield of farm animals became a necessity. Dairy cows are expected to yield up to 23 litres of milk a day. Green grass is not enough. The rendering industry took what would otherwise be waste products - the skull, the brain, the spinal cord, the unwanted bones and guts. The final product, MBM, was mixed with cereal, bagged up and shipped back to farms to fatten cows, pigs and sheep for the dinner table. The British dairy farmer was not doing anything different from his German or American counterpart.

On December 22, 1984, Sussex farmer Peter Stent called out his local vet, David Bee, to examine a sick cow who was losing weight, trembling and had an arched back. Soon another eight cows in the Jersey dairy herd fell ill with the same odd disintegrating muscular coordination. Some became aggressive. "They tried to attack a colleague. They really went for him. It was baffling; they changed from being placid into different beasts," says Bee, who believed the cows were suffering from some kind of fungal poisoning. Bee and a colleague gave the condition a nickname, "Stent Farm Syndrome". The cows all died but not before Cow 142 had been sent to a local veterinary laboratory where the intact brain was examined.

The animal pathologist recorded the cause of death as "spongiform encephalopathy - acute". It was a perplexing finding. But as summer came, the remaining cows in the herd were put out to grass and the sickness stopped. Bee, a specialist cattle veterinarian, didn't come across another case in the next two years and forgot about the Stent cluster until the official announcement of BSE at the end of 1987. Cow 142 would in time be recognised as the first documented mad cow.

Cow 142's spongiform pathology had been very easy to recognise because all British animal pathologists were familiar with the condition in sheep. Scrapie, the TSE that affects sheep, was first identified in Britain in the 1730s and is endemic to the national flock. Named after the way infected sheep rub themselves against fences and walls, scrapie can be a devastating disease. The only way to halt the infection is to destroy the flock and abandon use of the infected pasture area. The scrapie agent can lie dormant in the soil for years. But, crucially, scrapie is not transmissible to cows, or to humans, it is species-specific.

In the summer of 1985, however, mad cow clusters started breaking out in other farms and further tissue samples were sent to the Central Veterinary Laboratory in Surrey. In June 1986, a zoo antelope - a nyala, a bovine species - exhibited similar symptoms and its brain revealed the classic vacuoles, or holes, that gave it a sponge-like, or spongiform, appearance under the microscope. The evidence started building up. Sheep and cattle had been sharing the same British fields from the Roman era. Suddenly a sheep TSE had jumped the species barrier. Something was very wrong. Panic set in at the Ministry of Agriculture.

The appearance of what was known as "bovine scrapie" was almost immediately recognised as a potential veterinary catastrophe. "If the disease turned out to be bovine scrapie, it would have severe repercussions for the export trade (in cattle) and possibly also for humans if, for example, it was discovered that humans with spongiform encephalopathies had close association with cattle," wrote Ray Bradley, head of pathology at the Central Veterinary Laboratory, in the first memo about the new disease circulated to senior civil servants in December 1986. "It is for this reason I have classified this document confidential."

Bradley's memo set the tone for the government's official response for the next decade as civil servants and ministers waged a propaganda war to convince themselves and an increasingly sceptical population that British beef was safe to eat. The then agriculture minister John Gummer fed his four-year-old daughter Cordelia a hamburger on primetime television in May 1990 and various tame scientific committees met to endorse the claim that "there is no evidence that the bovine disease is transmissible to humans". Ministers kept repeating that the new BSE disease was just like scrapie - and not infectious to humans - despite the evidence of cross-transmission to the nyala, domestic cats and other zoo animals such as tigers. In December 1995, just four months before the link between BSE and vCJD was finally acknowledged, the then prime minister John Major wrote to the mother of a vCJD victim: "I should make it clear that humans do not get mad cow disease."

Major's brazen denial was one in a long line of institutional lies, self-justifications, omissions and contortions of the growing scientific evidence of the risk to the human population from mad cow disease. Driven by short-term political expediency, each and every arm of the British state, from civil servants in the Ministry of Agriculture to ministers in the Department of Health and 10 Downing Street, colluded in a conspiracy of denial. Telling the truth even about potential risks of infection, they reasoned, would cause "panic" and hit the meat trade at home and abroad. For two decades, Britain's rulers ignored and minimised the real health risk of this deadly TSE disease to millions of their own citizens.

In the meantime, BSE-infected cattle were flooding into the human food supply as the level of infection spiralled exponentially from the first Stent farm cluster. In 1986, two herds were believed to be infected. In 1987, there were 95 confirmed cases of BSE. The figure leaped to 2,296 in 1988, to 10,091 in 1989 and then to 24,396 in 1990. By 1996, there were 200,000 confirmed cases.

Measures were introduced banning the feeding of ruminant animal protein from cows to other cows. In 1989, the Specified Bovine Offal ban required slaughterhouses to remove from the human food chain the most infectious parts of the cow carcass - the spinal cord and the brain. Finally, in 1996, the British government banned all animal-rendered protein from being fed to farm animals and ordered the mass slaughter of all cattle over 30 months old, 4.7 million beasts, in a bid to remove BSE infection from the 10 million-strong national herd.

But the measures were almost always too little, too late. Because of the long incubation period in cattle, up to five years, large numbers of apparently healthy but infectious animals were fed to the British population as pies, sausages, steaks and Sunday roasts. In total, an estimated 750,000 BSE-infected cattle entered the British food supply.

How many people are infected? It is clear even from Queniborough that not everyone who eats a BSE-infected meal will develop mad cow disease. Some humans, because of their genetic make-up, may be immune. The geographical pattern of vCJD cases, heavily skewed towards industrial regions, seems to point towards diet, and heavy consumption of processed meat products such as pies, as a contributory factor. But it is also possible that different human genotypes merely incubate the rogue prion at different rates. What is certain is that most of Britain's 60m population have been exposed to the BSE agent.

As late as 1995, British slaughterhouses were still allowed to "mechanically recover meat", even though the end product was almost certain to contain the most dangerous BSE-infected parts of the cow. After a carcass was stripped by hand, the bones and spinal column, were fed into an industrial pressure cooker. The result was a reddish sludge known as mechanically recovered meat (MRM) which was classified as real meat and added to baby food, hamburgers, pies and sausages to bulk out the beef content. In theory, the highly infectious spinal cord of the cow had been stripped out of the bovine column before MRM recovery. In reality, as various members of the government were aware in 1990, the spinal column was still likely to contain fragments of lethal spinal cord and be drenched in cord particles from the butchering process. It's hard to think of a better way to spread the BSE agent around in human foodstuffs. The food industry itself is still refusing to disclose how much MRM was fed into the human food chain and what sort of products contained the meat substitute.

Queniborough lies in the eastern hinterlands of Leicester. In the late 1960s and early 1970s, there was a large wave of Asian immigration from India and Kenya which transformed the racial make-up of the city; a third of the population is British-Asian. But Queniborough, and its pastoral vistas, remain remarkably untouched by this social change. All five victims were white and lived within a three-mile radius of Queniborough for most of their lives. Most of them knew each other by sight or by name. None had undergone major surgery or blood transfusions. Nor had any of them received growth hormone injections. Their clean medical records ruled out the iatrogenic - medical - contamination which had resulted in a worldwide spate of CJD cases in the 1980s among height-stunted children who had received infected growth hormone pooled from the pituitary glands of human corpses.

The victims' age range - Stacey, the youngest, was 18 and Glen Day, the oldest, was 35 - meant that they were unlikely to have received the same batch of childhood vaccinations, such as polio, that had utilised bovine serum, ruling out another potential route of BSE infection. They went to different local schools at different times and so could not have been infected by eating the same school meals.

Puzzlingly, all of the families were low-income. Stacey's mother, Lorraine, who is disabled, lived on benefits. Arthur Beyless was a milkman. Christopher Reeve's father, Tony, was a farm labourer with six children. Christopher, who was born in 1976 and died in October 2000, was the only victim to work directly with cattle. From the age of 12, he would bunk off school to Barrowcliffe farm, about a mile from Queniborough, to work for William Raynes, the farmer. When he left school, the Raynes, who farm beef cattle, trained him as a cattleman and even sent him to college one day a week. But Christopher was no student and soon dropped out. He took another job, as a tractor driver, and was a familiar sight, buzzing around the village on a noisy moped.

Glen Day, who died in October 1998 - the same month as Pamela and Stacey - was a van driver. A lanky six-footer, he was a quiet man who is best remembered in the Britannia pub for his love of dogs; he would regularly buy other drinkers' dogs packets of crisps as a treat.

None of the Queniborough victims travelled far in life. But then neither did the BSE agent that killed them. Somewhere in the lush pastures of the Wreake and Soar river valleys, it incubated itself in the body of a cow or cows and ended up on the butcher's slab at Queniborough. No one knows how the BSE agent entered their bodies or how it passed through the gut wall. The most likely transmission route may be little areas on the intestinal wall known as Peyer's patches which are connected to the body's nervous system. Once inside the body, the rogue prions incubate, replicate and accumulate in the spleen, lymph nodes, the tonsils, the spinal cord and the brain.

Of all the victims, Pamela was the most adventurous. She left school at 16, in 1991, and moved to Southampton, 170 miles away. Back at home, Arthur Beyless keeps a picture of her 21st birthday party on the wall of his lounge. Pamela, surrounded by friends, is just about to blow out the candle on the cake. By 1996, Pamela was working as an accounts clerk for a bank, processing loan applications. She had to retrieve files and electronically input data. It was fastidious work. Somehow she kept making mistakes, getting it wrong. She started to get warnings at work about her performance. She became forgetful, running up credit card debts and not paying the bill. But the first time Arthur Beyless noticed anything wrong was on the phone. "I phoned her up to thank her for the Father's Day card. She gave this funny laugh. It was a monkey laugh. It was not the way Pam laughed. She would not have laughed, anyway. She would have said: 'That's all right, Dad.' It just stuck in my mind." A month later, Pamela started to have difficulty walking and had to return to her parents' home.

Arthur Beyless also keeps a video of his daughter, which he shows to visiting reporters but which he never watches himself. The video was taken by staff in Leicester Royal Infirmary in November 1996 as part of his daughter's medical tests. Pamela has a worried frown that is, in fact, the first sign of loss of control of her facial muscles. Behind the camera, the neurologist asks her to walk in a straight line but her gait is uncoordinated; her legs shuffle clumsily from side to side and her back is oddly arched. She sits down and the neurologist asks who is the prime minister of Britain. She shakes her head. She no longer knows what day it is or why she is at the hospital.

Pamela's fall, like Stacey's, was swift. By April 1997, she was in a coma. Pamela's body lived on for another year and seven months before succumbing to pneumonia. By then her brain was pitted with microscopic holes, a spongiform encephalopathy, and weighed less than a healthy human brain. There was no salvation for her.

Pamela was not born in Queniborough, but grew up in the suburbs of Leicester. In 1976, when she was two, her mother's brother, Leslie James, was killed in a car crash. He is buried in the churchyard at Queniborough. The family visited the grave every Sunday and afterwards had dinner with her grandparents, Eva and Mitchell James, who lived in the village. It would prove to be a fatal connection.

The James family were friendly with Bramley, who ran the traditional butcher's shop in the village. The family liked Ian and they liked his meat. When the Beylesses moved house, Eva James bought them a chest-style freezer cabinet as a present. And then they started to buy their meat in bulk from Ian Bramley. "It was not processed meat," says Arthur Beyless, still haunted by the knowledge that his beloved daughter was probably infected at the family dinner table. "It was all joints, steaks, chops, legs and shin. We'd order a quarter of a cow three or four times a year. I'd phone up and a few days later go round. It would all be boxed up ready. Pamela liked her meat but she ate the same meals as the rest of us. Basically she ate meat and two vegetables. She ate a lot of fast foods as youngsters do; burgers, doner kebab, pizza, and fish and chips. The only different thing she ate was saveloys."

Bramley was not the last small butcher in England. Across Britain, there would have been thousands of them. In the neighbouring village of Rearsby, a couple of miles from Queniborough, another butcher, John Smith, butchered his cattle as Bramley did. Christopher Reeve's mother shopped for her meat at Smith's. Smith even killed the cows himself. Every week, he would go to the local cattle markets and pick out four or five cows. The killing would take place in a small abattoir at the back of the shop. First the butcher would use a captive bolt stunner that punches a hole in the cow's skull and knocks it unconscious. Dying cattle still kick, so to protect the slaughter team, Smith took a long coiled steel spring, called a pithing rod, and stuck it through the hole down into the spinal column. "Pithing", by knocking out the brain's electrical centres, foreshortens the animal's death spasms and increases the blood flow to the severed neck arteries. The pithing rod would now be saturated in sticky brain matter and Smith would wipe it clean with a cloth.

After the killing, the slaughtermen would begin the butchery, starting with the head - cutting out the tongue, the tough cheek meat and the brains. Some of the older villagers still liked brains, a cheap cut of meat dating back to wartime rationing. "You dip them in hot water, then cold water, roll them in flour, fry with butter and serve with parsley sauce. Delicious," says Queniborough village historian Rosemary Smith.

Fried brains may have been a quaint delicacy but unbeknown to the villagers of Queniborough, it was the most fatal feast. The rogue prion accumulates in the brain and the spinal cord of infected animals, making those organs the most potentially infectious body parts. One of the initial puzzles of kuru had been the sexual imbalance of victims - far more women and children than adult men were afflicted. The riddle was solved only when it was discovered that Fore men left the soft body organs and the brains to the women.

Bramley used a small abattoir in Leicester and had the carcass and the bovine heads delivered directly to his Queniborough shop for butchering. Getting at those cattle brains was a messy business for both butchers. First they would take a cleaver, hack off the lower jaw to get a nice flat surface on the wooden butcher's block, then chop their way into the thick bovine skull. Brains are a blood-rich organ and the material would get splattered everywhere. After scooping out the brains, which weigh about 2lb, the butchers would wipe the block clean and start on the rest of the carcass. By then traces of brain material would have contaminated the butchers' knives, hands, clothes, the wiping cloths, the cutting block and other parts of the carcass.

None of the vCJD families ate brains but the use of the two traditional butchers by four of the five families was, according to Monk, the key link in the Queniborough cluster. "Relatives reported that the people with vCJD were 15 times more likely to have purchased and consumed beef from a butcher who removed the brain from a beast, compared with controls who purchased meat from outlets where cross-contamination with brain material was not a risk."

New EU hygiene regulations meant that small slaughterhouses such as Smith's Rearsby shop were going out of fashion. Slaughterhouses were getting bigger and bigger, and everything was becoming more mechanised. In comparison, Parkers, the main slaughterhouse in Leicester, killed 1,000 cattle and 5,000 sheep a week on an automated production line. Parkers was a killing factory in which every aspect of the slaughter business was compartmentalised. Skulls were dealt with in a separate area from the main production line. The risks of brain/meat cross-contamination were lower. Meat was shipped out in plastic shrink-wrappers and supplied to butchers who did little more than lay it out on trays like a supermarket shelf.

The other crucial factor was the cattle themselves. Queniborough is mainly dairy country. The local herds are Friesian , prized for their high milk yield. The local beef cattle trade was an offshoot. Male Friesian calves would be weaned from their mothers at six days, then fed protein supplements including what later turned out to be BSE-infected meat and bone meal feed. These calves were therefore potentially exposed to the infectious agent for longer than other beef cattle who would normally milk from their mother until they were six months old. Friesians are also slower to fatten, taking between 30 and 36 months to reach their prime weight. The combination of the early potential exposure and longer life span meant the meat trade in Leicestershire was rearing cattle that were more likely to be incubating BSE, and whose meat was a greater danger to humans.

Officially, after nine months on the case, the investigators have blamed the Queniborough outbreak on those local butchering practices back in the 1980s. The cluster may have originated in just two or three infected cattle, cut up, contaminated and sold in the two local butchers' shops. "The important thing we are saying is that it is not just a single factor that was the issue here. The cattle had to have the BSE agent when slaughtered and they had to have the brains removed or damaged for cross-contamination to occur," Bryant told a meeting of villagers. Based on their findings, it was "extremely unlikely" that there would be a large-scale epidemic.

BSE is probably the most cynical act of biological warfare ever waged against a civilian population by a western government. The British government put the narrow business interests of its farming and meat industries before the health of its own population, and that of other countries. MBM was identified as the source of the infection as early as 1988 and its use as cattle feed was banned in Britain. But that did not stop British exporters, with the blessing of the British government, selling 56,000 tonnes of their lethal product to their fellow Europeans over the next six years.

"Those people don't have any remorse for the people they have killed. I've met Stephen Dorrell, he was the health minister, who finally said, in March 1996, that BSE does cause vCJD. He's my local MP. He never said a dicky bird about being sorry. And he never will," Arthur Beyless says.

Through the export of BSE-infected cattle or contaminated feed, Britain managed to export mad cow disease to most of continental Europe, including France, Germany, Switzerland, Italy, Belgium, Portugal and Denmark. Some of that contaminated feed was then re-exported to Asia and Latin America, creating the risk of a global epidemic. Only the US remains theoretically immune.

Potentially, the Queniborough cluster study answered that other vital question - the incubation period. Working backwards from the "craft butcher hypothesis", the medical investigators were able, they believe, to narrow down the minimum incubation period to between 10 and 16 years. Their calculations were based on the ages of the victims and the two butchers' shops. Bramley, the Queniborough butcher, died in June 1982. His successor, David Clarke, took over in December 1982 and never sold brains. Smith, the Rearsby butcher, stopped trading in 1989. The arithmetic is simple. Pamela Beyless was eight years old when her father stopped buying meat at Bramley's. Sixteen years later, she died of vCJD. The fifth, unidentified victim was 19 when he died in 1999, 10 years after John Smith shut up shop.

It all sounds very plausible, but is it? Not according to some highly distinguished critics. "They have no idea, it is just guesswork, speculation. The aim is to reassure, rather than get at the truth," says Professor Richard Lacey, a dissident medical microbiologist who first alerted the British public to the link between BSE and vCJD. "It's the same as the whole basis of CJD over the last 15 years - not to get at the truth, but to reassure in the short term. You cannot do research on the association of cause and effect unless you know what the cause is. We do not yet know the cause of vCJD. Is it meat, milk, inhalation from cattle, maternal transmission? We haven't the faintest idea. And there is no explanation as to why so many young people are infected."

There is harsher criticism from some in Queniborough. "It's just another whitewash," says Mike Winterton, a grazer who selected cattle for slaughter for Smith. "They are blaming it all on one dead butcher and one retired butcher. No one from the health authority or the civil service is responsible."

Certainly the medical team's investigation provokes as many questions as it answers. How could Stacey Robinson, who was born in November 1979, be exposed to BSE-contaminated meat from Bramley's shop when he died in June 1982 and she was just 30 months old? What about the villagers who ate brains? And where did these BSE-infected cattle come from in 1982, when Cow 142 did not appear for another two years, in a farm hundreds of miles away, and the first confirmed case of BSE in Leicestershire was in 1988?

If both butchers were supplying contaminated meat to the village population, why are all the victims from lower income families? Why have no rich villagers also succumbed? Is the real link actually just cheap products, such as pies and sausages, which are far more likely to contain mechanically recovered meat? Some of the families also bought food in the local branches of Britain's two biggest retail supermarket chains. Arthur Beyless worked for the biggest, Tesco, with millions of customers, and filled the rest of his freezer with free beyond-sell-by-date meat pies. Has the entire British food industry been contaminated? Is mechanically recovered meat the human equivalent of the meat and bone meal that infected cows? Is the Queniborough cluster, like the Stent Farm cluster in cows, just the first real outbreak of a massive wave of mad cow infection in the British population?

The true answer is that no one knows. "We know the pattern of the BSE epidemic we saw in cows and we can back-calculate to the 1970s," Monk says. "There is likely to be some mirroring of that in the human epidemic, but we don't know where on that curve we are. Are we right at the beginning or at a later stage? We don't know where in the mirroring we are."

Ten years might be a plausible minimum incubation period, but what is the maximum incubation time? Part of the uncertainty lies in our last genetic defence against the BSE agent, the species barrier. BSE takes up to five years to kill a cow. But every time the rogue prion agent in BSE jumps a species, it mutates and the average incubation period lengthens. BSE cow prions take longer to turn into vCJD human prions and thus take longer to kill the human victim. Kuru can take at least 28 years to kill humans. How long will vCJD take? The answer will finally come not from the laboratory but from the trembling minds and bodies of the British population in 2010, maybe as late as 2020.

One thing is sure. All the talk about genotypes and epidemiological controls has not quelled the real sense of fear in the village of the damned. "I spend every day of my life worrying about it," Simon Robinson says. I saw what it did to my sister. We sat down at the same table, we ate the same meals. If I get the slightest headache - anything like that - I start worrying. I'm scared for my family. I'm especially scared for Josh. What is going to happen to me, my mum and my nephew? I feel fear for myself."

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