Prions
Mad Cow Home or Best Links

Chicken-related CJD cluster documented in Arkansas
BSE Sets Agenda for Imported Gelatin
EU to discuss meat rule exemption for U.S. Sept. 8 EU weighs sparing US, others food law EU meat safety rules: trade fight with U.S.? CJD from liver or albumin: your choice
Can nvCJD be shown dietary? CJD victim's wife calls for public inquiry
EU official ordered to retire after claims of withholding data
BSE Surveillance In The United States: An Update

Chicken-related CJD cluster documented in Arkansas

5/12/95 Account of X-Files episode 2-24
FBI Special Agents Dana Scully and Fox Mulder investigate the notorious kuru-type outbreak of 1995 in Dudley, Arkansas associated with a chicken-processing plant whose motto is, "Good People, Good Food." Some 27 people get the disease at a town potluck from eating a missing poultry inspector, George Kearns, who had CJD.

Another poultry worker is shot after she goes insane, giving Mulder a hunch that the townfolk really are what they eat. But the deeper they dig into the missing man's connections in the town, the stranger the town begins to look. Unusually long life spans and youthful appearances cause Mulder and Scully to suspect that there may be more to the local chicken-processing industry than meets the eye. Mulder stumbles onto the horrifying secret even as Scully's life is threatened by a masked murderer.

X-files. Our Town .... Original Air Date: 05/12/95 Season 2, Episode 24

Top Swedish X-File site ... Disgusting film clip ... Grotesque head shot

It's almost midnight...Aren't you sleepy yet?

Episode review by Sarah Stegall 
Mulder and Scully are assigned to a routine missing- persons case which catches Mulder's eye because of the association with so-called foxfire phenomena: the Ozark lights which spirit away travellers and leave large scorch marks. Once on the scene, however, it becomes clear that something more ominous is going on. More than 100 people have disappeared from the area within the last 50 years. Suspicion centers on the town's chicken-processing plant, whose motto, "Good People, Good Food", looks more sinister with every passing moment. The employees are developing a brain disorder so rare that the odds of two people in the same town having it are incalculable. Reluctantly, and to Mulder's delight, Scully develops a "sick theory": their missing man was recycled into the local chicken-feed operation, and his disease is now loose in the chicken-eating population. As it turns out, the answer is even worse than that: he wound up as the main course at the town potluck.

There are very few "universal" taboos in human cultures: incest and cannibalism come to mind. Both address the purity of the group, either moral or physical. While some cultures can accept incest among members of a privileged group--such as the ancient Egyptians-- they forbid it among the general population. NO culture endorses the indiscriminate eating of one another for food. From the myth of Atreus, who served his brother the stewed bones of his own sons, through the horrific tales of the Donner Party and Alferd Packer, to Jeffrey Dahmer, the taboo against eating human flesh runs deep and strong. Even cannibal cultures enforce the prohibition: you *never* eat one of your own. The very basis of cannibalism is either the absorption of magical powers (for strength, wisdom, longevity, etc.) or the reinforcing of the distinction between "them" and "us": *we* are people, and therefore not food. Those guys on the other side of the hill, however, are not People, and therefore can be served with croutons. In "Our Town", it is the crossing of this line, not the threat posed by Mulder and Scully, which signals the end for the community. Once the distinction between friend and food breaks down, with the murder and stewing of Doris Kearns, whatever societal norms are built on it must fall, and chaos ensues. This is symbolized in the murder of the paterfamilias, Mr. Chaco (Joe Milford), the ultimate authority figure in the community.

The town's motivation for cannibalism is neither hunger nor solidification of the group identity, despite what the title "Our Town" might imply. The motivation is as old as the knowledge of mortality: the town members are holding off not just death but old age. As Mulder discovers early on, not everyone in this town is what they appear to be (surprise!): the youthful granddaughter of the plant's founder is actually only three years shy of her fiftieth birthday. The old man himself is nearly a century old, yet appears to be no older than sixty. Clearly the fountain of youth has been found, but the price of a drink is pretty stiff. Wisely, writer Frank Spotnitz does not "explain" their success in staying young through either a magical or biochemical explanation; to inquire too closely into this premise would snap our fragile suspension of disbelief.

Director Rob Bowman paces the episode well, with some snappy dialogue and some excellent cinematography. I particularly liked the scene in the X-files office, where Scully's face is reflected in the video monitor where we see an abduction survivor raving about his experience in the woods near Dudley, Arkansas. Scully's skeptical expression is an interesting counterpoint to Mulder's bland acceptance of every word the raving mental patient is spewing. In fact, nearly all of Mulder and Scully's interaction in this episode is strong and effective: I love the scenes when they are discussing the case, tossing theories back and forth, working out the details of this jigsaw puzzle like two halves of the same mind.

Nevertheless, the episode is seriously flawed in several places. Dr. Dana Scully develops superhuman powers, as she manages to extract a substantial slice of brain tissue from a body without the bother of opening the skull. Mulder, who presumably dives to the rescue of a wrecked truck driver, is as dry as a bone in the next scene--does he carry a Ronco dry cleaning kit in the trunk of his car? Since the heads of all the victims are kept in Chaco's souvenir case, it is hard to see how their diseased brain tissue could make it into the stew the town shares, and thereby infect them. In Chaco's interview with Mulder and Scully, the old man indulges in long- winded non-sequiturs that no experienced interrogator would tolerate. And no, the ancient Anasazi did NOT practice cannibalism: what few remains have been discovered are simple burials that do not support this premise. I can only imagine the reference was inserted as a foreshadowing of next week's episode, entitled "Anasazi". If so, it was silly and unnecessary....

Note from webmaster: The above is a spoof or joke. The X-Files are a popular television show -- the actors are not really FBI agents nor is the woman lead an MD pathologist. No CJD cluster has been reported from Arkansas Arkansas nor has any chicken-processing plant there or elsewhere been reported to rendered humans.

Public perceptions are formed in part by how the media represents current events, and public policy is never far behind. That is the reason for coverage of the X-Files episode.

Far more people watch this show and its reruns than ever saw Oprah, read Neurology, or visit this Web site. Many people in the US today exhibit a diminished capacity to distinguish between fictional representations and reality. Once these perceptions are formed, any subsequent attempt to inform or change is viewed as a government plot or industry lie. In fact, things are so bad that disclaimers such as this one are necessary.

BSE Sets Agenda for Imported Gelatin

 by Charles Marwick  JAMA. 1997;277:1659-1660  - June 4, 1997 
THE FALLOUT from "mad cow disease" continues, although incidence of the disease itself has been decreasing steadily since January 1993 (JAMA. 1996;276;438-440). The latest worry is a renewal of the uncertainties surrounding the risk of human exposure to the agent or agents of spongiform encephalopathy in gelatin, which is manufactured from the bones and hides of cows and pigs.

The concern has prompted a Food and Drug Administration (FDA) committee to advise the agency to reimpose a restriction on the use of gelatin manufactured from parts of cows imported from countries that have reported cases of bovine spongiform encephalopathy (BSE). Humans are exposed to a wide variety of products that contain or use gelatin, from sausage casings and jellies to medicinal capsules and at least 1 vaccine.

In January, for example, Italian health authorities suspended distribution of a Haemophilus influenzae b conjugate vaccine (named HibTITER, manufactured by Wyeth-Lederle Vaccines and Pediatrics, Radnor, Pa) because the nutrient on which the organisms are cultivated contains bovine brain and heart, hence a possibility of contamination with BSE. The step was purely precautionary. No specific risk is known. At the time, the FDA issued a statement saying the agency was convinced that the vaccine is safe.

The Gelatin Exemption

In 1994, the FDA allowed bovine-derived materials to be imported for the manufacture of pharmaceutical grade gelatin from countries that reported cases of BSE. The decision relaxed a position the agency had taken the previous year, namely, that bovine materials from countries with BSE should not be used in FDA-regulated products--the so-called gelatin exemption.

"The decision reflected a conclusion that the available evidence did not suggest transmission of BSE via gelatin and was based on an assessment that the manufacturing conditions were likely to inactivate the infectious agent," said David Asher, MD, PhD, chief of the FDA's Laboratory of Method Development, Center for Biologics Evaluation and Research, addressing a meeting last month of the agency's Transmissible Spongiform Encephalopathies Advisory Committee.

In light of more recent events, the FDA decided to reconsider the gelatin exemption and other issues relevant to BSE, Asher said. The most dramatic of these events was the report last year of a new variant of Creutzfeldt-Jakob disease (CJD), a human disorder akin to BSE, found in 15 persons in Great Britain and 1 in France. A previous report of feline spongiform encephalopathy in Britain also suggested that the so-called species barrier was not absolute. In addition, Asher said, the FDA has not been provided with scientific evidence that the processing of gelatin removes all infectivity from the original materials; and finally, there was concern that some source material for gelatin--particularly imported gelatin--may contain bovine neural tissue.

Since 1986, when BSE was first reported in Britain, more than 165,000 cattle from about 33,000 herds have been diagnosed with the disease. It has been reported in cattle in France, Switzerland, Portugal, the Republic of Ireland, Northern Ireland, and the Netherlands, said Kiki Hellman, PhD, a senior scientist at the FDA's Center for Devices and Radiological Health. The disease has not been detected in cattle in the United States. The US Department of Agriculture (USDA) has an ongoing program of histopathologic examination of specimens of central nervous system tissue from US cattle that exhibit unusual neurologic symptoms, and no cases of BSE have been found. Since 1989, no cattle have been imported into the United States from the USDA's list of BSE-designated countries.

Evidence and Recommendation

For a day and a half, the committee listened to gelatin manufacturers describe the sources of the raw materials and processes by which gelatin is made. What the committee heard included data from a study that allegedly validated that neural tissues were removed from the source materials during gelatin manufacture; a summary of a March 1997 recommendation by the World Health Organization stating that bovine sources for preparing medicinal products and devices should be avoided; and, finally, a directive from the European Community Commission that banned "all high-risk materials of bovine and ovine origin" in the manufacture of pharmaceuticals regardless of their source, not just animals from countries reporting BSE.

After pondering the possibility that gelatin, manufactured from bones obtained from cows possibly exposed to BSE, could be infected with the disease agent and so trigger a spongiform disease in humans, the majority of the members concluded that the process of making gelatin does not fully inactivate the agent. It was, as one committee member put it, "a decision in the face of uncertainty." At the same time, said William Hueston, DVM, PhD, associate dean of Virginia-Maryland Regional College of Veterinary Medicine, College Park, Md, "there is no evidence to date of transmission of spongiform encephalopathy to animals or humans via gelatin."

The committee agreed to recommend that the agency adopt a risk-based approach based on the source and categories of use. Hueston laid it out for his fellow committee members: There is no concern about gelatin produced in the United States from US sources; there is a concern about gelatin produced from foreign sources. But, he added, "from the presentations we have heard it would appear that there are currently in place regulations that limit the introduction of the raw material into the United States, and this includes no material coming from countries that currently report cases of BSE." Finally, he said, there is concern about gelatin produced in other countries and imported into the United States.

So, he said, what must be considered is the BSE status of the country of origin of the source materials, the effectiveness of surveillance, and what the source materials are and the methods by which they are processed and put to use.

Gradation of Risk

From the reports presented at the meeting, Hueston said, it was evident that bovine source materials carry a higher risk than do porcine source materials; that bones, particularly skulls and backbones, are greater sources of risk than are hides; that alkaline processing procedures are more effective in terms of safety than is the acid-extraction process; and that the risk for users descends in severity from parenteral products to oral products to industrial products.

With nods of approval around the table, committee chair Paul W. Brown, MD, medical director of the National Institute of Neurological Disorders and Stroke, Bethesda, Md, said, "We could recommend that if any restrictions are put in place they be based on this type of risk assessment."

At the end of the meeting, Brown polled committee members for their response to a question put by the FDA: Does current scientific evidence justify continuing to exempt gelatin from the restrictions recommended by the FDA for other bovine-derived materials from BSE countries?

Of the members present and eligible to vote, 11 of 15 replied: "No," many of them enlarging on their response. For example, Lawrence B. Schonberger, MD, MPH, assistant director for public health at the Centers for Disease Control and Prevention, Atlanta, Ga, said that while gelatin was most likely safe, "the data on the risk of exposure were insufficient, particularly in light of the large numbers of people who are being exposed." Sidney M. Wolfe, MD, director of the Public Citizen Health Research Group, Washington, DC, said, "We're more worried now about the crossing of the species barrier; there is evidence of residual neural tissue in the materials used for gelatin, and we have no guarantee that the infectivity has been removed."

EU to discuss meat rule exemption for U.S. Sept. 8

August 8, 1997
Dow Jones News
BRUSSELS -- An E.U. official was cited as saying Friday that The E.U. Multidisciplinary Committee on Bovine Spongiform Encephalopathy will consider Sept. 8 whether U.S. tallow producers should be exempted from E.U. meat processing rules that could block $100 million a year of U.S. tallow exports.

The story notes that tallow is generally made from whole cattle carcasses. Tallow derivatives are used in an estimated 80% of pharmaceuticals, as well as in a range of other products including cosmetics and lubricants. The U.S. has threatened to complain to the World Trade Organization if forced to change slaughterhouse practices in order to safeguard U.S. tallow exports to the E.U. One third of E.U. tallow supplies - or about 500,000 metric tons a year - comes from non-E.U. countries.

At least four countries - the U.S., Canada, Australia New Zealand - have formally requested TSE-free status from the Commission. But the U.S. isn't expected to get it because it has had scrapie. [Canada also has scrapie and a single case of BSE in a bull imported from the UK -- webmaster]

EU meat safety rules could start trade fight with U.S.

August 6, 1997
Dow Jones News
BRUSSELS -- The U.K. Financial Times is cited in this story as reporting on Wednesday that new European Union meat safety rules, meant to prevent the spread of bovine spongiform encephalopathy, could provoke a trade war with the U.S.

The story notes that the forthcoming E.U. ban on the use, for any purpose, of specified risk material, including the head and spine of all cattle, sheep and goats more than a year old of cattle, sheep and goats at risk of harboring BSE. The ban will also affect uses such as tallow and derivatives, which the story says are used in about 80% of pharmaceuticals, as well as in cosmetics.

The U.S. has said the ban will affect $100 million of U.S. exports to the E.U. unless it brings its slaughterhouse rules in line with the E.U.'s, and, the story says, has threatened to complain to the World Trade Organization about the ban.

EU weighs sparing US, others food law

Reuters Business Report By Janet McEvoy
Fri, Aug 8, 1997
BRUSSELS, Belgium - The European Commission, facing a trans-Atlantic trade dispute, is studying whether to exempt the United States from new meat safety rules that will ban the use of tallow and other animal byproducts. A European Union source said Friday the group's executive commission was examining a request from Washington, along with similar demands from Australia, New Zealand and Canada, to be spared the new law because they were free of so-called mad cow disease.

The requests will be considered in September by the EU's Scientific Veterinary Committee, which will at the same time consider tightening up the rules, the source said. Earlier a spokeswoman for U.S. Trade Representative Charlene Barshefsky said Washington could take the 15-nation bloc to the World Trade Organization over the new rule, which the EU plans to implement on Jan. 1.

     "We have expressed our concern to the EU concerning the directive,"
the spokeswoman said in Washington. "If they don't change the directive,
we'll take them to the WTO."
She said the new rules -- aimed at controlling the spread of mad cow disease by banning the parts of cattle most likely to carry the disease >from entering the food chain -- would have a "severe negative impact" on U.S. exports. In addition to affecting beef tallow producers, the directive could affect pharmaceutical and cosmetics companies since tallow -- fat extracted >from cows, sheep and other animals -- is used in drugs, soaps, candles and other products.

To be exempted from the law, the EU source said, the United States would have to prove there was little risk of mad cow disease in its beef products but also other diseases.

The September meeting of the EU scientific committee will also consider fine-tuning the EU law, which was agreed to by EU farm ministers on July 23, the source said. That will involve looking at the kind of tissues included in the directive, and examining whether the age of the cattle covered, currently standing at 12 months and more, should be lowered, he said.

The EU source said that the bloc did not want to worry people about the safety of cosmetics, but that it was erring on the safe side as a potential risk had been identified. Last year the mad cow disease scare blew up when British health officials announced a possible connection between the disease and a similar human brain disease.

CJD victim's wife calls for public inquiry

 PA (PA News)
 Thu, Aug 7, 1997  By Matthew Cooper, PA News
The wife of an engineer who died from the new strain of Creutzfeldt Jakob Disease - the human form of mad cow disease - has called for a public inquiry into the BSE crisis. Keith Humphreys, 42, of Birmingham, died of the new variant of CJD on July 15, his widow Carol disclosed today.

The 37-year-old nurse, who lives in the Northfield area of the city, said:

"I am not bitter but I do feel that the public have a right to be informed about the dangers of contaminated beef. "Farmers are being compensated for the loss of cattle but people who have lost loved ones are not. It does not seem fair."
The death certificate on Keith, an engineer with Travel West Midlands, states that his death was caused by "Pneumonia as a result of New Variant CJD."

Carol, who said her husband died a slow and painful death, added:

"There are no words of comfort anybody can offer me. I had to watch somebody I love turn from a man who was always the life and soul of the party into somebody who was in constant pain and torment."
Mrs Humphreys said too little was being done to help CJD victims' families, adding that she believed Keith contracted the disease by eating beef between 1986 and 1989. Mr Humphreys family began to notice something was wrong in the summer of 1995 when he became very forgetful. In March last year a specialist told Mrs Humphreys her husband had CJD and had six to eight months to live.
"I just think a public inquiry is the only way forward - it's the only sensible thing that can happen because things can't be left as they are now."

The American Association of Vet Lab Diagnosticians has posted the Abstracts of the scientific program for the 40th AAVLD annual meeting for October 12, 1997.

BSE Surveillance In The United States: An Update.

 7 Aug 97 A.L. Jenny, A.J. Davis, W.D. Taylor, L.D. Miller, M. Fekadu.
Active surveillance for evidence of bovine spongiform encephalopathy (BSE) in the United States continues with collection of central nervous system tissue specimens from three groups of cattle. These groups include adult domestic cattle with clinical signs of nervous system disease, adult domestic cattle with recumbency as the primary clinical sign ("downers"), and imported cattle from the United Kingdom.

Samples from cattle with clinical signs of nervous system disease are generally received from three sources: state veterinary diagnostic laboratories, state public health (rabies) laboratories, and U.S. Department of Agriculture (USDA) field veterinary medical officers (VMOs). Samples from recumbent cattle are generally submitted by USDA field VMOs from selected slaughter plants. As the United Kingdom imports die or are euthanized, specimens are collected and forwarded to the National Veterinary Services Laboratories (NVSL) by state or Federal VMOs.

These samples are routinely examined by histopathology either at the NVSL or at state veterinary diagnostic laboratories. Selected samples are further tested with an immunohistochemical test routinely used at the NVSL in the detection of scrapie prion protein. In this report, laboratory results and statistics of histopathological and immunohistochemical testing of these central nervous system tissue specimens are discussed.

EU official ordered to retire after claims of withholding data

August 7, 1997
Nature
Munich -- The European Commission's deputy director-general for agriculture with responsibility for veterinary affairs, Fernando Mansito Caballero, has, according to this story, been ordered to take early retirement, five months after accusations that he deliberately withheld from the public information about the dangers of bovine spongiform encephalopathy (BSE) in 1990.

The move is widely believed to result from pressure from the European Parliament, which had accused the commission of mismanaging the BSE affair and demanded an investigation within the commission. Mansito allegedly said in 1990 that BSE should be discussed behind closed doors to avoid inciting public alarm.

However, the commission is cited as denying that Mansito's forced resignation is related to these accusations.

CJD from liver or albumin: your choice

Mon, 11 Aug 1997 This study might have relevence to CJD transmission through blood transfusion and/or to what parts of cow are safe to eat. BSE liver and plasma were tested of course, but at what titre-sensitivity? There is no proof in the correlation below since every CJD victim has a medical history of some sort. They didn't start with the albumin donor and do forward statistics on recipients. Still, CJD is a very rare disease and the connection to an albumin donor is of concern. -- webmaster

CJD after CJD from liver or albumin: your choice">liver transplantation

Ann Neurol 1995 Aug;38(2):269-272
Creange A, Gray F, Cesaro P, ..., Parchi P, Gambetti P, Degos JD
We report a 57-year-old woman who died from Creutzfeldt-Jakob disease 2 years after a liver transplantation. The liver donor had no history of neurological disease. In one albumin donor, possible Creutzfeldt-Jakob disease developed 3 years later. The patient initially had cerebellar symptoms. Neuropathology included "Kuru-type" plaques and prion protein (PrP) deposits involving the cerebellum predominantly. The patient was homozygote valine at codon 129 of the PrP gene while the liver was homozygote methionine. This observation raises the possibility of transmission of Creutzfeldt-Jakob disease by the graft itself or the associated albumin transfusions and, on a wider extent, by nonneural tissue.

Can nvCJD be shown dietary?

July 19, 1997 Lancet articles, Collinge, J et al.
1. The authors looked at two kuru brains of unspecified origin and age, unfortunately both129 val/val, and compared them to two nvCJD (necessarily met/met at this point), the idea being that the florid plaques and so on of nvCJD might be characteristic of a dietary source and so be similar to kuru. There were some underwhelming similarities.

2. In the following paper they looked at 5 UK farmers who had died of CJD diagnosed as sporadic but sometimes cited as occupational nv CJD. Here all 5 were very clearly in the region of type 1 and 2 region of Collinge's strain-typing gel, saying not nvCJD.

Mad Cow Home or Best Links