Prion Disease
Mad Cow Home ... Best Links

Renewed fears for baby of CJD mother
Irish farmer jailed for mad cow fraud
Blood: infectivity of buffy coat in new variant CJD
Imported BSE cases
Global BSE infection: it's official
Fish farmers get mad cow warning
Sick, hungry cows taken to market on way to incinerator
French officials to begin mad cow screening program
Eighth annual report of CJD Surveillance Unit
High risk tissues finally to be removed
Mad Cow Remains Fuel Power Station
US eye surgeons reuse blades

Renewed fears for baby of CJD mother

June 4 2000 Sunday Times Jonathon Leake
A WOMAN who became ill with variant CJD while pregnant has died - leaving behind the mystery of whether she has passed the disease to her child, writes Jonathan Leake. The 24-year-old Midlands woman, who had worked in catering, died last week. She had contracted pneumonia after becoming bedridden.

This weekend the dead woman's family gathered at their home near Nottingham in preparation for the funeral early this week. Her daughter, now seven months old and suffering from a degenerative brain disease, is being cared for by her maternal grandparents. Neither the baby nor her family can be identified for legal reasons.

The woman's mother spoke yesterday of her anger at her daughter's death, which she blamed on the "greed of the agricultural industry and the incompetence of officials and ministers in the last Conservative government". She has started a compensation claim on behalf of the child.

Doctors have spent the past few days conducting tests on the dead woman for any link to her child's illness. All that can be said with certainty is that the baby's symptoms have similarities to those found in new variant CJD.

Doctors have not, however, been able to find any sign of the so-called prion protein particles that are widely believed to be the cause of new variant CJD.

If the investigation finds she was infected by her mother, it would mean generations of children as yet unborn could be affected. There is evidence that prion disease can transmit from mother to offspring in sheep and cattle, possibly during pregnancy, birth or through suckling.

The dead woman's mother - grandmother to the child - said: "This disease would never have happened but for the disgusting practice of turning cows into cannibals and it would never have spread among humans if the agriculture ministry had heeded its own scientists' warnings when the disease first appeared in cows."

Magnetic resonance images taken of the youngster's brain have shown strong resemblances to those found of adult victims of variant CJD. There have also been similarities in various other diagnostic features. Doctors have not, however, been able to find any sign of the infective prion particles which are widely believed to be the cause of variant CJD. This is despite carrying out a whole battery of tests on the child, including microscopic examination of potentially infective tissue Doctors treating the mother and child have told the family that they believe the youngster's condition was, at the very least, caused by her mother's illness..

The implications of the investigation are huge. Almost everyone in Britain is thought to have eaten bovine material infected with the prion agent believed to cause CJD. About 750,000 infected animals entered the human food chain before 1996 when new rules came in. The potential size of a human epidemic remains, however, very uncertain because there is still no reliable test of who is incubating the disease.

Professor John Collinge of Imperial College London, head of the Medical Research Council's prion unit, believes that "it is logical to conclude that many more cases must result from the dietary exposure." If Collinge, who is also leading the investigation into the baby's condition, finds she was infected by her mother, it would open a whole new infective pathway and mean that generations of children as yet unborn could be at risk. There is some evidence that prion disease can transmit from mother to offspring in sheep and cattle, possibly during pregnancy, birth or through suckling.

Frances Hall, a spokesman for the BSE foundation which represents victims and their families, said her thoughts were with the woman's family. "It is terrible enough for a family to have one victim of this disease but for two to be damaged is beyond belief. We are all still waiting for the final results of the tests to see if it could have passed to the child. If so it will be very worrying."

One of the complicating factors is that all the 70-odd definite or probable victims share a particular genetic make-up, having two molecules called methionine at a particular position on the gene responsible for making prions. About 38% of Britons have similar genes. The baby, however, is understood to have a different genetic make-up, with a molecule called valine in place of one of the methionines. This configuration is found in about half the British population.

Doctors at the Medical Research Council's prion research unit at Queen may Hospital in London have speculated that the illness could show very different symptoms in such people and may only be confirmed by a post-mortem.

Other experts have, however, suggested that the child's illness is not related to her mother's and that she is simply one of a small number of children born every year with terrible and inexplicable handicaps. CJD and its animal equivalents such as BSE in cattle and scrapie in sheep are thought to be caused when naturally occurring proteins known as prions undergo a change in shape that alters their behaviour and makes them attack brain tissue.

In BSE and variant CJD the change in shape is initiated simply by contact with abnormal prions which can enter the body via contaminated food. Humans are thought to have acquired variant CJD be eating one of the 750,000 cows infected with BSE that entered the human British food chain before 1996.

In cows and sheep, however, scientists have found that infection may also pass down the generations - possibly from mothers to their offspring. It is not known whether this happens during pregnancy, birth, suckling or by some other means.

In Britain only one other woman has been known to give birth to a child while ill with new variant CJD and that youngster, a boy now aged five, remains completely healthy.

[Comment (webmaster): The number of babies born each year in the UK with undiagnosable neurological disease to healthy young mothers is very small, perhaps one in 5,000. Advanced prion disease in the mother might very well cause a loss of normal prion function or secondary brain or placental dysfunction that could adversely affect the baby without transmitted prion disease being responsible. The baby could survive the initial problem only to succumb to nvCJD at a later date. Distinctions between direct and indirect disease are in many ways moot to the families that must bear the burden of the disease.

Medline has 8 articles on 'prion and placenta'. This tissue has one of the highest levels of expression of any in the body.

1 :  Race R, Jenny A, Sutton D.
  Scrapie infectivity and proteinase K-resistant prion protein in sheep placenta, brain, spleen, and lymph node:
 implications for transmission and antemortem diagnosis.
 J Infect Dis. 1998 Oct;178(4):949-53.

 2 :  Wrathall AE.
Rev Sci Tech. 1997 Apr;16(1):240-64. Review.

 3 :  Saeki K, Matsumoto Y, Hirota Y, Matsumoto Y, Onodera T.
       Three-exon structure of the gene encoding the rat prion protein and its expression in tissues.
 Virus Genes. 1996;12(1):15-20.

 4 :  Tanji K, Saeki K, Matsumoto Y, Takeda M, Hirasawa K, Doi K, Matsumoto Y, Onodera T.
  Analysis of PrPc mRNA by in situ hybridization in brain, placenta, uterus and testis of rats.
Intervirology. 1995;38(6):309-15.

5 : Billette de Villemeur T, Pradel A.
[Iatrogenic Creutzfeldt-Jakob disease. Lessons from cases secondary to extracted growth hormone in France].
Transfus Clin Biol. 1994;1(5):333-7. French.

6 : Onodera T, Ikeda T, Muramatsu Y, Shinagawa M.
 Isolation of scrapie agent from the placenta of sheep with natural scrapie in Japan.
Microbiol Immunol. 1993;37(4):311-6.

7 : Bradley R.
   The research programme on transmissible spongiform encephalopathies in Britain with special reference to
bovine spongiform encephalopathy.
Dev Biol Stand. 1993;80:157-70. Review.

8 : Manson J, West JD, Thomson V, McBride P, Kaufman MH, Hope J.
The prion protein gene: a role in mouse embryogenesis?
Development. 1992 May;115(1):117-22.]

Irish farmer jailed for mad cow fraud

Reuters World Report Fri, Jun 23, 2000
An Irish court jailed a dairy farmer for five years on Friday after he was convicted of introducing a BSE-infected cow into his herd to qualify for special compensation payments, court sources said.

The Circuit Criminal Court in the southern city of Cork imposed the sentence on farmer James Sutton after the first successful prosecution under legislation to prevent the spread in Ireland of bovine spongiform encephalopathy, a fatal brain-wasting disease.

Sutton, who had huge debts, stood to gain 75,000 Irish pounds ($89,660) in compensation from the Department of Agriculture for the slaughter of cattle on his 80-acre farm in Clonakilty, west of Cork.

Sutton called in a veterinary surgeon in September 1996 to report that one of his cows was sick. The vet diagnosed BSE and Department of Agriculture inspectors ordered the herd to be destroyed. However, inspectors became suspicious about where the infected cow had come from and uncovered a network smuggling BSE-infected animals across the border from British-ruled Northern Ireland.

Five years jail foR BSE scam farmer

Fri, Jun 23, 2000 By Chris Parkin, PA News
A farmer who deliberately infected his cattle with BSE in order to claim compensation was jailed for five years in the Irish Republic today. James Sutton, from Clonakilty, Co Cork, was told by the judge at Cork Circuit Criminal Court that his action could have erorded confidence in Irish exports. Sutton, 49, pleaded guilty to conspiring with others in an attempt to defraud the state of IR 75,000.

The case marked the first successful prosecution by the Irish authorities under the terms of legislation specially introduced to combat the spread of mad cow disease. Sutton admitted buying an animal with the disease in 1996 and introducing it into his herd by switching a ear tag from another cow. He contacted his vet about the sick cow and the complete herd had to be slaughtered.

The Irish Department of Agriculture became suspicious, though, and a subsequent police investigation uncovered a scheme involving the smuggling to the Republic of cows with BSE from Northern Ireland to take advantage of premium compensation rates being paid to farmers.

Agriculture Department officer Declan Holmes said the scam could have had huge consequences, not just for the national cattle herd, but for the whole economy.

Judge A G Murphy said the crime was enormous, and on a par with the most serious trials he had encountered. He noted that Sutton lost his cattle, was about to lost his farm, had suffered marital break-up and been ostracised by his local community. But the judge said he had to impose a custodial sentence because of the gravity of the case.

Blood: infectivity of buffy coat in new variant CJD

Transfusion, Vol. 40, No. 6, 754-755, June 2000 Letters to the Editor Muttuswamy Sivakumaran, MSc, FRCP, MRCPath, PhD 
"I read with interest the timely article of Brown and coworkers, recently published in TRANSFUSION.1 Their experimental work has produced several important findings that are relevant to current blood transfusion practice in several countries. First, the observation is reassuring that components prepared from the plasma of mice infected with a mouse-adapted strain of human transmissible spongiform encephalopathy are associated with very low infectivity. However, their other finding, that plasma infectivity is not eliminated by WBC reduction filtration, is obviously a worrying one and may imply that additional measures may be required to reduce the "theoretical risk" of transmission of variant CJD (vCJD) through the use of FFP.

Their study also confirms that most of the infectivity in blood is associated with buffy coat. This finding has been, in my opinion, erroneously interpreted by Brown et al. as evidence that WBCs are the major source of infectivity in blood. I would like to point out that buffy coat and WBCs are not synonymous, and it is well known that buffy coat preparations contain large numbers of platelets.

Unfortunately, because of either a flaw in the method of Brown et al. or their omission of a vital piece of information (i.e., platelet contents of the buffy coat preparations used in the study), the validity of their conclusion that WBCs are the major source of infectivity in vCJD is questionable. An experiment carried out to ascertain the composition of buffy coat preparations made by the method described in their report (centrifugation at 2280 x g for 4 minutes at ambient temperature, which is followed by complete removal of supernatant plasma) using six normal mouse blood samples (Harlan Sera-Lab, Belton, UK) reveals that the average platelet content of the buffy coat preparations is at least 30 times greater than that of WBCs (the average platelet:WBC ratio of normal mouse blood samples was 76:1). This confirms my assertion that unmanipulated buffy coat preparations contain large numbers of platelets.

Because of the uncertainty of the composition of the buffy coat preparations used in the experiments of Brown et al., any attempt to draw conclusions as to the relative infectivities of WBCs and platelets in vCJD would be misleading. It would be reasonable to surmise that, although buffy coat has the highest infectivity concentration of any blood component, the relative contributions from WBCs and platelets remain unknown. Experiments using "purer" preparations of WBCs and platelets would be needed to resolve the above issue.

Needless to say, the question of whether WBCs or platelets account for the infectivity of buffy coat is of great importance, as the answer would have direct relevance for the strategies formulated to reduce the "theoretical" risk of transfusion-associated transmission of vCJD. For example, if platelets are found to be the "culprit," the removal of WBCs (WBC reduction) will have very little impact indeed! "

1. Brown P, CervenŠkovŠ L, McShane LM, et al. Further studies of 
blood infectivity in an experimental model of transmissible
spongiform encephalopathy, with an explanation of why blood 
components do not transmit Creutzfeldt-Jakob disease in
humans. Transfusion 1999;39:1169-78.[Abstract/Full Text]
The above letter was sent to Dr. Brown and Ms. CervenŠkovŠ, who offer the following reply:

"We thank Dr. Sivakumaran for this perceptive reading of our article about blood infectivity in an experimental model of transmissible spongiform encephalopathy (TSE). He is correct in thinking that the evidence presented in the article does not provide any basis for distinguishing infectivity in buffy coat as belonging to either of its components, WBCs or platelets.1 Wherever we have referred to WBCs, the proper term should have been buffy coat. We were probably subconsciously anticipating data we knew existed from as yet unpublished studies in hamsters, which indicate that washed platelets have a substantially lower concentration of infectivity than buffy coat and thus imply that most of the buffy coat infectivity is associated with WBCs.2

It is of course possible that the hamster model (like our own mouse model) will not be applicable to the situation in humans with TSE. A recent study of the normal precursor of the pathogenic amyloid protein ("prion protein," or PrP) in human blood components has shown that by far the highest concentration is associated with platelets, not WBCs.3 However, the tissue distribution of the precursor in normal animals may not reflect the distribution of PrP or infectivity in diseased animals: muscle, for example, contains high levels of the precursor in normal animals, but, in diseased animals, it contains neither the converted pathogenic form of PrP nor any detectable infectivity.4-6

The question of which component of buffy coat contains more infectivity therefore remains open, but it should be settled by experiments currently in progress to assay the infectivity in purified WBCs and platelets from the blood of primates inoculated with several different strains of human TSE, including new variant CJD. "

1. Brown P, CervenŠkovŠ L, McShane LM, et al. Further studies of 
blood infectivity in an experimental model of transmissible
spongiform encephalopathy, with an explanation of why blood components 
do not transmit Creutzfeldt-Jakob disease in
humans. Transfusion 1999;39:1169-78.[Abstract/Full Text]

2. Rohwer RG. Titer, distribution, and transmissibility of blood-born 
TSE infectivity. Presented at Cambridge Healthtech
Institute 6th Annual Meeting, "Blood Product Safety: TSE, Perception 
versus Reality," McLean, Virginia, February 13-15,
2000.

3. MacGregor I, Hope J, Barnard G, et al. Application of a 
time-resolved fluoroimmunoassay for the analysis of normal prion
protein in human blood and its components. Vox Sang 1999;77:88-96.

4. Bendheim PE, Brown HR, Rudelli RD, et al. Nearly ubiquitous tissue 
distribution of the scapie agent precursor protein.
Neurology 1992;42:149-56.

5. Hadlow WJ, Kennedy RC, Race RE. Natural infection of Suffolk 
sheep with scrapie virus. J Infect Dis
1982;146:657-64.

6. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, et al. Human spongiform 
encephalopathy: the National Institutes of Health series
of 300 cases of experimentally transmitted disease. Ann Neurol
1994;35:513-29.

Imported BSE cases

Tue, 30 May 2000  Sources: Ag-ministries in Ireland, Netherlands, Portugal; OIE
Portugal imported BSE cases from France, Denmark and the UK whereas Ireland imported cases from France (in 1999), Netherlands (1997), Denmark (1997), the UK and Northern Ireland. What goes around comes around: the UK ironically imported a case from the Netherlands (1997).

Comment (Dr. Marcus G. Doherr Institute for Animal Neurology, Berne.):

"One word of caution: when considering "imported" BSE cases, one should be aware of the fact that there are at least two possibilities:

a. Clinical BSE in imported cattle where the exposure to the infectious agent most likely, based on the time spent in the country of origin and the time spent in the destination country, took place in the country of origin. Example: a breeding cattle that spent the first 2.5 years of life in the country of origin, was exported to another country and came down with clinical BSE at the age of 4.5 years, i.e. 2 years after importation, most likely was exposed/infected in the country of origin.

b. Clinical BSE in imported cattle that spent a relatively short time in the country of origin and a sufficiently long time (longer than the expected incubation time of the disease) in the importing country indicates a likely exposure/infection in the importing (receiving) country.

Unfortunately this information (date of birth, date/age of export/import and date/age of detection) is not immediately available for most of the "imported" BSE cases.

BSE risk, country by country

25 May 2000 Report on the assessment of the geographical BSE-risk (May 2000)
Opinion (webmaster): Because Britain scarcely ceased export of both live cattle and megatonnes of bone meal during the BSE epidemic, a large number of other countries came to be at risk. The case of Germany is fairly typical, with 13000 imported British cattle and 5,000,000 pounds MBM. But it is important to keep in mind the scale of the epidemic which even today is overwhelmingly concentrated in the UK, rather than bash France and Denmark for every little case whereas 3 months of BSE in the UK this year is about the cumulative world total for all time. thus 734 cases admitted to in the first quarter of 2000.

Here are the risk assessment pdfs: serious documents that rate each exposed country, including the US, Canada, Australia, and Latin America:

- USA ... Canada ... Germany ...  France
 
- Austria ...  Belgium ... Denmark ... Ireland

- Italy ...  Netherlands ... Portugal ... Spain ... Switzerland

What mad cows?

10.6.00 New Scientist editorial
HOW does a country know when its cattle are carrying BSE? How can it prove they are not? These are not trivial questions. It is surprisingly difficult, especially in big herds, to spot the odd mad cow. When the early signs of BSE look like those of common ailments, and surveillance depends on farmers reporting the unusual, it's nigh-on impossible. And since discovering BSE can mean that your nation's herds have to be slaughtered and exports shut down, governments are reluctant to beef up their surveillance.

So the European Commission's finding that some apparently BSE-free countries are probably harbouring the disease is about as welcome as a fox in the hen house. Among BSE scientists, it has been an open secret for years that Germany, Italy and Spain are in a state of denial about BSE. It's time these nations ditched their passive surveillance techniques and began to search actively for BSE and, if needed, take precautions to protect people from the nasty disease it appears to cause.

As members of the European Union they will in any case be forced by law from 2001 to take active measures to search for the disease. But there's no such compulsion for other countries such as Canada whose position the study has also thrown into doubt. Indeed, there is no requirement for them even to undertake risk assessments similar to the Commission's study. The Paris-based World Organisation for Animal Health (OIE) should demand stricter proof for the rest of the world. After all, the OIE watches for sheep with bluetongue. Eating sheep infected with bluetongue doesn't kill people. Eating mad cows probably does. pg 4 continuation:

Global infection. It's official: even in countries that deny it, some cattle are likely to be harbouring BSE

10.6.00 Debora MacKenzie New Scientisst
Across Europe, tens of millions of people believe they have little chance of catching new variant Creutzfeld-Jakob disease (vCJD) because their countries are officially BSE-free. But scientists advising the European Commission say they have every reason to be worried. (Click on thumbnail below for a map of British beef exports.)

BSE, believed to be the cause of vCJD, is much more widespread than some countries will admit: the advisers say that Germany, Italy and Spain, officially BSE-free, are "likely to be infected". And infection "is unlikely but cannot be excluded" in six more European countries, as well as Canada, Australia and the US.

These figures have emerged from a two-year study for the Commission of the factors affecting the spread of BSE in 25 countries by independent scientists and experts in the countries concerned. They collected data on each country's import of cattle and meat and bone meal (MBM) from Britain and other BSE-infected countries, then calculated how well the importing country would have controlled any infection. To restrict BSE, cattle feed should be pressure-cooked, and should not contain MBM.

Smaller studies have already suggested a more widespread BSE epidemic (New Scientist, 3 May 1997, p 14), but this is the first official assessment. A draft has been put on the Web to collect comments from researchers and government experts.

American officials told New Scientist that they are preparing a reply. Werner Zwingmann, Germany's chief veterinary offeicer, says the assessment is based on probabilities that are "purely speculative". Brian Evans, Canada's chief veterinary officer, thinks the assessment is too harsh. A British cow imported to Canada developed BSE in 1993 and 63 others may have become cattle fodder. "We don't dispute that infection cannot be completely ruled out, but the measures Canada took at the time were appropriate," says Evans.

Germany imported 13 000 British cattle at the height of Britain's epidemic, plus 1200 tonnes of British MBM. The figures for Spain and Italy were similar. Some of this meat, including infection-bearing nervous tissue, was fed to local cattle without enough pressure-cooking to make it safe. These cows could have infected others. In all three countries, any BSE infectivity entering the system "would have been quickly amplified", the scientists conclude.

The Commission banned feeding cattle to cattle in 1994, but infection would have continued to circulate, says the assessment, through infected cattle remains contaminating feed mills, and insufficient pressure-cooking of feed. All three countries have refused Commission requests to remove high-risk tissue, such as brains and spinal cords, from cattle carcasses, insisting that their cattle are BSE-free. "We consider their BSE risk similar to countries with a low, admitted incidence of BSE, such as France," says Marcus Doherr of the Swiss Federal Veterinary Office, one of the assessment's organisers.

These countries say they have no sick cows in their herds. But Doherr says that their "passive" surveillance, which relies on farmers reporting sick animals, may entirely miss small numbers of cases. Passive monitoring detects only a third of such cases, according to more active surveillance in Britain and Switzerland, where cows not suspected of having BSE have still been tested after slaughter.

The US imported 126 cattle and 44 tonnes of MBM from Britain. Any infection would have been amplified and could still be circulating, while surveillance would not detect all cases, says the report.

Fish farmers get mad cow warning

The Dominion (Wellington)  June 19, 2000  MILNE Jonathan
New Zealand fish farmers have been told to stop feeding animal byproducts to their fish or risk letting a mad cow type of disease into the human food chain.

Farmers also had to get their act together on excluding chemicals containing dioxin from their farms, Treaty of Waitangi Fisheries Commission member John Mitchell told the commission's annual meeting in Wellington at the weekend.

Dr Mitchell, just back from a big aquaculture conference in France, said New Zealand's farmed seafood would be rejected by European countries if farmers could not prove they were meeting European Union health regulations.

Genetically engineered fish could not be exported to Europe either. New Zealand's aquaculture exports earned about $ 200 million in the past year. Fish could no longer be fed any food derived from beef, sheep, pork, or chicken industry byproducts because of the danger of the human variant of mad cow disease, Creutzfeld-Jacob disease, he said.

Cage-reared salmon, snapper and flounder farmers "had better be pretty damn sure they are using fishmeal -- byproducts from a fish factory that cannot be fashioned into food products for human consumption -- and clean fishmeal at that".

Salmon Farmers Association chairman Mark Gillard, from King Salmon, said meat-based and bone-based meal was used because it was cheap and efficient. He did not believe there was a health risk but farmers would have to change their practices when new European regulations came in.

"It would affect us. I'd like to know the reason why they would want to ban it first. You'd have to have some pretty good evidence that it shouldn't be used, because it's a pretty efficient use of something that can't otherwise be used," he said.

Dr Mitchell warned aquaculturalists: "Don't just assume that because it's been a practice to do certain things, traditionally in this corner of the world, that you can carry on doing those things. These are high-valued products that can generate high prices, but none of it's any consolation to you if you arrive at the border and someone turns you away."

He said there was also much concern in Europe after scandals in Belgium where dioxin was allowed to penetrate foods.

"It is a very grave concern that in the management of marine farms, with the vessels and machines operating around them, that the oil, diesel and other fuels cannot possibly escape into the environment where the fish are growing because many of those fuels carry dioxins and other toxic chemicals," he said.

Sick and hungry cows driven taken to market on way to incinerator

Mon, Jun 19, 2000 By Amanda Brown, Environment Correspondent, PA News
Huge numbers of sick and hungry animals are being processed through Britain's livestock markets, the hidden victims of the farming crisis, according to a report out today. Animal Aid has looked at the implementation of the Government's 1998 welfare strategy for animals passing through markets and discovered that there remain endemic problems of brutality and serious neglect.

Chief among the victims are worn out ewes, cattle en route to incineration under the BSE emergency measures and discarded calves, for whom there is negligible demand. These animals result mostly from irresponsible over-production by Britain's heavily subsidised farmers, says the pressure group in its document, Bartered Lives.

It produces evidence of such animals arriving diseased and emaciated, and being subjected to aggressive treatment during what is often a long, stressful experience. The provision of water is rare.

The Animal Aid report demonstrates that similar treatment in Britain's typically ramshackle markets, extends to animals for whom there is a commercial demand.

Undercover film published with the report shows animals beaten heavily with sticks, kicked in the head and prodded repeatedly about the face with electric goads -- sometimes by children. There is no accredited training requirement for those handling animals at markets.

Animal Aid director Andrew Tyler said: "Twenty-one months ago, largely in response to Animal Aid's previous report, the Government announced a new welfare strategy. But without resources for enforcement, that strategy is meaningless. Farmers have only themselves to blame for financial problems arising from the over-production of animals and the BSE crisis.

"To deprive worn out ewes and cattle of food or veterinary care in the last months of their lives is barbaric. Driving them through the medieval bartering system prior to their slaughter compounds the offence. The day at market, `fun' as it might be for socialising farmers, has no place in the 21st century."

French officials to begin mad cow screening program

AP WorldStream Thu, Jun 8, 2000
BMJ 2000 320(7251): p. 1692e
French officials on Thursday said that a long-planned program to screen cattle for mad cow disease would debut next week, with specialists testing 48,000 dead animals before the end of the year.

At a news conference, France's top food official, Marion Guillou, said the study's final results would be released in late 2000 or in early 2001, though initial data would be available in the fall. Until now, only animals suspected of carrying mad cow disease, or bovine spongiform encephalopathy, have been tested. The new program screens dead animals who have not shown signs of the disease but who are considered high-risk.

The vast operation is modeled on a program in Switzerland and is expected to cost 271 million francs (38.7 million dollars). Some 12,000 of the tests are required by the EU as part of a Europe-wide program.

Mad cow disease remains a key health concern in France, where 18 cases of the disease have been reported so far this year. Europe's beef scare was triggered in 1996, when the European Union imposed a ban on British beef after a link was established between the disease and Creutzfeldt-Jakob disease, a fatal brain-wasting condition in humans.

Herd killed as France reports new BSE case

Mon, Jun 19, 2000 Reuters World Report
A herd of 54 cattle was destroyed in France after the discovery of a new case of "mad cow" disease, the farm ministry said on Monday. The new outbreak brought to 21 the number of cases of bovine spongiform encephalopathy (BSE), a fatal brain-wasting disease, reported in France this year. France now looks set to exceed the 30 cases of BSE detected in 1999. One hundred and one cases of the disease have been reported in France since the epidemic was first detected in 1991.

The ministry said in a statement the infected animal behind the latest scare was born in 1992 in the Maine et Loire region. The animal and the 53 cattle in its herd were destroyed, in accordance with French law.

Officials have said the number of French cases of BSE is expected to rise sharply in the next few months as France begins testing 48,000 cattle for the illness in a bid to measure the epidemic's extent. France remains locked in a legal battle with the European Commission over its refusal to lift a ban on imports of British beef because of fears it is not free from BSE.

Britain has reported more than 176,000 cases of BSE, making the French epidemic look small by comparison. But while the number of cases in Britain is falling, the French outbreak is still growing despite measures introduced almost a decade ago to combat the spread of BSE through contaminated animal feed. France introduced tougher controls on cattle feed in 1996 and had originally predicted that BSE cases would taper off in 2001, given that the disease has a five-year incubation period. But the government has recently stopped forecasting when it expects the epidemic to die out.

New case of mad cow discovered in France

AP WorldStream  Mon, Jun 19, 2000
Officials have discovered another case of mad cow disease in central France, the Agricultural Ministry said Monday. The animal was the 21st afflicted cow reported in France this year. In 1999, French authorities discovered 31 cases of mad cow disease, or bovine spongiform encephalopathy.

Officials slaughtered the milk cow, born in 1992 in the Maine and Loire region, and its herd of 54, as a precaution, the ministry statement said in a statement. New cases of the disease are expected to break out in France until 2002, five years after authorities took rigorous measures to prevent more outbreaks. Mad cow disease has an average incubation period of five years. The French Agriculture Ministry has said it will begin tests of ailing and dead cattle to determine how widespread the disease has become.

Eighth annual report of CJD Surveillance Unit

Sat, 17 Jun 2000 Full text at  CJDSU  website  
The eighth annual report of the National Creutzfeldt-Jakob Disease Surveillance Unit, jointly funded by the Department of Health and the Scottish Executive, was published today. The report looks back over the period from May 1990, when the Unit was set up, to 31 December 1999 in relation to sporadic, familial and iatrogenic CJD and variant CJD (vCJD). It provides analyses of data collected by the Unit on the dietary, occupational and medical histories of patients.

Highlights of the Eighth Annual Report 1999 Creutzfeldt-Jakob Disease Surveillance in the UK

1. "The average number of cases of sporadic CJD identified annually since 1990 was higher than in previous surveillance periods extending back to 1970. It is impossible to say with certainty to what extent these changes reflect an improvement in case ascertainment and to what extent, if any, they reflect changes in incidence. "

[They are seeing a 400% increase in section 2.1. A look-back study [2.9 Extent of misclassification of death from CJD Study] mentioned later says no cases were missed. So improving ascertainment cannot explain this. -- webmaster]

2. "Analysis of the incidence of vCJD by standard region suggests that the incidence of vCJD in the "North" of the UK may be higher than in the "South" (p=0.02).... the apparent differential incidence of vCJD between "North" and "South" should be treated with caution in view of the absence of an a priori hypothesis and the small numbers of cases on which this finding is based." [Note p=0.02 is well past the threshold of statistical significance. -- webmaster]

3. "The two youngest cases were aged 14 years at onset while the oldest case was aged 53 years... "

[This conflicts with a later statement in section 2.8 Study of Progressive Intellectual & Neurological Deterioration (PIND): "773 children have been reported.... 56 cases are in the process of being followed up. So far one case of definite vCJD and 2 suspected cases have been identified." -- webmaster]

2.7 Transfusion Medicine Epidemiology Review A collaborative study between the CJDSU and the UK Blood Transfusion Services has been in progress since 1997 to examine the possibility of transmission of CJD via blood transfusion. Patients with sporadic CJD and matched controls, who were reported to have donated blood, were identified and a single look-back exercise was performed. The details of the recipients who received components from these donors are held on the CJDSU database in order to check whether any subsequently develop CJD. This process was extended to vCJD cases. So far, 30 recipients have been identified in the CJD study and 12 in the vCJD study. None has appeared as suspect cases on the CJDSU register.

The reverse process is also underway. The study group is sporadic CJD patients and matched controls who have received blood. The relevant donors are traced and then linked to the CJDSU register. To date, 282 donors have been identified and none appear on the register. This study was also extended to vCJD. Only one vCJD patient was known to have received blood; 103 blood components were involved. None of the donors appear on the register. (Collaborators on this project: Dr P.E. Hewitt and Dr C.A. Llewelyn).

3.1 Medical risk factors for vCJD Thirty-one of the cases were reported to have had some sort of operation/surgical procedure (other than dental procedures) prior to the onset of their illness compared with 19 controls (Table 9). There is no evidence to suggest that cases were more likely than controls to have had operations in the past.

Table 9 Reported operations/surgical procedures from 51 cases of variant Creutzfeldt-Jakob disease and 27 controls.

% of cases (n = 51) % of controls (n= 27)
Any operation 61 70 Abdominal operation1 22 26 Neurological operation 0 0 Orthopaedic operation 10 7 Eye operation 2 7 Tonsillectomy 8 19 Appendicectomy 4 19 1 Includes appendicectomy

Five of the 51 cases were reported by relatives to have had a history of blood transfusion compared with 2 of the 27 controls. It is of note in relation to the TMER study that a record of blood transfusion was not traced in 4 cases.

3.2 Dietary risk factors for vCJD The reported consumption of various different meats and meat products by cases and controls in the period since 1980 (except for 5 cases and 4 controls where the period is from 1985 due to a change in the questionnaire) is shown in Table 10.

4.3.2 Western Blot Analysis Five cases of CJD that contained detectable PrPres were seen that could not be accommodated in the above classification system. Two MM sporadic CJD, one VV sporadic CJD and a case of GSS displayed a non-glycosylated PrPres mobility reproducibly intermediate between Type 1 and Type 2. Our own studies show that a similar mobility type results from metal ion chelation prior to proteinase K treatment of Type 1 extracts. We are currently investigating the possibility that these intermediate types represent endogenously or artifactualy metal-depleted PrP.

The remaining unclassifiable case was a young VV individual with atypical clinical features, referred to the NCJDSU from outside the UK. Isotyping of a cortical biopsy showed Type 1 PrPres, however after a protracted illness, analysis of autopsy cortical material showed Type 2 PrPres and a glycosylation ratio markedly different from the biopsy material and more closely resembling that of GSS. This finding and recently published data showing isotype diversity in a subset of sporadic CJD cases has prompted us to undertake a neuroanatomical survey of isotypes in sporadic CJD and vCJD. Preliminary results confirm isotype diversity within and between cases of sporadic CJD but indicate a homogeneous isotype in vCJD.

Peripheral Tissues The possible presence of CJD infectivity in peripheral tissues of individuals with preclinical CJD presents a risk to public health. It is therefore important to determine the tissue distribution of PrPres as a surrogate marker for infectivity in peripheral tissues. The challenge involved in such studies is that PrPres levels are lower than in the CNS. The Protein Lab is currently involved in the parallel development of three approaches to address this question, namely Dissociation Enhanced Lanthanide Fluoro Immuno Assay (DELFIA), Capillary Electrophoresis and modifications of the standard Western blot procedure. Initial studies of peripheral tissues by Western blotting proved inconclusive however centrifugal concentration of PrPres has allowed us to increase the sensitivity of our Western blotting procedure by up to a factor of 100. Using this technique we have analysed post-mortem tonsil with the following results:

These studies confirm the specificity of tonsilar PrPres to vCJD and show tonsilar PrPres to be more extensively glycosylated than PrPres from the brain of the same individual. The negative result from the growth hormone therapy iatrogenic CJD tonsil is of interest since it demonstrates that peripheral infection with the CJD agent may be insufficient in itself to result in a PrPres positive tonsil. We plan to extend this work to: i) further cases of CJD, ii) other forms of CJD, iii) other lymphoreticular organs, iv) the peripheral nervous system, and v) organs commonly used for transplantation.

4.4 Brain banking activities The bank of fixed and frozen tissues in the surveillance unit was used extensively in 1999 for research purposes within the unit and with collaborators in the UK and overseas (see Table 19). No problems were encountered with Year 2000 compatibility in the Tissue Bank, and this facility is now being reorganised to allow the most efficient storage of frozen brain and organ samples, blood, DNA and CSF samples.

Researchers requesting and receiving tissue samples:

Professor C Abee, Alabama, USA 3
Dr P Minor, Hertfordshire, UK 4
Dr J  Anderson, Cambridge, UK 3
Dr M Mirakhur, Belfast, N. Ire. 5
Dr N Bogdanovic, Sweden 4
Dr D Moffat, Eastbourne, UK 1
Dr L Bridges,Leeds, UK 4
Dr Morgan, Scarborough, UK 1
Dr J  Broome, Liverpool, UK 2
Dr T Moss,Bristol, UK 2
Dr P Brown, Maryland,  USA 3
Dr I Nauroz, Kirkcaldy, UK 1
Dr M Bruce, Edinburgh, UK  7
Dr J Neal, Cardiff, UK  6
Dr M Carey, Birmingham, UK 12
Dr C OŪBrien, Swansea, UK 1
Dr J Cesbron, Lille, France 1
Dr R Perry, Newcastle, UK 1
Professor J Collinge, London UK 3
Dr J Polo, Santander,Spain 2
Dr D Crooks, Hull, UK 3
Professor S Prusiner, San Francisco, USA 12
Dr S Dealler, Leeds, UK 2
Dr Raafat, Birmingham, UK 1
Dr D Ellison, Southampton, UK 3
Dr H Reid, Manchester,UK 5
Dr M Esiri, Oxford, UK  1
Dr T Revesz, London, UK 4
Dr M Farrell, Dublin, Ireland 1
Dr K Robson, Nottingham,UK 1
Dr J Fraser, Edinburgh, UK 12
Dr E Ruban ,Leicester, UK 1
Dr P Gambetti, Ohio, USA 3
Professor A Sau, Marmara, Turkey 2
Dr J Geddes,London,UK 6
Dr Scoones, Middlesbourgh, UK 2
Professor F Gray, Cedex, France  1
Dr S Shanker, Bangalore ,India 1
Dr J Hope, Compton,UK 15
Dr R Somerville , Edinburgh, UK 2
Dr P Ince, Newcastle, UK 2
Dr M Stewart, Lancaster, UK 1
Dr G Jansen, Utrecht, Holland 5
Dr A Taratuto, Buenos Aires, Argentina 2
Dr C Jarius, Vienna, Austria 1
Dr W Timperley,Sheffield, UK 4
Dr R Kisilevsky, Ontario, Canada 1
Professor R Weller, Southampton, UK 3
Dr Kitamoto, Sendai, Japan 3
Dr S Wells, Bolton, UK 1
Dr S Lehmann,  Montpellier, France  3
Dr S Wharton, Edinburgh, UK 1
Dr P Liberski, Lodz, Poland 2
Ms. L Wheatley, Leicester, UK 1
Professor J Lowe, Nottingham, UK 8
Dr H Whitwell, New Zealand 1
Dr J Mac Kenzie, Aberdeen, UK 2
Dr P Wilkins, London, UK 1
Dr D Mann, Manchester, UK  2
Dr Wright, Dunedin, New Zealand 1
Dr J Manson, Edinburgh, UK 1
Dr J Wyatt, Leeds, UK 6
Dr A Marshall, Essex, UK 2
Dr K Zarkovic, Zagreb, Croatia  5

To be adopted: measures to reduce BSE risk

Fri, 23 Jun 2000  EU Commission press release
Commissioner David Byrne, responsible for Health and Consumer Protection has today welcomed the support of a majority of Member States at the Agriculture Council for a key decision strengthening consumer health protection by taking animal tissues likely to be infected with BSE out of the food and feed chain. The proposed decision can now be adopted by the European Commission in the absence of a simple majority of Member States against the proposal. The objective of the decision is to introduce harmonising rules for the removal of specified risk materials presenting a BSE risk as of October 1st.

Slaughterhouses and meat cutting and processing plants in the UK and Portugal will have to take out more tissues given their higher BSE risk. In all Member States, including those countries where so far no BSE case has been detected, the tissues most likely to present a BSE risk will need to be discarded.

"This is a major breakthrough in our work of the past years to make sure that the highest health protection standards are in place throughout the EU", said David Byrne. "I am very pleased with the simple majority reached by the Member States. This measure is the best possible safeguard to keep the meat products clear of BSE infectivity, and to protect consumers from the risk of new variant Creutzfeldt-Jakob disease. Together with the improved BSE monitoring and testing program agreed with the Member States in April we will have a solid and comprehensive package of measures in place to prevent future crises and deliver on our food safety promises. "

The measures reflect the key recommendations of the Commission's Scientific Steering Committee, which has repeatedly insisted on the need to remove specified risk materials (SRM's) from the food and feed chain in all EU countries. The discovery of a first BSE case in Denmark in March this year further strengthened the doubts about the BSE-free status of countries where so far no BSE case had been identified.

The Decision as voted upon today will require all Member States to make slaughterhouse and authorised meat cutting and processing plants remove (short list):

- the skull (including the brains and eyes), the tonsils, the spinal cord and the ileum of cattle above 12 months;

- the skull (including the brains and eyes), the tonsils and the spinal cord of sheep and goat above 12 months or of younger animals that have a permanent incisor erupted through the gum;

- the spleen of sheep and goat of all ages.

In the UK and Portugal slaughterhouses and authorised meat cutting and processing plants will in addition be required to remove (long list):

- the entire head (excluding the tongue and including the brains, eyes, trigeminal ganglia and tonsils), the thymus, the spleen, the intestines and the spinal cord of cattle above 6 months;

- the vertebral column (including dorsal root ganglia) of cattle above 30 months. The vertebral column may also be removed at the point of sale.

The short list of SRMs to be removed will apply equally to third countries from 1 April 2001 onwards if their BSE-free status is not established by a scientific risk assessment. National measures, which already foresee that third countries have to remove SRMs, can stay in place until the coming into effect of the Community measure.

The Decision will also prohibit the use of certain slaughtering techniques which entail a risk of contamination of animal blood by the release of BSE infected tissue into the bloodstream as of 31 December 2000.

The provisions of this Decision will be repealed when the proposed Council and European Parliament Regulation for the prevention and control of certain transmissible encephalopathies enters into force. The regulation is foreseen to be discussed by the Council in autumn.

This Decision will replace a previous Commission Decision 97/534/EC on the use of risk BSE materials whose entry into force had been repeatedly delayed. It will be subject to review in the light of new scientific evidence and in function of progress made in controlling and preventing BSE infectivity through risk management measures.

US eye surgeons reuse blades

05-23-2000Mercury News BY JULIE SEVRENS
A Bay Area eye center has notified 2,700 patients that they could have been exposed to infectious diseases because the blades used in laser surgery may have been routinely reused on other patients before being discarded. Letters have been sent to patients of the LaserVue Eye Center alerting them that disposable microkeratome blades were routinely reused on patients undergoing laser eye surgery at the company's San Francisco and Santa Rosa locations.

For more than two years, doctors at both sites reportedly rinsed the equipment -- which is used to cut flaps in a patient's corneas -- in a water solution before using them again, even though medical protocol calls for blades to be thrown away after use. Furthermore, the blade holders were sterilized only after every fourth patient, a violation of medical guidelines, according to a state health department investigation. The department requested that the firm send letters to its patients informing them of the practice of reusing the blades. The doctors have since stopped the practice, and the state medical board is reviewing the case.

It is possible, though the chances are very slight, that infectious diseases such as hepatitis B and C as well as the AIDS virus [and CJD -- webmaster] could have been transmitted in this manner.

``A fourth-grader would understand you need to follow at least these basic sterilization procedures,'' said Geoff Gordon-Creed, a San Francisco attorney who has filed a class-action lawsuit against the center and two of its physicians.

Drs. Sanjay Bansal and Swati Singh told health department investigators they reused blades in an attempt to give patients the best eye care possible. The physicians contended that they only reused those blades that served them well in prior surgeries, and that they threw away any that had visible blood on them. ``That was the reason why the blades were being (reused) -- for precision purposes -- not to compromise the procedure in any way or put anyone at a health risk,'' said Patrice Smith, a spokeswoman for LaserVue. ``Much of the procedure is in the blade itself. The ability of the blade to cut well.''

Other eye surgeons said they were appalled by the practice of reusing surgical blades and believe the LaserVue case is rare among eye clinics. ``There's no excuse for it,'' said Dr. Gary Kawesch of the Laser Eye Center of Silicon Valley, which has offices in San Jose and Pleasanton. ``The concept of not sterilizing is something that is a very basic principle of surgery. The potential for disease transmission is huge.''

Most clinics discard the microkeratome blades after performing laser surgery on a single patient, Kawesch said. Very few surgeons he's aware of reuse blades, but when they do they sterilize them between patients. Most eye surgeons use ethylene oxide gas or steam to sterilize blades. The blades, he says, cost $40 to $65 apiece, and one is used to complete the laser surgery on both eyes of a patient.

``I feel that for the fees patients are paying they deserve a fresh blade,'' Kawesch said. The surgery costs about $5,000 for both eyes.

Manufacturers recommend in their literature that blades be used only once, then thrown out, according to Dr. Hamid Sajjadi, who performs laser eye surgeries at the Ellis Eye Center and Silicon Valley Eye Laser Center. Although diseases are more commonly transmitted through blood, there is evidence they can spread through contact with tears and other conjunctival tissue, said Dr. Jon Rosenberg, medical epidemiologist with the state health department.

The first patients began receiving their letters earlier this month, and many remain in a state of disbelief. ``I thought it was a joke,'' said Debbie Shubin, a Sebastopol mortgage broker who paid $4,400 to have her eyes operated on last year. ``I could not believe that Dr. Bansal, with all of these diseases that are out there, would reuse an unsanitary blade on more than one patient. `I would never have had the surgery had I known he was going to be reusing blades,'' she said. ``I don't know anybody who would put themselves through that.''

Mad Cow Remains Fuel Power Station

AP Online Sun, Jun 4, 2000
British cattle slaughtered during the crisis over mad cow disease are finding new life -- as a source of electricity. The Glanford power station in Lincolnshire, central England, has begun incinerating the remains of animals destroyed due to fears of bovine spongiform encephalopathy, or mad cow disease. The station had previously burned poultry litter, but switched over to cattle meat and bonemeal about two weeks ago. Its consumption of 250 metric tons a day produces enough electricity to power a small town.

BSE is a brain-wasting cattle disease that appeared in Britain in the mid 1990s. Experts believe its human form, a variant of Creutzfeldt-Jakob disease, can be contracted by eating contaminated beef. In 1996, the European Union banned imports of British beef and Britain banned cattle older than 30 months from the food chain. Millions of cattle were slaughtered, and almost half a million tones of dried meat and bonemeal has since been stockpiled in secure sheds.

The Environment Agency granted permission to Glanford's owners, Fibrogen, as part of a scheme to destroy the massive stockpile of animal waste. Fibrogen has a three-year contract to burn 85,000 tons of animal remains a year, and two other contractors are believed to be interested in opening similar incinerators.

Fibrogen Managing Director Rupert Fraser said the station meets stringent environmental standards and there is no danger of BSE-infected material escaping into the atmosphere. "We are very confident the risk to the public is absolutely negligible," he said. "It's infinitesimally small."

BSE carcasses burned for electricity

Saturday, 3 June, 2000 By BBC environment correspondent Tim Hirsch
Half a million tonnes of cattle remains are to be incinerated. Every day, a fleet of bright red 40-tonne lorries travels up and down Britain's motorways carrying a cargo most of us would prefer not to think about: the dried remains of nearly a million cattle slaughtered each year under the anti-BSE protection measures.

But someone has got to think about it, and we're all still paying for it - the cull of all cattle aged over 30 months has cost us more than £2bn since it was introduced in 1996, and has created a mountain of some 460,000 tonnes of meat and bone meal (MBM) which has to be disposed of.

Until now virtually all of it has been piling up in secure storage sheds, the biggest of which is on the edge of an RAF base at Barkston Heath near Grantham in Lincolnshire - the converted aircraft hangars are stuffed full with nearly 100,000 tonnes of MBM.

Waste stockpile: Each 26-tonne lorryload of the brown, sand-like powder contains the remains of around 200 cattle, mostly dairy cows which have reached the end of their milking lives. But a start is finally being made in getting that enormous stockpile down.

An hour's drive to the North of that store, on an industrial estate outside Scunthorpe, a power station which used to burn poultry waste has now converted to MBM incineration. The Fibrogen plant at Flixborough has just come on stream, and already generates enough electricity to power a small town.

Around 250 tonnes of the meat and bone meal are burned each day - the grisly fuel is delivered into an enclosed shed and checked by inspectors wearing body protection and breathing filters.

It is then fed up along a system of covered conveyor belts into a furnace, producing steam which turns the power turbines. About a quarter of the volume of the MBM remains as ash, which is captured and sent off to landfill sites.

The cattle producing this material are not confirmed BSE cases - those are cremated separately in special plants.

Pollution concern: But since some cows will have been infected without developing the disease, the Fibrogen plant has to meet strict rules set down by the Environment Agency designed to ensure that nothing dangerous escapes into the atmosphere. They include a requirement that all the fuel must be subjected to at least 850 degrees C for a minimum of two seconds.

The plant's managing director Rupert Fraser says that means the risks to the public are infinitesimally small - and it performs an important service by using up the cattle waste.

But some local residents are not convinced by those reassurances, and mounted an unsuccessful campaign to prevent the power station from being allowed to operate.

Even with its capacity to burn 85,000 tonnes of MBM a year, the Fibrogen plant alone does not even keep up with the new waste material being produced by the slaughter. It is only when two new incinerators come on stream later in the year that a serious dent will start to be made in the stockpile.

Mad Cow Home ... Best Links