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First reported case of BSE in the Netherlands
Second reported case of BSE in the Netherlands
BSE in Poland -- 29 this year?
CJD infectivity of blood reported
VA hospitals look for transmission by blood
Squirrel brains and CJD
Hogg lies about carcass disposal
First anniversary: Deaths from new CJD strain 'may run to thousands'
Kuru brain update
nvCJD victims family to sue the British Government
Neurologists See Risk In Unlabeled Blood Reagent
Court case threat over beef labelling
Financial Times bashes MAFF
Britain finally agrees to label its export beef
Yet another isolated case of E. coli from minced beef

First case of BSE in the Netherlands

Friday 21 March ... Jan Braakman, journalist Agrarisch Dagblad Netherlands

The Dutch have created a special Dutch mad cow site to cover this issue. The site will be updated daily if needed (in Dutch).

A BSE-case in the Netherlands was confirmed. It is the first confirmed case of BSE in the Netherlands. The dairy farmer (living in the village of Wilp in the province of Gelderland) consulted his veterinarian in the second week of March because the cow exhibited distressed behavior. The veterinarian suspected BSE.

The brains of the five year old cow were invstigated at the Institute of Animal Health (ID-DLO) in Lelystad because of BSE-suspicion. The dairy-farmer had a herd of 110. After confirmation,the entire herd was culled; brains will be investigated at ID-DLO for the disease. Also blood and urine-samples were taken. All the relatives of the cow were traced back through the computerized identification and registration-system that is used in the Netherlands. Most of the relatives were still at the farm where the BSE-cow lived. According to the Dutch Agriculture Ministry and AP, none of its meat reached the human food chain.

The cow's name is Anja 3. She is a daughter of the dairy-cow Anja (already dead, lived at the same farm) and the Holstein-Frisian AI (artificial insemination) by bull Cowboy (already dead, imported from Germany) whose roots go back to the United States. Mother Anja had one other daughter (Anja 4, lived at the same farm), one granddaughter (Anja 6, lived at the same farm) and one son (already dead)

Anja 3's grand-mother and grand-grand-mother (both dead) lived at the same farm in Wilp. Anja 3 gave birth to one still living daughter (Anja 5) and two already slaughtered steer calves. Three calves that originate from the grandmother were traced at two other farms. They also were culled and their brains will be examined.

Not much yet known about the feed-providers. Investigation into that is done by the Inspection of the ministry of Agriculture. Research into the infection routes being considered (according to the ministry of Agriculture) include:

1 - contaminated feed [Ruminant MBM was banned in the Netherlands in 1989.]
2 - maternal infection
3 - 'spontanuous' BSE
The Dutch ministry of agriculture announced that none of the 105 cattle from the herd where the BSE-cow named Anja 3 came from had BSE-infectivity. All 'relatives' at the farm and at two other farms were investigated. None was BSE-positive.

The Institute of Animal Health (ID-DLO) in Lelystad has still one cow under investigation. That animal was born at the same day at the same farm as BSE-cow Anja 3. She lived at another farm now and was traced by the computerized identification and registration system.

Controls at three feedmills that delivered feed since 1992 to the farm did not yet result in a trace that leads to feed-contamination. Further investigation is being done by the government inspection (AID).

Netherlands records second case of mad cow disease

Reuter Information Service April 7, 1997

THE HAGUE -- The Netherlands reported its second case of bovine spongiform encephalopathy, also called BSE or mad cow disease, Monday just over two weeks after the first. The government said the disease had been diagnosed in a cow in the northern province of Friesland which was slaughtered in late March, adding that it had provided full details of the case to the European Commission in Brussels.

As a precaution the farm has been sealed off and all of the livestock there will be killed. The slaughterhouse has also been closed and all of its stocks of meat will be destroyed. The parents and descendants of the infected cow will be traced to see if they also have the disease in an attempt to pinpoint the source of the infection.

Experts are still trying to piece together how the first Dutch BSE case, reported on March 21, was caused. The Netherlands has also been struggling in recent weeks to contain an outbreak of swine fever which has already led to an EU export ban and the destruction of several thousand pigs.

Since 1986, about 178,000 BSE cases have been confirmed in the European Union and Switzerland, according to figures published by the European Commission. The majority of those cases -- around 172,000 -- have occurred in Britain. ------------- Mad- cow disease was confirmed today in a six year old cow in the village of Kollum (Province of Friesland). The cow has been culled at a slaughterhouse in Tolbert (Friesland). The farm and the slaughterhouse are restricted. The whole herd (40 dairy cattle, 30 calves, 40 sheep and 10 goats) will be inspected on BSE-infection. The meat will be destroyed. The meat from other animals at the slaughterhouse will be destroyed.

Offspring of the cow and ancestors will be traced. If those animals live at other farms, they will be taken away for further inspection. Feed history will be investigated. The European Commission has been informed.

BSE Reporting in Europe

From: British Dairying Magazine (p17 March '97)

Claims from other EU countries that they have little or no BSE are looking increasingly silly. Evidence is mounting that thousands of cases should have been reported while some, notably Belgium have still to report any. One of the latest pieces in the jigsaw is the news that Poland has confirmed 51 cases - 29 of them this year.

The pattern of acknowledging BSE is remarkably similar to Switzerland, which up to last year had disclosed 230 cases in a manner which persuaded UK vets at Tolworth [UK/MAFF/VS HQ] that it was operating a fully accountable reporting system. It is regarded as significant that each of these countries is outside the EU and has a common border with Germany.

CJD infectivity of blood

25 Mar 1997 Charles Arthur, The Independent

"Paul Brown of the NIH made a presentation yesterday to the WHO in Geneva about infectivity of CJD-infected mice blood. He demonstrated that intracerebral injection of this blood could pass the disease to healthy mice. Work not yet published. Some discussion still going on today and tomorrow at WHO about implications."

John Collinge told me today that the transgenic mice with human PrP genes, which he injected with BSE, have now all died, apparently of natural causes. They are studying the results. He intends to carry on to a second stage (in which brain homogenate from the first mice will be given to a second generation of mice). These mice were VALINE homozygous at codon 129. (All the human victims of v-CJD who have been gene-typed have been methionine homozygous at c129.) Collinge is presently preparing a separate experiment with transgenic Met homozygotic mice.

Matthew Parker became the 15th Briton to die of v-CJD, on Sunday. Two other Britons still alive with the disease - Vicky Rimmer (in coma for two years; clinically, a remarkable case) and Donna Mellowship."

VA hospitals look for transmission by blood

Lookback notification action of Washington, DC 20420 August 8, l995
Management of patients identified in CJD lookback notification

1. PURPOSE: The purpose of this Veterans Health Administration (VHA) Directive is to provide guidance to medical centers on the medical management of Department of Veterans Affairs (VA) patients identified by VA's Creutzfeldt Jakob Disease (CJD) lookback notification action of January 6, 1995.

2. BACKGROUND a. On January 6, 1995, VHA initiated a voluntary lookback notification of all VA patients who may have received certain lots of blood derivatives or blood components produced from donors with CJD. CJD has never been shown to be transmitted by the transfusion of blood components or through plasma derivatives. The Centers for Disease Control and Prevention has characterized the risk of transmission from blood derivative products as "small and immeasurable" and "theoretical."

b. Notwithstanding the theoretical nature of the transmission risk, VA believed it had an ethical obligation to inform patients of the exposure to potentially contaminated products. VA voluntarily elected to notify all patients who may have received these blood component or plasma derivative products while under VA's care.

c. CJD is a rare degenerative disease of the brain which is one of the transmissible encephalopathies. It usually presents as a rapidly evolving dementia with myoclonic jerks and an abnormal EEG with periodic waveforms. CJD is rapidly progressive with death occurring in less than a year after onset of symptoms. In most cases, it is sporadic in occurrence with an incidence of approximately one case per year per million people. However, the illness has also been transmitted iatrogenically through the use of contaminated brain depth electrodes, corneal transplant, dual grafts and pituitary growth hormone therapy. The period between exposure and onset of disease in iatrogenic cases varies from sixteen months to over two decades. In cases associated with growth hormone therapy, the predominant symptoms were gait and balance problems. This is in contrast to sporadic cases where dementia and myoclonic jerks are the predominant symptoms.

d. Identification and evaluation of neurological symptoms in patients identified in the lookback notification is important to accurately differentiate potential CJD from other dementias commonly seen in VA's population, especially Alzheimer's disease and multi-infarct dementia. ...

b. Patient Evaluation, Autopsy, and Reporting. (1) Evaluations at the patient's request. At the request of the patient identified through VA's CJD lookback notification process, VA will provide a medical evaluation related to the lookback notification without regard to the eligibility status. The evaluation should be performed by the patient's primary care provider. In addition to other relevant evaluation criteria, the examination should include completion of the evaluation form in attachment B.

(2) Patients identified by the CJD lookback notification should be evaluated during all admissions using the evaluation outlined in the information sheet. Autopsy and reporting procedures outlined in the information sheet should be followed.

c. Blood Donation. There is presently no evidence to support the exclusion of patients identified by the lookback notification from blood donor programs unless a diagnosis of CJD has been made.

5. REFERENCES

a. FDA Talk Paper, Nov. 17, 1994. Voluntary Market Withdrawal of Blood Products. 
b. VA teletype dated 1/6/95. Voluntary Lookback Notification of Patients in CJD Component/Derivative Recall Process. 
c. FDA Hearing Transcript. Blood Products Advisory Committee. December 15, 1994. 
d. FDA "Dear Colleague" letter. March 29, 1995. 

Squirrel brains and CJD

Neurology Web-Forum
This response submitted by Bill Alford on 8/19/96.

Dr. Eric Weisman (a behavior neurologist) and myself have recently noted at least three patients who suddenly contracted CJD, and who admittedly had consumed the brains of squirrels. The findings were so revelent, that theniversity of Kyhas requested the brains of 100 squirrels be submitted for examination and research. I had a patient that died secondarily of CJD - she was super productive weeks prior to her diagnosis, then vegitated for less than a month before her demise. A sad situation!!!

Deaths from new CJD strain 'may run to thousands'

March 21 1997 MICHAEL HORNSBY Tomes

TENS of thousands of people may die from the new strain of Creutzfeldt-Jakob disease, the brain condition linked to BSE, scientists said yesterday. A year to the day since the Government admitted the likelihood of the link, the scientists told the first national conference of relatives of CJD victims that it was still impossible to predict how many people were likely to die. "We still have to say that there is a range from the odd 100 or so cases right through to tens of thousands," John Pattison, Professor of Medical Microbiology at University College London, said.

Dr Pattison, who heads the Government's advisory committee on CJD and BSE, said at Warwick University that the number of cases of the new strain of CJD arising in the next two or three years would be crucial. On March 20 last year, Stephen Dorrell, the Health Secretary, disclosed that ten cases of a variant of CJD with a distinctive brain pathology, had probably been caused by eating beef contaminated with BSE. Since then six more cases have been diagnosed.

Peter Smith, Professor of Tropical Epidemiology at the London School of Hygiene and Tropical Medicine, said: "It would be premature to assume that there would be no big epidemic of the new strain of CJD simply because no more than 16 cases had been confirmed so far." The key uncertainty, Professor Smith said, was the length of the incubation period. If this varied widely between individuals, there could be a surge in the number of people developing the disease. "If the number of cases in each of the next three years is roughly constant, and less than about 20 a year, a final size of the epidemic may well be a few hundred cases or fewer.

"If there are 25 or more cases confirmed this year, with a doubling or tripling in each of the following two years, that would be compatible with ... an epidemic of many thousands of cases."

Exposure of the public to contaminated meat is thought to have occurred in the mid-1980s before safeguards were introduced, suggesting an incubation period of about ten years in those who have died from the new strain. It differs from the classical form of the disease in attacking people under 40. The scientists said that if a typical incubation period turned out to be no more than ten to 15 years, the total cases of the new disease would be relatively low. But it was possible that those who had died had a genetic susceptibility which caused the disease to develop more quickly.

Dr Pattison said: "It may be that the cases we have seen so far are a small group of people with a short incubation period, for reasons we do not understand, and that the peak of the disease will be seen in later years." John Collinge, a specialist in molecular genetics at St Mary's Hospital Medical School in London, told the conference that the evidence for a link between BSE and new-variant CJD was "persuasive" and should be accepted as "a working hypothesis". No cure for CJD was in sight, Professor Collinge said. But some forms of treatment were worth exploring. The most hopeful was the use of drugs to influence the behaviour of the mutant prion protein thought to cause the disease. The disease appeared to be caused when this rogue protein attacked its healthy neighbours.

The conference was organised by the CJD Support Network, set up two years ago to help families of victims by the Alzheimer's Disease Society. Dot Churchill, whose son Stephen, 19, was the first victim of the new variant and who died in May 1995, said: "All the families want to see a full independent inquiry into the Government's handling of BSE."

Strang in attack on Hogg over BSE error

March 11 1997 Times ... BY POLLY NEWTON, POLITICAL REPORTER

DOUGLAS HOGG was criticised last night after he admitted giving inaccurate information to his Labour Shadow about the disposal of cattle infected with BSE. The mistake prompted Labour calls for a Commons statement from the Agriculture Minister on the Government's handling of the BSE crisis.

In a written parliamentary answer last Thursday, Mr Hogg told Gavin Strang, the Shadow Agriculture Minister, that three cattle suspected of having "mad cow" disease, had been buried rather than incinerated last year. Yesterday, the Ministry of Agriculture said the three carcasses had in fact been used for research.

European and government guidelines say carcasses infected with BSE should be burned to avoid any possibility of land or water supplies becoming contaminated. The Ministry of Agriculture has told Dr Strang that the remains of 6,120 BSE-infected cattle were buried at landfill sites before the practice was stopped, but it refuses to give details of where and when on the grounds of "disproportionate cost".

Dr Strang yesterday sent a letter to the minister demanding that information. The Labour backbencher Helen Jackson, who has put down several questions about BSE, also raised the issue in the Commons.

Court case threat over beef labelling

20 March 1997 ... From the European Parliament Socialist Group

The European Parliament's environment committee today unanimously backed legal action against EU farm ministers over labelling of beef. The Euro MPs hit out after ministers cut them out of the decision-making process and adopted plans for obligatory labels to come into force in 2000 as well as a passport system for cattle from 1998.

Committee chairman Ken Collins (Lab., Strathclyde East) said: The ministers have learned nothing from the mad cow crisis. They have gone back to their bad old ways of taking decisions in secret. Following BSE we need a maximum of openness -- not decisions behind closed doors.' Mr Collins wrote on behalf of the committee to EP President Jose Maria Gil Robles protesting at a flagrant abuse of democratic principles' and urging European Court action over the legal basis of the labelling decision.

German social democrat leader in the EP Willi Goerlach -- former farm minister of Hesse -- denounced the ministers' decision as a slap in the face for democracy.' Backing the call for court action, he said: The Council operates in line with the slogan that the European beef market comes before democracy.'

Socialist Group spokeswoman on the all-party consumer committee Dagmar Roth-Behrendt of Germany denounced the farm ministers' decision as totally disgraceful.' She added: This shows a lack of commitment to democracy which frightens me to death.

Commission President Jacques Santer may now be obliged to join with the Parliament in going to the Court. I have no doubt or hesitation in saying that court action is necessary.'

Britain agrees to label its beef

March 20 1997 MICHAEL HORNSBY ' Times

BRITAIN agreed to compulsory labelling for beef exported to other European Union countries yesterday, despite fears that this could lead to an indefinite Continental boycott of the British product. The climbdown came a year after the Government precipitated a ban on British beef by disclosing that a new strain of Creutzfeldt-Jakob disease (CJD), a fatal brain condition, was probably caused by eating beef infected with BSE. The ban, which halted beef exports worth more than 500 million a year, is still in force. Exports of by-products, such as tallow and gelatine, remain blocked.

In an unexpected move on the third day of talks between European Union farm ministers in Brussels, the Government withdrew its opposition to the labelling scheme which it had earlier said violated the single market's free-trade rules. Douglas Hogg, the Agriculture Minister, was summoned home from the meeting on a three-line whip on Monday night, leaving Britain represented only by officials when the labelling decision was taken. "By introducing a labelling system, European consumers will know exactly what products they are dealing with and where they are from," said Jozias Van Aartsen, the Dutch Farm Minister and the current president of the Council of Ministers.

The labelling scheme is intended to restore consumer confidence in beef. Sales have been worse hit in many Continental countries than in Britain, where consumption is now close to where it was before the Government's announcement on CJD. The labelling scheme will be optional for meat sold on a member state's home market, but compulsory from the start of the year 2000 for meat exported to other countries in the EU if those countries have adopted the labelling plan.

nvCJD victims family to sue the British Government

19 Mar 1997 Times

Peter Hall, one of the first 10 cases of nvCJD, died in February, 1996. The 20-year-old student was first given three kinds of anti-depressants, after showing initial psychiatric symptoms. In May 1995, his short-term memory was shot, and by early July, loss of balance was registred.

Frances Hall, Peter's mother, said to British press that their son became "completely apathetic - not talking, except in a whisper. And even when the television was on, just sitting in his wheelchair staring at the wall." (Daily Express, February 18, 1996) In August, he was incapable of feeding himself, incontinent, and sometimes hallucinatory. Peter Hall's elder brother John, 25, a student at Newcastle University, has now been granted legal aid to sue the UK Government for alleged negligence in failing to protect the public.

A coroner has ruled that Peter Hall probably contracted Creutzfeld Jakob Disease through eating contaminated beef. Geoffrey Burt, the Durham coroner, told an inquest last fall that he was convinced that BSE-contaminated beef was to blame:

"I am satisfied it is more likely than not that Peter contracted this disease prior to 1990 by eating some form of contaminated product, possibly a beef burger." (The Times, August 20, 1996)

The Department of Health answered the coroner:

"Our position remains unaltered. There is no scientific proof that human beings can contract CJD from beef." (The Times, 8/20, 1996)

Financial Times bashes MAFF

Financial Times of London ... Friday March 14 1997

Sometimes there is nothing else to be done. Britain's ministry of agriculture, fisheries and food should be razed to the ground . The rubble, sealed in lead-lined vessels, should be buried in the deepest trench below the North Sea. Its senior officials should be conscripted to countryside chain-gangs, tasked with repairing the ravages of industrial farming. Then we can begin a serious discussion about food safety.

Douglas Hogg and his comedy of ministers at MAFF are a distraction. Of course, it may be that history records their complacent incompetence as the final indignity of John Major's administration. mad cow disease, we know, was the catalyst for the disintegration of Mr Major's European policy. And this week's furore over filthy abattoirs does nothing to dispel the image of a government forever tossed in the thrall of events. Incompetence and cover-ups are lethal bedfellows.

The collapse of public confidence in food safety , however, has deeper roots than the bombastic bungling of Mr Hogg. It lies in the producer culture of MAFF, in Whitehall's ever-present obsession with secrecy and, above all, in a failure to understand how the world has changed since Britain ploughed its fields for victory. The weekly Hogg-roast in the House of Commons is great fun. It is not the answer.

Think about it. During the last few decades, there has been a revolution in the way Britain feeds itself. Scarcity has been replaced by abundance and, crucially, consumers have been robbed of the information required to make considered, rational choices.

MAFF is a creature of wartime food shortages, living still in the era of ration-books. It had a simple task, laid down in the 1947 Agriculture Act: to feed the nation. Its allies were the farmers. Consumers too would benefit, but only in the sense that higher production would fill the shelves of the nation's corner shops. Quantity not quality was what counted.....

Neurologists See Risk In Unlabeled Reagent

Lancet Volume 275, Number 5296, Issue of 3 January 1997, p. 17

Researchers in two neurology labs were surprised to learn last month that a reagent they were using--known as N-2--may have been derived from the blood of someone who died of Creutzfeldt-Jakob disease (CJD), a fatal nerve disorder that can be transmitted by direct contact with infected tissue. While many agree that the risk of transmission through lab materials is vanishingly small, the scientists remain concerned and say the reagent should have carried a warning label.

Neurologists at the Harvard-affiliated Children's Hospital in Boston, who asked not to be named, say they heard "through the grapevine" that the batch of N-2 they were using was possibly contaminated with the CJD agent. The researchers were particularly upset when they checked the N-2 labels and saw no mention of its human derivation. Since then, some scientists have contacted the manufacturer--Life Technologies of Grand Island, New York--and received replacement batches of N-2.

But Life Technologies has not put out a warning note. In a faxed response to Science's questions, official Keith Gittermann explained that the company was told by its supplier, the Bayer Corp., that the American Red Cross has withdrawn several lots of a blood product used to make N-2 because a blood donor was later diagnosed with CJD. But "outside experts," Gittermann says, have determined that the risk to lab workers is negligible; therefore, the company "has not recalled any lots of product."

This decision appears in line with an advisory put out by the Food and Drug Administration on 11 December. It says materials derived from possible CJD patients need not be quarantined or destroyed if they are "intended for further manufacturing into noninjectable products." However, FDA does say that such products should be labeled "biohazard."

Pathology and immunocytochemistry of a kuru brain

 Brain Pathol 7: 547-553 (1997)
 Hainfellner JA, Liberski PP, Guiroy DC, Cervenakova L, Brown P, Gajdusek DC, Budka H
Institute of Neurology, University of Vienna, Austria. 
We report here results of modern staining techniques including anti-prion protein (PrP) immunocytochemistry to a set of archival brain specimens of a 16 year-old male who died from kuru in 1967. Brain suspensions transmitted disease to chimpanzees and New World monkeys. The PrP gene is homozygous for valine at the polymorphic codon 129.

E Coli discovery in Hong Kong an isolated case, officials say

Mar 17, 1997 Nando

Hong Kong authorities said Monday the discovery of fatal E coli bacteria, which was found in beef in the territory this month, was an isolated case. Assistant Director of Health, Doctor Leung Pak-yin, told a news conference that the strain, blamed for the deaths of at least 11 people in Japan and nearly 20 in Scotland, was found in only two of the 55 samples taken, and no sickness had been reported.

The Department of Health started the investigation after raw minced beef on sale at a Japanese-owned Hong Kong supermarket was found to contain the bacteria. Leung said two samples from this source tested positive. The department also tested 53 other samples, from a slaughterhouse, as well as from wholesale and retail outlets, but said none of these tested positive.

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