Thursday, February 17, 2011

Imported cow from Switzerland aged over 30 months not tested for BSE

Imported cow aged over 30 months not tested for BSE

Wednesday 16 February 2011

The Agency has been notified that meat has entered the food supply from an Over Thirty Month (OTM) cow imported from Switzerland that had not been tested for BSE.

It is very unlikely that the cow was infected with BSE and, as specified risk material (SRM) was removed, any risk to human health is extremely low. SRM is those parts of an animal likely to be infected if the animal has BSE.

Nevertheless, according to BSE regulations the untested cow, the one slaughtered before and the two slaughtered after must not enter the food supply. Negative BSE test results were received for the 'one before' and 'two after'.

The cow had been imported into the UK in December 2009 and was slaughtered at William Taylor & Son Ltd's abattoir in Bamber Bridge, near Preston, on 15 October 2010 at just over 41 months of age. BSE testing is mandatory for cattle born in Switzerland if slaughtered for human consumption at over 30 months of age. The missing BSE test result was discovered on 17 November during routine cross checks of slaughter and BSE test data. By the time the failure was discovered, all of the associated carcasses had left the premises.

The Agency has established that the carcase of the Swiss-born bovine was sold as fresh meat and is likely to have been eaten. We also traced the batch of carcases that included the 'one before' and 'two after'. This showed that:

the majority of the meat was no longer in the food supply and is likely to have been eaten a small portion of the batch was found in cold storage and has since been destroyed a small portion of the batch was mixed with a large quantity of meat from other batches and used in Iceland's own brand 1.4kg steak pies with 'best before' dates of 23.11.11 and 26.11.11. Although any food safety risk from consuming these pies would be extremely low, Iceland has taken the decision to withdraw the affected pies from sale some had been exported to Ireland and the authorities there have been informed.

Sweden probes possible mad cow death Published: 16 Feb 11 07:00 CET | Double click on a word to get a translation Online:

Share A woman in her fifties from Värnamo in central Sweden died recently of the rare brain-debilitating disease Creutzfeldt-Jakob (CJD), Sveriges Radio (SR) reported on Tuesday.

.Fruits, vegetables may trigger child allergies (13 Feb 11) .Dengue fever rises among Swedes (6 Feb 11) .Sweden set for diabetes explosion: researchers (13 Nov 10) "A patient who had been sick for about six months died the other week. In the last month we established that she had Creutzfeldt-Jakob disease," infectious disease specialist Peter Iveroth told SR.

Health officials will now carry out an investigation into the woman's death to determine if it may have been caused by the variant of CJD associated with mad cow disease.

The woman's doctor, however, doubts there is any link between her death and Bovine Spongiform Encephalopathy (BSE), commonly known as mad cow disease.

"It's a very hard disease to diagnose and we aren't 100 percent certain of the diagnosis," the doctor said, adding the patient was not believed to have Variant Creutzfeldt-Jakob (vCJD).

Variant Creutzfeldt-Jakob (vCJD) is thought to be passed on to humans who have eaten meat contaminated with BSE.

Over 150 people around the world have died from the disease since it first emerged in 1995.

The other variant of CJD is unrelated to mad cow disease and non-transmissible.

"In this case it is our assessment that it is the older (non-transmissible) variant ... we have had that variant in Sweden for many years, before the occurrence of mad cow," the doctor said, describing sporadic CJD as "extremely unusual."

Swedish radio reported results for the patient's complete autopsy could take up to six months.

DR. Adriano Agutzy is one of the world's experts on prion diseases. He says he has ruled out most of the other explanations, and now his main working hypothesis is that at least some Sporadic C.J.D. in Switzerland could be another form of human Mad Cow Disease.


But this by no means excludes that B.S.E. may manifest itself in humans with different characteristics, and maybe B.S.E. in Switzerland is also different from B.S.E. in the U.K., and then variant C.J.D. will also be different. So I think from the U.K. experience, it's impossible to draw the conclusion that B.S.E. will only give rise to what we know as variant C.J.D.

BSE May Have Caused Some Cases Of CJD As Well As vCJD

By James Meikle The Guardian - UK 11-29-2

Measures to protect the public from bovine spongiform encephalopathy (BSE)-like diseases were called into serious question last night as researchers suggested that the BSE epidemic in cattle might have caused 2 separate fatal brain conditions, not one as thought. Their stunning conclusion, that eating cheap cow meat might be responsible for some cases of sporadic Creutzfeldt-Jakob disease (CJD), as well as variant CJD [abbreviated as vCJD or CJD (new var.) in ProMED-mail] or human BSE, will cause further reassessment of risks still posed by food and by infection through cross-contamination of surgical instruments and blood.

Experiments with mice by John Collinge and colleagues at University College London appear to strengthen the possibility that more animals, and by extension humans, can act as infective carriers of the killer diseases well before full-blown symptoms occur. They cast another shadow over the safety of sheep, reinforcing concerns that they were infected with BSE as well as scrapie, a disease not known to be harmful to humans, and that one disease had "masked" the other.

The Department of Health will have to reconsider the operation and size of its compensation scheme for families that have a human BSE sufferer. The money is not available for sporadic CJD victims or relatives, and there is no way yet that scientists can distinguish between cases that arose from spontaneous changing in the form of the prion protein linked to both diseases, and those that might be diet-related. The research, outlined in the journal of the European Molecular Biology Organisation (EMBO) [see comment below], could mean huge changes in the counselling of sporadic CJD patients and their families. Professor Collinge said last night: "When you counsel those who have the classical sporadic disease, you tell them that it arises spontaneously out of the blue. I guess we can no longer say that."

The results come from a continuing long term study of laboratory mice [engineered to express] the human prion protein, then injected in the brain with BSE-infected material. Some showed a molecular signature indistinguishable from human BSE; others a signature the same as that left by one of 3 forms of sporadic CJD.

Deaths from sporadic CJD reported in Britain in the 1990s peaked at around 60 a year between 1997 and 1999, far more than the 28 variant CJD cases in 2000, the worst year so far for variant CJD. Urgent reviews will be made as to whether some sporadic cases might be BSE-related, although hard evidence would be extremely difficult to find. The pattern of infectivity through the body is markedly different between sporadic CJD and variant CJD, meaning surveillance might now become more complicated.

There are no blood tests yet available. Hemophiliac patients who were treated with blood clotting factors by the Scottish blood transfusion service between 1987 and 1989 are being informed that one donor of blood for the concentrates had later contracted variant CJD. All hemophiliacs in Scotland are now treated with genetically engineered substitutes, but these are not universally available in England, where warnings of possible contamination have been issued several times. The Haemophilia Society last night said it would increase pressure on the Department of Health to ensure that alternatives were available to patients in England.

Gillian Turner, of the CJD Support Network, said: "This research will be welcomed by many families who have been affected by sporadic CJD. They have been concerned for a long time that it was diet-related." Lester Firkins, of the Human BSE Foundation, said: "This could throw all the work that people have been doing on modelling [the possible spread of diseases] up in the air again. You might see clustering of cases if you have more numbers."

Sporadic CJD was identified in 1920s. The disease affects mainly the middle-aged and elderly. The incidence is roughly one in a million, between 29 and 63 a year since 1990. The disease is found worldwide. Time between obvious symptoms and death is typically a few months. Until now it has been assumed that a normal prion protein in the brain spontaneously changed into abnormal dangerous form. BSE or similar diseases could now be factor

vCJD was identified in 1996, with the average age of onset in the late 20s. Found mainly in Britain: 129 cases with 117 deaths so far, plus 6 in France, 1 each in Italy, Ireland, Canada & the USA. Largely blamed on consumption of cheap cattle meat and offal during 1980s. Tough food controls were meant to have significantly reduced risk. Concern remains over whether sheep might also have become infected with BSE and entered the food chain. First symptoms often [imitate] psychological problems. Duration is often well over a year. Several physical symptoms are similar to sporadic CJD.,2763,849293,00.html


Research article Open Access

A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001–2004

Jessica Ruegger†1, Katharina Stoeck†2,3, Lorenz Amsler4,5, Thomas Blaettler2,6,

Marcel Zwahlen7, Adriano Aguzzi2, Markus Glatzel2,8, Klaus Hess1 and

Tobias Eckert*4,9

Address: 1Department of Neurology, University Hospital Zurich, Zurich, Switzerland, 2Institute of Neuropathology, University Hospital Zurich,

Zurich, Switzerland, 3Department of Neurology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Hamburg, Germany, 4Federal

Office of Public Health, Bern, Switzerland, 5CSL Behring, Bern, Switzerland, 6Bristol-Myers Squibb, Wallingford, CT, USA, 7Institute of Social and

Preventive Medicine, University of Bern, Bern, Switzerland, 8Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Hamburg-

Eppendorf, Hamburg, Germany and 9Swiss Tropical Institute, Basel, Switzerland

* Corresponding author †Equal contributors


Background: In 2001, the observed annual mortality from Creutzfeldt-Jakob disease (CJD) in Switzerland increased from less than 1.5 to 2.6 per million inhabitants. An underlying cause could not be identified.

Methods: To analyse potential risk factors for sCJD in Switzerland, close relatives of 69 sCJD-patients and 224 frequency age-matched controls were interviewed in a case-control study using a standardised questionnaire. 135 potential risk factors including socio-demographics, medical history, occupation and diet were analysed by logistic regression adjusting for age, sex and education.

Results: sCJD patients were more likely to have travelled abroad, worked at an animal laboratory, undergone invasive dental treatment, orthopaedic surgery, ophthalmologic surgery after 1980, regular GP visits, taken medication regularly, and consumed kidney. No differences between patients and controls were found for residency, family history, and exposure to environmental and other dietary factors.

Conclusion: Although some factors were significantly more frequent among sCJD-cases, this study did not reveal specific explanations for the increased incidence of deaths due to sporadic CJD observed in Switzerland since 2001. Results have to be interpreted with caution due to multiple testing and possible recall bias in association with a long incubation period. The most plausible reason for the increase in Swiss sCJD cases after 2000 is an improved case ascertainment. Therefore, underreporting of cases might well have occurred before the year 2001, and the "real" yearly incidence of sCJD might not be lower than, but rather above 2 per million inhabitants.


The third hypothesis to explain the increase in annual mortality rates from sCJD in Switzerland between 2001 and 2004 is a better case ascertainment. Over recent years in Europe, as a general tendency incidences have been rising, however not to such extent as in Switzerland. Given that our results do not support strong evidence for the hypotheses of a zoonotic or iatrogenic cause, ascertainment bias due to a heightened perception and awareness of the disease in physicians must be regarded as the most likely cause for the observed increase. One factor that might be jointly responsible for this increase might be altered reporting requirements in 1999. Since that year all suspected cases in Switzerland had to be reported. In this respect, also the role of chance must be considered, as it is possible that the observed increase of Swiss sCJD-deaths was due to random fluctuation. The rise in annual mortality rate from the years before 2000 to the period 2001– 2004, however, was statistically significant, and therefore, chance must be considered a less likely explanation. In the most recent years, the observed incidence in sCJD deaths (2005: 10; 2006: 13, 2007: 15) dropped to levels just slightly above those before 2000. When recent incidence data until 2007 are included, however, the rise in the annual mortality rate after 2001 was still significant. The sudden increase in 2001 and the slow decrease afterwards are well in line with the media coverage of the CJD topic in the respective years.

Worst-case scenario If BSE is the cause of Swiss sporadic CJD - the worst-case scenario - researchers have to work out how the cattle disease can cause two different forms of the human disease: variant CJD in Britain and elsewhere in Europe, and sporadic CJD in Switzerland.

This could be explained if British and Swiss cattle harboured different strains of the infectious prion protein - but preliminary experiments suggest that they are the same.

Alternatively, Swiss CJD could have leapt to humans through the injection of vaccines or medicines produced using serum made from cows, suggests Cornelia van Duijn of the European collaborative group on CJD incidence at the Erasmus University Medical School in Rotterdam, The Netherlands. Finding the source of the disease has "got to be priority number one for Europe", she says.

Researchers now hope to establish whether sporadic CJD came from BSE. They will use 'strain typing': infecting mice with the prions causing the two diseases and seeing if the symptoms match up. "It's the best way to establish or exclude any suspected link," says Moira Bruce of the Institute for Animal Health Neuropathogenesis Unit in Edinburgh, UK. These experiments will take at least a year.

Imperial College London

References 1.Glatzel, M. et al. Sharply increased Creutzfeld-Jakob disease mortality in Switzerland. Lancet 360, 139 - 141 (2002). | Article | ISI |


The scientific paper upon which this report is based is published in the current issue of the EMBO Joural, Vol. 21, No. 23, 6358-6368, 2002.

Title: BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein.

Authors: Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth and John Collinge. MRC Prion Unit and Department of Neuro-degenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK.

Abstract: Variant Creutzfeldt-Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrP\Sc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

In an interview with the London Times Health Editor, Professor Collinge stated that the number of cases of sporadic CJD cases had been increasing at about the same rate as the vCJD figures, and that the Swiss had reported recently a 2 to 3 fold increase in sporadic CJD. He also expressed concern that the transgenic mouse experiments had revealed instances of subclinical infection; although appearing normal in life, these mice when examined post-mortem exhibited extensive brain lesions. Subtle changes in mice are hard to detect, but changes might be more obvious in humans in terms of psychiatric symptoms. A subclinical form of CJD in humans would increase the hazard of transmission of disease via surgical instruments.

Professor Collinge stated that it was now a matter of urgency to conduct a large scale study of tonsil tissue in order to establish how widespread CJD infection is. He said that he remained concerned about the ultimate size of the CJD epidemic. The tonsil study that has been carried out so far employed a relatively crude test. He stated that new cases of kuru, the neurological disease caused by cannibalism some 50 years ago are still coming to light. Consequently these are very early days for a human prion epidemic, which could have a 30 year incubation period. - Mod.CP]

[see also: CJD, increased incidence - Switzerland 20020714.4756 CJD, long incubation period 20020612.4478 CJD, surgical instrument re-use - UK 20021030.5671 CJD, suspected cluster - USA (Wisconsin) 20020721.4827 CJD (new var.) - UK: 10th Annual Report 20020711.4727 CJD (new var.) - UK: update Sep 2002 20020908.5258 2001 ---- CJD, death rate increase - Switzerland 20011225.3110 CJD, surgical transmission? - Canada (Ontario) 20010512.09 2000 ---- CJD, possible surgical transmission - USA (Louisiana) 20001027.1872] ..................cp/jw

Wednesday, July 28, 2010

Atypical prion proteins and IBNC in cattle DEFRA project code SE1796 FOIA Final report


"All of the 15 cattle tested showed that the brains had abnormally accumulated prion protein."

Saturday, February 28, 2009

NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009

SEAC 102/2

Thursday, December 23, 2010

Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009 Volume 17, Number 1-January 2011

Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

Monday, November 22, 2010

Atypical transmissible spongiform encephalopathies in ruminants: a challenge for disease surveillance and control



October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,


Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.


Sat Apr 28, 2007 07:10


i have been working on something for a while 'the big lie', the following data to be added in, but i thought due to what i think the importance of this is, i thought i would go ahead and put this out now for those that might be interested. ........tss




1. This submission, which has been agreed with colleagues in HEF(M). alerts PS(L) to the contents of the forthcoming annual report of the CJD Surveillance Unit and presents options for publication. It also highlights concern over the presentation of results which could be misrepresented by the media and others as evidence of a lilnk between CJD and the consumption of veal. ...


2. PS(L) is invited to agree the recommendation at para 13.


7. The main findings in the case-control study were STATISTICALLY SIGNIFICANT ASSOCIATIONS BETWEEN CONSUMPTION OF VEAL OR VENISON AND THE DEVELOPMENT OF CJD (INCREASED RISKS OF 2-13x). There was also evidence of a dose-response relationship between dietary exposure and development of the disease. (Last year's findings showed an apparent association between eating black pudding and risk of CJD which was neither statistically significant nor biologically plausible - interestingly, this has not been (replicated was marked out with something i cannot read), and then this complete sentence was marked through to be replaced ;


IP PS(L) wishes to probe this further we think it best to explain the matter VERBALLY. The problem is how to present the findings in this year's annual report in a way which avoids unnecessary public alarm and limits the scope for media scare stores. (or the facts...TSS)

This is of considerable concern given recent development. In particular Ministers will be particularly concerned about the European dimension given the recent troubles with the Germans.

9. DH doctors advise - and we understand Dr Wills agrees - that the association the study found between the developments of CJD and veal consumption cannot be regarded as demonstrating a causal relationship or give any reason to change the advice that eating beef and veal is safe. IF PS(L) wishes to probe this further we think it best to explain the matter verbally. The problem is how to present the findings in this year's annual report in a way which avoids unnecessary public alarm and limits the scope for media scare stories.

Next steps ...

snip... full text ;


7. The main findings in the case-control study were STATISTICALLY SIGNIFICANT ASSOCIATIONS BETWEEN CONSUMPTION OF VEAL OR VENISON AND THE DEVELOPMENT OF CJD (INCREASED RISKS OF 2-13x). There was also evidence of a dose-response relationship between dietary exposure and development of the disease. (Last year's findings showed an apparent association between eating black pudding and risk of CJD which was neither statistically significant nor biologically plausible - interestingly, this has not been (replicated was marked out with something i cannot read), and then this complete sentence was marked through to be replaced ;

see watered down report here ;

Seven main threats for the future linked to prions

The NeuroPrion network has identified seven main threats for the future linked to prions.

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed. Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat


Wednesday, March 31, 2010

Atypical BSE in Cattle

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.


please see all seven threats listed in the USA, and more...FULL TEXT ;

Thursday, August 12, 2010

Seven main threats for the future linked to prions

Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

Thursday, February 17, 2011

Environmental Sources of Scrapie Prions

Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

Sunday, April 18, 2010


Wednesday, February 16, 2011



Our findings demonstrate that cervid PrPSc, upon strain adaptation by serial passages in vitro or in cervid transgenic mice, is capable of converting human PrPC to produce PrPSc with unique biochemical properties, likely representing a new human prion strain. The newly generated CWD-huPrPSc material has been inoculated into transgenic mice expressing human PrP to study infectivity and disease phenotype and this data will be published elsewhere. ...end


Wednesday, September 08, 2010


Friday, February 11, 2011

Creutzfeldt-Jakob disease (CJD) biannual update (2010/1) Emerging infections/CJD



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