Sunday, December 2, 2012

CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’

CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’
 
 
 
 
 
 
10/31/2012




Managing Future Risks from BSE and other Emerging Issues




Summary Report of a Foresight Exercise




Background Canada’s cattle industry experienced a severe shock with the finding of several cases of Bovine Spongiform Encephalopathy (BSE) in native-born cattle starting in 2003. The first case in a Canadian-born animal was reported in May 2003. Since then, a total of 19 cases of BSE have been found in Canadian cattle, with the latest case detected in February 2011 in a dairy cow born in 2004.



Lucrative export markets proved fragile resulting in very significant economic losses despite the industry’s success maintaining reasonable stability in domestic markets. The cost of market closures and the subsequent actions taken to sustain the Canadian cattle and beef industries has been variously estimated to exceed $10 billion.



Seventeen of Canada’s BSE cases (10 dairy, three beef-cross and four beef) were of the “classical” type of BSE (c-BSE), the form responsible for the vast majority of cases in most BSE-affected countries. The other two (both beef animals) were “atypical” cases as they occurred in significantly older animals, but had slightly different biochemical characteristics from the classic form. Observations in other BSE-affected countries and ongoing research indicate that that atypical cases might represent spontaneous forms of BSE. Uncertainties remain regarding the implications of these atypical cases for both the human food supply and the animal feed chain.



While the situation has improved significantly, Canada faces ongoing challenges to the design and costs of its BSE prevention programs and to international recognition of its BSE status:



1. Two major beef exporting countries (Mexico and the USA) that now share Canada’s “controlled risk” status for BSE might advance to “negligible risk” status some years before Canada, thereby gaining trade advantages. Brazil, the world`s largest beef exporting country, recently was reclassified by the World Organization for Animal Health (OIE) from ``controlled risk`` to ``negligible risk`` status for BSE.



2. There are unresolved conflicts between pressures on the one hand to harmonize feed control standards with the USA in order to stay cost-competitive and, on the other hand, to accelerate measures to gain international recognition of Canada’s BSE status as ``negligible risk``:



a. Canada’s regime to safely dispose of specified risk materials (SRM) is more demanding and costly than that of the USA, but it is still less stringent than those of the most demanding countries (EU, Japan).



b. Canada’s feed control regime is less rigorous than some others (EU, Japan) and has been criticized by the OIE for its lack of a testing program and for limited stringency of rendering practices.



3. BSE surveillance in Canada has declined from 55,000 animals tested in 2007 to about 35,000 animals per year more recently. Is this level sufficiently robust to support the case that will need to be made for recognition of Canada as a “negligible risk” country?



4. There remain uncertainties about other prion diseases that are known to occur in Canada: chronic wasting disease (CWD) and scrapie. In the case of CWD, uncertainties about the potential host range combined with the ongoing spread of the CWD agent in the environment of Western Canada may provide arguments for trading partners who might wish to maintain or raise protective barriers.





The Foresight Exercise To assist in planning for the management of these and future risks to the industries of similar magnitude, two Workshops were convened to explore possible future scenarios using a Foresight process. Participants were drawn from the cattle and beef industries, academia and governments, including regulatory agencies. Further information on these Workshops is provided in Appendices 1 to 4.





snip...




Appendix 4: Scenario Stories




The following is a summary of the scenarios developed by Workshop 1 participants.





SCENARIO 1: ‘BLUE SKIES’


Sustainable Production and Robust Markets


Canada


Science


- Affirmed the health benefits of beef in balanced diets.


- Produced a cost-effective test for BSE and other diseases that is widely used, providing input for a nation-wide data system. The system enables comprehensive surveillance of the health of Canada’s herd, increasing consumer confidence.


- Developed and validated more cost-effective methods to safely manage SRM


- Government and private investment from industries, led to significant breakthroughs in TSE (transmissible spongiform encephalopathy) science and genetic research in feed grains, forage and cattle.




SCENARIO 2: ‘BOVINE SITUATION EXTREME’


Unsustainable Production and Fragile Markets


Canada


Science


- Financial support has weakened due to financial problems of Government and the industries, leading to sharp curtailments of research.


- Work on a vaccine for the variant TSE continues feverishly.


- No major scientific breakthroughs related to the prevention and cure of prion diseases occurred.





SCENARIO 3: ‘THE THIN STEMMED GLASS’


Sustainable Production and Fragile Markets


Canada


Science


- BSE is not linked to classical CJD, but a TSE is found that is linked to Alzheimer’s disease.


- Science breakthroughs in neurodegenerative diseases.


- New product development continues.





SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’


Unsustainable Production and Robust Markets


Canada


Science


- Experimental evidence indicates that abnormal prions may persist for undetermined periods of time in buried materials.


- A new prion disease has occurred in cattle, possibly originated from CWD. Research funds are not available to investigate its nature and origin.


- The origin, transmission and prevalence of atypical BSE remain unclear.


- L-type atypical BSE has been demonstrated to be transmissible to humans.


- New testing procedures indicate the presence of sub-clinical carriers in the cattle population.


- New scientific knowledge provides no evidence that CWD is transmissible from Cervids to humans.









Tuesday, November 6, 2012


Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update








SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012


Summary Report BSE 2012


Executive Summary







Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012







Saturday, August 4, 2012


Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation








Tuesday, November 02, 2010


BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992








Thursday, June 14, 2012



R-CALF USA Calls USDA Dishonest and Corrupt; Submits Fourth Request for Extension



R-CALF United Stockgrowers of America








Monday, June 18, 2012 R-CALF


Submits Incomplete Comments Under Protest in Bizarre Rulemaking “Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products”







CENSORSHIP IS A TERRIBLE THING $$$





Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$



THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$








Thursday, February 10, 2011


TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31







Wednesday, August 11, 2010


REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Thursday, August 19, 2010


REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Friday, March 4, 2011


Alberta dairy cow found with mad cow disease








Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA







Wednesday, June 13, 2012


MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD







Wednesday, April 4, 2012


Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68






Thursday, February 16, 2012


Bovine Spongiform Encephalopathy BSE


31 USA SENATORS ASK PRESIDENT OBAMA TO HELP SPREAD MAD COW DISEASE 2012







Wednesday, February 16, 2011


IN CONFIDENCE


SCRAPIE TRANSMISSION TO CHIMPANZEES


IN CONFIDENCE







Sunday, April 18, 2010


SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010







Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas






Thursday, February 23, 2012


Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012







Wednesday, April 4, 2012


20120402 - Breach of quarantine/Violation de la mise en quarantaine of an ongoing Scrapie investigation







Monday, June 11, 2012


another atypical Nor-98 Scrapie case documented in Canada for 2012







Monday, April 25, 2011


Experimental Oral Transmission of Atypical Scrapie to Sheep


Volume 17, Number 5-May 2011 However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing finding that the biochemical properties of the resulting PrPSc have changed on transmission (40).







***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.







*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.


119








*** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.







Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.


Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.


(i) the unsuspected potential abilities of atypical scrapie to cross species barriers


(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier


These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.







Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues







Monday, November 30, 2009


USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE




I strenuously urge the USDA and the OIE et al to revoke the exemption of the legal global trading of atypical Nor-98 scrapie TSE. ...TSS





Thursday, November 29, 2012


Chronic wasting disease on the Canadian prairies








Tuesday, November 13, 2012



PENNSYLVANIA 2012 THE GREAT ESCAPE OF CWD









Wednesday, November 14, 2012


PENNSYLVANIA 2012 THE GREAT ESCAPE OF CWD INVESTIGATION MOVES INTO LOUISIANA and INDIANA








Friday, November 09, 2012


Chronic Wasting Disease CWD in cervidae and transmission to other species











OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles


Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA



Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.



Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.



Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.


In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.



Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.


The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.








Wednesday, March 28, 2012


VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $








*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.





AS OF AUGUST 2012 ;


CJD UPDATE USA


1 Listed based on the year of death or, if not available, on year of referral; 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; 3 Disease acquired in the United Kingdom; 4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case; *** 5 Includes 8 cases in which the diagnosis is pending, and 18 inconclusive cases; *** 6 Includes 10 (9 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded. *** The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2224 cases of sporadic Creutzfeldt-Jakob disease (sCJD).








Tuesday, November 6, 2012


Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update








Friday, November 23, 2012


sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA


snip...


Greetings BSE-L members et al, and others,


Confucius is confused again on the infamous ‘classification pending sporadic creutzfeldt jakob disease’ cpsCJD, (because nvCJD has been ruled out).


Confucius is confused about why the increase of these cpsCJD cases in the USA and Canada which we have been seeing, but I saw no reports in the UK surveillance reports of the infamous North American Classification Pending Sporadic Creutzfeldt Jakob disease cases.


if truly a supposedly sporadic spontaneous disease, would you not see these cpsCJD cases popping up all over the world in random ???


or, could these cpsCJD cases be of a North American zoonotic or iatrogenic from North American zoonoses sub-clinical source ???


or both ???


with so many documented Transmissible Spongiform Encephalopathy TSE prion disease in so many different species here in North America, and consumption there from, I believe that this should be at the forefront of research. ...



Confused Confucius...flounder



snip...see full text ;





Friday, November 23, 2012



sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA







Saturday, October 6, 2012


TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2011 Annual Report







Wednesday, May 16, 2012


Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?


Proposal ID: 29403






see the Duke, Pa, Yale, and Mexican study here, showing the misdiagnosis of CJD TSE prion disease as Alzheimers ;







Monday, August 20, 2012



CASE REPORTS CREUTZFELDT-JAKOB DISEASE: AN UNDER-RECOGNIZED CAUSE OF DEMENTIA









Thursday, August 02, 2012


CJD case in Saint John prompts letter to patients


Canada CJD case in Saint John prompts letter to patients







layperson




TSS

Friday, November 30, 2012

PROPOSED DECISION TO STOP BSE TESTING OF HEALTHY CATTLE SLAUGHTERED FOR HUMAN CONSUMPTION FSA 12/12/04 Open Board – 11 December 2012

PROPOSED DECISION TO STOP BSE TESTING OF HEALTHY CATTLE SLAUGHTERED FOR HUMAN CONSUMPTION


Food Standards Agency FSA 12/12/04 Open Board – 11 December 2012



david.carruthers@foodstandards.gsi.gov.uk



1 SUMMARY 1.1



The current system for monitoring Bovine Spongiform Encephalopathy (BSE) in cattle includes BSE testing of all healthy cattle aged over 72 months slaughtered for human consumption. The European Commission is proposing to allow qualifying Member States (MS), including the UK, to decide to stop testing these cattle. 1.2 The Board is recommended to agree to advise Ministers that it would be acceptable on grounds of negligible risk to consumers and proportionality to stop BSE testing of all healthy cattle aged over 72 months slaughtered for human consumption in the UK.



snip...




Food Standards Agency FSA 12/12/04 Open Board – 11 December 2012 16



Annexe F



Ability of BSE test to detect different forms of BSE



1. Testing is carried out using approved commercial diagnostic tests for the disease-specific, abnormal form of the prion protein (PrP). A sample of the brainstem of the animal at the level of the obex is required to be used for the test. Studies have established that in Classical BSE abnormal PrP deposition in the brainstem first occurs at the obex level, where a substantial amount of this protein accumulates during the late incubation phase. Consequently, as described in the EFSA Report, targeting the obex for testing is considered to be the most sensitive approach for detecting cases of Classical BSE.



*** 2. In relation to the two forms of atypical BSE (H and L types) that have been reported, the EFSA Report points out that, while these conditions are detectable by the current approved tests, a full evaluation of their performance in detecting atypical BSE cases has not been carried out and the suitability of the obex as the target tissue for early and sensitive detection of these conditions remains largely unknown.



*** 3. The impact of stopping testing of healthy slaughtered cattle on the ability of the monitoring system to detect the emergence of a hypothetical new type of TSE disease in cattle is unknown, since the characteristics of any such new TSE disease and whether the current testing system would detect it are necessarily unknown. By definition, novel TSEs with forms of PrP substantially different from the currently-recognised forms may evade the existing tests.











RCVS Response Consultation on BSE Testing


12 May 2011


53kb PDF




Joint consultation by Department for Environment, Food and Rural Affairs, Welsh Assembly Government and Food Standards Agency on proposals for changes to BSE testing of cattle slaughtered for human consumption 1. The following response is made on behalf of the Royal College of Veterinary Surgeons (RCVS). The RCVS is the regulatory body for veterinary surgeons in the UK. The role of the RCVS is to safeguard the health and welfare of animals committed to veterinary care through the regulation of the education, and ethical and clinical standards, of veterinary surgeons and nurses, thereby protecting the interests of those dependent on animals, and assuring public health. It also acts as an impartial source of informed opinion on relevant veterinary matters.


2. The RCVS considers that in relation to Bovine Spongiform Encephalopathy (BSE), monitoring and controlling the epidemic and protecting the public and animals from exposure to the agents that cause BSE are of paramount importance. Nevertheless, it is important that controls and testing regimes are appropriate and proportionate to the current risks of the disease.


3. The RCVS commends the launch of this non-formal consultation before adopting the proposed changes to BSE testing made possible by the amendment to Commission Directive 719/2009/EC and the decision of Ministers to seek the opinion of the Food Standards Agency (FSA) and independent expert advice of the Spongiform Encephalopathy Advisory Committee (SEAC) on any potential risks before adopting changes.


4. SEAC comprises experts at the forefront of Transmissible Spongiform Encephalopathy (TSE) research and has provided independent expert scientific advice to the Government in a transparent fashion for 20 years. The RCVS therefore supports the recommendations and observations of SEAC in relation to the proposed changes to the BSE testing of healthy cattle and strongly urges the Government to take account of its views.


5. SEAC advises that in the short-term the additional risk to human health of raising the age for the slaughter of healthy cattle to 72 months is insignificant and notes that the Veterinary Laboratories Agency (VLA) modelling concurs with the conclusion. In presenting their advice, however, SEAC sounds an important note of caution: that such conclusions are only valid if the prevalence of BSE in UK cattle continues to fall or remains the same and that the validity of the analysis depends upon the quality of surveillance and its ability to detect any re-emergence of the disease.


6. The RCVS therefore considers it imperative that the testing and surveillance programme is kept under review and that measures are implemented to ensure that the programme is capable of identifying any changes in BSE prevalence. To this end, the RCVS considers that provision should be made for the random testing of apparently healthy animals being slaughtered between the ages of 48 months and 72 months to verify the science behind the new testing regime and to ensure that health of the public is safeguarded appropriately. In this regard, the RCVS also supports the proposal of SEAC that the VLA model should be used ‘to examine a range of hypothetical rates of increase of BSE infection and the ability of current surveillance measures to detect the change’.


7. As SEAC notes, changes in the prevalence of BSE are most likely to be detected in fallen stock and casualty animals. The RCVS therefore considers that assessments should be made to ensure the adequacy and sensitivity of such testing for identifying any emerging trends in BSE.


8. The RCVS regards the removal of Specified Risk Material (SRM) as the primary means via which the public are protected from agents that cause BSE. Whilst there are no proposals to change SRM measures, the RCVS wishes to express its strong belief that current SRM measures should not be relaxed.


9. Given recent evidence of fraud relating to cattle that are found to be TB test-positive and the Government’s moves to tackle this by requiring that DNA tags are applied immediately to cattle that test positive for TB, the RCVS considers that provisions need to be made to ensure that any fraud in relation to BSE testing is tackled and that cattle over the age of 72 months are not passed off as being younger at the time of slaughter. Such fraud could be addressed by linking the passport and ear tag to some form of biological indicator of age such as the ossification of the vertebrae or appropriate dental changes.


10. If you require any clarification on the above comments, please do not hesitate to contact me. Alternatively, representatives from the RCVS would be happy to meet with you to discuss and expand upon our position


Anthony Roberts


RCVS Policy and Public Affairs Officer








Tuesday, November 02, 2010


BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992






Beckett admits BSE test blunder



Margaret Beckett last night admitted she blundered over how the Government announced the news that scientists seeking evidence of BSE in sheep had actually used cows' brains. The original statement about the bungled research failed to explain one of the gravest errors ever made by Government scientists researching the disease, and did not even mention the word 'cow'. As opposition MPs demanded an explanation of how £217,000 had been wasted, the Environment, Food and Rural Affairs Minister conceded that the way she had announced the mix-up was ' perhaps in error'. Mrs Beckett said: 'I gave instructions, perhaps in error, that the statement drafted to explain what we knew - and that was a limited amount - should be put into the public domain as soon as possible.' Shadow Minister Peter Ainsworth said the handling of the publication of the error had been 'despicable' and called on whoever made the decision to be sacked. Fingers had pointed to Lucian Hudson, the Government's new Director of Communications, who oversaw the release. But last night it emerged that Mrs Beckett had overruled advisers and drafted a statement herself, and insisted it be released late on Wednesday. A Whitehall insider said: 'She should have waited until the morning and held a full specialist briefing. But she was nervous that the news would leak and the department would look as if it had tried to cover something up. News releases are never done by one person and scientists were consulted on this one. But the wording was Mrs Beckett's own.' Mrs Beckett yesterday wrote to Mr Ainsworth to 'set the record straight'. If she had not made the news immediate, she said, his party would have 'accused me of trying to conceal the information for as long as possible'. The Department for Environment, Food and Rural Affairs (Defra) was first alerted to the error on Wednesday morning. A Government laboratory had been sent a sample of the brains by the Institute of Animal Health, which was carrying out the research, to ensure there was no crosscontamination. There was horror when it appeared the scientists had been studying cows' brains rather than sheep. There followed an afternoon briefing with officials to inform them of the blunder, before Ministers were told in the early evening. Mrs Beckett said: 'We immediately put in hand a scientific audit, which people are carrying out this weekend, to try to find out what happened, what went wrong and whether there's still anything to learn from the experiment.' Government scientists have called for immediate new research into the risks of eating British lamb, although the Food Standards Agency has not changed its advice, which is that the risk of BSE in sheep 'remains theoretical'. Last night Liberal Democrat agriculture spokesman Colin Breed said: 'Mrs Beckett must now outline what she intends to do to allay fears about potential BSE in sheep. She has some awkward questions to answer over Defra's scientific policy.'









Wednesday, December 21, 2011


Potential mad cows that entered food supply without being tested for BSE 2011: UK END OF YEAR REVIEW







Thursday, September 6, 2012


UK Breaches of BSE controls in consignments of beef 2011 communications missing four reports








ATYPICAL BSE AND SPORADIC CJD NOVEMBER-DECEMBER 2012



Dispatch


Transmission of New Bovine Prion to Mice


Thierry G.M. Baron* , Anne-Gaëlle Biacabe*, Anna Bencsik*, and Jan P.M. Langeveld† Author affiliations: *Agence Française de Sécurité Sanitaire des Aliments, Lyon, France; †Central Institute for Animal Disease Control, Lelystad, the Netherlands


Abstract


We previously reported that cattle were affected by a prion disorder that differed from bovine spongiform encephalopathy (BSE) by showing distinct molecular features of disease-associated protease-resistant prion protein (PrPres). We show that intracerebral injection of such isolates into C57BL/6 mice produces a disease with preservation of PrPres molecular features distinct from BSE.



Conclusions Our data show that the recently identified bovine H-type isolates involve an infectious agent that can induce development of a disease across a species barrier, while maintaining the specific associated PrPres molecular signature. This evidence in favor of a new bovine prion strain in cattle suggests that BSE is not the only transmissible prion disease in cattle. The origin of such cases has not been determined (7). These cases suggest either the existence of alternative origins of such diseases in cattle or phenotypic changes of PrPres after infection with the BSE agent. However, based on analysis of molecular features of prion diseases in cattle, this situation is similar to that in humans (5), in which different subtypes of sporadic Creutzfeldt-Jakob disease agents are found.



Dr Baron is head of the Unité Agents Transmissibles Non Conventionnels, Agence Française de Sécurité Sanitaire des Aliments, in Lyon. His research focuses on diagnosis of prion diseases of ruminants and characterization of the disease-

associated prion protein and infectious agents, with particular emphasis on atypical forms of these diseases.








Tuesday, November 6, 2012


Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update







SPORADIC CJD RISING IN MANY COUNTRIES, INCLUDING THE U.K. ??? ZOONOSIS ???





SEE STEADY RISE OF SPORADIC CJD IN THE U.K. WHERE IN 1990, IT WENT FROM 28 SPORADIC CJD CASES, TO 2011, WITH A RECORD HIGH OF 90 CASES OF SPORADIC CJD, THE MOST EVER DOCUMENTED IN ONE YEAR IN THE U.K. ...TSS







SEE STEADY RISE OF SPORADIC CJD IN THE U.S.A. WHERE IN 1996 AND BEFORE, THERE WERE 28 CASES OF SPORADIC CJD, TO 2010, WITH A RECORD HIGH OF 216 CASES OF SPORADIC CJD, THE MOST EVER DOCUMENTED IN ONE YEAR IN THE U.S.A., WITH 2011, COMING IN A CLOSE SECOND, AT 214 CASES OF SPORADIC CJD, AND STILL COUNTING. ALSO, MOST DISTURBING, IS THE TYPE DETERMINATION PENDING CLASSIFICATION OF CJD, WHICH I CALL cpsCJD, ON THE RISE AS WELL, AND OF COURSE, THE INFAMOUS VPSPr SPORADIC CJD, IS ALSO RISING, PLEASE SEE MORE BELOW ;









SEE ALSO CANADA WITH THE SAME SPORADIC TYPE CJD PROBLEMS, AND RISE THERE FROM ;



3. Final classification of 48 cases from 2009, 2010, 2011 and 2012 is pending.








Friday, November 23, 2012


sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA







Alternatively, other yet unknown routes of transmission or genetic determinants must be considered. This said, H-type BSE might persist after eradication of C-type BSE. What are the implications of this scenario? Studies in mice provided experimental evidence that H-type BSE may shift its disease phenotype to that of C-type BSE (3) upon transmission. It has therefore been hypothesized that the C-type BSE epidemic originated from spontaneously occurring H-type BSE cases. If this was the case there would be a constant risk that C-type BSE re-emerges in the cattle population once the feed-ban is discontinued. Consequently, some measures of disease control would need to be maintained indefinitely. Since the standards for the determination of a countries’ BSE risk status currently do not differentiate between BSE subtypes (28), BSE risk assessments will certainly need to take such considerations into account. This highlights the need for continuing research into the relationship between classical and atypical BSE variants to provide the scientific basis for future disease surveillance and control policies.








Saturday, October 6, 2012


TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2011 Annual Report







Subject: Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate



Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate



Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1



1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America



Abstract



Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.



Methodology/Principal Findings



Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.



Conclusion/Significance




Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, *** the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.




Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017



Editor: Neil Mabbott, University of Edinburgh, United Kingdom



Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008



Copyright: © 2008 Comoy et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.



Funding: This work has been supported by the Network of Excellence NeuroPrion.



Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad.



* E-mail: emmanuel.comoy@cea.fr



snip...




In summary, we have transmitted one atypical form of BSE (BASE) to a cynomolgus macaque monkey that had a shorter incubation period than monkeys infected with classical BSE, with distinctive clinical, neuropathological, and biochemical features; and have shown that the molecular biological signature resembled that seen in a comparatively uncommon subtype of sporadic CJD. We cannot yet say whether BASE is more pathogenic for primates (including humans) than cBSE, nor can we predict whether its molecular biological features represent a clue to one cause of apparently sporadic human CJD. However, the evidence presented here and by others justifies concern about a potential human health hazard from undetected atypical forms of BSE, and despite the waning epizoonosis of classical BSE, it would be premature to abandon the precautionary measures that have been so successful in reversing the impact of cBSE. We would instead urge a gradual, staged reduction that takes into account the evolving knowledge about atypical ruminant diseases, and both a permanent ban on the use of bovine central nervous system tissue for either animal or human use, and its destruction so as to eliminate any risk of environmental contamination.








Monday, July 9, 2012


Spread of Classic BSE Prions from the Gut via the Peripheral Nervous System to the Brain


Study Finds "Mad Cow Disease" in Cattle Can Spread Widely in Autonomic Nervous System before Detectable in the Central Nervous System






Thursday, August 12, 2010


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


snip...







EFSA Journal 2011 The European Response to BSE: A Success Story


This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;


Monday, October 10, 2011


EFSA Journal 2011 The European Response to BSE: A Success Story


snip...


EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.


snip...










see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;








Saturday, May 26, 2012


Are USDA assurances on mad cow case 'gross oversimplification'?


SNIP...


What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”


The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. "(The agency) has no foundation on which to base that statement.”


“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State.


In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said


The argument about feed is critical because if feed is the cause, not a spontaneous mutation, the California cow could be part of a larger outbreak.



SNIP...







Monday, August 6, 2012


TAFS BSE in USA August 6, 2012


BSE in USA







Sunday, May 6, 2012


Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012 IOWA State University OIE






2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006






Comments on technical aspects of the risk assessment were then submitted to FSIS.


Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:






Owens, Julie


From: Terry S. Singeltary Sr. [flounder9@verizon.net]


Sent: Monday, July 24, 2006 1:09 PM


To: FSIS RegulationsComments


Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)


Page 1 of 98







FSIS, USDA, REPLY TO SINGELTARY









U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001











2012 atypical L-type BSE BASE California reports


Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012







SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012


Summary Report BSE 2012


Executive Summary







Saturday, August 4, 2012


Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation







CENSORSHIP IS A TERRIBLE THING $$$


Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$


THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$





Thursday, February 10, 2011


TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31







Wednesday, August 11, 2010


REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Thursday, August 19, 2010


REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Friday, March 4, 2011


Alberta dairy cow found with mad cow disease







Reasons for the New Regulation Order No. 23 (as well as amending Order No. 149) of the State Committee for Veterinary Medicine name BSE as the reason for new import requirement. The legal title for Order No. 23 is "On Urgent Measures Aimed at Prevention and Elimination of BSE and Other Prion Infections in Cattle”. Neither Order explains how the threat of introduction of BSE can be addressed through the inspection of producers of all products of animal origin including fish, dairy products, poultry and pork. It is not clear what other concerns are addressed through the proposed inspections. Formal Notification of Trading Partners On August 3rd, Ukraine's Notification and Enquiry Point issued a legal Notification G/SPS/N/UKR/3/Rev.1 found on the Official WTO Website (Committee on Sanitary and Phytosanitary Measures)








Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas







Monday, November 30, 2009


USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE








ANY RELAXING OF ANY BSE TESTING RULES WOULD NOT BE BASED ON SOUND SCIENCE, BUT BASED ON INDUSTRY LED SCIENCE AND MONEY $$$



we now know that indeed atypical BSE is transmissible to cattle and other species, and atypical BSE have been documented in older cattle to date. so relaxing any BSE testing on older cattle would be a huge step backwards, and could risk everything that has been done over the past 27 years to try and eradicate BSE. ...




TSS

Wednesday, November 14, 2012

Prionics: New test to combat scrapie and mad cow disease in sheep and goats

MEDIA RELEASE


Schlieren-Zurich, November 14, 2012


Prionics: New test to combat scrapie and mad cow disease in sheep and goats


An innovative new test is now available to detect TSE (scrapie and mad cow disease) in sheep and goats. With the Prionics®-Check PrioSTRIP SR, Prionics brings to the market the first test to achieve high diagnostic sensitivity on preclinical TSE cases. The test was approved today by the European Commission for use in TSE testing in small ruminants*. The Prionics®-Check PrioSTRIP SR can detect scrapie earlier than conventional tests and will therefore significantly contribute to TSE control programs.


The Prionics®-Check PrioSTRIP SR was extensively tested in a laboratory evaluation directed by the European Commission. The product fulfilled all the requirements of the EU evaluation and has now been approved as a rapid test for detection of TSE in the central nervous system of small ruminants (i.e. sheep and goats).


Superior detection of preclinical scrapie


The Prionics®-CHECK PrioSTRIP SR detected EU reference samples of classical and atypical scrapie in sheep and goats as well as BSE in sheep with a sensitivity and specificity of 100%. The Prionics®- CHECK PrioSTRIP SR also proved to be the first test kit to achieve a high diagnostic sensitivity on preclinical TSE cases. Most importantly, the test can detect infection as early as 7 months post exposure, which is earlier - during the incubation period - than the reference tests. “Early detection of scrapie is a critical factor in disease control programs” says Ernst Zollinger, Head of Marketing and Sales at Prionics. “In contrast to BSE, scrapie is an outbreak disease which could not be eliminated for centuries. The earlier the infection is detected means that the costs associated with managing scrapie can be minimized.”


PrioSTRIP®SR: Fast and simple


The Prionics®-Check PrioSTRIP SR is not only the most sensitive test, but is also the fastest and simplest sheep TSE test on the market, making it an excellent tool to monitor the incidence of TSE in small ruminants. This fast high throughput laboratory assay delivers results in just over one hour and is based on immunochromatography, using antibody-mediated detection of scrapie and BSE prions. The Prionics®-Check PrioSTRIP SR is an economical test as it requires only minimal laboratory equipment and produces little waste. This fast and convenient test uses the same technology as the established and successful PrioSTRIP® test for BSE in cattle, meaning that the same test principle can be used for the detection of BSE in cattle and TSE in small ruminants.


About TSE in small ruminants


Small ruminants (sheep and goats) can be infected with scrapie or BSE under natural circumstances. While scrapie is thought to be non-infectious to humans, it is unclear whether BSE in sheep and goats could cause Creutzfeldt-Jakob disease in humans, similar to that of BSE in cattle. Small ruminant testing programs, initiated by the European Community to identify the incidence of TSE in sheep and goats, are ongoing.


About Prionics


Prionics AG, based in Zurich, Switzerland, is a leading provider of farm animal diagnostics and is a recognized center of expertise in BSE and prion diseases. Prionics produces and markets innovative diagnostic solutions for major farm animal diseases, aiding in the protection of consumer health.


Prionics is the main sponsor of the 16th International Symposium of the World Association of Veterinary Laboratory Diagnosticians in Berlin June 5-8, 2013.


For more information please visit www.prionics.com or contact:


Marjan van der Haar, PhD


Marketing Communications


Tel: +41 44 200 20 57 / +41 79 352 53 16


Email: media@prionics.com











New test to combat scrapie and mad cow disease in sheep and goats



Zurich, November 14, 2012 - An innovative new test is now available to detect TSE (scrapie and mad cow disease) in sheep and goats. With the Prionics®-Check PrioSTRIP SR, Prionics brings to the market the first test to achieve high diagnostic sensitivity on preclinical TSE cases. The test was approved today by the European Commission for use in TSE testing in small ruminants*. The Prionics®-Check PrioSTRIP SR can detect scrapie earlier than conventional tests and will therefore significantly contribute to TSE control programs.


The Prionics®-Check PrioSTRIP SR was extensively tested in a laboratory evaluation directed by the European Commission. The product fulfilled all the requirements of the EU evaluation and has now been approved as a rapid test for detection of TSE in the central nervous system of small ruminants (i.e. sheep and goats).


Superior detection of preclinical scrapie


The Prionics®-CHECK PrioSTRIP SR detected EU reference samples of classical and atypical scrapie in sheep and goats as well as BSE in sheep with a sensitivity and specificity of 100%. The Prionics®-CHECK PrioSTRIP SR also proved to be the first test kit to achieve a high diagnostic sensitivity on preclinical TSE cases. Most importantly, the test can detect infection as early as 7 months post exposure, which is earlier - during the incubation period - than the reference tests. “Early detection of scrapie is a critical factor in disease control programs” says Ernst Zollinger, Head of Marketing and Sales at Prionics. “In contrast to BSE, scrapie is an outbreak disease which could not be eliminated for centuries. The earlier the infection is detected means that the costs associated with managing scrapie can be minimized.”


PrioSTRIP® SR: Fast and simple


The Prionics®-Check PrioSTRIP SR is not only the most sensitive test, but is also the fastest and simplest sheep TSE test on the market, making it an excellent tool to monitor the incidence of TSE in small ruminants. This fast high throughput laboratory assay delivers results in just over one hour and is based on immunochromatography, using antibody-mediated detection of scrapie and BSE prions. The Prionics®-Check PrioSTRIP SR is an economical test as it requires only minimal laboratory equipment and produces little waste. This fast and convenient test uses the same technology as the established and successful PrioSTRIP® test for BSE in cattle, meaning that the same test principle can be used for the detection of BSE in cattle and TSE in small ruminants.


About TSE in small ruminants


Small ruminants (sheep and goats) can be infected with scrapie or BSE under natural circumstances. While scrapie is thought to be non-infectious to humans, it is unclear whether BSE in sheep and goats could cause Creutzfeldt-Jakob disease in humans, similar to that of BSE in cattle. Small ruminant testing programs, initiated by the European Community to identify the incidence of TSE in sheep and goats, are ongoing.









>>> Superior detection of preclinical scrapie The Prionics®-CHECK PrioSTRIP SR detected EU reference samples of classical and atypical scrapie in sheep and goats as well as BSE in sheep with a sensitivity and specificity of 100%.




a test is only as good as the ones giving that test. your country can be full of TSEs, and you can have a TSE prion test with a specificity of 100%, and still not find much of anything, only by chance, when part of your 2004 enhanced BSE surveillance program consisted of only testing healthy cattle brains. case in point ;


Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program


An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.


snip...


Topics that will be covered in ongoing or planned reviews under Goal 1 include:


soundness of BSE maintenance sampling (APHIS),


implementation of Performance-Based Inspection System enhancements for specified risk material (SRM) violations and improved inspection controls over SRMs (FSIS and APHIS),


snip...


The findings and recommendations from these efforts will be covered in future semiannual reports as the relevant audits and investigations are completed.


4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half








-MORE Office of the United States Attorney District of Arizona FOR IMMEDIATE RELEASE For Information Contact Public Affairs February 16, 2007 WYN HORNBUCKLE Telephone: (602) 514-7625 Cell: (602) 525-2681




CORPORATION AND ITS PRESIDENT PLEAD GUILTY TO DEFRAUDING GOVERNMENTS MAD COW DISEASE SURVEILLANCE PROGRAM


PHOENIX -- Farm Fresh Meats, Inc. and Roland Emerson Farabee, 55, of Maricopa, Arizona, pleaded guilty to stealing $390,000 in government funds, mail fraud and wire fraud, in federal district court in Phoenix. U.S. Attorney Daniel Knauss stated, The integrity of the system that tests for mad cow disease relies upon the honest cooperation of enterprises like Farm Fresh Meats. Without that honest cooperation, consumers both in the U.S. and internationally are at risk. We want to thank the USDAs Office of Inspector General for their continuing efforts to safeguard the public health and enforce the law. Farm Fresh Meats and Farabee were charged by Information with theft of government funds, mail fraud and wire fraud. According to the Information, on June 7, 2004, Farabee, on behalf of Farm Fresh Meats, signed a contract with the U.S. Department of Agriculture (the USDA Agreement) to collect obex samples from cattle at high risk of mad cow disease (the Targeted Cattle Population). The Targeted Cattle Population consisted of the following cattle: cattle over thirty months of age; nonambulatory cattle; cattle exhibiting signs of central nervous system disorders; cattle exhibiting signs of mad cow disease; and dead cattle. Pursuant to the USDA Agreement, the USDA agreed to pay Farm Fresh Meats $150 per obex sample for collecting obex samples from cattle within the Targeted Cattle Population, and submitting the obex samples to a USDA laboratory for mad cow disease testing. Farm Fresh Meats further agreed to maintain in cold storage the sampled cattle carcasses and heads until the test results were received by Farm Fresh Meats.


Evidence uncovered during the governments investigation established that Farm Fresh Meats and Farabee submitted samples from cattle outside the Targeted Cattle Population. Specifically, Farm Fresh Meats and Farabee submitted, or caused to be submitted, obex samples from healthy, USDA inspected cattle, in order to steal government moneys.


Evidence collected also demonstrated that Farm Fresh Meats and Farabee failed to maintain cattle carcasses and heads pending test results and falsified corporate books and records to conceal their malfeasance. Such actions, to the extent an obex sample tested positive (fortunately, none did), could have jeopardized the USDAs ability to identify the diseased animal and pinpoint its place of origin. On Wednesday, February 14, 2007, Farm Fresh Meats and Farabee pleaded guilty to stealing government funds and using the mails and wires to effect the scheme. According to their guilty pleas:


(a) Farm Fresh Meats collected, and Farabee directed others to collect, obex samples from cattle outside the Targeted Cattle Population, which were not subject to payment by the USDA;


(b) Farm Fresh Meats 2 and Farabee caused to be submitted payment requests to the USDA knowing that the requests were based on obex samples that were not subject to payment under the USDA Agreement;


(c) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Data Collection Forms to the USDAs testing laboratory that were false and misleading;


(d) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Submission Forms filed with the USDA that were false and misleading;


(e) Farm Fresh Meats falsified, and Farabee directed others to falsify, internal Farm Fresh Meats documents to conceal the fact that Farm Fresh Meats was seeking and obtaining payment from the USDA for obex samples obtained from cattle outside the Targeted Cattle Population; and


(f) Farm Fresh Meats failed to comply with, and Farabee directed others to fail to comply with, the USDA Agreement by discarding cattle carcasses and heads prior to receiving BSE test results. A conviction for theft of government funds carries a maximum penalty of 10 years imprisonment. Mail fraud and wire fraud convictions carry a maximum penalty of 20 years imprisonment. Convictions for the above referenced violations also carry a maximum fine of $250,000 for individuals and $500,000 for organizations. In determining an actual sentence, Judge Earl H. Carroll will consult the U.S. Sentencing Guidelines, which provide appropriate sentencing ranges. The judge, however, is not bound by those guidelines in determining a sentence.


Sentencing is set before Judge Earl H. Carroll on May 14, 2007. The investigation in this case was conducted by Assistant Special Agent in Charge Alejandro Quintero, United States Department of Agriculture, Office of Inspector General. The prosecution is being handled by Robert Long, Assistant U.S. Attorney, District of Arizona, Phoenix. CASE NUMBER: CR-07-00160-PHX-EHC RELEASE NUMBER: 2007-051(Farabee) # # #








Friday, May 4, 2012


May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States







PAUL BROWN COMMENT TO ME ON THIS ISSUE


Tuesday, September 12, 2006 11:10 AM


"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency." ........TSS







OR, what the Honorable Phyllis Fong of the OIG found ;


Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program ­ Phase II and Food Safety and Inspection Service


Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III


Report No. 50601-10-KC January 2006


Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain








Tuesday, January 1, 2008


BSE OIE USDA


Subject: OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle dealers i.e. USDA


Date: May 14, 2007 at 9:00 am PST


OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle dealers i.e. USDA


STATEMENT BY DR. RON DEHAVEN REGARDING OIE RISK RECOMMENDATION


March 9, 2007








Tuesday, November 02, 2010


IN CONFIDENCE


The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".


BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992








2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006







Comments on technical aspects of the risk assessment were then submitted to FSIS.


Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:








Owens, Julie


From: Terry S. Singeltary Sr. [flounder9@verizon.net]


Sent: Monday, July 24, 2006 1:09 PM


To: FSIS RegulationsComments


Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)


Page 1 of 98








FSIS, USDA, REPLY TO SINGELTARY








U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001












2012 atypical L-type BSE BASE California reports


Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012







SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012


Summary Report BSE 2012


Executive Summary







Saturday, August 4, 2012


Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation







CENSORSHIP IS A TERRIBLE THING $$$


Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$


THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$




Thursday, February 10, 2011


TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31







Wednesday, August 11, 2010


REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Thursday, August 19, 2010


REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Friday, March 4, 2011


Alberta dairy cow found with mad cow disease







Reasons for the New Regulation Order No. 23 (as well as amending Order No. 149) of the State Committee for Veterinary Medicine name BSE as the reason for new import requirement. The legal title for Order No. 23 is "On Urgent Measures Aimed at Prevention and Elimination of BSE and Other Prion Infections in Cattle”. Neither Order explains how the threat of introduction of BSE can be addressed through the inspection of producers of all products of animal origin including fish, dairy products, poultry and pork. It is not clear what other concerns are addressed through the proposed inspections. Formal Notification of Trading Partners On August 3rd, Ukraine's Notification and Enquiry Point issued a legal Notification G/SPS/N/UKR/3/Rev.1 found on the Official WTO Website (Committee on Sanitary and Phytosanitary Measures)







Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas







Monday, November 30, 2009


USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE







Thursday, August 12, 2010


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


snip...







EFSA Journal 2011 The European Response to BSE: A Success Story


This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;


Monday, October 10, 2011


EFSA Journal 2011 The European Response to BSE: A Success Story


snip...


EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.


snip...










see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;







2011 Monday, September 26, 2011


L-BSE BASE prion and atypical sporadic CJD







Saturday, March 5, 2011


MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA







Wednesday, August 01, 2012


Behavioural and Psychiatric Features of the Human Prion Diseases: Experience in 368 Prospectively Studied Patients







Monday, August 06, 2012


Atypical neuropathological sCJD-MM phenotype with abundant white matter Kuru-type plaques sparing the cerebellar cortex







Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA







Friday, August 24, 2012


Iatrogenic prion diseases in humans: an update






Monday, July 23, 2012


The National Prion Disease Pathology Surveillance Center July 2012







OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles


Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA


Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.


Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.


Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.


In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.


Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.


The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.







Wednesday, March 28, 2012


VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $







*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.


AS OF AUGUST 2012 ;


CJD UPDATE USA


1 Listed based on the year of death or, if not available, on year of referral; 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; 3 Disease acquired in the United Kingdom; 4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case; *** 5 Includes 8 cases in which the diagnosis is pending, and 18 inconclusive cases; *** 6 Includes 10 (9 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded. *** The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2224 cases of sporadic Creutzfeldt-Jakob disease (sCJD).







Sunday, September 23, 2012


EU-Approved Rapid Tests for Bovine Spongiform Encephalopathy Detect Atypical Forms: A Study for Their Sensitivities









**** Tuesday, November 6, 2012



Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update









TSS