Thursday, January 5, 2012

Ten years of BSE surveillance in Italy: Neuropathological findings in clinically suspected cases

doi:10.1016/j.rvsc.2011.10.008 | How to Cite or Link Using DOI

Permissions & Reprints

Ten years of BSE surveillance in Italy: Neuropathological findings in clinically suspected cases

B. Iulinia, C. Maurellaa, M.D. Pintorea, E. Vallino Costassaa, D. Corbellinia, C. Porcarioa, A. Pautassoa, C. Salatab, D. Gelmettic, T. Avanzatoa, G. Pal├╣b, A. D’Angelod, M. Caramellia, C. Casalonea, ,



Reference Center for Transmissible Spongiform Encephalopathy (CEA), Istituto Zooprofilattico Sperimentale of Piemonte, Liguria and Valle D’Aosta, Italy


Department of Histology, Microbiology and Medical Biotechnologies, University of Padova, Italy


Istituto Zooprofilattico Sperimentale of Lombardia and Emilia Romagna, Italy


Department of Animal Pathology, University of Turin, Italy

Received 20 June 2011; Accepted 16 October 2011. Available online 13 November 2011.


Between 2001 and 2010, 244 clinically suspected cases of bovine spongiform encephalopathy (BSE) were reported in Italy. This report summarizes the neuropathological findings in cattle displaying clinical signs consistent with a diagnosis of BSE. All animal specimens were submitted for confirmatory testing; samples testing negative underwent neuropathological examination to establish the differential diagnosis. Immunohistochemistry for scrapie prion protein (PrPSc) at the level of frontal cortex was carried out to exclude atypical BSE.

Neuropathological changes were detected in 34.9% of cases; no histological lesions were found in 52.3% of subjects; 12.8% of samples were found unsuitable for analysis. BSE was detected in one case, but no cases of atypical BSE were observed.

This study identified the diseases most commonly encountered in the differential diagnosis of BSE; furthermore, it demonstrated that the surveillance system is necessary for monitoring neuropathological disease in cattle and for the detection of BSE cases.

Keywords: Bovine; Brain; BSE; Neuropathology; Immunohistochemistry; Surveillance

"BSE was detected in one case, but no cases of atypical BSE were observed."

i don't understand this statement ???

atypical BSE has been detected and confirmed in Italy.

please see ;

We recently reported two Italian atypical cases with a PrPTSE type identical to BSE-L, pathologically characterized by PrP amyloid plaques and known as bovine amyloidotic spongiform encephalopathy (BASE).

The L-type has been found in cattle in Italy (10), Japan (11), Germany (12) and Belgium (13). So far, the H-type has been described in cattle from France (14), Germany (12) and the United States (15). The U.S. cases were animals born and raised in the U.S. (Texas, Alabama).

Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease

Cristina Casalone*,†, Gianluigi Zanusso†,‡, Pierluigi Acutis*, Sergio Ferrari‡, Lorenzo Capucci§, Fabrizio Tagliavini¶, Salvatore Monaco‡,?, and Maria Caramelli*

+ Author Affiliations

*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; ‡Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; §Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico ”Carlo Besta,” Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003)

Next Section Abstract

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called ”species barrier” between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease.

The transmissible spongiform encephalopathies (TSEs), or prion diseases (1), encompass a group of progressive neurodegenerative disorders, including Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep, and bovine spongiform encephalopathy (BSE) (1-4). These disorders are characterized by brain deposition of an insoluble, protease-resistant isoform of the host-encoded cellular prion protein (PrPC), named PrPSc (1, 4, 5) In different TSE phenotypes, PrPSc exhibits disease-specific properties, including distinctive cleavage sites after proteolytic treatment, ratio of glycoforms, and deposition patterns, all features useful in providing a means of strain identification (6-10).

Although not contagious, TSEs are potentially infective, and in humans may present as sporadic, inherited, and acquired diseases. Human-to-human transmission of TSE is well documented and has occurred either through oral or mucocutaneous route of infection, as in kuru (11), or after medical and surgical procedures, as in iatrogenic CJD (12). Recently, animal-to-human transmission has also occurred. Epidemiological (13), experimental transmission (14), and biochemical PrPSc typing (8) have provided strong evidence that the single prion strain responsible for BSE has infected humans, causing variant CJD (vCJD), in addition to several animal species. In BSE and BSE-related disorders, including vCJD, the molecular typing of disease-associated PrPSc shows identical PrP fragment sizes and predominance of the high molecular mass glycoform both in natural hosts and in experimentally inoculated animals. To date, at variance with CJD in humans and scrapie in sheep, only a single strain and a single PrPSc type have been detected in BSE.

The spreading of the BSE agent across mammalian species barriers has aroused considerable concern for the following reasons: (i) the possible existence of new or previously unrecognized cattle TSE strains, potentially pathogenic for humans; and (ii) the occurrence of phenotypic variation of the BSE strain, with propagation of a new agent encoding distinctive molecular and biological properties.

In Italy, an active surveillance system on BSE in cattle was started in January 2001, and by August 2003 a total of 103 BSE cases had been diagnosed of 1,638,275 statutory tested brainstem samples. Confirmatory positive results have been obtained in all cases by immunohistochemical and Western immunoblot demonstration of disease-specific protease-resistant PrPSc.

To assess molecular and neuropathological characteristics in Italian BSE cases, we have over the last few months collected whole brains of eight Italian cattle that were PrPSc-positive in Western immunoblots. In two cattle, older than other affected bovines, the PrPSc glycotype was clearly different from the BSE-associated PrPSc molecule, and widespread PrP-amyloid plaques were seen in supratentorial brain regions. Unlike typical BSE, the brainstem was less involved and no PrP deposition was detected in the dorsal nucleus of the vagus nerve. Given the biochemical and pathological similarities with sporadic CJD (sCJD) cases linked to type-2 PrPSc (9) and methionine/valine (M/V) polymorphism at codon 129 in the prion protein gene (PRNP), these findings have prompted ongoing strain typing in inbred mice. Although the present findings dictate caution, here we show that a PrPSc type associated with sCJD and the previously undescribed bovine PrPSc show convergent molecular signatures.


Phenotypic Similarities Between BASE and sCJD. The transmissibility of CJD brains was initially demonstrated in primates (27), and classification of atypical cases as CJD was based on this property (28). To date, no systematic studies of strain typing in sCJD have been provided, and classification of different subtypes is based on clinical, neuropathological, and molecular features (the polymorphic PRNP codon 129 and the PrPSc glycotype) (8, 9, 15, 19). The importance of molecular PrPSc characterization in assessing the identity of TSE strains is underscored by several studies, showing that the stability of given disease-specific PrPSc types is maintained upon experimental propagation of sCJD, familial CJD, and vCJD isolates in transgenic PrP-humanized mice (8, 29). Similarly, biochemical properties of BSE- and vCJD-associated PrPSc molecules remain stable after passage to mice expressing bovine PrP (30). Recently, however, it has been reported that PrP-humanized mice inoculated with BSE tissues may also propagate a distinctive PrPSc type, with a ”monoglycosylated-dominant” pattern and electrophoretic mobility of the unglycosylated fragment slower than that of vCJD and BSE (31). Strikingly, this PrPSc type shares its molecular properties with the a PrPSc molecule found in classical sCJD. This observation is at variance with the PrPSc type found in M/V2 sCJD cases and in cattle BASE, showing a monoglycosylated-dominant pattern but faster electrophoretic mobility of the protease-resistant fragment as compared with BSE. In addition to molecular properties of PrPSc, BASE and M/V2 sCJD share a distinctive pattern of intracerebral PrP deposition, which occurs as plaque-like and amyloid-kuru plaques. Differences were, however, observed in the regional distribution of PrPSc. While in M/V2 sCJD cases the largest amounts of PrPSc were detected in the cerebellum, brainstem, and striatum, in cattle BASE these areas were less involved and the highest levels of PrPSc were recovered from the thalamus and olfactory regions.

In conclusion, decoding the biochemical PrPSc signature of individual human and animal TSE strains may allow the identification of potential risk factors for human disorders with unknown etiology, such as sCJD. However, although BASE and sCJD share several characteristics, caution is dictated in assessing a link between conditions affecting two different mammalian species, based on convergent biochemical properties of disease-associated PrPSc types. Strains of TSE agents may be better characterized upon passage to transgenic mice. In the interim until this is accomplished, our present findings suggest a strict epidemiological surveillance of cattle TSE and sCJD based on molecular criteria.

Sunday, May 01, 2011

STUDY OF ATYPICAL BSE 2010 Annual Report May 2011

Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

"BSE-L in North America may have existed for decades"

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.


The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

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


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

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

T. Singeltary

Bacliff, TX, USA


An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.


12 years independent research of available data


I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.


I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

Monday, May 23, 2011

Atypical Prion Diseases in Humans and Animals 2011

Top Curr Chem (2011)

DOI: 10.1007/128_2011_161

# Springer-Verlag Berlin Heidelberg 2011

Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar


Although prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the "scrapie form" (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

M.A. Tranulis (*)

Norwegian School of Veterinary Science, Oslo, Norway

S.L. Benestad

Norwegian Veterinary Institute, Oslo, Norway

T. Baron

Agence Nationale de Se´curite´ Sanitaire, ANSES, Lyon, France

H. Kretzschmar

Ludwig-Maximilians University of Munich, Munich, Germany

Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type

snip...SEE MORE HERE ;

Sunday, June 07, 2009


Sunday, May 10, 2009

Identification and characterization of bovine spongiform encephalopathy cases diagnosed and NOT diagnosed in the United States

Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

Saturday, February 28, 2009


"All of the 15 cattle tested showed that the brains had abnormally accumulated PrP"

2009 SEAC 102/2

Wednesday, July 28, 2010

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

Sunday, September 6, 2009


Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.

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


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.


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 ;

Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?



This is a highly competitive field and it really will be a pity if we allow many of the key findings to be published by overseas groups while we are unable to pursue our research findings because of this disagreement, which I hope we can make every effort to solve.




2. The discovery might indicate the existence of a different strain of BSE from that present in the general epidemic or an unusual response by an individual host.

3. If further atypical lesion distribution cases are revealed in this herd then implications of misdiagnosis of 'negative' cases in other herds may not be insignificant.


This minute is re-issued with a wider distribution. The information contained herein should NOT be disseminated further except on the basis of ''NEED TO KNOW''.

R Bradley








All material for publication including written works to be published in scientific journals, books, proceedings of scientific meetings, abstracts of verbally delivered papers and the like should be scrutinized for risk to the Ministry before dispatch to the publishers.............

full text;


- 10 -

19. On 18th February, 1987 (YB87/2.18/1.1) I reported to Dr Watson and Dr Shreeve on a further case which we had received from Truro VIC. The brain had shown neuronal vacuolation and in brain extracts there were fibrils that were similar in size and appearance to SAFs from sheep with scrapie. The Virology Department was studying the brain further and considering a transmission study. A few weeks before this, I had discussed the possibility of a transmission study with Michael Dawson, a research officer in the Virology Department and an expert in viral diseases in sheep, and we were considering carefully the safety aspects. In my note I raised the question of whether we should disclose the information we had more widely to the VIS because this may assist in getting any other cases referred to CVL but there was the difficulty that we knew very little about the disorder and would be unable to deal with queries that might be raised.

20. On 23rd February, 1987 (YB87/2.23/1.1) I sent Mr Wells a note asking him to prepare a statement for publication in Vision, the in-house newsheet prepared by the VIS for the SVS, setting out details of what we had discovered. On 24th February, 1987 (YB87/2.25/2.1) Gerald Wells indicated in a note to me that he had discussed the proposed article with Mr Dawson and they both believed that it could be damaging to publish anything at that stage. They believed cases would be referred to CVL in any event because they were unusual and they did not feel "Vision" was an appropriate publication because its confidentiality was questionable and might lead to referrals to veterinary schools rather than CVL. Gerald Wells was also concerned about the resources available in his section to deal with referred cases. I replied (YB87/2.25/2.1) indicating a draft statement was needed by the Director before a decision on publication could be made. Gerald Wells prepared a draft statement (YB87/3.2/2.1) and sent it to me on 2nd March, 1987. In his cover note (YB87/3.2/1.1) he commented that he believed the distribution of any statement about the new disease outside of CVL to be premature because there was so little information available about the new disease. I passed on a copy of Gerald Wells' note to Dr Watson (YB87/3.2/3.1). I discussed the matter of publication with Dr Watson. No decision had been taken to publish any material at that stage and I sent a note to Gerald Wells letting him know the position and confirming that his views and those of Michael Dawson would be taken into account when a decision was taken.

- 11 -

21. In March, 1987 serious consideration was given to possible transmission (e.g. to hamsters) and other experiments (other than the collection of epidemiological data by the VIS and clinicopathology which had been in progress since the first cases were recognised in November, 1986).

22. On 23rd April, 1987 I sent a report (YB87/4.23/1.1) to Dr Watson and Dr Shreeve informing them that nine control brains were being examined for SAFs and a cow which appeared to be affected with BSE had been purchased for observation. The cow had come from the farm where the original cases had developed and had arrived at CVL on 22nd April, 1987.

23. On 15th May, 1987 Dr Watson informed me that the proposed "Vision" draft would be circulated to VICs in England and Wales if it was approved by management. On 22nd May, 1987 I was copied in on a note (YB87/5.22/2.1) from B.M Williams, (who I believe was Head of the VIS at this time but retired shortly after this), to Dr Watson. This confirmed that the draft prepared for publication in Vision was approved but that the final paragraph should be amended to make it clear that knowledge of the new disease should not be communicated to other research institutes or university departments. At a meeting with Dr Watson on 2nd June, 1987 he informed me that no communication should be made with NPU until after the meeting with the CVO on 5th June, 1987 (see my note of 3rd June, 1987 – YB87/6.3/1.1). We needed much more data and information to answer inevitable queries. ...


*This case study accompanies the IRGC report “Risk Governance Deficits: An analysis and illustration of the most common deficits in risk governance”.

The Bovine Spongiform Encephalopathy (BSE) Epidemic in the United Kingdom

By Belinda Cleeland1


A6 Misrepresenting information about risk

From the very beginning of the BSE outbreak, not only was knowledge misrepresented by the British government, but in some cases it was even withheld. For example, after the initial diagnosis of BSE by the SVS in late 1986, there was an embargo placed on the sharing, or making public, of any BSE-related information that ran until mid-1987. Also, up until at least 1990, outside scientists that requested access to BSE data to conduct further studies were denied, despite the fact the improved scientific understanding of the disease had the greatest potential to minimise the impact of the epidemic. Even government scientists within the CVL have acknowledged that there was a culture of suppressing information, to the point that studies revealing damaging evidence (e.g. that there was a causal link between BSE and the new encephalopathy found in cats) were denied publication permission [Ashraf, 2000].

The withholding of such information allowed the government to publicly assert that BSE was just like another version of scrapie – not transmissible to humans – and that there was “clear scientific evidence that British beef is perfectly safe” [UK House of Commons, 1990].2 This was certainly a misrepresentation of the knowledge held at the time, and one that was only possible due to the suppression of some scientific findings and recommendations. Of course, the main reason for this misrepresentation of knowledge was the protection of agricultural and industrial interests – the specific stakeholder favoured in this case was the British beef industry, which stood to lose billions of pounds if a large number of its animals had to be slaughtered, if export bans were put in place, or if costly regulations were implemented.

To protect the interests of the beef industry, the government would assert on many occasions that British beef was safe to eat and that regulatory controls already implemented would prevent any 2 This comment was made by the Agriculture Minister to the House of Commons.

contaminated material from entering the food chain. This was also a misrepresentation of knowledge, as the government was fully aware that their measures were not designed to eliminate exposure, but only to diminish the risk [van Zwanenberg & Millstone, 2002:161].

What’s more, many uncertainties relating to the transmissibility of the disease were either down-played or ignored, resulting in an overstatement of certainty that British beef was completely safe to eat and that BSE was not transmissible to humans. The way uncertainty was dealt with in this case was the result of a number of factors, including the desire to protect specific stakeholder interests.

One crucial factor was the underlying element of risk political culture in the UK that linked the identity of the actor to the consistency of his policy positions. This led to consistency of position being prioritised over accuracy [Dressel, 2000], and resulted in the government insisting on the absence of risk to the population, maintaining this public position despite mounting evidence to the contrary. Although aware of them, policy-makers chose not to overtly acknowledge the levels of uncertainty and the complexity of the risks involved with BSE and its spread because the ramifications of these were too great for the interests they were trying to safeguard.

B1 Responding to early warnings

The incorporation of rendered meat and bone meal into animal feed creates a number of risks related to the transmission, recycling and amplification of pathogens. Such risks were recognised well before the emergence of BSE. In the US in the mid-1970s, concerns that scrapie may be linked to CJD (although there is no evidence that scrapie is transmissible to humans) led to some regulations being placed on the incorporation of sheep or goat carcasses into human and animal foods [van Zwanenberg & Millstone, 2002:158]. In the UK, too, the Royal Commission on Environmental Pollution recommended in 1979 that minimum processing standards be implemented by the rendering industries in order to minimise the potential for disease spread [RCEP, 1979]. The incoming Thatcher government withdrew these proposed regulations, preferring to let industry decide for itself what standards to use. In retrospect, the failure to act at this point to mitigate the general risk of disease transmission may have had a crucial impact on the later outbreak of BSE, given that the disease “probably originated from a novel source in the early 1970s” [BSE Inquiry, 2000b].

Early warnings that BSE might be transmissible to humans were, in fact, observed by scientists and government officials throughout the period from 1986 (the time of first diagnosis in cattle) to 1995 (when vCJD was first observed in humans). Such observations are noted in, for example, the minutes of a meeting of the National Institute for Biological Standards and Control in May 1988, where the probability of transmission of BSE to humans is assessed as more than remote. The diagnosis in 1990 of a domestic cat with a previously unknown spongiform encephalopathy resembling BSE indicated that the disease could infect a wider range of hosts. Responses to such early warnings of potential dangers to human health were either too weak or came too late. This may have been partly a result of an ‘unwillingness to know’ due to the economic harm this knowledge would cause the UK beef industry (related to deficit A6); and partly due to institutional capacities and procedures (related to deficits B5, 9 and 10).

Tuesday, July 28, 2009



Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$


Wednesday, January 4, 2012

A Bovine Prion Acquires an Epidemic Bovine Spongiform Encephalopathy Strain-Like Phenotype on Interspecies Transmission

Monday, January 2, 2012
EFSA Minutes of the 6th Meeting of the EFSA Scientific Network on BSE-TSE Brussels, 29-30 November 2011
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
2011 Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD
Owens, Julie
From: Terry S. Singeltary Sr. []
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
Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 INTRODUCTION The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:
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:
Saturday, June 19, 2010
Friday, August 20, 2010
USDA: Animal Disease Traceability August 2010
Friday, November 18, 2011
country-of-origin labeling law (COOL) violates U.S. obligations under WTO rules WT/DS384/R WT/DS386/R

Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011 (SEE VIDEO)

2011 Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD




No comments:

Post a Comment