Monday, January 2, 2012

EFSA Minutes of the 6th Meeting of the EFSA Scientific Network on BSE-TSE Brussels, 29-30 November 2011


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BRUSSELS, 29-30/11/2011

Minutes of the 6th Meeting of the EFSA Scientific Network on BSE-TSE

Brussels, 29-30 November 2011


Item Topic

1 Welcome and apologies

2 Adoption of the agenda

4 Briefing on Action Points from previous meeting (BIOHAZ Unit)

3 Briefing by Members and Observers on BSE-TSE related activities since last meeting occurring in their respective Countries

5 The TSE Roadmap II (European Commission)

6 Current, recent and future prospects of BSE-TSE activities of EFSA’s Panel on Biological Hazards (BIOHAZ Unit)

7 Inspections of the Food and Veterinary Office related to BSE and TSE: Findings and trends (European Commission)

8 Update on the epidemiological situation of BSE and Scrapie in the EU (Network member from Italy)

9 Rapid TSE tests employed in the EU: From the application to the approval (BIOHAZ Unit)

10 Scrapie(s): What we know, what we do not know and what we may never know (Dr. Olivier Andreoletti, Member of the Panel on Biological Hazards)

11 VI years of the EFSA Scientific Network on BSE and TSE (BIOHAZ Unit)

12 Round table discussion on the future of the EFSA Scientific Network on BSE and TSE

13 Date and venue for next meeting and AOB

14 Closure of the meeting


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Belgium and Luxembourg (Stefan Roels), Bulgaria (Ilian Boykovski), Czech Republic (Pavel Vodrazka), Cyprus (Penelope Stylianou), Denmark (Larry G. Paisley), Estonia (Olga Piirik), Finland (Leena Sahlström), France (Jean Charles Le Blanc), Germany (Anne Buschmann), Greece (Dimitrios Dilaveris), Hungary (Zsuzsanna Szogyenyi), Ireland (Hazel Sheridan), Italy (Giuseppe Ru), the Netherlands (Aline De Koeijer), Poland (Miroslaw Polak), Romania (Theodora Vasile), Slovenia (Ivan Ambrozic), Slovak Republic (Janiuk Lubomir), Spain (Maria del Carmen Sanchez Morillo) and Sweden (Maria Noremark).


Switzerland (Peter Braam), Croatia (Branko Sostaric), the Former Yugoslav Republic of Macedonia (Spase Donov), Turkey (Hıkmet Un), Albania (Bernarda Haderi), Bosnia and Herzegovina (Senad Prasovic), Montenegro (Nikola Pejovic) and Serbia (Slavica Nikolic Stajkovic).

European Commission:

DG Health and Consumer Protection (Polyvios Neocleous, Martial Plantady and Fabien Schneegans).

Invited Experts:

Olivier Andreoletti (Member of the EFSA Scientific Panel on Biological Hazards) EFSA

Marta Hugas (Chair, Head of Unit, Biological Hazards Unit), Luis Vivas-Alegre (Biological Hazards Unit) and Pietro Stella (Biological Hazards Unit).


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1. Opening, welcome and apologies for absence The chair welcomed the participants to the 6th meeting of the EFSA Scientific Network on BSETSE. Apologies were received from Austria (Herbert Budka), Latvia (Edvins Olsevskis), Lithuania (Petras Maciulskis), Portugal (Maria Jose Pinto), United Kingdom (Patrick Burke and Irene Hill), Norway (Bjorn Nass) and Kosovo under UN Security Council Resolution 1244 (Ilirjana Zymberaj). Apologies were also received from Koen Van Dyck (Directorate General for Health and Consumers, European Commission) and from Vicky Lefevre and Heinz Schimmel (Directorate General Research of the European Commission).

2. Adoption of the agenda

The agenda was adopted including three additional items:

• Briefing by the Swiss Network Observer on the reporting of two cases of BSE in cattle in Switzerland, with an unusual prion protein signature as published online ahead of print in the Journal of Emerging Infectious Diseases as a letter to the editor. This briefing is presented under point 9 below.

• Informing the Network Members and Observers on a recent editorial article published in The EFSA Journal and authored by Prof. Herbert Budka (Network Member representative for Austria and Member of the EFSA BIOHAZ Panel). This article titled “The European Response to BSE: A Success Story” reflects on past, present and future issues related to the BSE epidemic in the EU.

• A communication from the Norwegian Network Member on the update of the epidemiological situation of Scrapie in Norway.

3. Briefing by EFSA on Action Points from the 2010 Network meeting The Network Members and Observers were briefed by the BIOHAZ Unit on the state of the specific actions identified at the previous Network: continue to promote the participation of Candidate and Potential candidate countries as observers, plus the preparation of a presentation on the updated epidemiological situation of TSEs in farmed animals in the EU. The later was kindly prepared by Giuseppe Ru, the Italian Network Member and is further discussed under point 8 below.

4. Briefing by Members and Observers on BSE-TSE related activities since the 2010

Network meeting

All Members and Observers addressed to the group the details on their activities since the last Network meeting. Special emphasis was made in the epidemiological situation of the different countries both in bovines and small ruminants. Some particular details outlines of the briefing include:

• The representative from Belgium and Luxembourg highlighted that since the last meeting the discussion on the disinfection of laboratories decommissioned for analysing


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samples for TSE has evolved and now laboratory guidance are available, and would be translated into English.

• The representative from The Netherlands informed the group about the organisation of the next PRION conference in Amsterdam, on 9-10 May 2012.

• The representative from Poland advised on the finding in 2006 of two BSE cases in Poland with Western blot profiles similar to those now reported in Switzerland. This information has been shared with the Swiss national Reference Laboratory but it was not published in a scientific paper.

5. The TSE Road map 2

The Commission presented the details of The TSE Road map 2, the strategy paper on TSEs for 2010-2015 from the European Commission. The presentation addressed key future expected changes in particular in the context of the EU-wide feedban on proteins of animal origin to farmed animals, of the BSE monitoring and of the monitoring and control of TSEs in small ruminants. Some of the issues discussed after the presentation included:

• The need to review the monitoring and reporting of BSE by the EU MSs, where currently discrimination of Atypical BSE cases is neither legally required nor reported. This is also an issue that is not currently considered in the OIE.

• The future seeking of alignment with OIE requirements with regards measures for eradication of TSEs in small ruminants (i.e. EU measures more stringent).

• What EU MSs would be able to apply to the sample-size based for healthy slaughtered cattle BSE monitoring scheme.

6. Current, recent and future prospects of BSE-TSE activities of EFSA’s Panel on Biological Hazards

The work carried out by the BIOHAZ Panel on BSE and other TSEs since the last Network meeting was presented by the BIOHAZ Unit. Details included description of the Scientific Opinions and Technical Reports adopted, and both the ongoing and future tasks.

7. Inspections of the Food and Veterinary Office related to BSE and TSE: Findings and trends

The Commission presented an overview of the tasks of the FVO regarding the verification of official controls carried out by the national competent authorities on BSE-TSE risk management issues described in legislation. The presentation also included historical information on number of audits carried out in the different EU MSs, audits carried outside the EU and on the general findings of those audits.

8. Update on the epidemiological situation of BSE and Scrapie in the EU The Italian representative presented an epidemiological review of the trend of Classical BSE, Atypical BSE, Classical Scrapie and Atypical Scrapie in different countries (i.e. EU Member States, European countries and non-European countries). The presentation included updated data


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also for 2011 that was kindly shared by the European Commission. The review of some of the possible reasons for the difference in the epidemiological situation of Classical and Atypical Scrapie between the different MSs was made.

9. Reporting of two cases of BSE with an unusual prion protein signature in Switzerland The Swiss representative presented the details of the molecular diagnosis results of two BSE cases that occurred in Switzerland in 2011 that presented a prion protein phenotype distinct from those of Classical, L-type and H-type Atypical BSE. It was highlighted that the autolysed state of the obex samples could account for the differences found. Nevertheless, bioassay studies are needed and already ongoing in order to fully typify the disease phenotype of those two cases. The Polish representative highlighted that in the past two cases of BSE with a similar profile to the one reported in here were found.

10. Rapid TSE tests employed in the EU: from the application to the approval

The BIOHAZ Unit provided a comprehensive review of different aspects related to the approval process of the rapid TSE tests employed in the EU for the monitoring of TSEs in farmed animals. The presentation included the detailed aspects of the approval process and provided an update on current tests under approval and on future prospects. Additional EFSA activities related to the post-approval evaluation of rapid TSE tests based on ad hoc requests from the European Commission were also outlined.

11. Scrapie(s): What we know, what we do not know and what we may never know Dr Olivier Andreoletti (Member of the EFSA Scientific Panel on Biological Hazards) presented to the attendees the current state of the art on several scientific issues related to TSEs in small ruminants. The presentation was structured on different areas of knowledge with implications for TSE risk assessment, ranging from active surveillance of TSEs in small ruminants, breeding for resistance in goats, state of the art on research on ante-mortem testing and knowledge of agent biodiversity.

12. VI years of the EFSA Scientific Network on BSE and TSE

The BIOHAZ Unit provided an overview of the 6 years of history of the BSE-TSE Network. It included considerations on the yearly increase in the number of Members and Observers, on the changes in the scope and final formalisation of the Network in line with EFSA policies, and on the activities carried out outside the regular Network meetings.

13. Round table discussion on the future direction of the EFSA Scientific Network on BSE and TSE

The Network Members and Observers consider that future developments of the Scientific Network on BSE-TSE should be proportional to the future interest in the field of BSE-TSE. All the attendees found the Network as a useful tool for exchange of information, and it was felt by all that the current frequency of the meetings (i.e. once a year) was appropriate.


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Particular issues discussed for future action points were to:

• Consider the possibility for including issues related to scientific and technical aspects for the treatment and safe disposal Animal By-Products (ABP) as done in the past or the need for a dedicated EFSA Network on this subject.

• Invite the OIE to a Network meeting in order to brief the representatives on the details of the application process for the revision of the BSE risk categorisation of the countries and on the consideration given to the impact of Atypical BSE cases.

• Develop a more structure frame for the reporting of the Members and Observers on their BSE-TSE related activities at the beginning of the meetings. This could be supported by the use of a form that would be circulated in advance to the meeting requesting background on different BSE-TSE issues that are normally briefed only orally. This could also be used to get information on Atypical BSE cases and to identify topics for discussion in advance for the meeting. However, this should not jeopardise the regular use of the available electronic tools for discussion and data exchange when needed (e.g. EFSA Sciencenet, e-mail distribution list).

• Invite the EU Reference Laboratory for Animal Proteins in Feedingstuffs (EURL-AP) to a Network meeting in order to present the state of the art both in science and in practice regarding testing of feedingstuffs for the presence of animal proteins.

14. Date and venue for next meeting.

It was agreed to hold a Network meeting in 2012, preferably after the summer break. The exact date and venue remains to be confirmed.

15. AOB

Dr. Larry G. Paisley, Network Member from Denmark, informed that this was his last Network meeting before retirement. Special recognition was given to Dr. Paisley by the Chair of the Network to his contribution to and participation in all these years of the EFSA Scientific Network on BSE-TSE.

16. Closure of the meeting

The meeting closed at 12.30 pm on 30th November 2011.

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

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...

LET us review the failures of the O.I.E. on BSE TSE Prion risk factors shall we.

THE O.I.E. Transmissible Spongiform Encephalopathy TSE PRION eradication efforts and regulations there from have been drawn up NOT to protect animals or humans from the TSE prion agent (aka all strains and types of mad cow disease), but to protect TRADE. The almighty ‘BSE GOLD CARD’. But, when the USDA and the O.I.E. decided to do away with TSE Prion safeguards by dissolving the BSE GBR RISK assessments, and shoving down the throats of just about every country out there the infamous BSE M.R.R. policy (all the time this process was going on, to undermine TSE science for trade, two atypical mad cows in the USA tissue samples sat up on the shelf not to be confirmed for 4 months on one sample, and 7+ months on the other sample, with the 7+ month sample finally being confirmed literally after an act of congress i.e. the Honorable Phyllis Fong of the O.I.G, and only after the BSE M.R.R. policy was validated, the legal trading of all strains of TSE globally), and then to follow up with the OMISSION OF ATYPICAL SCRAPIE ALL TOGETHER as a risk factor of humans and animals of trade protocol for the O.I.E. trade, all the attempts and money to eradicate BSE, Scrapie, and all TSE from the globe was sent back to ground zero 1984-1985 when the documentation of the BSE agent was first documented. all this because of the long incubation period, money, and politics. what will the ramification of all this be over the next decade?

A review from pre-BSE GBR risk assessment to the bought and paid for by your local cattle dealer science of the BSE M.R.R. policy of the O.I.E., thanks to the U.S.D.A. PLEASE remember, all this only happened after the U.S.A. lost it’s ‘BSE GOLD STAR’ category.

1st let’s look at passed history, regulations, the dissolving of those regulations, to present BSE MRR and typical vs atypical regulations, and then lastly the present science on TSE Prion to date, and the possible ramifications there from in the future.

Comment from Terry S Singletary Document ID: APHIS-2006-0041-0028Document

Type: Public Submission

Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Docket ID: APHIS-2006-0041RIN:0579-AC01

see attachment ;

Comment from Terry S Singletary Sr Document ID: APHIS-2006-0041-0006Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Docket ID: APHIS-2006-0041RIN:0579-AC01 Topics: No Topics associated with this document View Document


[Federal Register: January 9, 2007 (Volume 72, Number 5)] [Proposed Rules] [Page 1101-1129] From the Federal Register Online via GPO Access [] [DOCID:fr09ja07-21]

please see attachment to right at bottom as well...tss

Wednesday, January 28, 2009 OIE amending the Annex to Decision 2007/453/EC establishing the BSE status of Member States or third countries or regions COMMISSION DECISION of 30 October 2008


(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries.The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to then Central Bureau............

Comment from Terry S Singeltary, CJD WATCH/VOICE Document ID: APHIS-2007-0033-0002Document Type: Public Submission This is comment on Proposed Rule: Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Docket ID: APHIS-2007-0033RIN:0579-AC53 Topics: No Topics associated with this document View Document

Public Comments Submitted to FDA Dockets Following

Federal Register Notice Announcement of

TSEAC Meeting

e-mail from Terry S. Singeltary Sr. received 10/14/2005


File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [] Monday, January 08,200l 3:03 PM freas ...

Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

TO :

May 8, 2009

Greetings again Dr. Freas, TSEAC et al,

I would kindly, once again, wish to comment at this meeting about the urgent actions that need to be taken asap, to the Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009. Due to my disability from my neck injury, I will not be attending this meeting either, however I hope for my submission to be read and submitted. ...

IN reply to ;

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

Transmissible Spongiform Encephalopathy and the O.I.E.

OIE Terrestrial Animal Health Standards Commission / September 2010

The EU takes note of the fact that atypical scrapie is not an OIE listed disease. Nevertheless, it will remain notifiable in the EU. Moreover it must be stressed that any emergence of this disease should be notified to the OIE by Members and that scientific data should continue to be gathered.


Zoonotic Potential

Has transmission to humans been proven? (with the exception of artificial circumstances) AND

Is human infection associated with severe consequences? (death or prolonged illness)

Saturday, December 18, 2010

OIE Global Conference on Wildlife Animal Health and Biodiversity – Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011

I see again that the OIE has done little to help eradicate all animal TSE from the globe, and in fact in my opinion, have help enhance the spread of BSE and other animal TSE globally by their industry friendly regulations. I tried to warn the OIE in 2002 about CWD and the potential, but very real threat of CWD to humans. I was told that they were seriously considering this. what happened ? NOW, the OIE and the USDA collaborate to make legal the trading of all strains of atypical BSE legal, and in fact have done so with the atypical scrapie, when science has made perfectly clear the risk factors to humans and other species. I have said it once (see below), and i will say again ;

"THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization."

JUST about every country that went by the infamous O.I.E. B.S.E. guidelines, most all came down with B.S.E. ...TSS

NOW, some history on the failed OIE BSE/TSE policy, and why the OIE allowed BSE and other TSE to spread around the globe $$$


i proposed to OIE years ago to include CWD. but with these new atypical case of TSE showing up in cattle and sheep, it will be interesting to see how the OIE handles the USA demands on weakening the BSE/TSE regs for exporting countries;

Date: Fri, 12 Jul 2002 16:11:42 -0700

Reply-To: B S E-l

Sender: Bovine Spongiform Encephalopathy

From: TSS

Subject: CWD/USA — CWD/OIE?


Greetings List Members,

speaking with someone at the OIE about my concerns with CWD and the non-testing for TSEs in USA cattle, i find it very sad that the OIE does not follow CWD related issues. BUT, they voice my same concerns and said changes are in the makings. sadly, the changes will take about 2 years?


''I agree with you Dr Terry. The OIE, namely the International Animal Health Code Commission is working on making proposals to Member Countries to change the OIE lists so to avoid some the problems mentioned in you e-mail. This will take at least two years before adoption by the International Committee.''


two years is a very long time, on an issue of such importance to both humans and animals...

kind regards, terry


PAGE 25 Transmission Studies Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculam (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in all of these species with the shortest incubation period in the ferret...

Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs.


and why do we not want to do TSE transmission studies on chimpanzees $


5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.



same reason CJD/TSE is not reportable Nationally in the USA. same reason no CJD questionnaire exists in the USA that is issued to all victims and families of victims asking real questions pertaining to route and source of agent. no autopies for all demented of young AND OLD! same reason the USA is steadfast refusing to this day to rapid TSE test all cattle for human/animal consumption. the USA simply does not want to know$

hell, we should just retain it all, and just play like it has not happened for the next 40 years as well. hmm, something else to ponder ;

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man

so, when/where is our first case transmission study of TSE on man going to be? i wish to witness this and have a few suggestions for our first human guinea-pigs ;-)

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, TEXAS USA 77518

----- Original Message -----

From: "Terry S. Singeltary Sr."


Sent: Saturday, June 04, 2005 8:07 AM

Subject: BSE OIE CHAPTER 2.3.13 (The Weakening of a already terribly flawwed BSE/TSE surveillance system)

################Bovine Spongiform Encephalopathy #####################

C H A P T E R 2 . 3 . 1 3 .



SNIP...please see full text ;

putting the cart before the horse OIE TSE policy. ...TSS

Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

Sunday, December 12, 2010

EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010

Thursday, December 23, 2010

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

Thursday, November 18, 2010

Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep

Sunday, October 3, 2010

Scrapie, Nor-98 atypical Scrapie, and BSE in sheep and goats North America, who's looking ?

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.


The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404





A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates.

One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.



Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

Wednesday, February 16, 2011




Sunday, April 18, 2010


Monday, April 25, 2011

Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011

Monday, November 30, 2009


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

Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

Thursday, July 14, 2011

Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)

Monday, June 27, 2011

Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease


If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...

Thursday, June 2, 2011

USDA scrapie report for April 2011 NEW ATYPICAL NOR-98 SCRAPIE CASES Pennsylvania AND California

Monday, June 20, 2011 2011

Annual Conference of the National Institute for Animal Agriculture ATYPICAL NOR-98 LIKE SCRAPIE UPDATE USA

Saturday, January 29, 2011

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

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

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"

Sunday, June 26, 2011

Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

Monday, February 7, 2011

FDA’s Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

Thursday, December 29, 2011

Aerosols An underestimated vehicle for transmission of prion diseases?


please see more on Aerosols and TSE prion disease here ;

Saturday, December 31, 2011

Depopulation Plan Being Developed for Captive Deer Facility in Macon County after second CWD positive confirmation

please see this game farm that was shut down, and the incredible infection rate ;

Tuesday, December 20, 2011


Wednesday, December 21, 2011

CWD UTAH San Juan deer hunting unit

Monday, November 14, 2011

WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

Wednesday, November 16, 2011

Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

Sunday, November 13, 2011


Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates


Wednesday, September 08, 2010


Wednesday, January 5, 2011



David W. Colby1,* and Stanley B. Prusiner1,2


Saturday, December 3, 2011

Isolation of Prion with BSE Properties from Farmed Goat

Volume 17, Number 12—December 2011

Wednesday, October 12, 2011

White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation

Thursday, December 22, 2011

Risk of Prion Disease Transmission through Bovine-Derived Bone Substitutes: A Systematic Review

Clin Implant Dent Relat Res. 2011 Dec 15. doi: 10.1111/j.1708-8208.2011.00407.x. [Epub ahead of print]

Saturday, November 19, 2011

Novel Prion Protein in BSE-affected Cattle, Switzerland

Friday, January 6, 2012

OIE 2012 Training Manual on Wildlife Diseases and Surveillance and TSE Prion disease

Saturday, December 3, 2011

Candidate Cell Substrates, Vaccine Production, and Transmissible Spongiform Encephalopathies

Volume 17, Number 12—December 2011



Friday, December 23, 2011

Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model

Volume 18, Number 1—January 2012 Dispatch

Monday, December 26, 2011

Prion Uptake in the Gut: Identification of the First Uptake and Replication Sites

Friday, December 30, 2011

Detection of central nervous system tissue as bovine spongiform encephalopathy specified risk material in traditional Turkish meat products, farmers, and sporadic CJD in Turkey

Research Article

Friday, December 30, 2011

Feds back Quebec R+D for SRM removal equipment Canada

Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD

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.


Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD, and GSS Blood Specimens: the Baxter Study Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3 1Fondation Alliance BioSecure, France; 2University of South Alabama, USA; 3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK; 5Baxter BioSience, Austria Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy (TSE) have documented blood infectivity in both the pre-clinical and clinical phases of disease. Results in a (presumably more appropriate) non-human primate model have not been reported. Objective: To determine if blood components (red cells, white cells, platelets, and plasma) from various forms of human TSE are infectious. Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease (vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from chimpanzees infected with either sCJD or Gerstmann-Sträussler-Scheinker disease (GSS). Animals were monitored for a period of 5 years, and all dying or sacrificed animals had post-mortem neuropathological examinations and Western blots to determine the presence or absence of the misfolded prion protein (PrPTSE). Results: No transmissions occurred in any of the animals inoculated with blood components from patients with sporadic or variant CJD. All donor chimpanzees (sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase plasmapheresis, several months earlier than the expected onset of illness. One monkey inoculated with purified leukocytes from a pre-clinical GSS chimpanzee developed disease after 36 months. Conclusion: No infectivity was found in small volumes of blood components from 4 patients with sporadic CJD and 3 patients with variant CJD. ***However, a single transmission from a chimpanzee-passaged strain of GSS shows that infectivity may be present in leukocytes, and the shock of general anaesthesia and plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.

Saturday, September 5, 2009



Wednesday, June 29, 2011
TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products

Wednesday, August 24, 2011
All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD

Wednesday, August 24, 2011

There Is No Safe Dose of Prions

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 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 Background: 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. Methods: 12 years independent research of available data Results: 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. Conclusion: 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. page 114 ;

Saturday, March 5, 2011


Creutzfeldt-Jakob Disease Public Health Crisis (SEE VIDEO)



Terry S. Singeltary SR.

P.O. Box 42

Bacliff, Texas USA 77518

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