Sunday, March 29, 2015

Uncommon prion disease induced in macaque ten years after scrapie inoculation

O35

 

J. Mikol1, S. Luccantoni-Freire1, E. Correia1, N. Lescoutra-Etchegaray1, V. Durand1, C. Dehen1, J.P. Deslys1, E. Comoy1

 

1Institute of Emerging Diseases and Innovative Therapies, Service of Prion Diseases, Atomic Energy Commission, 18 Route du Panorama 92265 Fontenayaux- Roses, France

 

E-mail: jacqueline.mikol@wanadoo.fr

 

Uncommon prion disease induced in macaque ten years after scrapie inoculation

 

Introduction: Bovine Spongiform Encephalopathy (BSE) is the single animal prion disease reputed to be zoonotic, inducing variant of Creutzfeldt-Jakob Disease (vCJD) in man, and therefore strongly conditioned the protective measures. Among different sources of animal prion diseases, we show here that after more than ten years of incubation, intracerebral injection of a sheep scrapie isolate can induce a prion disease in cynomolgus macaque, a relevant model of human situation towards several prion strains. Neuropathological studies showed classical and uncommon data.

 

Material and method: The cynomolgus macaque was intracerebrally exposed to a classical scrapie isolate issued from a naturally infected sheep flock. Upon onset of clinical signs, euthanasia was performed for ethical reasons. Classical methods of biochemistry and neuropathology were used.

 

Results: The three elements of the triad were present:

 

spongiosis was predominant in the cortex, the striatum, the cerebellum. Neuronal loss and gliosis were moderate.

 

The notable data were the following

 

(i) the brain was small, the atrophy involved mostly the temporal lobe in which axonal loss was histologically demonstrated

 

(ii) the spongiosis of the Purkinje cells was so intense that most of them were destroyed

 

(iii) there was a neuronal loss and a massive gliosis of the dorsomedialis nucleus of the thalamus

 

(iv) iron deposits were present in the lenticular nucleus. PrPres heavily distributed in the cortex, the basal ganglia and the cerebellum consisted in synaptic deposits and aggregates. Western Blot exhibited a type 1 PrPres in all parts of the brain.

 

Conclusion: We described here the successful transmission of a scrapie prion disease to a non-human primate after an extended incubation period, leading to a fatal, non-relapsing neurological disease with all the features of a prion disease. The cerebral lesional profile we observed was original in comparison to other animal prion diseases (c-BSE, L-type BSE, TME) we previously experimentally transmitted in this model.

 


 

Tuesday, December 16, 2014

 

Evidence for zoonotic potential of ovine scrapie prions

 

Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics

 

Abstract

 

Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 

Subject terms: Biological sciences• Medical research At a glance

 


 

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.

 

snip...

 

R. BRADLEY

 


 

Suspect symptoms

 

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 

28 Mar 01 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb...

 

2001

 

Suspect symptoms

 

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 

28 Mar 01

 

Like lambs to the slaughter

 

31 March 2001

 

by Debora MacKenzie Magazine issue 2284.

 

FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

 

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.

 

Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.

 

Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.

 

As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.

 

"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.

 

But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.

 

People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.

 

But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."

 

There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.

 

Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.

 


 

Friday, January 30, 2015

 

*** Scrapie: a particularly persistent pathogen ***

 


 

Thursday, March 26, 2015

 

Increased Infectivity of Anchorless Mouse Scrapie Prions in Transgenic Mice Overexpressing Human Prion Protein

 


 

Thursday, March 26, 2015

 

National Scrapie Eradication Program Monthly Report - February 2015

 


 

Thursday, March 26, 2015

 

Variant CJD and blood transfusion: are there additional cases?

 

Vox Sanguinis (2014) 107, 220–225 ORIGINAL PAPER © 2014 International Society of Blood Transfusion DOI: 10.1111/vox.12161

 



Sunday, May 18, 2008

MAD COW DISEASE BSE CJD CHILDREN VACCINES

http://bseinquiry.blogspot.com/2008/05/mad-cow-disease-bse-cjd-children.html

 


 


 

 

TSS

Friday, February 20, 2015

A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015)

Transcript - Briefing (February 18, 2015) Date/Date: February 18, 2015 4:00 p.m.

 

Location/Endroit: Teleconference, Ottawa, Ontario

 

Principal(s)/Principaux:

 

Dr. Martine Dubuc, Vice-President, Science, CFIA, and Delegate for Canada to the World Organisation for Animal Health Paul Mayers, Vice-President, Policy and Programs, CFIA Subject/Sujet: The Canadian Food Inspection Agency Holds a Technical Briefing to Provide More Information on a BSE (Bovine Spongiform Encephalopathy) Find in Alberta.

 

Moderator: Bon après-midi et merci d'être ici avec nous pour notre séance d'information technique de l'Agence Canadienne d'inspection des aliments. Thank you for joining us today for the Canadian Food Inspection Agency's technical briefing. Mon nom est Guy Gravelle et je suis le gestionnaire des relations avec les médias pour l'ACIA ainsi que le modérateur pour aujourd'hui.

 

In the room today we have a few delegates from the CFIA – Paul Mayers, Vice President for Policy and Programs for the CFIA ainsi que Dr. Martine Dubuc, Vice-présidente de la direction des sciences et déléguée pour le Canada à l'Organisation mondial de la santé animale. Thank you for joining us here today.

 

We will begin today's technical briefing with an update on the investigation into BSE in Alberta with a statement from the CFIA. We will then open the phone lines up for a question and answer period. J'aimerais maintenant céder la parole à Paul Mayers.

 

Paul Mayers: Thank you very much Guy. Good afternoon everyone and thank you for calling in today. We would like to provide an update on the Canadian Food Inspection Agency's investigation into bovine spongiform encephalopathy or BSE in a beef cow from Alberta. First of all I'd like to remind you that no part of the animal's carcass entered the human food or animal feed systems.

 

Canada's suite of internationally recognized safeguards effectively protects the safety of food and animal feed. There is no risk to food safety. The CFIA's investigation into BSE in Alberta has determined that the affected cow was born in March 2009. We were also able to confirm the location of the birth farm yesterday evening. We can also confirm that both the birth and index farm are located in northern Alberta near Edmonton.

 

The index farm is in the municipality of Spruce Grove. At this time the CFIA cannot release the municipality of the birth farm as the agency is still engaged in an active investigation at this premise. The CFIA was able to trace the animal to its original birth farm through the combination of reviewing the records at the index farm as well as a tattoo in the ear of the animal.

 

Both the index farm and birth farm continue to cooperate with the CFIA throughout this investigation. With regards to the birth farm, the CFIA is continuing its investigation efforts focusing on a review of on farm animal and feed records to verify if there are other animals that are of equivalent risk.

 

The CFIA will determine the source of the feed used at the birth farm in 2009 and assess any potential risk factors in relation to the feed used. As a precautionary measure, CFIA inspectors will review the records of the feed mills from which the feeds were sourced to verify compliance with the enhanced feed ban. The enhanced feed ban was put in place to accelerate Canada's progress towards the reduction of this disease.

 

As this progression continues, the detection of a small number of cases among the 30,000 samples tested yearly as part of our BSE surveillance program is not unexpected. This has been the case for most countries that are managing BSE. These cases are extremely rare in Canada. This has been the first case in four years. The number of cases seen in Canada has been steadily declining.

 

This is due to the strong measures Canada has in place. The enhanced feed ban, the comprehensive program of surveillance and the removal and disposal of specified risk materials serve to effectively protect public health and minimize the potential for recycling infection in the cattle population. Bill C18, currently before Parliament, includes provisions which will enhance the Feeds Act and the Health of Animals Act providing further regulatory tools for the CFIA in these types of situations.

 

The government of Canada is committed to protecting human and animal health and takes the management of BSE very seriously. The case has been reported to the World Organisation for Animal Health, also known as the OIE, in line with Canada's international obligations and our commitment to transparency. Canada is officially recognized under the OIE's science based system as a controlled BSE risk country.

 

This status clearly acknowledges the effectiveness of Canada's surveillance, mitigation and disease management measures and acknowledges the work done by all levels of government, the cattle industry, veterinarians and ranchers to effectively manage BSE in Canada. The OIE has stated that this one case won't change this status.

 

Therefore we expect our trading partners on the basis of this status to not make any changes to market access for Canadian beef. However, market access decisions are for individual countries to make. South Korea has imposed a temporary restriction on the importation of Canadian beef pending further information on the confirmed BSE find. Indonesia has temporarily suspended importation of non-edible by-products.

 

Again, the CFIA is strongly committed to protecting animal health. Our investigation is underway and we are mobilizing all necessary resources to address this situation. More information on BSE and the actions Canada has taken to address the disease are available at the CFIA website at inspection.gc.ca. Thank you very much.

 

Moderator: Thank you Mr. Mayers. Maintenant la déclaration de l'ACIA en français, Dr. Dubuc.

 

Dr. Martine Dubuc: Bonjour à tous et merci de votre présence aujourd'hui. Nous souhaitons présenter une mise à jour concernant l'enquête de l'Agence Canadienne d'inspection des aliments sur le cas d'encéphalopathie spongiforme bovine ou communément appelé ESB chez une vache d'une boucherie de l'Alberta.

 

D'abord je tiens à vous rappeler qu'aucune partie de la carcasse de l'animal ne s'est retrouvée dans l'alimentation humaine et celle des animaux. Les mesures de protection du Canada qui sont reconnues à l'échelle internationale protègent efficacement la salubrité des aliments et également la salubrité des aliments du bétail.

 

Il n'y a aucun risque pour la salubrité des aliments. L'enquête de l'Agence Canadienne d'inspection des aliments sur le cas d'ESB en Alberta a permis de déterminer que la vache infectée est née en mars 2009. Hier soir nous avons également été en mesure de confirmer l'exploitation où l'animal est né.

 

Nous pouvons également confirmer que l'exploitation où la vache est née et l'exploitation de référence sont situées dans le nord de l'Alberta près d'Edmonton. L'exploitation de référence est située dans la municipalité de Spruce Grove. Pour le moment l'ACIA ne peut pas divulguer dans quelle municipalité se situe l'exploitation où l'animal est né car l'ACIA mène encore une enquête axée sur ces lieux.

 

L'ACIA a pu retracer l'animal jusqu'à l'exploitation ou il est né grâce à l'examen des registres à l'exploitation de référence ainsi qu'à un tatouage dans l'oreille de l'animal. L'exploitation de référence et l'exploitation où est né l'animal continuent de collaborer avec l'ACIA dans le cadre de cette enquête.

 

En ce qui concerne l'exploitation où la vache est née, l'ACIA poursuit son enquête en mettant l'accent sur un examen des dossiers liés à l'alimentation animale ainsi qu'à l'élevage des animaux à la ferme dans le but de vérifier si d'autres animaux posent un risque équivalent.

 

L'ACIA déterminera la source des aliments de bétail qui étaient utilisés en 2009 à l'exploitation où la vache est née et évaluera tous les facteurs de risque possible en lien avec les aliments du bétail utilisés. À titre de mesures de précaution des inspecteurs de l'ACIA examineront les registres des (inaudible) d'où proviennent les aliments de bétail afin de vérifier s'ils respectaient l'interdiction renforcée frappant les aliments de bétail au Canada.

 

Cette interdiction visait à permettre au Canada d'atténuer le plus rapidement possible la propagation de la maladie. Cette progression se poursuit et nous attendons à ce qu'un petit nombre de cas soit détecté parmi les 30,000 échantillons qui sont analysés à chaque année dans le cadre de notre programme de surveillance de l'ESB et souvent non inattendu.

 

Il en est ainsi pour la majorité des pays qui luttent contre l'ESB. Ces cas sont extrêmement rares au Canada. C'est le premier cas que nous détectons dans les quatre dernières années. Le nombre de cas détecté au Canada ne cesse de diminuer. Cette diminution est attribuable aux mesures strictes que le Canada met en place.

 

L'interdiction renforcée frappant les aliments de bétail, le programme exhaustif de surveillance ainsi que le retrait et l'élimination des matières à risque spécifiées ont pour but de protéger efficacement la santé publique et de minimiser les risques que l'infection se reproduise chez tels bovins. Le projet de loi C18 qui est actuellement examiné par le Parlement comprend également des dispositions qui renforceront la loi relativement aux aliments de bétail et la loi sur la santé des animaux fournissant ainsi d'autres outils réglementaires à l'Agence et ses inspecteurs dans ces types de situation.

 

Le gouvernement du Canada est déterminé à protéger la santé humaine et animale et prend très au sérieux la gestion des cas d'ESB. Le cas a été également signalé à l'Organisation Mondiale de la santé animale aussi connu sous le nom de l'OIE conformément aux obligations du Canada sur la scène internationale et à son engagement en matière de transparence.

 

Le Canada est officiellement reconnu en vertu du système scientifique de l'Organisation Mondiale de la santé animale comme étant un pays à risque maitrisé. Ce status reconnait clairement l'efficacité des mesures de surveillance, d'atténuation et de gestion des maladies en vigueur au Canada et reconnait également le travail effectué par tous les ordres du gouvernement, par l'industrie bovine, par les vétérinaires et par les éleveurs afin de gérer de façon efficace l'ESB au Canada.

 

L'OIE a confirmé que ce cas spécifique n'aura pas d'impact sur notre status. Compte tenu de ce status nous nous attendons également à ce que nos partenaires commerciaux n'apportent aucun changement à l'accès au marché au bœuf canadien. Il revient toutefois à chaque pays de prendre des décisions relatives à l'accès au marché. Le Corée du Sud a ainsi imposé des restrictions temporaires sur l'importation de bœuf canadien dans l'attente d'obtenir de plus amples renseignements sur le cas confirmé d'ESB.

 

L'Indonésie a suspendu récemment temporairement l'importation des sous-produits de viande non comestibles. Encore une fois l'ACIA est fermement résolue à protéger la santé des animaux. Notre enquête est toujours en cours et nous mobilisons toutes les ressources nécessaires dont nous avons besoin pour régler cette situation. Pour obtenir plus d'information sur l'ESB et sur les mesures que le Canada a pris pour lutter contre cette maladie, nous vous invitons à consulter le site web de l'Agence à l'adresse inspections.gc.ca.

 

Je vous remercie de votre attention.

 

-30-

 


 

 Wednesday, February 18, 2015

 

OIE Bovine spongiform encephalopathy ,Canada

 


 

Saturday, February 14, 2015

 

Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta

 


 

Tuesday, February 17, 2015

 

Could we spot the next BSE?, asks BVA President

 


 

 

 

TSS

APHIS Freedom of Information Act (FOIA) Appeal Mouse Bio-Assays 2007-00030-A Sheep Imported From Belgium and the Presence of TSE Prion Disease Kevin Shea to Singeltary 2015


APHIS Freedom of Information Act (FOIA) Appeal Mouse Bio-Assays 2007-00030-A Sheep Imported From Belgium and the Presence of TSE Prion Disease Kevin Shea to Singeltary 2015

 

 

Greetings BSE-L Members et al,

 

you can’t believe what I got in the US Postal mail today. the wife would not pick it up yesterday, because there was a $6.00 charge for a certified letter from USDA Kevin Shea for about 5 pages. I went to the PO today, told the girls in the back that if it’s an affidavit, a warrant, summons, I don’t want it, send it back. but it was certified. scared me. but the curiosity got to me, so i coughed up 6 bucks, and took a chance. low and behold, after my last appeal to this decade plus old quest was turned down, even though I already had the answer from another source, APHIS et al finally stumbled across those old mouse bio-assays. they had them all along.

 

what the industry sent me first was better, because it had some of the good stuff i.e. redacted.

 

this all started way back around the year 2,000, when in my opinion, the USDA et al let these sheep in the USA from Belgium, when they should not have because of atypical BSE in Belgium. I started asking for the these mouse bio-assays back in or around 2003 or before, then I had to get official with FOIA, because no one would answer my questions.

 

well, it’s February 20, 2015, over a decade later, and I don’t know how many denials, here’s what was in the mail yesterday, February 19, 2015 ;

 

United States Department of Agriculture

 

Animal and Plant Health Inspection Service Marketing and Regulatory Programs Animal and Plant Health Inspection Service Legislative and Public Affairs Freedom of Information 4700 River Road Unit 50 Riverdale, MD. 20737-1232

 

FEB 10 2015

 

Terry S. Singletary Sr. P.O. Box 42 Bacliff, Texas 77518

 

Re: FOIA Appeal # 2007-00030-A

 

Dear Mr. Singletary:

 

This letter is in response to the Freedom of Information Act (FOIA) appeal that you submitted regarding FOIA request 07-566. Your appeal challenged the APHIS FOIA Office's search for the "Mouse Bio-Assays" on the sheep imported from Belgium. We apologize for the delayed response.

 

The APHIS FOIA Office received your appeal, on July 7, 2007 and assigned it FOIA case number 2007-00030-A.

 

In response to your appeal, the APHIS FOIA Office performed a second search of records responsive to your initial request. The Agency has since found four (4) pages of responsive records for the "Mouse Bio-Assays" dated October 22,2009. Although these records postdate both your initial request and subsequent appeal by approximately two years; we enclose them in the interest of responsiveness to your request.

 

We now consider this appeal closed and will take no further action. If you are dissatisfied with this decision, you have the right to judicial review in an appropriate United States District Court in accordance with 5 U.S.C. 552, (2)(4)(B).

 

Prior to seeking judicial review, you may contact the Office of Government Information Services (OGlS). OGIS was created within the National Archives and Records Administration when the Open Government Act of 2007 amended the FOIA. OGIS provides mediation of FOIA disputes between appellants and federal agencies. Participation in mediation does not affect your right to judicial review. Contact information for OGIS can be found at http:/www.archives.gov/ogis.

 

Sincerely,

 

Kevin Shea Administrator Enclosure

 

snip...end

 

the next 4 pages is exactly what I received from an industry source way back on Saturday, February 27, 2010. see ;

 

Saturday, February 27, 2010

 

FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27, 2010 http://foiamadsheepmadrivervalley.blogspot.com/2010/02/final-report-of-testing-of-belgian.html

 

see history below ;

 

From: Terry S. Singeltary Sr. Sent: Thursday, February 19, 2015 10:04 PM To: Terry S. Singeltary Sr. Subject: mad sheep mad river valley...NOT...never was

 

Veterinary Laboratories Agency – Weybridge

 

New Haw, Addlestone, Surrey KT15 3NB United Kingdom

 

Telephone +44 (0)1932 341111 Facsimile +44 (0)1932 347046 '

 


 

Veterinary Laboratories Agency

 

Your ref: MPL-6197-7-37

 

Our ref: FT1294

 

This is the FINAL report for contract MPL-6197-7-37 The testing of the Belgian (Vermont) sheep.

 

Background

 

Brain homogenate (10% in normal saline) from each case was inoculated intracerebralty into panels of 20 Rlll and 20 Tg338 mice.

 

FT1294/0001 (Sample 4677) was inoculated into mice on the 14/12/06

 

FT1294/0011 (Sample 4703) was inoculated into mice on the 20/12/06

 

Method

 

The brain from each mouse was examined histologically for any evidence of TSE-related vacuolation, and immunolabelled using anti-PrP antibody Rb486 as described elsewhere1, All slide interpretation was undertaken blind with regard to the clinical status of the mouse, or the source of the inoculum.

 

Final bioassay results

 

FT1294/0001 (Sample 4677)

 

Tg338 mice - All 20 mice are negative by histopathology, and immunohistochemistry

 

Rlll mice - All 20 mice are negative negative by histopathology, and immunohistochemistry

 

FT1294/0011 (Sample 4703)

 

Tg338 mice - All 20 mice are negative by histopathology, and immunohistochemistry

 

Rlll mice - All 20 mice are negative by histopathology, and immunohrstochernistry

 

The survival times for these mice can be seen in the figures below. Additional data sets from positive and negative inocula (J Spiropoulos, pers. comm.) have been included for comparison._

 

1 Beck KE, Chaplin M, Stack M: Sallis RE, Simonini S, Lockey R, Spiropoulos J. Lesion Profiling at Primary Isolation in Rlll Mice Is Insufficient in Distinguishing BSE from Classical Scrapie. Brain Pathol. 2009 epub ahead of print

 

FINAL report for contract MPL-6197-7-37

 

VLA is an Executive Agency of the Department for Environment, Food and Rural Affairs (Defra)

 

snip... (2 pages of charts and graphs of survival and comparison of tg338 data NOT included here...TSS)

 

CONCLUSION

 

These mice have survived for long enough to have demonstrated the presence of classical scrapie, atypical scrapie, or ovine BSE if any of these strains was present in the inoculum.

 

Both samples are negative by bioassay.

 

Dr. Marion M Simmons

 

22nd October 2009

 

February 27, 2010, INVESTIGATION OF MAD SHEEP OF MAD RIVER VALLEY COMPLETE. THEY WERE NOT INFECTED WITH ANY TSE. ...TSS

 

Greetings again BSE list members,

 

The investigation of the Mad Sheep of Mad River Valley may be complete now, but, I still have questions.

 

PLEASE SEE MY FINAL FOIA HERE ;

 

Monday, September 1, 2008

 

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [No. 00-072-1] September 1, 2008

 

Greetings again BSE-L members,

 

I had a pleasant surprise this past Saturday. I got an unexpected package from O.I.G. on my old F.O.I.A. request, of the final test results of the infamous mad sheep of mad river valley. IF you all remember, back on Thu, 24 Apr 2008 15:00:20 -0500 I wrote ;

 

Greetings,

 

With great disgust, I must report, that after years and years of wrangling over the infamous mad sheep of mad river valley, I have failed in getting an official answer via FOIA on the outcome of the TSE testing of those imported Belgium sheep. The USA Government refuses to tell the public, exactly what the testing outcome was, and in doing so, shows just how corrupt this administration has been. and the excuse given in their answer to my final appeal, which they have now officially denied, was bizarre to say the least ;

 

"I am denying your FOIA appeal. This is the final agency decision. You may seek judicial review of this decision in the United States district court for the judicial district in which you reside or have your principal place of business or in the District of Columbia, pursuant to 5 U.S.C. & 552(a)(4)(B)."

 

FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1] ...snip...end...TSS

 

NOW, out of the wild blue, AFTER them telling me they denied my FOIA appeal for the final time, any further action would have to be judicial review in the United States district court, I get 25+ pages, a lot of redacted names, etc, but this is the first time they sent me anything about this in the 6 years of waiting for my FOIA request. IT will take me a long time to get this online due to the fact you cannot hardly read it, very poor quality and eligibility of text. BUT, the just of it is, somebody (REDACTED) screwed those tests up. I will work to get all the data online next week or so, but it is odd how much they were concerned for human and animal health from an atypical scrapie of foreign origin back then, but yet when we document it here in the USA, you don't hear a word about it. it's a completely different story.

 

IN SHORT ;

 

August 15, 2000

 

OIG case # NY-3399-56 REDACTED, VT

 

''Enclosed is OIG's notification that they have scheduled an investigation of the following individual. REDACTED is alleged to have provided possibly inaccurate test results involving diseased sheep. However, because the results were determined to be inconclusive, no actual violation was actually committed.''

 

snip...

 

IN SHORT ;

 

August 15, 2000

 

OIG case # NY-3399-56 REDACTED, VT

 

''Enclosed is OIG's notification that they have scheduled an investigation of the following individual. REDACTED is alleged to have provided possibly inaccurate test results involving diseased sheep. However, because the results were determined to be inconclusive, no actual violation was actually committed.''

 

snip...

 

[only bush et al could have interpreted it that way. don't all criminals wish this is the way the system worked. ...tss]

 

JULY, 28, 2000

 

Case Opening Memorandum

 

snip...

 

An investigation regarding the subject identified below will be conduced and a report submitted at the conclusion of the investigation. If you have or should later receive additional information concerning this matter, please forward it to this office.

 

If you believe that administrative action should be taken before all criminal and other legal matters are completed, please coordinate that action with this office in order not to jeopardize the ongoing investigation.

 

The fact that this subject is under investigation should not be discussed with anyone who does not have a need to know and all inquiries on the investigation should be referred to the office of Inspector General.

 

snip...end

 

FOR OFFICIAL USE ONLY FEBRUARY 7, 2002

 

SUBJECT OIG CASE NY-3399-56 REDACTED VT HEALTH/SANITATION VIOLATION

 

TO: William Buisch, Regional Director Eastern Region, VS Raleigh, NC

 

Enclosed is the official investigation report on REDACTED. If you will recall, REDACTED is alleged to have provided possible inaccurate test results involving diseased sheep.

 

OIG is closing their file upon issuance of the Report of Investigation (copy enclosed). We are, therefore, also closing our case file.

 

REDACTED

 

Resource Management Systems and Evaluation Staff

 

Enclosure

 

cc:

 

REDACTED IES, Riverdale, MD (w/cy of incoming)

 

APHIS:RMSES: REDACTED 2/7/02 "NY-3399-56-REDACTED Closure''

 

END...TSS

 

NOW, the question is, who screwed those test up, and was it done on purpose, just to cover someone's ass for letting those sheep in here in the first place ???

 

WHICH tests were compromised, one of them, all of them, and, can we trust the outcome of any of these test under the circumstances here ???

 

i.e.

 

"It is significant that four of the sheep which first tested positive on REDACTED Western blot tests, thereby providing the type of confirmation the plaintiffs argue is lacking on the current record."

 

UNDER what circumstances were these test compromised ???

 

MY basic, simple question, was not answered in layman term, i.e. exactly what strain of TSE did those sheep have ???

 

IS this the best we can do ???

 

>>>"REDACTED is alleged to have provided possibly inaccurate test results involving diseased sheep. However, because the results were determined to be inconclusive, no actual violation was actually committed.''<<<

 

PLEASE SEE FULL TEXT HERE ;

 


 

Saturday, February 27, 2010

 

*** FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27, 2010 IN SHORT ; August 15, 2000 OIG case # NY-3399-56 REDACTED, VT ''Enclosed is OIG's notification that they have scheduled an investigation of the following individual. REDACTED is alleged to have provided possibly inaccurate test results involving diseased sheep. However, because the results were determined to be inconclusive, no actual violation was actually committed.''

 

FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27, 2010

 

(10 YEARS LATER, FOIA, none of the sheep had any TSE at all...tss)

 


 

Thursday, April 24, 2008

 

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

 


 

Monday, September 1, 2008 RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [No. 00-072-1]

 


 

 FOIA MAD SHEEP MAD RIVER VALLEY

 

Tuesday, November 13, 2007

 

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

 

To: Garfield.O.Daley@aphis.usda.gov

 

CC: phyllis.Fong@usda.gov; bse-L@aegee.org;

 

Re: FOIA APPEAL 07-566 DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

 

November 13, 2007

 

Greetings Garfield O. Daley, Acting FOIA Director, and USDA et al,

 

SNIP

 

for those interested, please see full text answer below received from USDA et al below on latest appeal ;

 


 


 

Tuesday, November 13, 2007

 

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

 

To: Garfield.O.Daley@aphis.usda.gov

 

CC: phyllis.Fong@usda.gov; bse-L@aegee.org;

 

Re: FOIA APPEAL 07-566 DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

 


 

EXACTLY WHAT are these people capable of doing ???

 

JUST HOW FAR will they go ???

 

Mad Sheep The True Story Behind the USDA‚ War on a Family Farm Linda Faillace

 

The page-turning account of a government cover-up, corporate greed, and a courageous family‚ fight to save their farm.

 


 


 


 

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

 

From: Terry S. Singeltary Sr.

 


 

Sent: Sunday, February 25, 2007 12:35 PM

 

Subject: FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP

 

Greetings USDA,

 

I respectfully request the final results of the mouse bio-assays test that were to have supposedly began 2+ years late, 5 years ago, on the imported sheep from Belgium ?

 

WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported sheep from Belgium that were confiscated and slaughtered from the Faillace's, what sort of TSE did these animals have ?

 

WERE they atypical scrapie, BSE, and or typical scrapie ?

 

HOW much longer will you refuse to give us this information ? and for what reason ?

 

WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

 

snip.

 

full text ;

 


 

UNITED STATES DEPARTMENT OF AGRICULTURE OFFICE OF INSPECTOR GENERAL WASHINGTON D.C. 20250

 

DEC 28, 2007

 

Mr. Terry S. Singeltary, Sr. P.O. Box 42 Bacliff, Texas 77518

 

Subject: FOIA Appeal-Log No. 08-00034 (No. 07-00060)

 

Dear Mr. Singeltary:

 

This is in response to your December 3, 2007, Freedom of Information Act (FOIA), 5 U.S.C. & 552, appeal of the November 20, 2007, decision of Ms. Deirdre MacNeil, FOIA/Privacy Act (PA) Attorney, Office of Inspector General (OIG), Department of Agriculture (USDA). As explained below, your FOIA appeal is denied.

 

As background, on March 1, 2007, you requested the "final results of the TSE Mouse-bioassays of those Atypical TSE in the Vermont Sheep." FOIA requires the release of agency records except where one or more of the nine enumerated exceptions apply. On November 20, 2007, Ms. MacNeil responded to your request by sending you seven pages from Hotline files PS-3340-0024, which was responsive to your request. Ms. MacNeil withheld identifying information pursuant to Exceptions 6 and 7(C) of the FOIA. See 5. U.S.C.& 552(b)(6) and (7)(C). On December 3, 2007, you appealed Ms. MacNeils decision.

 

12-3-07

 

To The Honorable Inspector General USDA,

 

I respectfully "APPEAL" the decision to withhold information I requested under the F.O.I.A. About the final results of the T.S.E. Mouse-bioassays of the Atypical T.S.E. in the Vermont Sheep imported from Belgium and later confiscated and slaughtered under a "Extra Ordinary Declaration of Emergency due to Atypical T.S.E. in U.S.A. sheep.

 

Log Number 07-00060 FOIA 07-566

 

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

 

Exemption 6 permits the Government to withhold information about individuals in "personnel and medical files and similar files the disclosure of which would constitute a clearly unwarranted invasion of personal privacy." 5 U.S.C. & 552 (b)(6). To warrant protection under Exemption 6, information must first meet a threshold requirement by falling within the category of personnel and medical files and similar files. Id. Information fits into a "similar file" if it contains information regarding a particular individual. See United States Dept of State V. Washington Post Co., 456, 601-02 (1982). The threshold is met in this case, as the memorandum contains information regarding particular named individuals.

 

Exemption 7(C) protects from disclosure law enforcement information, the disclosure of which "could reasonably be expected to constitute and unwarranted invasion of personal privacy." 5 U.S.C. & 552(b)(7)(C. Under Exemption 7(C), it has been held that a protectible privacy interest exists in the identities of investigative agents. See Senate of

 

Mr. Terry S. Singeltary, Sr. Page 2

 

Puerto Rico v. United States Dep't of Justice, 823 F.2d 574, 588-89 (D.C. Cir. 1987); Nishnic v. United States Dep't of Justice, 671 F. Supp. 776, 789 (D.D.C. 1987). Such a privacy interest exists in this case, as the withheld information contains the identities, including names and identifying information, of investigative agents in the memorandum.

 

Once it is determined that a privacy interest exists, Exemptions 6 and 7(C), of FOIA require a balancing of interests between the public interest served by disclosure and an individual's right to privacy. See, e.g., Senate of Puerto Rico, 823 F.2d at 587; Dep't of the Air Force v. Rose, 425 U.S. 352, 372 (1976). Determination of whether disclosure is warranted turns not upon the particular purpose for which the document is requested, but upon the nature of the requested document and its relationship to the central purpose of FOIA, which is to "open agency action to the light of public scrutiny." United States Dep't of Justice v. Reporters Comm. for Freedom of the Press, 489 U.S. 749, 772-73 (1989) (quoting Rose, 425 U.S. at 372). I have determined that the release of the withheld information, of investigative agents in the memorandum, would not serve the public interest. Therefore, I am denying your appeal with respect to the withholdings pursuant to Exemptions 6 and 7(C).

 

In addition to appealing the exemptions pursuant to 6 and 7(C), you appear to take issue with USDA's Animal $ Plant Health Inspection Service's (APHIS) response to your FOIA requests with APHIS. You may contact APHIS regarding the status of any such requests by contacting Mr. Garfield Daley, Acting FOIA Officer, at (301)734-5273, 4700 River Road, Unit 50, Riverdale, MD, 20737-1232

 

Finally, in your appeal, you seek answers to a series of questions posed to various USDA officials, including the Inspector General, However, FOIA allows requesters to access records only. It does not require Federal agencies to answer questions, render opinions, provide subjective evaluations, or create explanatory materials, See, e.g., NLRB v. Sears, Roebuck & Co. 421 U.S. 132, 162 (1975); Zemansky v. Epa, 767 f2d 569, 574 (9th Cir. 1985); Flowers v. IRS, 307 F. Supp. 2d 60, 71 (D.D.C. 2004); Citizens Progressive Alliance v. U.S. Bureau of Indian Affairs, 241 F. Supp. 2d 1342, 1364-65 (D.N.M. 2002); Hudgins v. IRS, 620 F. Supp. 19, 21 (D.D.C. 1985). As FOIA requires an agency only to produce responsive non-exempt records to a requester, OIG is not obligated to answer questions regarding the TSE occurrence as you requested. Therefore, I am denying your appeal with respect to your questions.

 

Mr. Terry S. Singeltary, Sr. Page 3

 

For these reasons, I am denying your FOIA appeal. This is the final agency decision. You may seek judicial review of this decision in the United States district court for the judicial district in which you reside or have your principal place of business or in the District of Columbia, pursuant to 5 U.S.C. & 552(a)(4)(B).

 

Sincerely,

 

David R. Gray

 

FOR

 

Phyllis K. Fong

 

Inspector General

 

=======END...TSS...4.24.08=======

 

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

 

From: Terry S. Singeltary Sr.

 

To: Boyd.Rutherford@usda.gov

 

Sent: Sunday, February 25, 2007 12:35 PM

 

Subject: FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP

 

Greetings USDA,

 

I respectfully request the final results of the mouse bio-assays test that were to have supposedly began 2+ years late, 5 years ago, on the imported sheep from Belgium ?

 

WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported sheep from Belgium that were confiscated and slaughtered from the Faillace's, what sort of TSE did these animals have ?

 

WERE they atypical scrapie, BSE, and or typical scrapie ?

 

HOW much longer will you refuse to give us this information ? and for what reason ?

 

WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years,why not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

 

snip...

 

full text ;

 


 


 

FURTHERMORE, I respectfully request up front, that any fees for this FOIA be wavered due to the fact this information should be free to the public and is in the best interest for the public to have these final results, no financial gain from this FOIA information is to be made either. ...

 

Thank You,

 

kind regards,

 

Terry S. Singeltary Sr.

 

P.O. Box 42

 

Bacliff, Texas USA 77518

 

Imported

 

Belgium/Netherlands

 

Sheep Test Results

 

Background

 

Factsheet

 

Veterinary Services April 2002

 

APHIS

 

snip...

 

Additional tests will be conducted to determine exactly what TSE the animals have BSE or scrapie. These tests involve the use of bioassays that consist of injecting mice with tissue from the infected animals

 

Page 15 of 98

 

8/3/2006

 

and waiting for them to develop disease. This testing may take at least 2 to 3 years to complete.

 


 

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E.

 

(PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES

 


 

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E

 

(PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2]

 


 


 


 

Imported Belgium/Netherlands Sheep Test Results Background Factsheet Veterinary Services April 2002 APHIS

 

snip...

 

Additional tests will be conducted to determine exactly what TSE the animals have BSE or scrapie. These tests involve the use of bioassays that consist of injecting mice with tissue from the infected animals

 

Page 15 of 98

 

8/3/2006

 

and waiting for them to develop disease. This testing may take at least 2 to 3 years to complete.

 


 

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES

 


 

DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2]

 


 

 

> > DEPARTMENT OF AGRICULTURE

> >

> > Office of the Secretary

> >

> > [Docket No. 00-072-2]

> >

> > Declaration of Emergency Because of an Atypical Transmissible

> > Spongiform Encephalopathy (Prion Disease) of Foreign Origin

> >

> > A transmissible spongiform encephalopathy (TSE) (prion disease) of

> > foreign origin has been detected in the United States. It is different

> > from TSE's previously diagnosed in the United States. The TSE was

> > detected in the progeny of imported sheep. The imported sheep and

> > their progeny are under quarantine in Vermont. Transmissible

> > spongiform encephalopathies are degenerative fatal diseases that can

> > affect livestock. TSE's are caused by similar, as yet uncharacterized,

> > agents that usually produce spongiform changes in the brain.

> > Post-mortem analysis has indicated positive results for an atypical

> > TSE of foreign origin in four sheep in Vermont. Because of the

> > potentially serious consequences of allowing the disease to spread to

> > other livestock in the United States, it is necessary to seize and

> > dispose of those flocks of sheep in Vermont that are affected with or

> > exposed to the disease, and their germ plasm. The existence of the

> > atypical TSE of foreign origin represents a threat to U.S. livestock.

> > It constitutes a real danger to the national economy and a potential

> > serious burden on interstate and foreign commerce. APHIS has

> > insufficient funds to carry out the seizure and disposal of animals

> > and germ plasm necessary to eliminate this disease risk. These funds

> > would be used to compensate the owners of the animals and germ plasm

> > for their seizure and disposal in accordance with 21 U.S.C. 134a.

> > Therefore, in accordance with the provisions of the Act of September

 

Page 16 of 98

 

8/3/2006

 

> > 25, 1981, as amended (7 U.S.C. 147b), I declare that there is an

> > emergency that threatens the livestock industry of this country and

> > hereby authorize the transfer and use of such funds as may be

> > necessary from appropriations or other funds available to agencies or

> > corporations of the United States Department of Agriculture to seize

> > and dispose of animals that are affected with or exposed to this TSE,

> > and their germplasm, in accordance with 21 U.S.C. 134a.

 

> >

 

> > Dated: This declaration of emergency shall become effective July 14,

> > 2000. Dan Glickman, Secretary of Agriculture. [FR Doc. 00-18368 Filed

> > 7-19-00; 8:45 am] BILLING CODE 3410-34-P

>

>

> >

> > I was told that ;

> >

> >

 

-------- Original Message --------
Subject: Re: AW: [BSE-L] USDA did not test possible mad cows - Dr. Detwiler, what about those sheep?
Date: Sun, 13 Jun 2004 11:27:24 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de
References: <13 .2d20eaae.2df84fb9="" aol.com=""> <40c8c7a0 .1080107="" wt.net="">


######## Bovine Spongiform Encephalopathy #########
Greetings list members, Thought I should let the list know that Dr. Detwiler kindly replied to my
question about the delayed 'atypical' TSE testing in the Vermont sheep and
tried to explain what caused the delay. If I interpreted it correctly,
seems it was the fault of the U.K. ;
-------- Original Message --------
Subject: Sheep
Date: Sat, 12 Jun 2004 14:26:04 EDT
From: LAVET22@aol.com
To: flounder@wt.net
Mr. Singeltary. I hope this finds you well. As you are aware I left the USDA last
year. I can only update you on the sheep before that time. Contact was
established with the UK on doing the bioassay studies. They agreed.
However, we were prioritized after their own needs, hence the delay. I
am aware that there are now additional labs in Europe running the mouse
bioassay strain typing. You will have to contact USDA for further word.

Linda Detwiler
=========
My reply to Dr. Detwiler;
-------- Original Message --------
Subject: Re: Sheep
Date: Sat, 12 Jun 2004 13:53:57 -0500
From: "Terry S. Singeltary Sr."
To: LAVET22@aol.com
References: <54 .2bd2ac1e.2dfca4bc="" aol.com="">
hello Dr. Detwiler, thanks for your kind reply. > However, we were prioritized after their own needs, hence the delay. not sure i understand that? > You will have to contact USDA for further word. already done that, and there answer was; >5/20/04
>
>Dear Mr. Singeltary,
>
>The Western blot tests on these animals were completed in April of this
>year. That means that we can begin the mouse inoculations. To get the
>results of the Western blot tests, you will need to submit a Freedom of
>Information Act request through our FOIA office. The FAX number there is
>301-734-5941.
>
>Have a nice day,
>
>Jim Rogers
>APHIS LPA
>
and with my previous attempts for information via the FOIA through
this administration (as you are probably very well aware of) they have
all been ignored/refused. so any further attempts would be fruitless i am
sure.
thanks anyway... kindest regards,
Terry
LAVET22@aol.com wrote: > Mr. Singeltary. snip... TSS Terry S. Singeltary Sr. wrote: > ######## Bovine Spongiform Encephalopathy
> #########
>
> Greetings Dr. Detwiler,
>
> glad to see you are still with us, you had become very silent lately.
> hope you are enjoying semi retirement.
>
> recently, i inquired through the BSE-L and via USDA official about
> those Vermont sheep via belgium which there was an Extraordinary
> Declaration of Emergency declared here in the USA due to
> atypical scrapie. The thread is;
>
> Confiscation of Sheep in Vermont and testing results ? Thu, 20 May 2004
> 12:10:03 -0500 "Terry S. Singeltary Sr." Bovine
> Spongiform Encephalopathy BSE-L
>
>
>
>> Imported
>> Belgium/Netherlands
>> Sheep Test Results
>> Background
>> Factsheet
>> Veterinary Services April 2002
>> APHIS
>
>
>
> snip...
>
>> Additional tests will be conducted to determine
>> exactly what TSE the animals have BSE or scrapie.
>> These tests involve the use of bioassays that consist
>> of injecting mice with tissue from the infected animals
>> and waiting for them to develop disease. This testing
>> may take at least 2 to 3 years to complete.
>
>
>
> http://www.aphis.usda.gov/lpa/pubs/fsheet_faq_notice/fs_ahvtsheeptr.pdf
>
> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E.
> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES
>
> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-32
>
>
> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E
> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2]
>
> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-31
>
>
> or if those old urls dont work, go here;
>
> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E
> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES
> - Terry S. Singeltary Sr. 7/20/00 (0)
>
>
> I was told that ;
>
>
> -------- Original Message --------
> Subject: Re: hello Dr. Sutton...question please...scrapie...TSS
> Date: Thu, 20 May 2004 14:36:09 -0400
> From: Jim.D.Rogers@aphis.usda.gov
> To: flounder@wt.net
>
>
>
> Dear Mr. Singeltary,
>
> The Western blot tests on these animals were completed in April of this
> year. That means that we can begin the mouse inoculations. To get the
> results of the Western blot tests, you will need to submit a Freedom of
> Information Act request through our FOIA office. The FAX number there is
> 301-734-5941.
>
> Have a nice day,
>
> Jim Rogers
> APHIS LPA
> =========
>
>
> Dr. Detwiler, my question is, why have these very important test been
> delayed for so long when we were told they were to have been started
> some 2+ years ago?
>
> who made this call to delay these very important test and why ?
>
> thank you,
> with kindest regards,
>
> Terry
>
>
> Linda Detwiler wrote:
>
>> ######## Bovine Spongiform Encephalopathy
>> #########
>>
>> I m responding to Roland's post about my quote in the article by Steve
>> Mitchell. I spent a fair amount of time on the phone with Mr.
>> Mitchell on more than
>> one occasion. The quote was one aspect of our conversation. Even
>> the quote
>> included "probably". I explained about proper location and sampling
>> condition of the brain. I also added in our conversation that the
>> best methodology is
>> to utilize both a test for PrP as well as histopathology when examining
>> brains from cattle with CNS disease. This is why as early as
>> 1993-94 the USDA
>> began using IHC in its TSE testing regime at the National Veterinary
>> Services
>> Laboratory. However, utilizing only a PrP test eliminates the
>> possibility of
>> diagnosing another neurologic disease.
>>
>> All of the tests have advantages and disadvantages. For example, I
>> have been
>> to a number of laboratories in Europe and watched as technicians took
>> the
>> test samples from the brain stem. They sample hundreds per night.
>> If they get
>> distracted the may take the sample lateral, or rostral to the
>> obex. If this
>> animal was in an earlier stage of disease, there may be a very small
>> amount of
>> PrPsc and limited to a single location in the brain. If that sample
>> missed
>> the obex, the sample would be negative and life goes on. With any
>> test using
>> a homogenate I am not aware that there is any cross check for location.
>>
>> With IHC, the pathologist can determine location, however it too has
>> drawbacks in regard to sample condition. There is no perfect test.
>> There are
>> limitations to the tests themselves and there are limitations to all
>> of the aspects
>> of collection. Hence utilizing multiple tests especially for CNS
>> cases is
>> prudent. It is also prudent to examine other locations of the brain
>> in the event
>> a disease changes or something new emerges.
>>
>>
>> Linda Detwiler
>>
>> ######### http://mailhost-alt.rz.uni-karlsruhe.de/warc/bse-l.html
>> ##########
>>
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Saturday, February 27, 2010

 

*** FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP

 

February 27, 2010 IN SHORT ;

 

August 15, 2000

 

OIG case # NY-3399-56 REDACTED, VT

 

''Enclosed is OIG's notification that they have scheduled an investigation of the following individual. REDACTED is alleged to have provided possibly inaccurate test results involving diseased sheep. However, because the results were determined to be inconclusive, no actual violation was actually committed.''

 

snip...

 

PLEASE SEE FULL TEXT HERE ;

 


 

> > Date: Thu, 20 May 2004 14:36:09 –0400

 


 

> > To: flounder@wt.net

 

> > > snip...

 

> > > FULL TEXT AND THREAD BETWEEN TSS, MAFF, USDA AND DR. DETWILER HERE ;

 


 

OpenDocument

 

7. WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

 

SOME DISTURBING TSE DATA FROM BELGIUM ;

 

Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium

 

B. Van Everbroeck1, A. Michotte2, R. Sciot3, C. Godfraind4, M. Deprez5, S. Quoilin6, J. -J. Martin1 and P. Cras1, 7

 

(1) Born-Bunge Institute (BBI), University of Antwerp (UA), Campus Drie Eiken (CDE), Antwerp, Belgium

 

(2) Department of Neuropathology, Academic hospital, Free University of Brussels, Brussels, Belgium

 

Page 17 of 98

 

8/3/2006

 

(3) Department of Pathology, Catholic University of Leuven, Leuven, Belgium

 

(4) Pathology Laboratory, Catholic University of Louvain, Brussels, Belgium

 

(5) Laboratory of Neuropathology, University of Liège, Sart Tilman, Liège, Belgium

 

(6) Institute of Public Health-Louis Pasteur, Brussels, Belgium

 

(7) Laboratory of Neurobiology, BBI, UA, CDE, Universiteitsplein 1, B-2610 Wilrijk, Belgium Received: 28 October 2005 Accepted: 28 March 2006 Published online: 12 July 2006 Abstract From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium. In addition to epidemiological data, information on cerebrospinal fluid biomarkers, prion protein gene and brain neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were referred to the surveillance system. In 85 patients a ‘definite’ diagnosis of sporadic CJD (sCJD) could be made, whereas 26 patients remained ‘probable’. We further identified two unrelated patients with an E200K mutation, and two patients with a seven octapeptide repeat insertion in one family. In one patient a familial history was noted but genetic analysis was not performed. In 72 patients different final diagnoses were made, Alzheimer’s disease being the most frequent (N = 20). The demographic parameters of the Belgian population were similar to those observed in the rest of Europe. We did notice a significantly increased age-specific incidence (‰>‰6/106/year) of sCJD patients between 70 and 90 years old in the period 2002– 2004 compared to 1998–2001 and retrospectively obtained data (1990–1997, p < 0.01). We undertook a detailed clinical and biochemical analysis to investigate this increase but could not identify any reason other than an increased vigilance for the diagnosis.

 

In conclusion, our study identified that in the past sCJD may have been underestimated in patients over age 70 although these patients are both clinically and neurobiochemically similar to the general sCJD phenotype. Keywords Diagnosis - Epidemiology - Prion disease - Transmissible spongiform encephalopathy

 


 

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

 


 

Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium

 

H. De Bosschere, DVM, PhD

 

S. Roels, DVM, PhD

 

E. Vanopdenbosch, DVM, Lic Page 18 of 98

 

8/3/2006

 

Veterinary and Agrochemical Research Centre (CODA/CERVA) National Reference Laboratorium for Veterinary TSEs

 

Groeselenberg 99, B-1180

 

Ukkel (Brussels), Belgium

 

KEY WORDS: Bovine spongiform encephalopathy, BSE, Western blot, atypical BSE.

 

ABSTRACT

 

For many years, researchers believed that only one bovine spongiform encephalopathy (BSE) strain existed, in contrast to the many different scrapie strains found. However, only very recently reports emerged about unconventional BSE strains seen in Italy, France, and Japan. The present case describes an atypical strain of BSE in Belgium in a 64-month old East-Flemish cow with an electrophoretic profile and other features similar to those described in Japan.

 

INTRODUCTION

 

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of fatal neurodegenerative diseases including sheep and goat scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans. They are characterized by the accumulation of an abnormal protein, called PrPsc, which is formed post translationally from the normal isoform (PrPc).1,2 At present, the agent causing TSEs is still incompletely characterized, although PrPsc is believed to be its major if not unique constituent.3

 

Research in mice showed the existence of different scrapie strains.4,5 Scrapie strain discrimination is currently based on biologic typing in a panel of inbred mice, using incubation time and brain pathology scoring as criteria.6 However, no large-scale studies of the molecular features of PrPsc have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biologic and molecular properties even after experimental or accidental passages into different species, such as mice, humans, primates, and sheep.7–10 However, very recently, variant forms of BSE have been reported in Japan, Italy, and France.11-13 These forms were characterized by atypical histopathologic, immunohistochemical, or biochemical phenotypes. The present case is the description of the first atypical BSE case in Belgium.

 

MATERIALS AND METHODS

 

Since January 2001, all cattle older than 30 months are tested for TSE via a rapid test (TeSeE-kit, Bio-Rad, Nazareth, Belgium) after EC regulation 999/2001.14,15 Samples positive according to the enzyme-linked immunosorbent assay (ELISA) screening are further subjected to scrapie-associated fibrils (SAF), histopathology, immunohistochemistry, and Western blot (WB) testing 16,17 at the National Reference Laboratory (NRL).

 

RESULTS

 

A positive ELISA sample from a 64-month-old East-Flemish cow or Belgian white and red (Figure 1) was presented at the NRL for confirmation. The animal was reported healthy before slaughter. The optical density (OD) titers at the local laboratory were 2.324 and 2.116.16 The OD titers at the NRL were 0.953 and 0.708 (sample taken at the contralateral side of the first sampling side of the obex region). The histopathology of the obex, pons, and midbrain

 

Page 19 of 98

 

8/3/2006

 

showed no spongiform changes; immunohistochemistry of the brainstem revealed no signal of PrPsc accumulation typical for BSE; and SAF was negative. However, WB analysis (Bovine WB, Bio-Rad, France; antibodies 12F10 and SAF60) of the same homogenate that was prepared from the obex region for ELISA revealed a small amount of PrPsc with an electrophoretic profile different from that of typical BSE-associated PrPsc.18,19 The band on the gel of the non-glycosylated form of PrPsc of the present case clearly showed a lower migration pattern compared with that of a typical BSE case (Figure 2).

 

DISCUSSION

 

For many years, researchers assumed that only one BSE strain existed.7–10 Only in the past months, reports of atypical BSE cases were announced.11–13 The Japanese case11 describes a very young bull (23 months) characterized by the absence of spongiform changes and PrPsc deposits immunohistochemically. The WB analysis revealed an electrophoretic profile different from that of typical BSE, characterized by low content of the di-glycosylated molecular form of PrPsc and a faster migration of the nonglycosylated form of PrPsc. In Italy,12 two BSE affected cattle with a previously unrecognized neuropathologic profile and PrPsc type were seen. These cases were determined using a different staining pattern on immunohistochemistry, a difference in size and glycoform ratio of PrPsc on immunoblot and a difference in regional distribution of lesions. The two cases in France13 showed variant molecular features with a different PrPsc electrophoretic profile from other BSE cases, mainly characterized by a higher molecular mass of the nonglycosylated PrPsc. The present case shows the most similarities (ie, identical electrophoretic profile, only ELISA and WB positive and histopathology and immunohistochemistry negative) with the Japanese case,11 although the cow in the Japanese case was only 23 months old, and the cow in this case was 64 months old.

 

The fact that these strains were detected worldwide and in several breeds suggest that there is no local or breed dependent feature involved. It could be that the WB techniques have become more specific within the past year in the detection of minor differences in di-, mono-, and nonglycosylated molecular forms of PrPsc. Infection of cattle by scrapie could also be considered since scrapie can be transmitted by direct contact between animals or through environmental contamination.13

 

In conclusion, this Belgian case should be added to the list of atypical BSE strains only very recently detected worldwide and may contribute to further research studies about epidemiologic significance. Current continued research on BSE would appear to reveal different BSE strains in analogy with the different scrapie strains.

 

ACKNOWLEDGMENTS

 

The authors wish to thank Rita Geeroms, Patrick Van Muylem, Stephanie Durand, Raphaël Foubert and Amina Chama for their technical assistance. Mario Vanpoucke is acknowledged for providing references.

 

REFERENCES

 

snip...

 


 

[PDF]Response to Public Comments - USDA Food Safety and ...

 

www.fsis.usda.gov/.../BSE_Risk_Asse... Cached

 

Food Safety and Inspection Service Loading...

 

Oct 31, 2005 - documents sent to the docket. ...

 

2 Expanded “Mad Cow” Safeguards Announced to Strengthen Existing ...

 

FSIS and APHIS that the estimated percentage of poultry litter fed to ..... 0579-AB93, Federal Register, Vol.70 No. .....

 

Comment #7: WHY is it that the Farm of the Mad Sheep of Mad River Valley were.

 


 

Comment #6: WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported sheep from Belgium that were confiscated and slaughtered from the Faillace's, what sort of TSE did these animals have?

 

Response: It is not clear how the test results referred to in this comment are relevant to the Harvard BSE Risk Assessment Update. Sheep were not considered in the risk assessment.

 

Comment #7: WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

 

Response: This comment pertains to policy. As such, it is not addressed here.

 

 [PDF]Owens, Julie Page 1 of 98 8/3/2006 - USDA Food Safety ...

 

www.fsis.usda.gov/.../2006-0011-1.p... Cached

 

Food Safety and Inspection Service Loading... by F Greetings - ‎2006 - ‎Related articles Aug 3, 2006 -

 

Subject: [Docket No. FSIS-2006-0011] ... [Federal Register: July 12, 2006 (Volume 71, Number 133)]. [Notices] ..... group as compared to prior years, we found that conflicting APHIS instructions .....

 

WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these.

 

 


 

 

 

> >> Imported

 

> >> Belgium/Netherlands

 

> >> Sheep Test Results

 

> >> Background

 

> >> Factsheet

 

> >> Veterinary Services April 2002

 

> >> APHIS

 

> > > >

 

> > > > snip...

 

> > > >> Additional tests will be conducted to determine

 

> >> exactly what TSE the animals have BSE or scrapie.

 

> >> These tests involve the use of bioassays that consist

 

> >> of injecting mice with tissue from the infected animals

 

> >> and waiting for them to develop disease. This testing

 

> >> may take at least 2 to 3 years to complete.

 


 

> > > > > > DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E.

 

> > (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES

 


 

> > > > > > > > > DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E

 

> > (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2]

 


 

> > > > > > > > > or if those old urls dont work, go here;

 

> > > > DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E

 

> > (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES

 

> > - Terry S.

 

> > Singeltary Sr. 7/20/00 (0)

 

> > > > > [Federal Register: July 20, 2000 (Volume 65, Number 140)] [Notices]

 

> > [Page 45018]

 

>From the Federal Register Online via GPO Access

 

> > [wais.access.gpo.gov] [DOCID:fr20jy00-32]

 

> > > >

 

-----------------------------------------------------------------------

 

> > > > DEPARTMENT OF AGRICULTURE

 

> > > > Office of the Secretary

 

> > > > [Docket No. 00-072-1]

 

> > > > Declaration of Extraordinary Emergency Because of an Atypical

 

> > Transmissible Spongiform Encephalopathy (Prion Disease) of Foreign Origin

 

> > > > A transmissible spongiform encephalopathy (TSE) (prion disease) of

 

> > foreign origin has been detected in the United States. It is different

 

> > from TSE's previously diagnosed in the United States. The TSE was

 

> > detected in the progeny of imported sheep. The imported sheep and

 

> > their progeny are under quarantine in Vermont. Transmissible

 

> > spongiform encephalopathies are degenerative fatal diseases that can

 

> > affect livestock. TSE's are caused by similar, as yet uncharacterized,

 

> > agents that usually produce spongiform changes in the brain.

 

> > Post-mortem analysis has indicated positive results for an atypical

 

> > TSE of foreign origin in four sheep in Vermont. Because of the

 

> > potentially serious consequences of allowing the disease to spread to

 

> > other livestock in the United States, it is necessary to seize and

 

> > dispose of those flocks of sheep in Vermont that are affected with or

 

> > exposed to the disease, and their germ plasm. The existence of the

 

> > atypical TSE of foreign origin represents a threat to U.S. livestock.

 

> > It constitutes a real danger to the national economy and a potential

 

> > serious burden on interstate and foreign commerce. The Department has

 

> > reviewed the measures being taken by Vermont to quarantine and

 

> > regulate the flocks in question and has consulted with appropriate

 

> > officials in the State of Vermont. Based on such review and

 

> > consultation, the Department has determined that Vermont does not have

 

> > the funds to compensate flock owners for the seizure and disposal of

 

> > flocks affected with or exposed to the disease, and their germ plasm.

 

> > Without such funds, it will be unlikely to achieve expeditious

 

> > disposal of the flocks and germ plasm. Therefore, the Department has

 

> > determined that an extraordinary emergency exists because of the

 

> > existence of the atypical TSE in Vermont. This declaration of

 

> > extraordinary emergency authorizes the Secretary to seize, quarantine,

 

> > and dispose of, in such manner as he deems necessary, any animals that

 

> > he finds are affected with or exposed to the disease in question, and

 

> > their germ plasm, and otherwise to carry out the provisions and

 

> > purposes of the Act of July 2, 1962 (21 U.S.C. 134-134h). The State of

 

> > Vermont has been informed of these facts.

 

> > > > Dated: This declaration of extraordinary emergency shall become

 

> > effective July 14, 2000. Dan Glickman, Secretary of Agriculture. [FR

 

> > Doc. 00-18367 Filed 7-19-00; 8:45 am] BILLING CODE 3410-34-P

 


 

 


 

 

THE REST IS HISTORY, around 2005, what they were trying to stop at the Faillaces front door, was then finally documented anyway, and since then, the Nor-98 ‘foreign animal disease’ Winking smile , has spread from coast to coast in North America.

 

*** 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.

 

Saturday, July 6, 2013

 

Small Ruminant Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy

 

Research Article

 


From: TSS (216-119-138-157.ipset18.wt.net)
Subject: Re: More on the Mad Sheep from Mad River Valley
Date: August 12, 2000 at 8:44 am PST

In Reply to: More on the Mad Sheep from Mad River Valley posted by Terry S. Singeltary Sr. on August 12, 2000 at 8:42 am:
######### Bovine Spongiform Encephalopathy #########
>BSE-suspected Vermont Sheep Get Another
>Reprieve, USDA Backs Off Slaughter Order
>
>BURLINGTON, Vt., August 10 (United Press International) -- Two
>flocks of exotic sheep scheduled for slaughter this week in Vermont
>because of fears some of the animals might be infected with a form
>of the deadly mad cow disease have won yet another reprieve.
>
>The U.S. Department of Agriculture has temporarily backed off plans
>to seize and destroy the animals, the Burlington Free Press reported
>Thursday.
>
Actually something else happened in the interim:



Vermont sheep still grazing as lawyers reach deal


Thu, Aug 10, 2000 Reuters


BURLINGTON, Vt., Aug 10 (Reuters) - Lawyers for owners of Vermont sheep
suspected of having a neurological ailment similar to mad cow disease
struck a deal with prosecutors on Thursday for a fuller court hearing on
the animals' fate.


Under the agreement, lawyers for the farmers will forgo afederal
appeals case in return for a more detailed hearing
Under the agreement, lawyers for the farmers will forgo afederal
appeals case in return for a more detailed hearing in a lower court, said
Tom Amidon, who represents sheep farmer Houghton Freeman.
"So the sheep are on the farm awhile," Amidon said. He hoped a new
hearing would allow for closer examination of claims by the U.S.
Agriculture Department, which wants to seize and eliminate the 350 sheep
under scrutiny as a health precaution.


U.S. District Judge Garvan Murtha ruled last week the Agriculture
Department could carry out its plan. But the farmers planned to appeal
before Thursday's agreement changed the legal picture.
"We will drop our appeal to the second circuit and in return the U.S.
attorneys office in Vermont will allow us to hold a fuller court hearing,"
Amidon said.


Four sheep on Vermont farms near Warren, Vermont, tested positive last
month for a disease known as TSE or transmissible spongiform
encephalopathy, according to USDA officials.


TSE can cause scrapie, a fatal disease in sheep. It is also part of a
family of diseases that includes deadly BSE, also known as bovine
spongiform encephalopathy or mad cow disease.


No cases of BSE or mad cow disease have ever been found in the United
States, but the human form of the disease is blamed for 75 deaths in
Britain during the 1980s.


Breeding pairs for the two flocks of diary sheep came from Belgium and
the Netherlands in the mid-90s.

Amidon said the Belgian government has sent a letter to the USDA
requesting the return of all 350 sheep and that Freeman had already lined
up a plane to transport them. Whether that will eventually occur remains

############ http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############


BSE INQUIRY DFAs

 
Sunday, May 18, 2008
 
BSE Inquiry DRAFT FACTUAL ACCOUNT DFA
 
BSE Inquiry DRAFT FACTUAL ACCOUNTS DFA's
 
 
Sunday, May 18, 2008
 
BSE, CJD, and Baby foods (the great debate 1999 to 2005)
 
 
Sunday, May 18, 2008
 
MAD COW DISEASE BSE CJD CHILDREN VACCINES
 
 
 

2015

 

 

SCIENTIFIC COMMITTEE OF THE BELGIAN FEDERAL AGENCY FOR THE SAFETY OF THE FOOD CHAIN

 

 

Subject: RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE

 

SCIENTIFIC COMMITTEE OF THE BELGIAN FEDERAL AGENCY FOR THE SAFETY OF THE FOOD CHAIN

 

RAPID ADVICE 17-2014

 

Subject: Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE (Dossier SciCom 2014/22) Rapid advice approved by the Scientific Committee on 22nd October 2014.

 

Summary

 

The Scientific Committee was asked to answer two questions in regard to a proposal from the European Commission to no longer obligate Member States with a negligible BSE risk status to remove and dispose the specified risk materials as specified in Annex V to Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 laying down rules for the prevention, control and eradication of certain transmissible spongiform encephalopathies. The aim of this modification of the Regulation is to ensure that conditions for imports of commodities from third countries are not more favorable than the conditions applying to Member States with the same OIE BSE negligible risk status. More specifically it was asked to the Scientific Committee:

 

- If there is a difference in public health risk between the casings imported from third countries with a “negligible risk status for BSE” and casings that come from the 18 EU Member States with a “negligible risk status for BSE”?

 

- If there is a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM and if the other risk materials for BSE (the skull including the brains and eyes, the spinal cord, the tonsils and the spine) are indeed considered as SRM?

 

Due to lack of availability of data on true prevalence and tissue infectivity of BSE (classical as well as atypical BSE) the Scientific Committee was not able to thoroughly investigate the questions.

 

Removal of specified risk materials from cattle at slaughter prevents BSE infected materials from entering the human food chain.

 

The Scientific Committee is of the opinion that, taking into consideration the uncertainties in regard to the true prevalence of BSE (classical as well as atypical BSE) in countries with a “negligible risk status for BSE” and given the problems related with the early detection of asymptomatic BSE and given the zoonotic significance of atypical BSE, that stopping with the routine removal of specified risk materials during bovine slaughter will increase the risk for public health.

 

2/14

 

The Scientific Committee is not able to compare the public health risk of casings from third countries and from the 18 EU Member States, both with a negligible risk status for BSE, because of lack of data on true BSE prevalence and BSE tissue infectivity (classical BSE and atypical BSE) in the considered countries.

 

The Scientific Committee is also not able to properly answer the second question if there is a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM due to lack of quantitative data on tissue infectivity of different specified risk materials in slaughtered bovines in these countries. There is also no information on tissue infectivity of atypical BSE cases. It is known however that intestines are the portal of entry of prions and that they are already infective before the prions reach the central nervous system.

 

The final decision pertaining the need of removal of all or part of the specified risk materials is a risk management decision and goes beyond the competencies of the Scientific Committee.

 

Samenvatting Sneladvies over de risico’s voor de volksgezondheid van worstenvellen in landen met een “verwaarloosbaar risicostatuut voor BSE” en over de risico’s van wijziging van een lijst van gespecifieerde risicomaterialen (GRM) voor BSE

 

snip...

 

In conclusion the Scientific Committee is not able to answer this question with an acceptable degree of uncertainty because of lack of data on true prevalence of BSE (classical as well as atypical forms of BSE) in the considered countries. It reiterates its concern regarding the import of certain animal products from third countries with a ‘negligible BSE risk status’ as stated in rapid advice SciCom 16-2013.

 

snip...

 

2. Is there a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM while the other risk materials for BSE (the skull including the brains and eyes, the spinal cord, the tonsils and the spine) are indeed considered as SRM?

 

Once again the Scientific Committee is not able to properly answer this question because of lack of quantitative data on tissue infectivity of different specified risk materials in slaughtered bovines in EU Member States with a “negligible risk status for BSE”.

 

BSE infected animals may enter undetected the food chain due to the low sensitivity of the diagnostic tests. Further on the classical BSE agent accumulates from the first months post exposure in particular segments of the bovine intestines and persists till clinical onset. In addition no information is available about the infectivity of tissues by the atypical BSE agent, especially in the intestines.

 

If intestines from cattle in EU Member States with a “negligible risk status for BSE” are no longer removed as SRM and are allowed to enter the food chain the public health risk will be increased. The degree of rise in risk level cannot be determined. According to EFSA Journal 2014;12(2):3554, the TSEi model indicated that the removal of the last four meters of the small intestine and of the caecum from the food and feed chain would result in a major reduction of the Classical BSE exposure risk associated with intestine and mesentery in cattle.

 

Referring to its previous advice 16-2013 the Scientific Committee repeats that stopping with the routine removal of all specified risk materials during bovine slaughter will increase the risks of exposure of the population to BSE because of the uncertainty related to the detection of BSE. This uncertainty is the consequence of the long incubation period (especially in cases of atypical BSE), the low sensitivity of the available diagnostic methods, the apparent spontaneous nature of atypical BSE, the lack of a clear clinical picture of atypical BSE cases and the reduction in number of tests in healthy slaughtered animals.

 

The final decision pertaining the need of removal of all or part of the specified risk materials is a decision to be taken by the risk manager and goes beyond the competencies of the Scientific Committee.

 

5. Conclusion

 

Removal of specified risk materials from cattle at slaughter prevents BSE infected materials from entering the human food chain.

 

The Scientific Committee is of the opinion that, taking into consideration the uncertainties in regard to the true prevalence of BSE (including classical as well asaAtypical BSE) in countries with a “negligible risk status for BSE” and given the problems related with the early detection of asymptomatic BSE and given the zoonotic character of atypical BSE, stopping with the routine removal of all specified risk materials during bovine slaughter will increase the risk for public health.

 

12/14

 

The Scientific Committee is not able to compare the public health risk of casings from third countries and from the 18 EU Member States both with a negligible risk status for BSE because of lack of data on true BSE prevalence (classical BSE and atypical BSE) in the considered countries.

 

The Scientific Committee is not able to properly answer the question if there is a significantly increased public health risk if, in the EU Member States with a “negligible risk status for BSE”, the intestines are no longer removed as SRM due to lack of quantitative data on tissue infectivity of different specified risk materials in slaughtered bovines in these countries. There is also no information on tissue infectivity by the agent of atypical BSE.

 

On behalf of the Scientific Committee, The President Prof. Dr. E. Thiry (Sgd.) Brussels, 06/11/2014

 

References

 

snip...end

 


 

Thursday, July 24, 2014

 

Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA

 


 

P7.09

 

Biochemical screening for identification of atypical bse in belgium, 1999-present

 

Authors

 

Alexandre DobIy: Caroline Rodeghiero, Riet Geeroms; Stephanie Durand, Jessica De Sloovere, Emanuel Yanopdenbosch, Stefan Roels,

 

Content

 

Background: Recently atypical forms of BSE have been described. Western blot analyses showed that, in comparison to the classic BSE (C-type), they are demonstrable by a higher or lower molecular weight of the unglycosylated PrPres. They Viere thus named H-type and L-type BSE (L-type is also called BASE). In addition they show a lower proportion of diglycosylated PrPres than C-type. These emerging types represent different strains of BSE. They show unique incubation periods and histological lesions. Such types have been described on different continents. Indeed they might correspond to "sporadic" forms of BSE. In 2004 we already described one L-type in Belgium.

 

Objective: We retrospectively analysed the bovines at least 7-year-old in the Belgian archive of BSE ­diagnosed cattle in order to determine the prevalence of the two types of atypical BSE in Belgium.

 

Methods: We analysed homogenates from 39 bovines of 93 months old in median (min: 84, max: 181 months). The most recent one was diagnosed in 2006. We used Western blot with a panel of anti-PrP antibodies (Ab). They detect different regions of the PrP protein, from N-terminal to C-terminal: 12B2, 9A2, Sha31. SAFB4, 94B4. Their combination is aimed at an efficient typing diagnostic. We detected bound Ab with SuperSignal West Dura (Pierce) and analysed PrPres, signals with an image-analysis software (Quantity One, Bio-Rad).

 

Results: The results are still under analysis. We will detail the most crucial characteristics for typing PrPres. These include 1) the apparent molecular mass of the an-, mono- and diglycosylated bands, 2) the binding affinity to the five Ab (e.g.12B2 for H-type), 3) the presence of a fourth (unglycosylated) band and 4) the glycoprofile based on the relative proportions of the visible bands.

 

Discussion: The emergence of atypical types of BSE is partially due to a better knowledge of prion strains and more efficient diagnostic techniques. As the area in the brain where the PrPres is deposited can differ drastically between the types, it is essential to ascertain that the sampling techniques and analyses are adapted to these new types. As these new strains seem more virulent than classic types, they represent one of the next challenges in the field of prions.

 


 


 

Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium

 

H. De Bosschere, DVM, PhD

 

S. Roels, DVM, PhD

 

E. Vanopdenbosch, DVM, Lic

 

Veterinary and Agrochemical Research Centre (CODA/CERVA)

 

National Reference Laboratorium for Veterinary TSEs

 

Groeselenberg 99, B-1180

 

Ukkel (Brussels), Belgium

 

KEY WORDS: Bovine spongiform encephalopathy, BSE, Western blot, atypical BSE.

 

ABSTRACT

 

For many years, researchers believed that only one bovine spongiform encephalopathy (BSE) strain existed, in contrast to the many different scrapie strains found. However, only very recently reports emerged about unconventional BSE strains seen in Italy, France, and Japan. The present case describes an atypical strain of BSE in Belgium in a 64-month-old East-Flemish cow with an electrophoretic profile and other features similar to those described in Japan.

 

INTRODUCTION

 

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of fatal neurodegenerative diseases including sheep and goat scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans. They are characterized by the accumulation of an abnormal protein, called PrPsc, which is formed post-translationally from the normal isoform (PrPc).1,2 At present, the agent causing TSEs is still incompletely characterized, although PrPsc is believed to be its major if not unique constituent.3

 

Research in mice showed the existence of different scrapie strains.4,5 Scrapie strain discrimination is currently based on biologic typing in a panel of inbred mice, using incubation time and brain pathology scoring as criteria.6 However, no large-scale studies of the molecular features of PrPsc have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biologic and molecular properties even after experimental or accidental passages into different species, such as mice, humans, primates, and sheep.7-10 However, very recently, variant forms of BSE have been reported in Japan, Italy, and France.11-13 These forms were characterized by atypical histopathologic, immunohistochemical, or biochemical phenotypes. The present case is the description of the first atypical BSE case in Belgium.

 

snip...

 

In conclusion, this Belgian case should be added to the list of atypical BSE strains only very recently detected worldwide and may contribute to further research studies about epidemiologic significance. Current continued research on BSE would appear to reveal different BSE strains in analogy with the different scrapie strains.

 


 

 

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

 

Subject: Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium

 

Date: Fri, 04 Feb 2005 10:59:33 –0600

 

From: "Terry S. Singeltary Sr."

 

To: Bovine Spongiform Encephalopathy

 

CC: cjdvoice@yahoogroups.com

 

Atypical Case of Bovine Spongiform Encephalopathy in an East-Flemish Cow in Belgium

 

H. De Bosschere, DVM, PhD

 

S. Roels, DVM, PhD

 

E. Vanopdenbosch, DVM, Lic

 

Veterinary and Agrochemical Research Centre (CODA/CERVA)

 

National Reference Laboratorium for Veterinary TSEs

 

Groeselenberg 99, B-1180

 

Ukkel (Brussels), Belgium

 

KEY WORDS: Bovine spongiform encephalopathy, BSE, Western blot, atypical BSE.

 

ABSTRACT

 

For many years, researchers believed that only one bovine spongiform encephalopathy (BSE) strain existed, in contrast to the many different scrapie strains found. However, only very recently reports emerged about unconventional BSE strains seen in Italy, France, and Japan. The present case describes an atypical strain of BSE in Belgium in a 64-month-old East-Flemish cow with an electrophoretic profile and other features similar to those described in Japan.

 

INTRODUCTION

 

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of fatal neurodegenerative diseases including sheep and goat scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in humans. They are characterized by the accumulation of an abnormal protein, called PrPsc, which is formed post-translationally from the normal isoform (PrPc).1,2 At present, the agent causing TSEs is still incompletely characterized, although PrPsc is believed to be its major if not unique constituent.3

 

Research in mice showed the existence of different scrapie strains.4,5 Scrapie strain discrimination is currently based on biologic typing in a panel of inbred mice, using incubation time and brain pathology scoring as criteria.6 However, no large-scale studies of the molecular features of PrPsc have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biologic and molecular properties even after experimental or accidental passages into different species, such as mice, humans, primates, and sheep.7 10 However, very recently, variant forms of BSE have been reported in Japan, Italy, and France.11-13 These forms were characterized by atypical histopathologic, immunohistochemical, or biochemical phenotypes. The present case is the description of the first atypical BSE case in Belgium.

 

MATERIALS AND METHODS

 

Since January 2001, all cattle older than 30 months are tested for TSE via a rapid test (TeSeE-kit, Bio-Rad, Nazareth, Belgium) after EC regulation 999/2001.14,15 Samples positive according to the enzyme-linked immunosorbent assay (ELISA) screening are further subjected to scrapie-associated fibrils (SAF), histopathology, immunohistochemistry, and Western blot (WB) testing16,17 at the National Reference Laboratory (NRL).

 

RESULTS

 

A positive ELISA sample from a 64-month-old East-Flemish cow or Belgian white and red (Figure 1) was presented at the NRL for confirmation. The animal was reported healthy before slaughter. The optical density (OD) titers at the local laboratory were 2.324 and 2.116.16 The OD titers at the NRL were 0.953 and 0.708 (sample taken at the contralateral side of the first sampling side of the obex region). The histopathology of the obex, pons, and midbrain showed no spongiform changes; immunohistochemistry of the brainstem revealed no signal of PrPsc accumulation typical for BSE; and SAF was negative. However, WB analysis (Bovine WB, Bio-Rad, France; antibodies 12F10 and SAF60) of the same homogenate that was prepared from the obex region for ELISA revealed a small amount of PrPsc with an electrophoretic profile different from that of typical BSE-associated PrPsc.18,19 The band on the gel of the non-glycosylated form of PrPsc of the present case clearly showed a lower migration pattern compared with that of a typical BSE case (Figure 2).

 

DISCUSSION

 

For many years, researchers assumed that only one BSE strain existed.7 10 Only in the past months, reports of atypical BSE cases were announced.11 13 The Japanese case11 describes a very young bull (23 months) characterized by the absence of spongiform changes and PrPsc deposits immunohistochemically. The WB analysis revealed an electrophoretic profile different from that of typical BSE, characterized by low content of the di-glycosylated molecular form of PrPsc and a faster migration of the nonglycosylated form of PrPsc. In Italy,12 two BSE affected cattle with a previously unrecognized neuropathologic profile and PrPsc type were seen. These cases were determined using a different staining pattern on immunohistochemistry, a difference in size and glycoform ratio of PrPsc on immunoblot and a difference in regional distribution of lesions. The two cases in France13 showed variant molecular features with a different PrPsc electrophoretic profile from other BSE cases, mainly characterized by a higher molecular mass of the nonglycosylated PrPsc. The present case shows the most similarities (ie, identical electrophoretic profile, only ELISA and WB positive and histopathology and immunohistochemistry negative) with the Japanese case,11 although the cow in the Japanese case was only 23 months old, and the cow in this case was 64 months old.

 

The fact that these strains were detected worldwide and in several breeds suggest that there is no local or breed-dependent feature involved. It could be that the WB techniques have become more specific within the past year in the detection of minor differences in di-, mono-, and nonglycosylated molecular forms of PrPsc. Infection of cattle by scrapie could also be considered since scrapie can be transmitted by direct contact between animals or through environmental contamination.13

 

In conclusion, this Belgian case should be added to the list of atypical BSE strains only very recently detected worldwide and may contribute to further research studies about epidemiologic significance. Current continued research on BSE would appear to reveal different BSE strains in analogy with the different scrapie strains.

 

ACKNOWLEDGMENTS

 

The authors wish to thank Rita Geeroms, Patrick Van Muylem, Stephanie Durand, Raphaël Foubert and Amina Chama for their technical assistance. Mario Vanpoucke is acknowledged for providing references.

 

REFERENCES

 

1. Oesch B, Westaway D, Walchii M, et al: A cellular gene encodes PrP 27 30 protein. Cell 40:735 746, 1985.

 

2. Prusiner SB, De Armond SJ: Prion diseases and neurodegeneration. Annu Rev Neurosci 17:311 339, 1994.

 

3. Prusiner SB: Scrapie prions. Annu Rev Microbiol 43:345 374, 1989.

 

4. Bruce M, Dickinson AG: Biological evidence that scrapie agent has an independent genome. J Gen Virol 68:79 89, 1987.

 

5. Fraser H, Dickinson AG: Scrapie in mice: Agent strain differences in the distribution and intensity of grey matter vacuolation. J Comp Pathol 83:29 40, 1973.

 

6. Bruce M, McConnell I, Fraser H, Dickinson AG: The disease characteristics of different strains of scrapie in Sinc Congenic mice lines: Impications for the nature of the agent and host control of pathogenesis. J Virol 72:595 603, 1991.

 

7. Bruce M, Chree A, McDonnell I, et al: Transmission of bovine spongiform encephalopathy and scrapie to mice: Strain variation and the species barrier. Philos Trans R Soc Lon Ser B 343:405 411, 1994.

 

8. Bruce M, Will RG, Ironside JW, et al: Transmissions to mice indicate that new variant CJD is caused by the BSE agent. Nature 389:498 501, 1997.

 

9. Foster JD, Bruce M, McDonnell I, et al: Detection of BSE infectivity in brain and spleen of experimentally infected sheep. Vet Rec 138:546 548, 1996.

 

10. Lasmezas CI, Fournier J-G, Nouvel V, et al: Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-Jakob disease: Implications for human health. Proc Natl Acd Sci U S A 98:4142 4147, 2001.

 

11. Yamakawa Y, Hagiwara K, Nohtomi K, et al, for the Expert Commitee for BSE Diagnosis, Ministry of Health, Labour and Welfare of Japan: Atypical proteinase K-resistant prion protein (PrPres) observed in an apparently healthy 23-month-old Holstein steer. Jpn J Infect Dis 56:221 222, 2003.

 

12. Casalone C, Zanusso G, Acutis PL, et al: Identification of a novel molecular and neuropathological BSE phenotype in Italy: International Conference on Prion Disease: from basic research to intervention concepts. Gasreig, München, 8 10 October, 2003.

 

13. Biacabe AG, Laplanche JL, Ryder S, Baron T: A molecular variant of bovine spongiform encephalopathy. International Conference on Prion Disease: From basic research to intervention concepts. Gasreig, München, 8 10 October, 2003.

 

14. De Becker D, Roels S, Vanopdenbosch E: BSE onderzoek: opsporen van PrPres door middel van de BIO-RAD Platelia BSE-kit. Vlaams Diergeneeskundig Tijdschrift 69:382 384, 2000.

 

15. Roels S, Demeyer G, Tedik K, et al: Variance of mass (volume) taken with the calibrated syringe and of the results provided by the Bio-Rad Platelia BSE test upon storage of brainstem samples at 20°C. Anim Res 51:493 499, 2002.

 

16. Roels S, De Bosschere H, Saegerman C, et al: BSE and scrapie testing in Belgium: general overview. New Food: accepted, 2004.

 

17. Vanopdenbosch E, Dechamps P, Dufey J, et al: Le premier cas d encephalopathie spongioforme bovine diagnostique en Belgique. Annales de Médicine Vétérinaire 142:111 118, 1998.

 

18. Collinge J, Sidle KCL, Meads J, et al: Molecular analysis of prion strain variation and the aetiology of new variant CJD. Nature 383:685 690, 1996.

 

19. Hill AF, Desbruslais M, Joiner S, et al: The same prion strain causes vCJD and BSE. Nature 389:448 450, 1997.

 

Figure 1. Photograph of the East-Flemish cattle breed or the Belgian white and red.

 

Figure 2. Bovine Western blot (Bio-Rad, France) using antibodies 12F10 and SAF60. MM, Magic mark; Atyp. BSE, Atypical BSE case (present case); Ref1, Reference 1 of a classical BSE case; Ref2, Reference 2 of a classical BSE case. The third band of the non-glycosylated PrPsc of the Atyp. BSE case (left rectangle) shows a markedly faster migration compared to the Ref1 and Ref2 cases (right rectangle).

 


 

P.6.- Atypical case of bovine spongiform encephalopathy in an East-Flemish cow in Belgium.

 

H. De Bosschere1, S. Roels2, E. Vanopdenbosch3

 

1 Veterinary and Agrochemical Research Centre (CODA / CERVA), National Reference Laboratory for Veterinary TSE (Belgium & Luxemburg) , Unit Pathology, Department of Biocontrol, Groeselenberg 99, B-1180 Brussels (Ukkel), Belgium, (hedeb@var.fgov.be)

 

2 idem, (stroe@var.fgov.be)

 

3 idem, (emvan@var.fgov.be)

 

Bovine spongiform encephalopathy (BSE) is a prion disease with a fatal neurodegenerative pathogenesis. It is characterized by the accumulation of an abnormal protein (PrPres), formed posttranslationally from the normal isoform (PrPc). Research in mice showed the existence of different sheep scrapie strains. Scrapie strain discrimination is currently based upon biological typing in a panel of inbred mice. However, no large scale studies of the molecular features of PrPres have been reported for bovine BSE to date. Till now, the BSE strain seemed to maintain constant biological and molecular properties even after experimental or accidental passages into different species. Very recently, variant forms of BSE have been reported in Japan, Italy and France. These forms were characterized by atypical histopathological, immunohistochemical and/or biochemical phenotype compared to the classical BSE strain. The present case describes the first Belgian atypical BSE case. Since January 1st 2001, all cattle older than 30 months is tested for TSE via a rapid test following EC regulation 999/2001. Samples positive according to the ELISA screening are further subjected to scrapie associated fibrils (SAF), histopathology, immunohistochemistry and Western blot (WB) at the NRL. A positive ELISA sample from a 64 month-old East-Flemish or Belgian white and red cow was presented at the NRL for confirmation. The histopathology of the obex, pons and midbrain was negative, immunohistochemistry and SAF were also negative. However, WB analysis was positive with an electrophoretic profile different from that of a typical BSE case. The band on the gel of the non-glycosylated form of PrPres of the present case clearly showed a lower migratrion pattern compared to that of a typical BSE case. For many years it was assumed that there was only one BSE strain. Only very recently, reports of atypical BSE cases were announced in Japan, Italy and France. The Japanese case describes a very young bull (23 months) negative on histopathology and immunohistochemistry and a WB electrophoretic profile different from that of classical BSE. The Italians observed two BSE affected cattle with a a different staining pattern on immunohistochemistry, a difference in size and glycoform ratio of PrPres on WB and a difference in regional distribution of lesions. The French two cases showed variant molecular features with a different electrophoretic profile from other BSE cases. The present case shows the most similarities with the Japanese case (except for the age). The fact that these strains were detected worldwide and in several breeds suggest that there is no local or breed dependent feature involved. It could be that the WB techniques have become more specifique within the last year or infection of cattle by scrapie could also be considered. In conclusion, continued research on BSE reveals nowadays different BSE strains in analogy with the different sheep scrapie strains. Atypical BSE cases may question the significance and efficiency of the BSE epidemio-surveillance protocol and the validation of the confirmatory tests.

 

Keywords

 

Bovine spongiform encephalopathy, BSE, Western Blot, atypical BSE

 


 

Report on the assessment of the Geographical BSE-risk of BELGIUM July 2000

 

- 42 -

 

5. CONCLUSION ON THE GEOGRAPHICAL BSE RISK

 

5.1 The current GBR

 

The current geographical BSE-risk (GBR) level is III, i.e. BSE is confirmed in domestic cattle (last and only case in 1997) at a lower level. However, the observed incidence of clinical cases over the last 12 months (1 March 1999 to 29 February 2000) was 2.7 per 1 Million adult cattle. This figure is generated by a passive surveillance system that is not able to identify all clinical cases.

 

5.2 The expected development of the GBR

 

Assuming that measures in place continue to be appropriately implemented and no new external challenge occurs, the GBR is expected to decrease over time. However, this does not exclude that cattle infected by the BSE-agent in the past (before 1998/99) may be identified as clinical cases in the foreseeable future.

 

5.3 Recommendations for influencing the future GBR

 

Good implementation of the bans should be ensured.

 

In addition, expanding the surveillance system to target asymptomatic cattle in risk sub-populations such as adult fallen stock and adult cattle presented for emergency slaughter will allow verification of the current GBR-assessment and monitoring its future trend.

 


 


 


 

American Association of Zoo Veterinarians Infectious Disease Committee Manual 2013

 

BOVINE SPONGIFORM ENCEPHALOPATHY (BSE)

 

Little is known about atypical BSE. The origin and natural routes of transmission, if any, have yet to be determined. Almost all cases have been in older cattle (usually > 8 years of age) that have shown little resemblance to the clinic-pathological picture seen in classical disease. It has been suggested that the disease may be sporadic or be caused by a genetic mutation, but no convincing evidence has been found to support either of these ideas. The correct answer will probably only come by study of the future annual incidence curves of both types of disease. Regardless of the origin of atypical BSE, the possibility of recycling the disease in cattle and other ruminants, as well as the potential for transmission to humans, mandate a continuation of feed and specified-risk materials (SRM) bans, together with diagnostic testing programs for some time to come.

 

snip...

 

Naturally occurring cases of BSE in species other than cattle have been very limited and have been linked to exposure to contaminated feed or infected carcasses. The majority of cases originated in the UK and like BSE in cattle, have declined with the implementation of feed controls. None of the exotic animals were infected in the wild.

 

Experts who may be consulted: Linda A. Detwiler, DVM Clinical Professor Department of Pathobiology and Population Medicine

 

College of Veterinary Medicine Mississippi State University 732-580-9391 Fax: 732-741-7751 ldetwiler@belle-terre.com

 


 

Published in : European Journal of Epidemiology (2006), vol. 21, pp. 443-447

 

Status : Postprint (Author’s version)

 

Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium

 

B. Van Everbroeck1, A. Michotte2, R. Sciot3, C. Godfraind4, M. Deprez5, S. Quoilin6, J.-J. Martin1 & P. Cras1 1Born-Bunge Institute (BBI), University of Antwerp (UA), Campus Drie Eiken (CDE), Antwerp, Belgium; 2Department of Neuropathology, Academic hospital, Free University of Brussels, Brussels, Belgium; 3Department of Pathology, Catholic University of Leuven, Leuven, Belgium; 4Pathology Laboratory, Catholic University of Louvain, Brussels, Belgium; 5Laboratory of Neuropathology, University of Liège, Sart Tilman, Liège, Belgium; 6Institute of Public Health-Louis Pasteur, Brussels, Belgium

 

Abstract

 

From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium. In addition to epidemiological data, information on cerebrospinal fluid biomarkers, prion protein gene and brain neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were referred to the surveillance system. In 85 patients a 'definite' diagnosis of sporadic CJD (sCJD) could be made, whereas 26 patients remained 'probable'. We further identified two unrelated patients with an E200K mutation, and two patients with a seven octapeptide repeat insertion in one family. In one patient a familial history was noted but genetic analysis was not performed. In 72 patients different final diagnoses were made, Alzheimer's disease being the most frequent (N = 20). The demographic parameters of the Belgian population were similar to those observed in the rest of Europe. We did notice a significantly increased age-specific incidence (>6/106/ year) of sCJD patients between 70 and 90 years old in the period 2002-2004 compared to 1998-2001 and retrospectively obtained data (1990-1997, p < 0.01). We undertook a detailed clinical and biochemical analysis to investigate this increase but could not identify any reason other than an increased vigilance for the diagnosis. In conclusion, our study identified that in the past sCJD may have been underestimated in patients over age 70 although these patients are both clinically and neurobiochemically similar to the general sCJD phenotype.

 

Keywords : Diagnosis ; Epidemiology ; Prion disease ; Transmissible spongiform encephalopathy

 


 

To date, 27 cases of L-BSE and 24 cases of H-BSE have been report­ed worldwide (16), thus meaning that the prevalence of atypical BSE is considerably lower than that of C-BSE. However, recent studies showed that L-BSE is easily transmissible to transgenic mice expressing human (17,18) or bovine (19,20) prion protein, as well as to non-human primates (21), with shorter incubation periods than for the transmission of C-BSE to these animals.

 

***The virulent nature of L-BSE has stimulated new concern for human public health since the transmis­sion of C-BSE to humans resulted in variant Creutz­feldt-Jakob disease (vCJD) (4-7), a new emergent prion disease.

 


 

***Infectivity in skeletal muscle of BASE-infected cattle

 

***The present data offer novel information on the tropism of the BASE agent and highlight relevant public health issues. While the transmission barrier for classical BSE is high in most species, BASE prions are readily transmissible to a variety of mammals including non-human primates [11–13,35]. Accordingly, the possibility of spreading of BASE prions through skeletal muscle to other species should be taken into account and evaluated in risk analysis studies.

 


 


 

P.4.23

 

Transmission of atypical BSE in humanized mouse models

 

Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA

 

Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.

 

Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.

 

Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time. The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.

 

Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.

 

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.

 


 

2014

 

***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 

***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.

 

*** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 

snip...

 


 

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...

 


 


 

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...

 


 

snip...see more here ;

 

Saturday, January 31, 2015

 

RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE

 


 

Tuesday, December 16, 2014

 

*** Evidence for zoonotic potential of ovine scrapie prions

 

Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics

 

Abstract

 

Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 

Subject terms: Biological sciences• Medical research At a glance

 


 

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.

 

snip...

 

R. BRADLEY

 


 

FLASHBACK 2001

 

Suspect symptoms

 

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 

28 Mar 01 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb...

 

2001

 

Suspect symptoms

 

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 

28 Mar 01

 

Like lambs to the slaughter

 

31 March 2001

 

by Debora MacKenzie Magazine issue 2284.

 

FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

 

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.

 

Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.

 

Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.

 

As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.

 

"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.

 

But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.

 

People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.

 

But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."

 

There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.

 

Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.

 


 

Friday, January 30, 2015

 

*** Scrapie: a particularly persistent pathogen ***

 


 

Tuesday, February 17, 2015

 
***Could we spot the next BSE?, asks BVA President
 
 
Saturday, February 14, 2015
 
*** Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta
 
 
Tuesday, February 10, 2015
 
*** Alberta Canada First case of chronic wasting disease found in farm elk since 2002
 
 
Wednesday, December 31, 2014
 
NASDA BSE, CWD, SCRAPIE, TSE, PRION, Policy Statements updated with amendments passed during the NASDA Annual Meeting Updated September 18, 2014
 
 
Sunday, December 28, 2014
 
CHRONIC WASTING DISEASE CWD TSE PRION DISEASE AKA MAD DEER DISIEASE USDA USAHA INC DECEMBER 28, 2014
 
 

Subject: *** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease aka mad cow type disease

 

what is CJD ? just ask USDA inc., and the OIE, they are still feeding the public and the media industry fed junk science that is 30 years old.

 

why doesn’t some of you try reading the facts, instead of rubber stamping everything the USDA inc says.

 

sporadic CJD has now been linked to BSE aka mad cow disease, Scrapie, and there is much concern now for CWD and risk factor for humans.

 

My sincere condolences to the family and friends of the House Speaker Becky Lockhart. I am deeply saddened hear this.

 

with that said, with great respect, I must ask each and every one of you Politicians that are so deeply saddened to hear of this needless death of the Honorable House Speaker Becky Lockhart, really, cry me a friggen river. I am seriously going to ask you all this...I have been diplomatic for about 17 years and it has got no where. people are still dying. so, are you all stupid or what??? how many more need to die ??? how much is global trade of beef and other meat products that are not tested for the TSE prion disease, how much and how many bodies is this market worth?

 

Saturday, January 17, 2015

 

*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease

 


 

Thursday, January 15, 2015

 

41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE Prion: Case Report

 


 

*** HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL CDC ***

 

Sunday, November 23, 2014

 

*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European

 

the patient had resided in Kuwait, Russia and Lebanon. The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia. The specific overseas country where this patient’s infection occurred is less clear largely because the investigation did not definitely link him to a country where other known vCJD cases likely had been infected.

 


 

Sunday, December 14, 2014

 

ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report

 


 

Sunday, February 08, 2015

 

FDA SCIENCE BOARD TO THE FOOD AND DRUG ADMINISTRATION BOVINE HEPARIN BSE CJD TSE PRION Wednesday, June 4, 2014

 


 

Thursday, January 22, 2015

 

Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?

 


 

Saturday, December 13, 2014

 

Terry S. Singeltary Sr. Publications TSE prion disease

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 

snip...

 


 

 

 

TSS