SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) ONGOING SURVEILLANCE PROGRAM 2/14/12

UNITED STATES DEPARTMENT OF AGRICULTURE FOOD SAFETY AND INSPECTION SERVICE WASHINGTON, DC FSIS NOTICE

13-12

2/14/12

DISTRIBUTION: Electronic NOTICE EXPIRES: 3/1/13 OPI: OPPD

SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) ONGOING SURVEILLANCE PROGRAM

I. PURPOSE

This notice provides Food Safety and Inspection Service (FSIS) inspection program personnel (IPP) with instructions regarding the collection of brain samples for the Animal and Plant Health Inspection Service’s (APHIS) Bovine Spongiform Encephalopathy (BSE) ongoing surveillance program. The contents in this notice were last issued in FSIS Notice 05-10.

II. DEFINITION OF COLLECTION PROCEDURES

A. At federally-inspected slaughter establishments that have approved alternative off-site sample collection arrangements with APHIS:

1. APHIS will provide for the collection of brain (obex) samples from an allocated number of cattle 30 months and older condemned for any reason on ante-mortem inspection and from cattle of any age displaying Central Nervous System (CNS) symptoms.

2. At such establishments, FSIS IPP will provide the following to plant management, to the APHIS Area Veterinarian in Charge (AVIC), and to sample collecting contractors:

a. Condemn tag (Z-tag) numbers (not the Z-tag itself); and

b. Disposition information (i.e., the reason for condemnation under 9 CFR Part 309), including history, clinical signs and the condemnation code assigned.

B. At federally-inspected establishments that do not have approved alternative off-site sample collection arrangements with APHIS, FSIS Public Health Veterinarians (PHVs) are to:

2

1. Notify the APHIS AVIC when an animal has been condemned for CNS reasons and provide the information outlined in II.A.2.

2. If arrangements with the APHIS AVIC cannot be made, collect appropriate BSE samples from cattle of all ages that display CNS symptoms.

NOTE: Certain Alternative Off-Site Agreements that were in place during Enhanced Surveillance may no longer be in effect. Therefore, IPP may need to advise the establishment that they need to establish new agreements with APHIS and potential collectors.

III. FSIS PERSONNEL RESPONSIBILITIES

A. Upon receipt of this notice, the FSIS PHV is to ask establishment management whether:

1. It has an approved alternative off-site sample collection arrangement with APHIS for collecting allocated samples (as per paragraph II. A.); or

2. It is not an establishment that has an alternative off-site sample collection arrangement with APHIS. If the establishment does not have such an arrangement, then FSIS is to follow the directions in paragraph II.B.

B. If during the meeting establishment management states that it has submitted an application to APHIS to begin off-site sampling, until APHIS approves that arrangement, FSIS PHVs are to:

1. Identify all CNS animals condemned on ante-mortem with a "U. S. Condemned” tag;

2. If an animal is condemned for this reason, contact the APHIS AVIC and follow the directions in paragraph II.B.;

3. Ensure that the animals are humanely euthanized, unless APHIS requests that the animal not be euthanized; and

4. Not allow any CNS animal condemned on ante-mortem to move off the premises of the establishment until APHIS collects the sample or APHIS requests otherwise.

C. In a memorandum of interview (MOI), the FSIS PHV is to document who was present at the meeting with establishment management, the date and time of the meeting, how the establishment plans to proceed based on the choices set out in A. above, and any documents shared with management.

D. If the establishment states that it has submitted a request to APHIS to begin off-site sampling, the FSIS PHV is to update the MOI as to whether the establishment reached an agreement, and in general, what the agreement was.

E. The FSIS PHV is to maintain a copy of the MOI in the official government file, provide a copy to the plant management, and e-mail a copy to the APHIS AVIC. Any

3

changes in the agreement with APHIS for off-site sample collection are to be reflected in an updated MOI, which is then distributed as above.

IV. FSIS RESPONSIBILITIES RELATED TO APPROVED ALTERNATIVE OFF-SITE SAMPLE COLLECTION

A. The FSIS PHV is to complete the condemnation form, FSIS Form 6000-13 (Certification of Ante-mortem or Post-mortem Disposition of Tagged Animals), and FSIS Form 6150-1 (Identification Tag – Ante-mortem). The FSIS PHV is to pay special attention when providing a full description of the reason for the condemnation on FSIS Form 6000-13 and fill out fully FSIS Form 6150-1.

B. Incoming animal identification, except the Z-tag, is to be left on these animals to provide needed identifying information on collection forms at the approved alternative off-site collection location. IPP are, or someone under their supervision is to remove Z-tags before any carcasses leave the official establishment.

NOTE: Information supplied to plant management to take to the approved alternative off-site collection locations needs to be complete and accurate. FSIS PHVs need to provide a full description of the reason for the condemnation on FSIS Form 6000-13. APHIS will use this information to triage which condemned animals are sampled.

V. FSIS SAMPLE COLLECTION FOR CATTLE DISPLAYING CNS SYMPTOMS

A. If the establishment does not have an arrangement with APHIS for off-site sampling of cattle with CNS symptoms, the FSIS PHV is to follow the directions in paragraph II.B. The FSIS PHV is to make all final disposition decisions regarding whether to condemn cattle in accordance with 9 CFR part 309.

NOTE: FSIS PHVs can find information regarding BSE sampling (e.g., forms, sampling supply information) on the FSIS intranet under Public Folders, OPPD, PDD, BSE, at:

http://collaboration/sites/InfoExch/OPPD/default.aspx?RootFolder=%2fsites%2fInfoExch%2fOPPD%2fOPPD%2fPDD%2fBSE%20Training%20Info&FolderCTID=&View=%7b6F518F12%2d11E4%2d4A8C%2dB891%2dAACB8FDD96BD%7d



Additional BSE sampling information is provided on the APHIS website at:

http://www.aphis.usda.gov/animal_health/lab_info_services/downloads/BSE_Procedures_Manual.pdf



B. If the responsibility to collect the BSE sample falls to the FSIS PHV, the FSIS PHV, or the establishment under the supervision of the FSIS PHV, is to promptly remove the head in order to collect the brain sample. If the establishment does not arrange to remove the head, the FSIS PHV may need to collect the brain sample as a priority over other ante-mortem or post-mortem procedures.

4

C. The FSIS PHV, being mindful of other potential CNS disease conditions of public health significance that may be present, is to collect the brain sample either in the inedible area of the establishment or in an isolated area set aside for such collection to prevent human exposure or the creation of insanitary conditions. Establishment personnel and FSIS IPP are to take proper sanitary measures before returning to edible areas of the establishment after brain sample collection, in accordance with 9 CFR 416.5.

D. In situations where the FSIS PHV has missed the last UPS pick-up for the day, or the FSIS PHV collected the sample on a day when UPS does not pick up, the PHV is to refrigerate the samples until the next available UPS pick-up day. Remember, the sample is not to pass through or to be stored in areas of the establishment where the establishment produces edible product. The FSIS PHV is to maintain the sample’s chain-of-custody.

E. The FSIS PHV is to verify the collection, documentation, and control of all animal identification associated with cattle condemned during ante-mortem inspection that are to be sampled by FSIS. The FSIS PHV is to attach the “U. S. Condemned” tag to cattle condemned during ante-mortem inspection in accordance with 9 CFR 309.13. This documentation will facilitate traceback in the event that the sample result is positive for BSE. The FSIS PHV is to include in the documentation all pertinent information to facilitate trace back of the animal in question in accordance with 9 CFR 320.1.

F. The FSIS PHV is to verify that the presence of condemned cattle or parts does not create insanitary conditions (9 CFR Part 416). The establishment is responsible for the disposal of the condemned cattle in accordance with 9 CFR part 314. The FSIS PHV also is to verify that the establishment maintains records regarding the disposal of the condemned cattle in accordance with 9 CFR 320.1.

G. IPP may inform the establishment that it may choose to hold the carcass and parts until testing results are available. If the establishment chooses to dispose of any carcass or parts before it receives test results, IPP are to advise the establishment that it must dispose of the carcass in one of the following ways:

1. Render it at a facility for non-animal feed use (e.g., biofuel or cement);

2. Alkaline digestion;

3. Incineration; or

4. Lined landfills.

H. Documentation for Cattle Showing Signs of CNS Symptoms

1. For locations without high-speed internet connections, the FSIS PHV is to forward the completed BSE Veterinary Services Laboratory Submissions (BSE-VSLS) sample collection sheets to the corresponding APHIS,VS area office by FAX or by e-mail. The following site lists the VS office FAX numbers and e-mail where available:

http://www.aphis.usda.gov/import_export/downloads/vsavic.pdf



5

NOTE: The FSIS PHV can get copies of BSE-VSLS forms by contacting the local APHIS office. The APHIS AVIC in each area office may assist with sample delivery verification and troubleshooting.

2. For locations with high-speed connections, the FSIS PHV is to enter the relevant information into the BSE-VSLS.

NOTE: FSIS PHVs can refer to the BSE-VSLS training for assistance getting access to BSE-VSLS and inputting information. The FSIS PHV may contact the District Office (DO) if he or she needs a copy of the training CD.

VI. TEST RESULTS FOR FSIS SAMPLING FROM CATTLE SHOWING CNS SYMPTOMS

A. The FSIS PHV will receive, by e-mail, a report from the AVIC on the BSE test results. The AVIC will also send copies of the results to the DO.

B. If the test on the carcass condemned for CNS conditions is negative (reported as “not detected”), then any carcasses and parts the establishment has held may be released for rendering or other disposal in accordance with 9 CFR 314. C. If the test is inconclusive, the FSIS PHV will receive supervisory instruction on further actions.

D. For any sample confirmed positive for BSE, the FSIS PHV is to verify that the establishment disposes of the carcasses and parts in the proper manner as set out in paragraph V. G.

VII. PHIS PROCEDURES FOR BSE SAMPLING FROM CATTLE SHOWING CNS SIGNS

A. Enter disposition information into PHIS in ADR.

B. Enter BSE sampling information into the ADR APHIS Lab Sampling page in ADR.

VIII. RABIES

A. When an animal is condemned by the FSIS PHV on ante-mortem for rabies, the FSIS PHV is to contact the DO, which will advise APHIS. In these cases, APHIS will see that the animal is tested for rabies. APHIS will work with the laboratory to get appropriate samples forwarded for BSE surveillance from rabies negative animals.

B. Rabies booster vaccination for PHVs collecting BSE samples is still highly recommended. PHVs can be reimbursed for rabies vaccinations or boosters. Rabies vaccinations are voluntary. Only PHVs who are actually involved with BSE sample collection will be eligible for reimbursement on the vaccination series. PHVs are to make

6

arrangements for the vaccinations with their private physician. PHVs are to contact their Front-line supervisor for approval on reimbursement prior to beginning the three shot vaccination series. Refer questions regarding this notice to the Policy Development Division through askFSIS at http://askfsis.custhelp.com or by telephone at 1-800-233-3935.

Assistant Administrator

Office of Policy and Program Development

http://www.fsis.usda.gov/OPPDE/rdad/FSISNotices/13-12.pdf




Tuesday, November 02, 2010

IN CONFIDENCE

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

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

http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html




2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html




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

http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html





Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 INTRODUCTION The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:




http://www.fsis.usda.gov/Science/Risk_Assessments/index.asp).



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




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




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




http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf



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


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

To: FSIS RegulationsComments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



FSIS, USDA, REPLY TO SINGELTARY

http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf





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...



http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1






http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf





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



http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html





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...



http://www.neuroprion.org/en/np-neuroprion.html







Sunday, November 13, 2011

California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock

http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/california-bse-mad-cow-beef-recall-qfc.html





Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis



http://www.youtube.com/watch?v=zf3lfz9NrT4





http://www.youtube.com/watch?v=c0tWkNvhO4g





http://www.youtube.com/watch?v=zf3lfz9NrT4&feature=results_main&playnext=1&list=PL780BE2AF0B62A944





full text with source references ;



http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/terry-singeltary-sr-on-creutzfeldt.html







Friday, February 10, 2012



Creutzfeldt-Jakob disease (CJD) biannual update (2012/1) potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive



http://creutzfeldt-jakob-disease.blogspot.com/2012/02/creutzfeldt-jakob-disease-cjd-biannual.html





Sunday, February 12, 2012



National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas



http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html





Saturday, June 25, 2011

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




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




http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html





Sunday, June 26, 2011

Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/risk-analysis-of-low-dose-prion.html





Friday, December 23, 2011

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

Volume 18, Number 1—January 2012 Dispatch

http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/oral-transmission-of-l-type-bovine.html







TSS

Annual reports of Member States on BSE and Scrapie 2010 excluding North America (for obvious reasons i.e. corruption)

BSE/Scrapie - TSE in Goats

Annual reports of Member States on BSE and Scrapie 2010

http://ec.europa.eu/food/food/biosafety/tse_bse/docs/annual_report_tse2010_en.pdf

Tuesday, February 14, 2012

White House budget proposes cuts to ag programs including TSE PRION disease aka mad cow type disease

http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/white-house-budget-proposes-cuts-to-ag.html

Thursday, February 16, 2012

Bovine Spongiform Encephalopathy BSE

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

http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/bovine-spongiform-encephalopathy-bse-31.html

Thursday, February 23, 2012

EIGHT FORMER SECRETARIES OF AGRICULTURE SPEAKING AT USDA'S 2012 AGRICULTURE OUTLOOK FORUM INDUCTED INTO USA MAD COW HALL OF SHAME

http://madcowusda.blogspot.com/2012/02/eight-former-secretaries-of-agriculture.html

Thursday, February 23, 2012

Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012

snip...

RESEARCH



Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011



Experimental Oral Transmission of Atypical Scrapie to Sheep


Marion M. Simmons, S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos




To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specifi c prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These fi ndings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.



SNIP...



Although we do not have epidemiologic evidence that supports the effi cient spread of disease in the fi eld, these data imply that disease is potentially transmissible under fi eld situations and that spread through animal feed may be possible if the current feed restrictions were to be relaxed. Additionally, almost no data are available on the potential for atypical scrapie to transmit to other food animal species, certainly by the oral route. However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing fi nding that the biochemical properties of the resulting PrPSc have changed on transmission (40). The implications of this observation for subsequent transmission and host target range are currently unknown.



How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantifi ed, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confi rmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected.



Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011



http://wwwnc.cdc.gov/eid/article/17/5/pdfs/10-1654.pdf








OIE Scrapie Chapter Revision • Current draft recognizes Nor98-like scrapie as a separate disease from classical scrapie • USDA provided comments on the draft to OIE

http://www.animalagriculture.org/Solutions/Proceedings/Annual%20Meeting/2009/Sheep%20&%20Goat/Myers,%20Thomas.pdf

Atypical scrapie/Nor 98 October 2009

 

Last year, after examining member country submissions and investigating rigorous scientific research, the World Organisation for Animal Health (OIE) decided that Nor 98 should not be listed in its Terrestrial Animal Health Code. The Code sets out trade recommendations or restrictions for listed diseases or conditions, and the OIE determined there was no need for such recommendations around Nor 98.

http://www.nzfsa.govt.nz/publications/ce-column/ce-web-nor98.htm

http://www.biosecurity.govt.nz/files/pests/atypical-scrapie/atypical-scrapie-faq-oct09.pdf

Sutton reported that USDA has urged the World Organization for Animal Health (OIE) to categorize Nor98-like scrapie as a separate disease from classical scrapie. Currently, the OIE has proposed a draft revision of their scrapie chapter that would exclude Nor98-like scrapie from the chapter. USDA will be submitting it's comments on this proposal soon.

http://www.ohiosheep.org/Events/ScrapieNewsletterMarch09.pdf

 

snip...

 

 

 

http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/atypical-scrapie-nor-98-confirmed.html

Monday, November 30, 2009

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

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html

Sunday, February 12, 2012

National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas

 

 

 

http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html

 

 

 

TSS

Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012

Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012




PLEASE NOTE, the _spontaneous_ aspect of the rhetoric that USDA, CFIA, OIE, Australian, and every other Industry/Government is putting out, that atypical scrapie does not transmit to sheep, goat, or any other species, and that atypical Scrapie just happens spontaneously, is totally fabricated, and is not true. THERE IS NO SCIENCE showing this. IN fact, Science has shown that atypical scrapie does transmit, and it is very similar to some cases of sporadic CJD and GSS in humans. FOR the OIE et al to ratify a totally flawed trade policy, that makes legal the trading of atypical scrapie as a global commodity, is insane, and the ramifications of this insanity, will be played out for decades to come on man and animal. ...TSS


Fatal disease confirmed
in sheep 


Herd quarantine unlikely

Posted Feb. 23rd, 2012



by Mary MacArthur



A single case of atypical scrapie was confirmed in an Alberta sheep in January.


Dr. Bob Cooper, a veterinary program specialist with the Canadian Food Inspection Agency, said the case was discovered as part of the national surveillance program to eradicate scrapie in Canada.

The surveillance program tests samples of sheep and goats for scrapie in an effort to understand where the disease is found in Canada and how to eliminate it.

Scrapie is a fatal disease that affects the central nervous system of sheep and goats. It is in the same family as BSE in cattle and chronic wasting disease in deer and elk.

It is a reportable disease in Canada.

Unlike classical scrapie, atypical scrapie doesn’t require that sheep farms be quarantined or the flock investigated.

Cooper said there would be some follow up to determine where the sheep lived its life, but there will be no large scale investigation or quarantine.

The atypical scrapie case was posted on the CFIA website’s monthly reportable disease update.

Canada has developed the national voluntary scrapie flock certification program in an attempt to eliminate scrapie from the national flock.

Alberta Lamb Producer chair Phil Kolodychuk was relieved the case was atypical scrapie and not classic scrapie.

“It’s a bad disease. We’d like to get rid of it in our country,” he said.





http://www.producer.com/2012/02/fatal-disease-confirmed%E2%80%A8in-sheep-%E2%80%A9/









Current as of: 2012-01-31

Date confirmed Location Animal type infected
January 26* Alberta Sheep

*Atypical scrapie





http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/2012/flocks-infected-in-2012/eng/1329724998646/1329725166676









Increased Atypical Scrapie Detections

Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.


http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf






J Vet Diagn Invest 21:454-463 (2009)

Nor98 scrapie identified in the United States

Christie M. Loiacono,' Bruce V. Thomsen, S. Mark Hall, Matti Kiupe!, Diane Sutton, Katherine O'Rourke, Bradd Barr, Lucy Anthenill, Deiwyn Keane

Abstract.

A distinct strain of scrapic identified in sheep of Norway in 1998 has since been identified in numerous countries throughout Europe. The disease is known as Nor98 or Not-98-like scrapic. among other names. Distinctions between classic scrapie and Nor98 scrapie are made based on histopathologv and immunodiagnostic results. There are also differences in the epidemiology, typical signalment, and likelihood of clinical signs being observed. In addition, sheep that have genotypes associated with resistance to classic scrapie are not spared from Nor98 disease. The various differences between classic and Nor98 scrapie have been consistently reported in the vast majority of cases described across Europe. The current study describes in detail the patholo gic changes and diagnostic results of the first 6 cases of' Nor98 scrapic disease diagnosed in sheep of the United States.

Key words: Hisiopathology: Nor98: PrP imniunolabeling; scrapie: sheep.

snip...

Results

Case I

The first case identified as consistent with Nor98 scrapie had nonclassic PrP distribution in brain tissue, no PrPSC in lymph tissue, and nonclassic migration of protein bands on a Western blot test. The animal was an aged, mottled-faced ewe that was traced back to a commercial flock in Wyoming. ...

Case 2

The second case was a clinically normal 8-year-old Suffolk ewe that had been in a quarantined flock for 5 years at a USDA facility in Iowa.

Case 3

A 16-year-old, white-faced, cross-bred wether was born to a black-faced ewe. He lived his entire life as a pet on a farm in California.

Case 4

The fourth case of Nor98 scrapie was identified in an approximately 8-year-old Dorset ewe that was born into a flock of approximately 20 ewes in Indiana.

Case 5

The fifth case was a clinically normal, approximately 3-year-old, white-faced, cross-bred ewe from an approximately 400 head commercial flock in Minnesota.

Case 6

The sixth case of Nor98 scrapie was identified in a 4-year-old, white-faced ewe that was purchased and added to a commercial flock in Pennsylvania

snip...

see full text ;

http://ddr.nal.usda.gov/bitstream/10113/33943/1/IND44241920.pdf







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...


http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1




http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf






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



http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html








Monday, November 30, 2009


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


http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html







Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues


http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html







Sunday, December 12, 2010


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


http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/efsa-reviews-bsetse-infectivity-in.html





Wednesday, January 18, 2012

Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie

Journal of Neuropathology & Experimental Neurology: February 2012 - Volume 71 - Issue 2 - p 140–147

http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/selection-of-distinct-strain-phenotypes.html



Thursday, July 14, 2011


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


http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/histopathological-studies-of-ch1641.html





Wednesday, January 18, 2012

BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE

February 1, 2012


http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/bse-in-goats-can-be-mistaken-for.html





Thursday, December 23, 2010



Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009

Volume 17, Number 1 January 2011



http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/molecular-typing-of-protease-resistant.html







Thursday, November 18, 2010


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


http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html







hmmm, no I ponder why some of these sporadic CJD cases, are now being linked to a genetic TSE, that has NO link to the family ?

hmmm, could it be these atypical TSE in animals, that are linked to the human TSE in the USA, and also, these animals have been fed back and forth to each other ?

hmmm, why no link there $$$






P03.141

Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.



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




http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf







PR-26

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway

Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.

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

119




http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf







A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,? +Author Affiliations

*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)

Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.




http://www.pnas.org/content/102/44/16031.abstract





Monday, December 1, 2008

When Atypical Scrapie cross species barriers

Authors

Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.

Content

Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.

The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.

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

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

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

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

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




http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf







Thursday, January 26, 2012

Facilitated Cross-Species Transmission of Prions in Extraneural Tissue

Science 27 January 2012: Vol. 335 no. 6067 pp. 472-475 DOI: 10.1126/science.1215659

http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/facilitated-cross-species-transmission.html








Saturday, February 11, 2012


Prion cross-species transmission efficacy is tissue dependent


http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/prion-cross-species-transmission.html







Thursday, January 26, 2012


The Risk of Prion Zoonoses


Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167



http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html






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

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

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

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

snip...

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

PMID: 6997404

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract






12/10/76

AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE

Office Note CHAIRMAN: PROFESSOR PETER WILDY

snip...

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

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

snip...

76/10.12/4.6

http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf






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

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

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

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

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

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

http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html






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

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

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

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

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

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

http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html






Wednesday, February 16, 2011

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html







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

snip...

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

http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf







Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html





Monday, April 25, 2011


Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011


http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html





Sunday, April 18, 2010


SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010


http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html






Thursday, November 18, 2010


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


http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html








Wednesday, January 19, 2011


EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011


http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html








Monday, June 27, 2011


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


http://prionopathy.blogspot.com/2011/06/comparison-of-sheep-nor98-with-human.html






Archive Number 20100312.0803


Published Date 12-MAR-2010


Subject PRO/AH/EDR; Scrapie, atypical, ovine - Australia: (WA) susp



SCRAPIE, ATYPICAL, OVINE - AUSTRALIA: (WESTERN AUSTRALIA) SUSPECTED



*******************************************************************



A ProMED-mail post

ProMED-mail is a program of the International Society for Infectious Diseases

[1] Date: Fri 12 Mar 2010 Source: The Australian [edited]



A West Australian sheep has been found to have signs characteristic of the fatal brain disease atypical scrapie. It comes as Australia faces growing anger from its trade partners over the Rudd government's surprise decision to extend a ban on the importation of beef from countries exposed to mad cow disease for a further 2 years.

Australia's chief veterinarian, Andy Carroll, told the ABC an indicative case of the atypical scrapie had been confirmed but said it posed no risk to human or animal health or the safety of eating meat and animal products.

Nor does atypical scrapie carry the dire trade consequences associated with classical scrapie.

Classical scrapie is in the same transmissible spongiform encephalopathies (TSE) family as BSE, better known as mad cow disease, from which humans can be fatally infected.

Dr Carroll said samples from the sheep's brain were being sent to the World Reference Laboratory in Britain.

Neither atypical scrapie nor classical scrapie has been seen in Australia before, but a sheep in New Zealand tested positive to the atypical form last year [2009].

Atypical scrapie is a relatively recently discovered disease and the common scientific view is that it occurs spontaneously or naturally in very small numbers of older sheep in countries all over the world.

[Byline: Jodie Minus]

-- Communicated by: Sabine Zentis Castleview Pedigree English Longhorns Gut Laach 52385 Nideggen Germany

****** [2] Date: Wed 10 Mar 2010 Source: ABC News (Australian Broadcasting Corporation) [edited]

Animal health authorities are testing a sheep's brain for what could be Australia's 1st case of the disease atypical scrapie.

Although not confirmed, the sheep is thought to be from Western Australia.

This type of scrapie is described as a sporadic degenerative brain condition affecting older sheep, and is not contagious.

Ed Klim, from national advisory group SafeMeat, says a 2nd round of testing is now taking place. "We've been made aware that the Australian Animal Health Laboratory is conducting further routine testing on a sheep sample," he says.

"The disease isn't considered a health risk nor should have any impact on food safety or export markets for sheep meat of live sheep."

Australia's chief veterinarian and WA's Department of Agriculture of Food are both aware of the testing but will not comment.

-- Communicated by: Terry S Singeltary Sr

[Although atypical scrapie is not yet ruled out, it is important to realize this is a type of scrapie that thus far has only tended to appear as a sporadic condition in older animals. Currently it has not been shown to follow the same genetic tendencies for propagation as the usual scrapie.

However, the atypical phenotypic appearance has been shown to be preserved on experimental passage.

Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries, as Australia may join that list. It is likely that this case will be sent to the UK for definitive conformation.

[Ref: M Simmons, T Konold, L Thurston, et al. BMC Veterinary Research 2010, 6:14 [provisional abstract available at ]

"Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]

The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ]



http://www.promedmail.org/direct.php?id=20100312.0803






UPDATE ATYPICAL SCRAPIE AUSTRALIA CONFIRMED




First occurrence of atypical scrapie

Australia is free of scrapie, also known as ‘classical’ scrapie, and has been assessed as a ‘negligible bovine spongiform encephalopathy (BSE) risk’ (the lowest risk) by the World Organisation for Animal Health (OIE). Both diseases belong to a group of diseases termed transmissible spongiform encephalopathies (TSEs) or ‘prion diseases’.

Active surveillance occurs to validate Australia’s status for both diseases, through the National Transmissible Spongiform Encephalopathies Surveillance Program (NTSESP), consistent with OIE recommendations. Results are routinely reported in Animal Health Surveillance Quarterly.

The first case of atypical scrapie (another TSE) in Australia has been confirmed in a single sheep, through the NTSESP. This is not a surprising finding. Atypical scrapie is a rare, sporadic, degenerative brain condition that spontaneously occurs in a very small proportion of older sheep and, less commonly, in goats. Most countries that test large numbers of sheep for scrapie have found one or more cases of atypical scrapie.

Testing on samples from the affected sheep at the CSIRO Australian Animal Health Laboratory in March 2010 showed preliminary results consistent with atypical scrapie. The results were confirmed by the Veterinary Laboratory Agencies at Weybridge in the United Kingdom, an OIE reference laboratory.

Atypical scrapie is clinically, pathologically, biochemically and epidemiologically unrelated to classical scrapie, and has been recognised as a distinct disease of sheep and goats for about a decade. During this time, the disease has been diagnosed in more than 20 countries worldwide. It does not pose a risk to human health or to the productivity of the Australian sheep flock. There is evidence that it is not naturally spread to other animals. It is not known to have any causal relationship to other TSEs, including BSE in cattle, chronic wasting disease in deer, or any form of Creutzfeldt–Jakob disease in people.

As atypical scrapie is a different disease to classical scrapie, Australia’s internationally recognised status as free from scrapie will not change as a result of this case. Contributed by Reg Butler, Biosecurity Services Group, Australian Government Department of Agriculture, Fisheries and Forestry



http://www.animalhealthaustralia.com.au/fms/Animal%20Health%20Australia/ADSP/AHSQ/AHSQ%20Q1%202010.pdf






Scrapie

Scrapie has been recognised in sheep for more than 250 years, and occurs at a low annual incidence in many countries, but is not present in Australia or New Zealand.

Atypical scrapie In 2009, atypical/Nor98 scrapie was detected in one sheep brain from a consignment of sheep and goat brains sent from New Zealand to the European Union, for use as negative control materials for evaluating rapid tests for BSE and scrapie.50 In 2010, a case of atypical/Nor98 scrapie was diagnosed in a sheep in Australia.

Australian and New Zealand Standard Diagnostic Procedure, August 2010. Page 7 of 27

http://www.animalhealthaustralia.com.au/wp-content/uploads/2011/03/Australia-and-New-Zealand-Standard-Diagnostic-Protocols-for-TSE.pdf



The first case of atypical scrapie in Australia was recently detected through the active surveillance program for transmissible spongiform encephalopathies (TSEs). Atypical scrapie is a rare, degenerative brain condition that occurs spontaneously in a very small proportion of older sheep and goats. It is a different disease to classical scrapie and other known TSEs. Australia remains free from scrapie.



http://www.animalhealthaustralia.com.au/wp-content/uploads/2011/05/AHSQ-Q1-2010.pdf




http://www.animalhealthaustralia.com.au/






Thursday, October 7, 2010


Australia first documented case of atypical scrapie confirmed First occurrence of atypical scrapie


http://nor-98.blogspot.com/2010/10/australia-first-documented-case-of.html







RESEARCH



Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011



Experimental Oral Transmission of Atypical Scrapie to Sheep


Marion M. Simmons, S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos




To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specifi c prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These fi ndings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.



SNIP...



Although we do not have epidemiologic evidence that supports the effi cient spread of disease in the fi eld, these data imply that disease is potentially transmissible under fi eld situations and that spread through animal feed may be possible if the current feed restrictions were to be relaxed. Additionally, almost no data are available on the potential for atypical scrapie to transmit to other food animal species, certainly by the oral route. However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing fi nding that the biochemical properties of the resulting PrPSc have changed on transmission (40). The implications of this observation for subsequent transmission and host target range are currently unknown.



How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantifi ed, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confi rmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected.



Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011




http://wwwnc.cdc.gov/eid/article/17/5/pdfs/10-1654.pdf






OIE Scrapie Chapter Revision • Current draft recognizes Nor98-like scrapie as a separate disease from classical scrapie • USDA provided comments on the draft to OIE

http://www.animalagriculture.org/Solutions/Proceedings/Annual%20Meeting/2009/Sheep%20&%20Goat/Myers,%20Thomas.pdf




Atypical scrapie/Nor 98 October 2009

Last year, after examining member country submissions and investigating rigorous scientific research, the World Organisation for Animal Health (OIE) decided that Nor 98 should not be listed in its Terrestrial Animal Health Code. The Code sets out trade recommendations or restrictions for listed diseases or conditions, and the OIE determined there was no need for such recommendations around Nor 98.


http://www.nzfsa.govt.nz/publications/ce-column/ce-web-nor98.htm




http://www.biosecurity.govt.nz/files/pests/atypical-scrapie/atypical-scrapie-faq-oct09.pdf




Sutton reported that USDA has urged the World Organization for Animal Health (OIE) to categorize Nor98-like scrapie as a separate disease from classical scrapie. Currently, the OIE has proposed a draft revision of their scrapie chapter that would exclude Nor98-like scrapie from the chapter. USDA will be submitting it's comments on this proposal soon.


http://www.ohiosheep.org/Events/ScrapieNewsletterMarch09.pdf






SCRAPIE

The United States is unable to support the proposed new draft Code Chapter on Scrapie. The draft chapter, as written, departs significantly from the existing chapter, is confusing and is difficult to understand. This version of the scrapie chapter uses much of the same wording as the BSE chapter and is written as if the predominance of evidence revealed that scrapie was a food-borne disease similar to BSE in cattle which is inappropriate. Moreover, several of the new changes are not supported by current scientific evidence. As a result, detailed comments on individual articles would not meaningful at this time.

The United States is not supportive of the proposed draft chapter for the following reasons: 1. Inclusion of “atypical” scrapie: The scientific evidence indicates that “atypical” scrapie, also referred to as Nor-98, Nor-98-like, or non-classical scrapie, is not the same disease as classical scrapie. Further, “atypical” scrapie does not meet the criteria for listing diseases of trade concern by the OIE, as described in Chapter 2.1.1 of the Code. The United States recommends that the scope of this chapter be limited to classical scrapie in sheep and goats. Further, the United States recommends that OIE clearly adopt the position that “atypical” scrapie represents a distinct disease entity from classical scrapie and that it not be a listed disease.

• There is no evidence that “atypical” scrapie is a contagious disease. If it is contagious, available evidence suggests that it has a much lower transmission efficiency. (Hopp, et al, 2006; Green, et al, 2007; Benestad, et al 2008; McIntyre, et al, 2008)

• The disease appears to be ubiquitous in that it has been found wherever sufficient surveillance has been conducted. (Buschmann et al, 2004; De Bosschere et al, 2004; Orge, et al, 2004; Everest et al, 2006; Arsac, 2007; Benestad, et al 2008; Fediaevsky, et al, 2008)

• The disease does not appear to be economically significant in that the prevalence of clinical disease is low and it typically occurs in older animals. (Luhken, et al., 2007; Benestad, et al 2008).

• The disease is as likely as not to be the result of a spontaneous conversion of normal prion protein. (Benestad, et al 2008, De Bosschere et al 2007)

• Removal of exposed sheep is unlikely to reduce the prevalence of “atypical” scrapie infection and removing only those exposed sheep that are phenylalanine (F) at codon 141 is scientifically unsound since the disease is known to affect sheep of most other genotypes. Further, sheep with AHQ alleles have a similar risk of infection with “atypical” strains as sheep with F at codon 141. (Luhken, et al., 2007).

• If “atypical” scrapie is included as a listed disease, the surveillance and diagnostic requirements which are needed to identify these cases should be described in detail in both this Chapter and the Manual of Diagnostic Tests and Vaccines for Terrestrial

2

Animals. Data from Europe illustrates that using the proper test(s) is essential for the identification of atypical scrapie (Fediaevsky et al., 2008).

SNIP...

6. Overemphasis on importation and use of bovine meat and bone meal as a route of scrapie transmission: Given that the draft Chapter is not intended to address risk mitigation for BSE in small ruminants, we believe there is an over-emphasis on this potential route of transmission in the current draft.

The United States recommends that the requirements in this chapter be limited to the inclusion of products from sheep and goats (instead of from all ruminants) in feed or feed ingredients intended for consumption by animals.

• The use of products from sheep and goats as feed or feed ingredients for ruminant or non-ruminant animals represent one possible route of transmission (Philippe, et al, 2005) and a source of environmental contamination with the classical scrapie agent. However, this is not the primary route of transmission for the scrapie agent.

• The need for the exclusion of cattle-derived protein or other animal protein to mitigate BSE risk should be based on a country’s BSE risk status and should be addressed in Chapter 2.3.13 of the Code.

SNIP...

14. Failure to provide scientific justification for the list of permitted commodities in Item 1 of Article 2.4.8.1. .

We recommend that the list be re-evaluated and those items that have not been substantiated as presenting no risk be excluded or those with some risk but where the intended use mitigates the risk the use be specified.

• There is no known human health risk associated with scrapie. As such, if meat and meat products for human consumption are included in this list, sheep and/or goat milk intended for human consumption should also be added to the list of permitted commodities in Item 1 of Article 2.4.8.1.

• In the vast majority of sheep infected with classical scrapie, actual infectivity or PrPres has been identified in most tissues including the lymphoreticular system (tonsils, spleen, lymph nodes), the gastrointestinal tract, brain, and spinal cord (Hadlow et. al. 1979; Hadlow et al., 1980; van Kuelen et al., 1996; van Kuelen et al., 1999, Andreoletti et al., 2000; Heggebø et al., 2002; Caplazi et al., 2004). Infectivity and/or PrPres has also been identified in the placenta (see Hourrigan et al., 1979; Onodera et al., 1993; Pattison et al., 1972; Pattison et al., 1974; Race et al., 1998), blood (Hunter et al., 2002; Houston et al. 2008); peripheral nerves (Groschup et al., 1996), muscle (Pattison and Millson, 1962; Andreoletti et al., 2004; Casalone et al., 2005), salivary gland (Hadlow et al., 1980; Vascellari et al., 2007), kidney (Siso et al., 2006), and skin ( Thomzig et al., 2007). In addition, recent work has shown milk and/or colostrum from scrapie infected ewes transmitted the disease to 17 of 18 lambs (Konold et al., 2008).

• The data on the risk of low protein tallow made from scrapie infected tissues particularly for use in milk replacer is limited and some epidemiologic studies suggest an association of milk replacer use with scrapie risk. Taylor et al., 1997 examined the inactivation capacity of different rendering system in regards to scrapie. The presence of infectivity was determined by bioassay into mice. From the onset of this study, it was assumed that tallow was not the vehicle for the transmission of TSE. Hence only 2 tallow samples were examined.



http://www.aphis.usda.gov/import_export/animals/oie/downloads/tahc_mar-sep08/tahc-scrapie-77-mar08_cmt.pdf







• Most critical is that atypical scrapie shows higher prevalence in so-called resistant ARR homozygote and heterozygote genotypes, compared with classical scrapie. • Atypical scrapie has not been found naturally in VRQ/VRQ sheep, although such sheep can be infected artificially. VRQ sheep are, in contrast, highly susceptible to classical scrapie. In the UK, one case of atypical scrapie has been found in VRQ heterozygote (AF141RQ/VRQ) sheep. It is important to ascertain whether or not VRQ-carrying sheep are significantly resistant to infection with atypical scrapie or whether the data might result from a failure to detect PrPres in atypical scrapie due to a different pattern of PrP distribution in tissues. • Increased incidence of atypical scrapie in sheep with PrP alleles carrying the variant phenylalanine (F) at position 141 (leucine(L)/phenylalanine) has also been observed compared with classical scrapie. • It will be important to clarify the genotype effect, particularly in relation to ARR and L141F in transmission studies. • In classical scrapie, there is clear evidence for a PrP genotype effect on tissue distribution patterns of PrPres. This might also be true for atypical scrapie although the data are less complete. 4. Transmission of atypical scrapie It has recently18 been demonstrated that atypical scrapie is experimentally transmissible to mice and sheep, primarily through intracerebral injection. There are some data suggesting that it may also be transmissible orally to sheep of different genotypes. The subgroup noted that challenge experiments with atypical scrapie in sheep were underway in the UK, with one successful intracerebral challenge to date. The subgroup was informed that positive transmission of infectivity from atypical scrapie isolated from sheep with a range of genotypes had been observed in mice. This included ovinised transgenic mice overexpressing the VRQ allele. Nor98 atypical scrapie had also transmitted to ARR ovinised mice, with transmission experiments in AF141RQ ovinised mice planned. Biochemical features of the isolates were maintained after transmission, and were distinct from BSE and classical scrapie. High infectivity titres were observed in brain tissue from atypical scrapie, including from ARR/ARR sheep. Brain transmission experiments in mice carrying the human PrP gene were at an early stage. 18 Le Dur A., Béringue V., Andréoletti O., Reine F., Laï T.H., Baron T., Bratberg B., Vilotte J.- L., Sarradin P., Benestad S.L. and Laude H.(2005) A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes. PNAS 102, 16031-16036


http://www.seac.gov.uk/pdf/positionstatement-sheep-subgroup.pdf










BSE: TIME TO TAKE H.B. PARRY SERIOUSLY


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


http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1988/06/08004001.pdf







Tuesday, February 14, 2012


White House budget proposes cuts to ag programs including TSE PRION disease aka mad cow type disease


http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/white-house-budget-proposes-cuts-to-ag.html






Thursday, February 16, 2012


Bovine Spongiform Encephalopathy BSE


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


http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/bovine-spongiform-encephalopathy-bse-31.html






Thursday, February 23, 2012


EIGHT FORMER SECRETARIES OF AGRICULTURE SPEAKING AT USDA'S 2012 AGRICULTURE OUTLOOK FORUM INDUCTED INTO USA MAD COW HALL OF SHAME


http://madcowusda.blogspot.com/2012/02/eight-former-secretaries-of-agriculture.html






Monday, November 30, 2009


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


http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html








TSS