EFSA opinion on the BSE related public health risk

UK ministers may lift BSE-era ban on animal remains in chicken and pig feed

Exclusive: England and Wales proposals expected to follow Scottish consultation amid fears British farmers are being undercut

Daniel Boffey Chief reporter

Wed 8 Jan 2025 09.00 EST

Ministers may lift a ban introduced during the BSE crisis on the use of animal remains in feed for farmed chickens and pigs over fears that foreign producers are undercutting British farmers.

A consultation on permitting the use of processed animal protein (PAP) from poultry, pigs and insects has opened in Scotland, and it is understood that proposals will be made for England and Wales in the coming months.

https://www.theguardian.com/environment/2025/jan/08/uk-ministers-may-lift-bse-era-ban-on-animal-remains-in-chicken-and-pig-feed

With new studies out that Chronic Wasting Disease CWD TSE Prion of Cervid out, that CWD will transmit BY ORAL ROUTES, to cattle, PIGS, sheep, Cervid, monkeys, and with atypical BSE transmitting, both L type and H type transmitting by Oral routes, and with out breaks of Atypical BSE now showing up more in Europe and the USA, NOW IS NOT THE TIME TO LIFT MAD COW FEED BAN, BUT ENHANCE AND STRENGTHEN IT, SHOULD BE IN ORDER. …terry

CDC Journal ahead print into 2025 CWD not looking good

Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States Volume 31, Number 1—January 2025

https://wwwnc.cdc.gov/eid/article/31/1/24-0401_article

Detection of Chronic Wasting Disease Prions in Raw, Processed, and Cooked Elk Meat, Texas, USA R. Benavente et Al

Prions in Muscles of Cervids with Chronic Wasting Disease, Norway T. T. Vuong et Al

https://wwwnc.cdc.gov/eid/early-release#issue-269

CIDRAP develops state-of-the-art preparedness report for possible chronic wasting disease spillover January 8, 2025

https://www.cidrap.umn.edu/sites/default/files/CWD%20Report%202025_0.pdf

cwd scrapie pigs oral routes

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***

*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18(44%), and the tonsil in 10/25 (40%).

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091

https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017

https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105

Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105

Prion Conference 2023

Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure

Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA

Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.

Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).

Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.

Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.

"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."

=====end

Strain characterization of chronic wasting disease in bovine-PrP transgenic mice

Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.

"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."

=====end

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation

Justin J. Greenleea, Eric D. Cassmanna, S. Jo Moorea,b, and M. Heather West Greenleec

aVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA; bOak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, US; cDepartment of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, US

Aims: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure.

Material and Methods: Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n = 3) or from the US 2006 case with the E211K polymorphism (n = 4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation.

Results: Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57–4.0) in the brainstem, and IHC demonstrated PrPScthroughout the brain. All wild type recipient cattle and a single EK211 steer remained asymptomatic for the duration of the experiment (approximately 7 years post-inoculation) and no abnormal prion protein was detected in these cattle by EIA.

Conclusions: This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1 g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.

Funded by: US Department of Agriculture

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html

NOW, BE AWARE, OIE AND USDA HAVE NOW MADE ATYPICAL SCRAPIE AND ATYPICAL BSE A LEGAL TRADING COMMODITY, WITH NO REPORTING OF SAID ATYPICAL CASES, EXCEPT FOR A VOLUNTARY NOTE ON ANNUAL REPORT...i don't make this stuff up...terry

USA testing <25K cattle annually for BSE, BUT, even at those low testing figures, the USA did just confirm another case of BSE just here recently. Feed ban has failed terribly, and CWD is spreading in the USA, at an alarming rate. Recent transmission studies show oral transmission of CWD of Cervid to cattle. Studies also show links of sporadic CJD to BSE, Scrapie, and CWD. It’s a Whole new game of Prion poker now$$$

Wednesday, May 24, 2023

***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification

https://wahis.woah.org/#/in-review/5067

https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html

https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification

SATURDAY, MAY 20, 2023

***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE

https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html

https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed

MAY 19, 2023

https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse

2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;

***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;

Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023

''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...

https://www.regulations.gov/comment/APHIS-2023-0027-0002

https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf

1985

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

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

https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf

https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf

Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues

Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin

Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019.

Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues.

Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: https://doi.org/10.3389/fvets.2019.00430

Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.

Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305

https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665

https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668

Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.

https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0

***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***

Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment

https://www.regulations.gov/comment/FSIS-2022-0027-0002

https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed

PUBLIC SUBMISSION

Comment from Terry Singeltary Sr.

Posted by the Food and Drug Administration on May 17, 2016 Comment

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

https://www.regulations.gov/comment/FDA-2003-D-0432-0011

https://www.regulations.gov/docket/FDA-2003-D-0432

Transmission of scrapie prions to primate after an extended silent incubation period

*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.

*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.

*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efﬁciency comparable to that of cattle BSE. While the efﬁciency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.

https://www.nature.com/articles/srep11573

https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),

***is the third potentially zoonotic PD (with BSE and L-type BSE),

***thus questioning the origin of human sporadic cases.

==============

PRION 2015 CONFERENCE

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/

Cwd, cattle, sheep, raccoons, oh my

The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons

https://www.ars.usda.gov/research/publications/publication/?seqNo115=400777

A consultation on permitting the use of processed animal protein (PAP) from poultry, pigs and insects has opened in Scotland, and it is understood that proposals will be made for England and Wales in the coming months.

https://www.theguardian.com/environment/2025/jan/08/uk-ministers-may-lift-bse-era-ban-on-animal-remains-in-chicken-and-pig-feed

Prion Conference 2023

Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure

Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA

Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.

Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).

Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.

Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.

"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."

=====end

Strain characterization of chronic wasting disease in bovine-PrP transgenic mice

Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.

"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."

=====end

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation

Justin J. Greenleea, Eric D. Cassmanna, S. Jo Moorea,b, and M. Heather West Greenleec

aVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA; bOak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, US; cDepartment of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, US

Aims: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure.

Material and Methods: Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n = 3) or from the US 2006 case with the E211K polymorphism (n = 4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation.

Results: Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57–4.0) in the brainstem, and IHC demonstrated PrPScthroughout the brain. All wild type recipient cattle and a single EK211 steer remained asymptomatic for the duration of the experiment (approximately 7 years post-inoculation) and no abnormal prion protein was detected in these cattle by EIA.

Conclusions: This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1 g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.

Funded by: US Department of Agriculture

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html

Friday, December 14, 2012

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

snip.....

In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law. Animals considered at high risk for CWD include:

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES.

It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

snip...

https://web.archive.org/web/20170404125557/http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf

PLoS One. 2020 Aug 20;15(8):e0237410. doi: 10.1371/journal.pone.0237410. eCollection 2020.

Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease

Nathaniel D Denkers 1 , Clare E Hoover 2 , Kristen A Davenport 3 , Davin M Henderson 1 , Erin E McNulty 1 , Amy V Nalls 1 , Candace K Mathiason 1 , Edward A Hoover 1

PMID: 32817706 PMCID: PMC7446902 DOI: 10.1371/journal.pone.0237410

These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.

Snip…

Discussion

As CWD expands across North America and Scandinavia, how this disease is transmitted so efficiently remains unclear, given the low concentrations of prions shed in secretions and excretions [13, 14]. The present studies demonstrated that a single oral exposure to as little as 300nmg of CWD-positive brain or equivalent saliva can initiate infection in 100% of exposed white-tailed deer. However, distributing this dose as 10, 30 ng exposures failed to induce infection. Overall, these results suggest that the minimum oral infectious exposure approaches 100 to 300 ng of CWD-positive brain equivalent. These dynamics also invite speculation as to whether potential infection co-factors, such as particle binding [46, 47] or compromises in mucosal integrity may influence infection susceptibility, as suggested from two studies in rodent models [48, 49].

https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0237410

NOW, BE AWARE, OIE AND USDA HAVE NOW MADE ATYPICAL SCRAPIE AND ATYPICAL BSE A LEGAL TRADING COMMODITY, WITH NO REPORTING OF SAID ATYPICAL CASES, EXCEPT FOR A VOLUNTARY NOTE ON ANNUAL REPORT...i don't make this stuff up...terry

USA testing <25K cattle annually for BSE, BUT, even at those low testing figures, the USA did just confirm another case of BSE just here recently. Feed ban has failed terribly, and CWD is spreading in the USA, at an alarming rate. Recent transmission studies show oral transmission of CWD of Cervid to cattle. Studies also show links of sporadic CJD to BSE, Scrapie, and CWD. It’s a Whole new game of Prion poker now$$$

Wednesday, May 24, 2023

***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification

https://wahis.woah.org/#/in-review/5067

https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html

https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification

SATURDAY, MAY 20, 2023

***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE

https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html

https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed

MAY 19, 2023

https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse

2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;

***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;

Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023

''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...

https://www.regulations.gov/comment/APHIS-2023-0027-0002

https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf

Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues

Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin

Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019.

Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues.

Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: https://doi.org/10.3389/fvets.2019.00430

Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.

Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305

https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665

https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668

Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.

https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0

***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***

Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment

https://www.regulations.gov/comment/FSIS-2022-0027-0002

https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed

PUBLIC SUBMISSION

Comment from Terry Singeltary Sr.

Posted by the Food and Drug Administration on May 17, 2016 Comment

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

https://www.regulations.gov/comment/FDA-2003-D-0432-0011

https://www.regulations.gov/docket/FDA-2003-D-0432

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.

https://www.nature.com/articles/srep11573

Report on the epidemiological investigation of a BSE case in Scotland (RBSE24_00003) United Kingdom October 2024

Executive summary

On 9 May 2024, Scotland’s Chief Veterinary Officer (CVO) confirmed a case of classical bovine spongiform encephalopathy (BSE) in a 7.5-year-old cow on a beef suckler farm in Ayrshire, Scotland. This was the first case of classical BSE to be confirmed in the United Kingdom (UK) since 2021, and in Scotland since 2018. This report summarises the epidemiological investigations that have been carried out to describe and understand this single case of BSE.

The index case was a Simmental cross cow, born on 18 October 2016 in a holding in Dumfries and Galloway, Scotland. It was purchased and introduced into the incident herd on 27 June 2018, where it resided until its death.

The index case died on farm on 26 April 2024. The farmer did not suspect notifiable disease and the carcass was collected by the fallen stock company on the same day. The carcass was tested for BSE as per the UK’s statutory BSE surveillance procedures due to the cow’s age and because she was fallen stock.

A preliminary positive result was received on 1 May 2024. A final positive result was confirmed on 9 May 2024 by the Animal and Plant Health Agency (APHA) Weybridge. APHA Weybridge is the UK National Reference Laboratory (NRL) for transmissible spongiform encephalopathies (TSEs). It is also the World Organisation for Animal Health (WOAH) Reference Laboratory for BSE and scrapie.

Tracing investigations identified 2 offspring born in the 24 months prior to the clinical onset of disease and death of the index case (see also appendix 2, point (f)):

• The first one had a date of birth (DOB) of 13 May 2023. It was alive at the time of confirmation and placed under restrictions following BSE confirmation in the index case. It was transported alive to the NRL for TSEs in Weybridge for clinical observation. It was then euthanised and underwent a postmortem examination and BSE testing, with negative results.

• The second one had a DOB of 21 May 2022. It was already dead (slaughtered for human consumption and not eligible for BSE testing) when traced after the BSE case was confirmed.

Tracings investigations also identified 45 cohort animals born and/or reared with the index case during the relevant risk period (12 months either side of the date of birth of this case).

Of these, 43 were restricted and humanely culled on farm at their respective locations.

The carcasses were sampled for BSE testing and then disposed of as category 1 animal by products (ABP) at an approved ABP rendering facility. All the samples returned negative results for BSE.

The remaining 2 cohort animals were already dead when traced after the BSE case was confirmed. (They were slaughtered for human consumption and not tested for BSE as they were not eligible.)

Epidemiological investigations were undertaken at both the holding of birth and the holding of death of the positive BSE case. Following these investigations, the most likely source of infection remains undetermined. Four potential risk pathways were identified and assessed as very low likelihood events, all with high uncertainty. These 4 potential risk pathways were:

• accidental exposure to contaminated feed (possibly feed delivered before the reinforced feed ban that had remained attached to the side walls of a feed silo decommissioned in 2017) (see also appendix 2, point (b))

• maternal transmission

• environmental source 1: exposure to previous potential presence of the BSE agent on the natal farm via birth products

• environmental source 2: exposure to previous potential presence of the BSE agent on the natal farm from on farm or local cattle burials (when it was still legal to do so before 1 May 2003) via contaminated groundwater or other pathways (see also appendix 2, point (c))

The likelihood of any other potential risk pathways has been assessed as negligible. Any identified sources of infection have been effectively controlled through the following measures:

• The positive animal died on farm and was not destined to enter the food chain. As fallen stock, the entire carcass was category 1 ABP and was appropriately disposed of.

• Rearing cohorts and offspring cohorts were traced, culled and disposed of. All those culled cohorts and offspring were tested for BSE with negative results.

• Surveillance and testing of at-risk animals and fallen stock (see appendix 2, point D).

• Elimination of animal proteins from cattle feed as primary route of transmission (reinforced feed ban in effect since August 1996, see appendix 2, point B).

• Effective disposal of specified risk material (SRM) as per legislative requirements (see appendix 2, point E).

• Ban on burying fallen stock (dead animals) on farms since 1 May 2003 (see appendix 2, point C).

• The old feed silo was decommissioned in 2017.

The implementation of these control measures ensures that the risk of BSE agents being recycled within the bovine population has remained negligible. There is no evidence or other cause for concern that statutory official BSE or feed controls have been breached at any point in relation to this case or its herd of origin.

The detection of this case is evidence that the UK surveillance system for detecting and containing BSE is robust and effective. There is no threat to food safety, to human health or to animal health as a consequence of this case.

Introduction

snip...

K – Spontaneous origin

According to EFSA opinion, ‘the classification of a case as spontaneous is circumstantial and may change over time subject to additional information. It does not infer that there is no external cause; just that it could not be ascertained. A case of disease is classified as spontaneous by a process of elimination, excluding all other definable possibilities.’ (Ricci and others, 2017.)

As not all other pathways have been excluded, the likelihood of spontaneous origin is assessed as negligible.

Medium uncertainty reflects that the highest likelihood of any other pathways has been assessed as ‘very low, with high uncertainty’.

Pathway assessment Negligible likelihood, medium uncertainty.

snip...

Concluding remarks

Following an epidemiological investigation, 4 potential risk pathways have been identified as most likely source of infection. Each are assessed as a very low likelihood event, with high uncertainty.

1. Potential accidental exposure to contaminated feed concentrates at natal farm (old feed remnants of potentially contaminated feed – before the total feed ban in 1996– that might have remained in silo 1 and that could have accidentally been released in 2017).

2. Potential maternal transmission.

3. Environmental source

1: Potential exposure to previous potential presence of BSE on natal farm via birth products.

4. Environmental source

2: Potential exposure to previous potential presence of BSE on natal farm from on farm or local cattle burials via contaminated groundwater or other pathways.

The likelihood of any other potential risk pathways has been assessed as negligible.

The detection of this case is evidence that the surveillance system for detecting and containing BSE is solid and effective. There is no threat to food safety, to human health or to animal health as a consequence of this case. The implementation of control measures and continuous monitoring ensures that the risk of BSE agents being recycled within the bovine population has remained negligible. There is no evidence that any TSE regulations have been breached in this case. There is every reason to believe that current actions will contain any further potential exposure to cattle or the human food chain.

Acknowledgements

The views expressed in this report are those of the National Emergency Epidemiology Group (NEEG). We would like to express our thanks to the TSE experts within APHA, members of the One Health Team and the many other APHA colleagues who have assisted with this investigation. The NEEG is comprised of staff from APHA’s Veterinary, Operations and Science Directorates.

snip...see full report;

Report on the epidemiological investigation of a BSE case in Scotland (RBSE24_00003)

https://assets.publishing.service.gov.uk/media/67225af53ce5634f5f6ef578/bse-epidemiological-report-scotland-2024.pdf

Scotland Single case of disease confirmed in Dumfries and Galloway

Published 06 December 2024 12:45

Topic Farming and rural

Single case of disease confirmed in Dumfries and Galloway.

A case of atypical Bovine Spongiform Encephalopathy (BSE) has been confirmed in a cow on a farm in Dumfries and Galloway.

Precautionary movement restrictions have been put in place at impacted premises and cover animals which have been in contact with the case. Further investigations to identify the origin of the disease are ongoing. This is standard procedure for a confirmed case of atypical BSE.

The case was identified as a result of our routine yet intensive BSE surveillance and stringent control measures are in place. Atypical BSE is not known to be a risk to public health and the animal did not enter the human food chain. Food Standards Scotland have confirmed there is no risk to human health as a result of this isolated case.

The owners of the affected animals are working with authorities on next steps.

Agriculture Minister Jim Fairlie said:

“Following confirmation of a case of atypical BSE in Dumfries and Galloway, the Scottish Government and other agencies took swift and robust action to protect the agriculture sector.

“The fact we identified this isolated case so quickly is proof that our surveillance system for detecting this type of disease is working effectively.

“I want to thank the animal’s owner for their diligence. Their decisive action has allowed us to identify and isolate the case at speed which has minimised its impact on the wider industry."

Chief Veterinary Officer Sheila Voas said:

“The fast detection of this case is proof that our surveillance system is doing its job.

“We are working closely with the Animal and Plant Health Agency, and other partners to identify where the disease came from.

“I want to reassure both farmers and the public that this is an isolated case and of the aytypical strain of BSE which is not transmissible and not connected to contaminated feed. But, if any farmers are concerned, I would urge them to seek veterinary advice."

Ian McWatt, Deputy Chief Executive of Food Standards Scotland said:

“There are strict controls in place to protect consumers from the risk of BSE and consumers can be reassured that these important protection measures remain in place and that Food Standards Scotland Official Veterinarians and Meat Hygiene Inspectors working in all abattoirs in Scotland will continue to ensure that in respect of BSE controls, the safety of consumers remains a priority.

“We will continue to work closely with Scottish Government, other agencies and industry at this time.”

Background

Bovine spongiform encephalopathy (BSE): how to spot and report the disease - gov.scot

The Animal Plant and Health Agency (APHA) is investigating the source of the disease.

All animals over four years of age that die on farm are routinely tested for BSE under our comprehensive surveillance system. Whilst the disease is not directly transmitted from animal to animal, its cohorts, including offspring, have been traced and isolated, and will be destroyed in line with our legal requirements.

In addition to the measures we have in place for fallen stock and animal feed, there is a strict control regime to protect consumers. This includes the removal of specified risk material such as the spinal column, brain and skull from carcasses destined for human consumption.

https://www.gov.scot/news/bse-2/

News BSE Published 10 May 2024 10:30 Topic Farming and rural Disease confirmed in Ayrshire.

A case of classical Bovine Spongiform Encephalopathy (BSE) has been confirmed on a farm in Ayrshire.

Precautionary movement restrictions have been put in place at impacted premises and cover animals which have been in contact with the case. Further investigations to identify the origin of the disease are ongoing. This is standard procedure for a confirmed case of classical BSE.

The case was identified as a result of routine surveillance and stringent control measures. The animal did not enter the human food chain. Food Standards Scotland have confirmed there is no risk to human health as a result of this isolated case.

The owners of the affected animals are working with authorities on next steps.

Read more: BSE: how to spot and report the disease. Agriculture Minister Jim Fairlie said:

“Following confirmation of a case of classical BSE in Ayrshire, the Scottish Government and other agencies took swift and robust action to protect the agriculture sector. This included establishing a precautionary movement ban on the farm.

“The fact we identified this isolated case so quickly is proof that our surveillance system for detecting this type of disease is working effectively.

“I want to thank the animal’s owner for their diligence. Their decisive action has allowed us to identify and isolate the case at speed which has minimised its impact on the wider industry."

Chief Veterinary Officer Sheila Voas said:

“The fast detection of this case is proof that our surveillance system is doing its job.

“We are working closely with the Animal and Plant Health Agency, and other partners to identify where the disease came from.

“I want to reassure both farmers and the public that the risk associated with this isolated case is minimal. But, if any farmers are concerned, I would urge them to seek veterinary advice."

Ian McWatt, Deputy Chief Executive of Food Standards Scotland said:

“There are strict controls in place to protect consumers from the risk of BSE, including controls on animal feed, and removal of the parts of cattle most likely to carry BSE infectivity.

“Consumers can be reassured that these important protection measures remain in place and that Food Standards Scotland Official Veterinarians and Meat Hygiene Inspectors working in all abattoirs in Scotland will continue to ensure that in respect of BSE controls, the safety of consumers remains a priority.

“We will continue to work closely with Scottish Government, other agencies and industry at this time.”

Background

The Animal Plant and Health Agency (APHA) is investigating the source of the outbreak.

All animals over four years of age that die on farm are routinely tested for BSE under our comprehensive surveillance system. Whilst the disease is not directly transmitted from animal to animal, its cohorts, including offspring, have been traced and isolated, and will be destroyed in line with our legal requirements.

In addition to the measures we have in place for fallen stock and animal feed, there is a strict control regime to protect consumers. This includes the removal of specified risk material such as the spinal column, brain and skull from carcasses destined for human consumption.

Movement restrictions have also been put in place at three further farms – the farm of the animal’s origin and two more holdings where animals that have had access to the same feed are.

https://www.gov.scot/news/bse-1/

The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2023

Published: 28 November 2024

Adopted: 29 October 2024

DOI https://doi.org/10.2903/j.efsa.2024.9097

KEYWORDS atypical, BSE, classical, CWD, scrapie, surveillance, TSE

CONTACT biohaw@efsa.europa.eu

Abstract

This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye. In total, 948,165 cattle were tested by EU27 and XI (−3%, compared with 2022), with five atypical BSE cases reported (four H‐type: two in Spain, one in France and one in Ireland; one L‐type in the Netherlands); and 46,096 cattle by eight non‐EU reporting countries with two atypical BSE cases reported by Switzerland. Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1). In total, 284,686 sheep and 102,646 goats were tested in the EU27 and XI (−3.5% and −5.9%, respectively, compared to 2022). In the other non‐EU reporting countries 26,047 sheep and 589 goats were tested. In sheep, 538 cases of scrapie were reported by 14 MS and XI: 462 classical scrapie (CS) by 4 MS (104 index cases (IC) with genotypes of susceptible groups in 93.4% of the cases), 76 atypical scrapie (AS) (76 IC) by 12 MS. In the other non‐EU reporting countries, Iceland reported 70 cases of CS while Norway reported 7 cases of ovine AS. Ovine random genotyping was reported by six MS and genotypes of susceptible groups accounted for 6.9%. In goats, 183 cases of scrapie were reported, all from EU MS: 176 CS (47 IC) by seven MS and 7 AS (7 IC) by five MS. Three cases in Cyprus and one in Spain were reported in goats carrying heterozygous alleles at codon 146 and 222, respectively. In total, 2096 cervids were tested for chronic wasting disease by ten MS, none tested positive. Norway tested 14,224 cervids with one European moose positive.

© European Food Safety Authority

https://www.efsa.europa.eu/en/efsajournal/pub/9097

See full report;

https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2024.9097

The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022

European Food Safety Authority (EFSA)

First published: 28 November 2023

https://doi.org/10.2903/j.efsa.2023.8384

Approved: 19 October 2023 Abstract

This report presents the results of surveillance on transmissible spongiform encephalopathies (TSE) in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2022 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland [XI]) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye. In total, 977,008 cattle were tested by EU27 and XI (−4.3%, compared with 2021), and 52,395 cattle by eight non-EU reporting countries, with one case of H-BSE in France. In total, 295,145 sheep and 109,074 goats were tested in the EU27 and XI (−5.2% and −7.9%, respectively, compared to 2021). In the other non-EU reporting countries, 25,535 sheep and 633 goats were tested. In sheep, 557 cases of scrapie were reported by 17 MS and XI: 480 classical scrapie (CS) by five MS (93 index cases [IC] with genotypes of susceptible groups in 97.6% of the cases), 77 atypical scrapie (AS) (76 IC) by 14 MS and XI. In the other non-EU reporting countries, Norway reported 16 cases of ovine AS. Ovine random genotyping was reported by eight MS and genotypes of susceptible groups accounted for 7.3%. In goats, 224 cases of scrapie were reported, all from EU MS: 216 CS (42 IC) by six MS, and 8 AS (8 IC) by four MS. In Cyprus, two cases of CS were reported in goats carrying the heterozygous DN146 allele. In total, 3202 cervids were tested for chronic wasting disease by 10 MS. One wild European moose tested positive in Finland. Norway tested 17,583 cervids with two European moose, one reindeer and one red deer positive. In total, 154 animals from four other species tested negative in Finland.

https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2023.8384

https://www.efsa.europa.eu/en/search?s=Transmissible%20spongiform%20encephalopathy%20&sort=computed_sort_date&order=desc

''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094

OIE Conclusions on transmissibility of atypical BSE among cattle

Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.

https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf

Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019

34 Scientific Commission/September 2019

3. Atypical BSE

The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.

The Group acknowledged the challenges in demonstrating the zoonotic transmission of atypical strains of BSE in natural exposure scenarios. Overall, the Group was of the opinion that, at this stage, it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that may be different between atypical strains.

4. Definitions of meat-and-bone meal (MBM) and greaves

snip...

REFERENCES

SNIP...END SEE FULL TEXT;

http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf

Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/

Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/

Friday, October 4, 2024

another atypical bovine spongiform encephalopathy (BSE) in Ireland

https://woahoie.blogspot.com/2024/10/another-atypical-bovine-spongiform.html

WEDNESDAY, NOVEMBER 08, 2023

Ireland Atypical BSE confirmed November 3 2023

https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html

TUESDAY, NOVEMBER 14, 2023

Ireland Atypical BSE case, 3 progeny of case cow to be culled

https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html

SUNDAY, JULY 16, 2023

Switzerland Atypical BSE detected in a cow in the canton of St. Gallen

https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html

WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type

Switzerland Bovine Spongiform Encephalopathy Atypical L-Type

Switzerland - Bovine spongiform encephalopathy - Immediate notification

https://wahis.woah.org/#/in-review/4962

https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html

Monday, March 20, 2023

WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type

https://wahis.woah.org/#/in-review/4977

https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall

https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html

https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html

BRAZIL BSE START DATE 2023/01/18

BRAZIL BSE CONFIRMATION DATE 2023/02/22

BRAZIL BSE END DATE 2023/03/03

https://wahis.woah.org/#/in-review/4918

https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html

SPAIN BSE START DATE 2023/01/21

SPAIN BSE CONFIRMATION DATE 2023/02/03

SPAIN BSE END DATE 2023/02/06

https://wahis.woah.org/#/in-review/4888

https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html

NETHERLANDS BSE START DATE 2023/02/01

NETHERLANDS BSE CONFIRMATION DATE 2023/02/01

NETHERLANDS BSE END DATE 2023/03/13

https://wahis.woah.org/#/in-review/4876

https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html

PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...

Wednesday, May 24, 2023

***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification

https://wahis.woah.org/#/in-review/5067

https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html

https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification

SATURDAY, MAY 20, 2023

***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE

https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html

https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed

MAY 19, 2023

https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse

2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;

***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;

Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023

''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...

https://www.regulations.gov/comment/APHIS-2023-0027-0002

https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf

Monday, May 22, 2023

***> BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?

https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html

The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2023

Published: 28 November 2024

Adopted: 29 October 2024

DOI https://doi.org/10.2903/j.efsa.2024.9097

KEYWORDS atypical, BSE, classical, CWD, scrapie, surveillance, TSE

CONTACT biohaw@efsa.europa.eu

Abstract

This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye. In total, 948,165 cattle were tested by EU27 and XI (−3%, compared with 2022), with five atypical BSE cases reported (four H‐type: two in Spain, one in France and one in Ireland; one L‐type in the Netherlands); and 46,096 cattle by eight non‐EU reporting countries with two atypical BSE cases reported by Switzerland. Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1). In total, 284,686 sheep and 102,646 goats were tested in the EU27 and XI (−3.5% and −5.9%, respectively, compared to 2022). In the other non‐EU reporting countries 26,047 sheep and 589 goats were tested. In sheep, 538 cases of scrapie were reported by 14 MS and XI: 462 classical scrapie (CS) by 4 MS (104 index cases (IC) with genotypes of susceptible groups in 93.4% of the cases), 76 atypical scrapie (AS) (76 IC) by 12 MS. In the other non‐EU reporting countries, Iceland reported 70 cases of CS while Norway reported 7 cases of ovine AS. Ovine random genotyping was reported by six MS and genotypes of susceptible groups accounted for 6.9%. In goats, 183 cases of scrapie were reported, all from EU MS: 176 CS (47 IC) by seven MS and 7 AS (7 IC) by five MS. Three cases in Cyprus and one in Spain were reported in goats carrying heterozygous alleles at codon 146 and 222, respectively. In total, 2096 cervids were tested for chronic wasting disease by ten MS, none tested positive. Norway tested 14,224 cervids with one European moose positive.

© European Food Safety Authority

https://www.efsa.europa.eu/en/efsajournal/pub/9097

See full report;

https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2024.9097

The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022

European Food Safety Authority (EFSA)

First published: 28 November 2023

https://doi.org/10.2903/j.efsa.2023.8384

Approved: 19 October 2023 Abstract

This report presents the results of surveillance on transmissible spongiform encephalopathies (TSE) in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2022 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland [XI]) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye. In total, 977,008 cattle were tested by EU27 and XI (−4.3%, compared with 2021), and 52,395 cattle by eight non-EU reporting countries, with one case of H-BSE in France. In total, 295,145 sheep and 109,074 goats were tested in the EU27 and XI (−5.2% and −7.9%, respectively, compared to 2021). In the other non-EU reporting countries, 25,535 sheep and 633 goats were tested. In sheep, 557 cases of scrapie were reported by 17 MS and XI: 480 classical scrapie (CS) by five MS (93 index cases [IC] with genotypes of susceptible groups in 97.6% of the cases), 77 atypical scrapie (AS) (76 IC) by 14 MS and XI. In the other non-EU reporting countries, Norway reported 16 cases of ovine AS. Ovine random genotyping was reported by eight MS and genotypes of susceptible groups accounted for 7.3%. In goats, 224 cases of scrapie were reported, all from EU MS: 216 CS (42 IC) by six MS, and 8 AS (8 IC) by four MS. In Cyprus, two cases of CS were reported in goats carrying the heterozygous DN146 allele. In total, 3202 cervids were tested for chronic wasting disease by 10 MS. One wild European moose tested positive in Finland. Norway tested 17,583 cervids with two European moose, one reindeer and one red deer positive. In total, 154 animals from four other species tested negative in Finland.

https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2023.8384

https://www.efsa.europa.eu/en/search?s=Transmissible%20spongiform%20encephalopathy%20&sort=computed_sort_date&order=desc

Terry S. Singeltary Sr.

EFSA opinion on the BSE related public health risk

Blog Archive

About Me

Thursday, April 3, 2025

Four men and a business all convicted for diverting meat unfit for human consumption back into the human food chain

Thursday, January 9, 2025

UK ministers may lift BSE-era ban on animal remains in chicken and pig feed