Animal feed company convicted at Ballymena court EU Regulation No.999/2001 TSE Regulations
Animal feed company convicted at Ballymena court
Date published: 28 May 2025
Date published: 28 May 2025
Robin Rainey & Sons Limited, Portglenone Road, Randalstown were convicted today at Ballymena Court in relation to one charge of failure to comply with animal feeding requirements.
Green image with the word News in the centre
This contravenes EU Regulation No.999/2001, the Transmissible Spongiform Encephalopathies Regulations (Northern Ireland) 2018 and the Animal By-Products (Enforcement) Regulations 2015 (as amended).
Robin Rainey & Sons Limited pleaded guilty and fined £350 plus £15 offender levy.
The case was brought to the attention of DAERA following routine sampling on a sample of calf meal which tested positive for bone fragments and terrestrial muscle fibres.
Notes to editors:
Robin Rainey & Sons Limited was convicted on one charge of failed to comply with animal feeding requirements, in contravention of Article 7 of EU Regulation No.999/2001 and the Transmissible Spongiform Encephalopathies and Animal By-Products (Amendment etc.) (EU Exit) Regulations 2019, contrary to Regulation 5(5) of the Transmissible Spongiform Encephalopathies Regulations (Northern Ireland) 2018
Assuring food safety in Northern Ireland is essential to uphold public health standards, food production standards and is crucial for the commercial viability of the agri-food sector. All Food and Feed Business Operators should rightly employ all reasonable and practical steps to reduce the risk of any potential contaminants entering the food chain and all operators need to be fully aware of the consequences of not managing risks effectively.
Food safety scares undermine consumer confidence in the output of the agri-food sector. Contamination incidents early in the food chain can have a huge impact in terms of consumer confidence, public health and financial implications. The Feed sector works with supply chain partners, farmers processors, towards the common goal of assuring supply chain integrity. Under EU TSE Regulation (EC) No. 999/2001, the feeding of animal protein to ruminants is prohibited. The controls are implemented in Northern Ireland by the TSE Regulations (Northern Ireland) 2018. TSEs are caused by pathogens known as prions, which are responsible for a range of fatal brain diseases. The diseases include BSE in cattle, Scrapie in sheep and goats and Creutzfeldt-Jakob disease (CJD) and Kuru in humans.
In the UK, the first feed ban of this nature was introduced in 1988. In addition, it has been illegal to feed ruminants with all forms of mammalian protein since November 1994 and to feed any farmed livestock, including fish and horses, with mammalian meat and bone meal since 4 April 1996.
Follow DAERA on X formerly called Twitter and Facebook.(external link opens in a new window / tab)
All media queries should be directed to the DAERA Press Office: pressoffice.group@daera-ni.gov.uk or telephone: 028 9052 4619.
The Executive Information Service operates an out of hours’ service for media enquiries only between 1800hrs and 0800hrs Monday to Friday and at weekends and public holidays. The duty press officer can be contacted on 028 9037 8110.
https://www.daera-ni.gov.uk/news/animal-feed-company-convicted-ballymena-court
Single case of atypical BSE confirmed on a farm in Essex
https://bse-atypical.blogspot.com/2025/05/single-case-of-atypical-bseconfirmed-on.html
Atypical BSE in cattle
THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.
Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.
Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.
Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous
VET RECORD | 29 March–12 April 2025
disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.
The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8
Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.
In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.
Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.
Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.
“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”
Timm Konold, TSE lead scientist
Brenda Rajanayagam, workgroup leader for the data systems group
APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk
Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX
References
1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)
Atypical BSE In Cattle
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc
Green image with the word News in the centre
This contravenes EU Regulation No.999/2001, the Transmissible Spongiform Encephalopathies Regulations (Northern Ireland) 2018 and the Animal By-Products (Enforcement) Regulations 2015 (as amended).
Robin Rainey & Sons Limited pleaded guilty and fined £350 plus £15 offender levy.
The case was brought to the attention of DAERA following routine sampling on a sample of calf meal which tested positive for bone fragments and terrestrial muscle fibres.
Notes to editors:
Robin Rainey & Sons Limited was convicted on one charge of failed to comply with animal feeding requirements, in contravention of Article 7 of EU Regulation No.999/2001 and the Transmissible Spongiform Encephalopathies and Animal By-Products (Amendment etc.) (EU Exit) Regulations 2019, contrary to Regulation 5(5) of the Transmissible Spongiform Encephalopathies Regulations (Northern Ireland) 2018
Assuring food safety in Northern Ireland is essential to uphold public health standards, food production standards and is crucial for the commercial viability of the agri-food sector. All Food and Feed Business Operators should rightly employ all reasonable and practical steps to reduce the risk of any potential contaminants entering the food chain and all operators need to be fully aware of the consequences of not managing risks effectively.
Food safety scares undermine consumer confidence in the output of the agri-food sector. Contamination incidents early in the food chain can have a huge impact in terms of consumer confidence, public health and financial implications. The Feed sector works with supply chain partners, farmers processors, towards the common goal of assuring supply chain integrity. Under EU TSE Regulation (EC) No. 999/2001, the feeding of animal protein to ruminants is prohibited. The controls are implemented in Northern Ireland by the TSE Regulations (Northern Ireland) 2018. TSEs are caused by pathogens known as prions, which are responsible for a range of fatal brain diseases. The diseases include BSE in cattle, Scrapie in sheep and goats and Creutzfeldt-Jakob disease (CJD) and Kuru in humans.
In the UK, the first feed ban of this nature was introduced in 1988. In addition, it has been illegal to feed ruminants with all forms of mammalian protein since November 1994 and to feed any farmed livestock, including fish and horses, with mammalian meat and bone meal since 4 April 1996.
Follow DAERA on X formerly called Twitter and Facebook.(external link opens in a new window / tab)
All media queries should be directed to the DAERA Press Office: pressoffice.group@daera-ni.gov.uk or telephone: 028 9052 4619.
The Executive Information Service operates an out of hours’ service for media enquiries only between 1800hrs and 0800hrs Monday to Friday and at weekends and public holidays. The duty press officer can be contacted on 028 9037 8110.
https://www.daera-ni.gov.uk/news/animal-feed-company-convicted-ballymena-court
Abstract for Prion 2023
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas'
1, Samuel James Sharpe', Kristina Santiago-Mateo', Stefanie Czub', Waqas Tahirl,2, *
Affiliation: National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. ?Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.
*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (C-BSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined. Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of Prpso in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized.
Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for Prpsc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of Prpsc in their brains, having biochemical properties similar to that of Prps in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation
Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Volume 31, Number 5—May 2025
Dispatch
Administration of L-Type Bovine Spongiform Encephalopathy to Macaques to Evaluate Zoonotic Potential
Morikazu Imamura1Comments to Author , Ken’ichi Hagiwara, Minoru Tobiume, Minako Ohno, Hiromi Iguchi, Hanae Takatsuki, Tsuyoshi Mori, Ryuichiro Atarashi, Hiroaki Shibata, and Fumiko Ono1 Author affiliation: University of Miyazaki, Miyazaki, Japan (M. Imamura, M. Ohno, H. Iguchi, H. Takatsuki, T. Mori, R. Atarashi); National Institute of Infectious Diseases, Tokyo, Japan (K. Hagiwara, M. Tobiume); The Corporation for Production and Research of Laboratory Primates, Tsukuba, Japan (H. Shibata); Okayama University of Science, Imabari, Japan (F. Ono) Cite This Article
Abstract
We administered L-type bovine spongiform encephalopathy prions to macaques to determine their potential for transmission to humans. After 75 months, no clinical symptoms appeared, and prions were undetectable in any tissue by Western blot or immunohistochemistry. Protein misfolding cyclic amplification, however, revealed prions in the nerve and lymphoid tissues.
Worldwide emergence of classical bovine spongiform encephalopathy (C-BSE) is associated with variant Creutzfeldt-Jakob disease in humans (1). Two other naturally occurring BSE variants have been identified, L-type (L-BSE) and H-type. Studies using transgenic mice expressing human normal prion protein (PrPC) (2) and primates (3–5) have demonstrated that L-BSE is more virulent than C-BSE. Although L-BSE is orally transmissible to minks (6), cattle (7), and mouse lemurs (5), transmissibility to cynomolgus macaques, a suitable model for investigating human susceptibility to prions, remains unclear. We orally inoculated cynomolgus macaques with L-BSE prions and explored the presence of abnormal prion proteins (PrPSc) in tissues using protein misfolding cyclic amplification (PMCA) along with Western blot (WB) and immunohistochemistry (IHC). PMCA markedly accelerates prion replication in vitro, and its products retain the biochemical properties and transmissibility of seed prion strains (8).
The Study
Two macaques orally inoculated with L-BSE prions remained asymptomatic and healthy but were euthanized and autopsied at 75 months postinoculation. WB showed no PrPSc accumulation in any tissue (Table), IHC revealed no PrPSc accumulation, hematoxylin and eosin staining revealed no spongiform changes in brain sections, and pathologic examination revealed no abnormalities.
Snip…
Conclusion We noted no detectable evidence of PrPSc by WB or IHC in any tissues of L-BSE orally inoculated macaques. Nevertheless, PMCA successfully amplified PrPres from lymphatic and neural tissues. The PrPres exhibited electrophoretic patterns distinct from those detected by PMCA using L-BSE–affected cattle BH as the seed (Figure 3, panel C), indicating that the PrPSc used as the template for PrPres amplification in orally inoculated macaques did not originate from the bovine L-BSE prions used as inoculum. Instead, PrPSc were newly generated by the conversion of macaque PrPC by bovine L-BSE prions. Our results provide strong evidence that L-BSE can infect macaques via the oral route.
We found no evidence that PrPSc reached the brain in orally inoculated macaques; however, the macaques euthanized 6 years postinoculation might have been in the preclinical period. At low infection levels, lymph nodes play a vital role in prion spread to the central nervous system (11). Therefore, had the macaques been maintained for a longer period, they might have developed prion disease. Retrospective surveillance studies using the appendix and tonsil tissues suggested a considerable number of humans harboring vCJD in a carrier state (12). Thus, we cannot exclude that L-BSE orally inoculated macaques could similarly remain in a potentially infectious state.
The brain of L-BSE intracerebrally inoculated macaque accumulated prions with biochemical properties resembling bovine L-BSE prions (Figure 3, panel C; Appendix Figure 2); however, we observed no PrPSc accumulation in lymphoid tissues by WB or IHC (4). In contrast, macaques orally inoculated with C-BSE prions showed PrPSc accumulation in lymphoid tissues, including the spleen, tonsils, and mesenteric lymph nodes by WB and IHC (13). In our study, L-BSE orally inoculated macaques harbored C-BSE–like prions in their lymphoid and neural tissues. Interspecies transmission of L-BSE prions to ovine PrP transgenic mice can result in a shift toward C-BSE–like properties (14,15). Our data suggest that L-BSE prions may alter biophysical and biochemical properties, depending on interspecies transmission and inoculation route, acquiring traits similar to those of C-BSE prions. This transformation might result from structural changes in the L-BSE prion to C-BSE–like prions and other lymphotropic prions within lymphoid tissues or from the selective propagation of low-level lymphotropic substrains within the L-BSE prion population.
The first limitation of our study is that the oral inoculation experiment involved only 2 macaques and tissues collected at 6 years postinoculation, before disease onset. Consequently, subsequent progression of prion disease symptoms remains speculative. A larger sample size and extended observation periods are required to conclusively establish infection in orally inoculated macaques. Furthermore, we performed no bioassays for PMCA-positive samples, leaving the relationship between PMCA results and infectious titers undefined. Considering that PrPres amplifications from tissues from the orally inoculated macaque tissues required 2 rounds of PMCA, the PrPSc levels in positive tissues might have been extremely low and undetectable in the bioassay.
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Top
Dr. Imamura is an associate professor in the Division of Microbiology, Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan. His research interests are focused on elucidating the mechanisms underlying prion formation.
TopExternal Link
Acknowledgment This study was supported by the Health Labor Sciences Research Grant (H29-Shokuhin-Ippan-004, 20KA1003, and 23KA1004).
Top
References…snip…end
https://wwwnc.cdc.gov/eid/article/31/5/24-1257_article#r5
Single case of atypical BSE confirmed on a farm in Essex
Press release
Single case of atypical BSE confirmed on a farm in Essex
A single case of atypical Bovine Spongiform Encephalopathy (BSE) has been confirmed on a farm in Essex, the Animal and Plant Health Agency (APHA) confirmed today (Tuesday 20 May).
From: Department for Environment, Food & Rural Affairs and Animal and Plant Health Agency Published 20 May 2025
The animal showed some clinical signs of BSE and was humanely culled on farm and tested as part of Defra’s routine surveillance programme. There is no risk to public health or food safety from this case and the animal, as fallen stock, was not destined to enter the food chain.
Atypical BSE is a naturally occurring, non-contagious disease in cattle which occurs spontaneously. It is distinct from classical BSE which is linked to contaminated feed.
Chief Veterinary Officer Christine Middlemiss said: “A single case of atypical BSE has been confirmed on a farm in Essex. The animal died on farm and was tested as part of our strict routine controls and surveillance regime.
“Atypical BSE is distinct from classical BSE and is a spontaneously and sporadically occurring, non-contagious disease which is believed to occur at a very low level in all cattle populations. This is proof that our surveillance system for detecting and containing this type of disease is working.”
Dr James Cooper, Deputy Director of Food Policy at the Food Standards Agency said: “There is no food safety risk. 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 Agency Official Veterinarians and Meat Hygiene Inspectors working in all abattoirs in England will continue to ensure that the safety of consumers remains the top priority.”
Great Britain’s overall risk status for BSE remains at ‘controlled’ and there is no risk to food safety or public health.
The World Organisation for Animal Health and trading partners have been informed of the case. This does not affect the UK’s ability to export beef to other countries.
BSE is a notifiable animal disease. If you suspect it, you must report it immediately by calling the Defra Rural Services Helpline on 03000 200 301. In Wales, contact 0300 303 8268. In Scotland, contact your local Field Services Office. Failure to do so is an offence. This applies to pet and small holder animals as well as commercial cattle.
ENDS
Notes to editors: Bovine Spongiform Encephalopathy is a chronic degenerative disease affecting the central nervous system of cattle. It is not contagious, so it does not spread from animal to animal or between holdings.
Classical BSE was first diagnosed in the United Kingdom in 1986.
Atypical BSE is distinct from classical BSE and occurs at a very low level in all cattle populations. It is reported occasionally in countries with active BSE surveillance programmes.
The last case of atypical BSE in the UK was in December 2024 in Scotland. There has been a total of 4 cases since 2015 (including this latest case).
[The case was identified as a result of strict control measures we have in place. It was not destined for the human food chain and the Food Standards Agency have confirmed there is no risk to human health as a result of this isolated case.]
Published 20 May 2025
https://www.gov.uk/government/news/single-case-of-atypical-bseconfirmed-on-a-farm-in-essex
Volume 31, Number 5—May 2025
Dispatch
Administration of L-Type Bovine Spongiform Encephalopathy to Macaques to Evaluate Zoonotic Potential
Morikazu Imamura1Comments to Author , Ken’ichi Hagiwara, Minoru Tobiume, Minako Ohno, Hiromi Iguchi, Hanae Takatsuki, Tsuyoshi Mori, Ryuichiro Atarashi, Hiroaki Shibata, and Fumiko Ono1 Author affiliation: University of Miyazaki, Miyazaki, Japan (M. Imamura, M. Ohno, H. Iguchi, H. Takatsuki, T. Mori, R. Atarashi); National Institute of Infectious Diseases, Tokyo, Japan (K. Hagiwara, M. Tobiume); The Corporation for Production and Research of Laboratory Primates, Tsukuba, Japan (H. Shibata); Okayama University of Science, Imabari, Japan (F. Ono) Cite This Article
Abstract
We administered L-type bovine spongiform encephalopathy prions to macaques to determine their potential for transmission to humans. After 75 months, no clinical symptoms appeared, and prions were undetectable in any tissue by Western blot or immunohistochemistry. Protein misfolding cyclic amplification, however, revealed prions in the nerve and lymphoid tissues.
Worldwide emergence of classical bovine spongiform encephalopathy (C-BSE) is associated with variant Creutzfeldt-Jakob disease in humans (1). Two other naturally occurring BSE variants have been identified, L-type (L-BSE) and H-type. Studies using transgenic mice expressing human normal prion protein (PrPC) (2) and primates (3–5) have demonstrated that L-BSE is more virulent than C-BSE. Although L-BSE is orally transmissible to minks (6), cattle (7), and mouse lemurs (5), transmissibility to cynomolgus macaques, a suitable model for investigating human susceptibility to prions, remains unclear. We orally inoculated cynomolgus macaques with L-BSE prions and explored the presence of abnormal prion proteins (PrPSc) in tissues using protein misfolding cyclic amplification (PMCA) along with Western blot (WB) and immunohistochemistry (IHC). PMCA markedly accelerates prion replication in vitro, and its products retain the biochemical properties and transmissibility of seed prion strains (8).
The Study
Two macaques orally inoculated with L-BSE prions remained asymptomatic and healthy but were euthanized and autopsied at 75 months postinoculation. WB showed no PrPSc accumulation in any tissue (Table), IHC revealed no PrPSc accumulation, hematoxylin and eosin staining revealed no spongiform changes in brain sections, and pathologic examination revealed no abnormalities.
Snip…
Conclusion We noted no detectable evidence of PrPSc by WB or IHC in any tissues of L-BSE orally inoculated macaques. Nevertheless, PMCA successfully amplified PrPres from lymphatic and neural tissues. The PrPres exhibited electrophoretic patterns distinct from those detected by PMCA using L-BSE–affected cattle BH as the seed (Figure 3, panel C), indicating that the PrPSc used as the template for PrPres amplification in orally inoculated macaques did not originate from the bovine L-BSE prions used as inoculum. Instead, PrPSc were newly generated by the conversion of macaque PrPC by bovine L-BSE prions. Our results provide strong evidence that L-BSE can infect macaques via the oral route.
We found no evidence that PrPSc reached the brain in orally inoculated macaques; however, the macaques euthanized 6 years postinoculation might have been in the preclinical period. At low infection levels, lymph nodes play a vital role in prion spread to the central nervous system (11). Therefore, had the macaques been maintained for a longer period, they might have developed prion disease. Retrospective surveillance studies using the appendix and tonsil tissues suggested a considerable number of humans harboring vCJD in a carrier state (12). Thus, we cannot exclude that L-BSE orally inoculated macaques could similarly remain in a potentially infectious state.
The brain of L-BSE intracerebrally inoculated macaque accumulated prions with biochemical properties resembling bovine L-BSE prions (Figure 3, panel C; Appendix Figure 2); however, we observed no PrPSc accumulation in lymphoid tissues by WB or IHC (4). In contrast, macaques orally inoculated with C-BSE prions showed PrPSc accumulation in lymphoid tissues, including the spleen, tonsils, and mesenteric lymph nodes by WB and IHC (13). In our study, L-BSE orally inoculated macaques harbored C-BSE–like prions in their lymphoid and neural tissues. Interspecies transmission of L-BSE prions to ovine PrP transgenic mice can result in a shift toward C-BSE–like properties (14,15). Our data suggest that L-BSE prions may alter biophysical and biochemical properties, depending on interspecies transmission and inoculation route, acquiring traits similar to those of C-BSE prions. This transformation might result from structural changes in the L-BSE prion to C-BSE–like prions and other lymphotropic prions within lymphoid tissues or from the selective propagation of low-level lymphotropic substrains within the L-BSE prion population.
The first limitation of our study is that the oral inoculation experiment involved only 2 macaques and tissues collected at 6 years postinoculation, before disease onset. Consequently, subsequent progression of prion disease symptoms remains speculative. A larger sample size and extended observation periods are required to conclusively establish infection in orally inoculated macaques. Furthermore, we performed no bioassays for PMCA-positive samples, leaving the relationship between PMCA results and infectious titers undefined. Considering that PrPres amplifications from tissues from the orally inoculated macaque tissues required 2 rounds of PMCA, the PrPSc levels in positive tissues might have been extremely low and undetectable in the bioassay.
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Top
Dr. Imamura is an associate professor in the Division of Microbiology, Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan. His research interests are focused on elucidating the mechanisms underlying prion formation.
TopExternal Link
Acknowledgment This study was supported by the Health Labor Sciences Research Grant (H29-Shokuhin-Ippan-004, 20KA1003, and 23KA1004).
Top
References…snip…end
https://wwwnc.cdc.gov/eid/article/31/5/24-1257_article#r5
Single case of atypical BSE confirmed on a farm in Essex
Press release
Single case of atypical BSE confirmed on a farm in Essex
A single case of atypical Bovine Spongiform Encephalopathy (BSE) has been confirmed on a farm in Essex, the Animal and Plant Health Agency (APHA) confirmed today (Tuesday 20 May).
From: Department for Environment, Food & Rural Affairs and Animal and Plant Health Agency Published 20 May 2025
The animal showed some clinical signs of BSE and was humanely culled on farm and tested as part of Defra’s routine surveillance programme. There is no risk to public health or food safety from this case and the animal, as fallen stock, was not destined to enter the food chain.
Atypical BSE is a naturally occurring, non-contagious disease in cattle which occurs spontaneously. It is distinct from classical BSE which is linked to contaminated feed.
Chief Veterinary Officer Christine Middlemiss said: “A single case of atypical BSE has been confirmed on a farm in Essex. The animal died on farm and was tested as part of our strict routine controls and surveillance regime.
“Atypical BSE is distinct from classical BSE and is a spontaneously and sporadically occurring, non-contagious disease which is believed to occur at a very low level in all cattle populations. This is proof that our surveillance system for detecting and containing this type of disease is working.”
Dr James Cooper, Deputy Director of Food Policy at the Food Standards Agency said: “There is no food safety risk. 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 Agency Official Veterinarians and Meat Hygiene Inspectors working in all abattoirs in England will continue to ensure that the safety of consumers remains the top priority.”
Great Britain’s overall risk status for BSE remains at ‘controlled’ and there is no risk to food safety or public health.
The World Organisation for Animal Health and trading partners have been informed of the case. This does not affect the UK’s ability to export beef to other countries.
BSE is a notifiable animal disease. If you suspect it, you must report it immediately by calling the Defra Rural Services Helpline on 03000 200 301. In Wales, contact 0300 303 8268. In Scotland, contact your local Field Services Office. Failure to do so is an offence. This applies to pet and small holder animals as well as commercial cattle.
ENDS
Notes to editors: Bovine Spongiform Encephalopathy is a chronic degenerative disease affecting the central nervous system of cattle. It is not contagious, so it does not spread from animal to animal or between holdings.
Classical BSE was first diagnosed in the United Kingdom in 1986.
Atypical BSE is distinct from classical BSE and occurs at a very low level in all cattle populations. It is reported occasionally in countries with active BSE surveillance programmes.
The last case of atypical BSE in the UK was in December 2024 in Scotland. There has been a total of 4 cases since 2015 (including this latest case).
[The case was identified as a result of strict control measures we have in place. It was not destined for the human food chain and the Food Standards Agency have confirmed there is no risk to human health as a result of this isolated case.]
Published 20 May 2025
https://www.gov.uk/government/news/single-case-of-atypical-bseconfirmed-on-a-farm-in-essex
THURSDAY, MAY 22, 2025
Single case of atypical BSE confirmed on a farm in Essex
https://bse-atypical.blogspot.com/2025/05/single-case-of-atypical-bseconfirmed-on.html
Atypical BSE in cattle
THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.
Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.
Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.
Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous
VET RECORD | 29 March–12 April 2025
disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.
The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8
Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.
In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.
Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.
Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.
“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”
Timm Konold, TSE lead scientist
Brenda Rajanayagam, workgroup leader for the data systems group
APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk
Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX
References
1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)
Atypical BSE In Cattle
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc
FRIDAY, MAY 23, 2025
Epidemiological investigation of a single atypical BSE case in Dumfries and Galloway, Scotland (RBSE 24/00006)
https://bse-atypical.blogspot.com/2025/05/epidemiological-investigation-of-single.html
Epidemiological investigation of a single atypical BSE case in Dumfries and Galloway, Scotland (RBSE 24/00006)
https://bse-atypical.blogspot.com/2025/05/epidemiological-investigation-of-single.html
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