Tuesday, November 14, 2023

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

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

Atypical BSE case: 3 progeny of case cow to be culled

November 14, 2023 9:21 am

Atypical BSE case: 3 progeny of case cow to be culled

Three progeny animals of the 10-year-old cow in which a case of atypical bovine spongiform encephalopathy (BSE) was confirmed have been identified and will be culled, the Department of Agriculture, Food and the Marine confirmed.

The department said that EU regulations require the identification and culling of these progeny.

The EU regulation in question – which lays down rules for the prevention, control, and eradication of certain transmissible spongiform encephalopathies (TSE) – does not distinguish between atypical BSE and classic BSE where the requirement to identify progeny is concerned.

The regulation requires all progeny born to a cow with BSE in the two years prior to, or two years after, clinical onset of the disease to be identified.

The department confirmed last week that beef exports to China were suspended after tests confirmed a case of atypical BSE.

The department said that the animal in question was a 10-and-a-half-year-old cow and was identified during the department’s on-going systematic surveillance of fallen animals at knackeries.

The animal did not enter the food or feed chain and there are no public health risks associated with this occurrence.

The department subsequently confirmed that, following the identification of this atypical BSE case, three progeny animals of the case animal were identified and restricted immediately on the department’s Animal Identification and Movement (AIM) and animal health systems.

“In accordance with EU regulations, these animals will be culled and disposed of outside the food and feed chains and arrangements are being made in that regard currently,” the department said in a statement.

“These animals pose no public or animal health risk,” the statement added.

The department went on to reiterate: “Atypical BSE is recognised as being a sporadic spontaneous disease at a very low level in the bovine population.

“All atypical BSE cases identified in Ireland have been single sporadic cases. It is testament to the effectiveness of the routine surveillance system in place in Ireland that his case was identified,” the department said.


Ireland Atypical BSE confirmed November 3 2023

Beef exports to China suspended after case of Atypical BSE discovered in Irish cow

BSE, commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

BEEF EXPORTS TO China have been suspended after a case of Atypical BSE was detected during the testing of a cow in Ireland last Friday, the Department of Agriculture has said. 

The Department said that the decision was made by China and that the lifting of the suspension was at the discretion of the Chinese authorities. 

BSE (Bovine Spongiform Encephalopathy), commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

Classical BSE occurs when cows eat contaminated feed while the Atypical form is thought to occur spontaneously in bovine herds, according to the World Organisation for Animal Health This was the first Atypical BSE case detected in Ireland since 2020, the Department told The Journal. 

In statement the Department said the animal did not enter the food or feed chain and that no health risks were associated with the detected case. 

“Tests carried out at the Department’s Central Veterinary Research Laboratory confirmed a case of “atypical BSE” on 3 November. The animal in question was a 10 and a half year old cow and was identified during the Department’s on-going systematic surveillance of ‘fallen’ animals at ‘knackeries’. “The animal did not enter the food or feed chain and there are no public health risks associated with this occurrence. Atypical BSE is a rare spontaneous event that may occur in any bovine population. It is not related to feed contamination.”

The Department also said that the detection of such a case “does not impact on trade generally” but that Chinese protocols mean exports have been paused. 

“The protocol with China requires exports to be suspended pending submission and assessment of the epidemiological report,” the Department said.

“Therefore exports of beef to China are now temporarily suspended. The timeframe for resumption is a matter for the Chinese authorities.” 

The Department also said that the detection of this case is an example of the testing system working.

“Ireland’s BSE controls are robust and effective and consistent with legal requirements and best international practice.

“The discovery of this case exemplifies the strength of Ireland’s controls and surveillance system; demonstrable proof that our food and feed safety controls are effective,” the statement read. 

Beef is a major Irish export. Over 90% of Irish beef is sold abroad and the Government has just conducted a visit to South Korea, during which beef trade was a key topic on the agenda.

Reacting to the announcement this evening, the Irish Farmers’ Association’s Livestock Chair Brendan Golden said suspension of access to the Chinese market was disappointing but it must be resolved quickly.

Golden said: “Given that we had only recently regained access, it’s a setback that we could do without.”

Golden added that he hopes, once the case is reviewed by Chinese Authorities, there will be no delay in getting exports to the region restored. 

Beef exports to China suspended after case of Atypical BSE discovered in Irish cow

BSE, commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

BEEF EXPORTS TO China have been suspended after a case of Atypical BSE was detected during the testing of a cow in Ireland last Friday, the Department of Agriculture has said. 

The Department said that the decision was made by China and that the lifting of the suspension was at the discretion of the Chinese authorities. 

BSE (Bovine Spongiform Encephalopathy), commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

Classical BSE occurs when cows eat contaminated feed while the Atypical form is thought to occur spontaneously in bovine herds, according to the World Organisation for Animal Health This was the first Atypical BSE case detected in Ireland since 2020, the Department told The Journal. 

In statement the Department said the animal did not enter the food or feed chain and that no health risks were associated with the detected case. 

“Tests carried out at the Department’s Central Veterinary Research Laboratory confirmed a case of “atypical BSE” on 3 November. The animal in question was a 10 and a half year old cow and was identified during the Department’s on-going systematic surveillance of ‘fallen’ animals at ‘knackeries’. “The animal did not enter the food or feed chain and there are no public health risks associated with this occurrence. Atypical BSE is a rare spontaneous event that may occur in any bovine population. It is not related to feed contamination.”

The Department also said that the detection of such a case “does not impact on trade generally” but that Chinese protocols mean exports have been paused. 

“The protocol with China requires exports to be suspended pending submission and assessment of the epidemiological report,” the Department said.

“Therefore exports of beef to China are now temporarily suspended. The timeframe for resumption is a matter for the Chinese authorities.” 

The Department also said that the detection of this case is an example of the testing system working.

“Ireland’s BSE controls are robust and effective and consistent with legal requirements and best international practice.

“The discovery of this case exemplifies the strength of Ireland’s controls and surveillance system; demonstrable proof that our food and feed safety controls are effective,” the statement read. 

Beef is a major Irish export. Over 90% of Irish beef is sold abroad and the Government has just conducted a visit to South Korea, during which beef trade was a key topic on the agenda.

Reacting to the announcement this evening, the Irish Farmers’ Association’s Livestock Chair Brendan Golden said suspension of access to the Chinese market was disappointing but it must be resolved quickly.

Golden said: “Given that we had only recently regained access, it’s a setback that we could do without.”

Golden added that he hopes, once the case is reviewed by Chinese Authorities, there will be no delay in getting exports to the region restored. 


WEDNESDAY, NOVEMBER 08, 2023 

Ireland Atypical BSE confirmed November 3 2023 


''The department went on to reiterate: “Atypical BSE is recognised as being a sporadic spontaneous disease at a very low level in the bovine population.''

THERE IS NOT A SHRED OF EVIDENCE THAT THE ATYPICAL BSE CASES ARE A SPONTANEOUS EVENT, AND IF THERE WERE, THAT WOULD BE THE WORST NIGHTMARE.

ON THE CONTRARY, ATYPICAL BSE TRANSMIT BY ORAL ROUTES TO CATTLE. THIS SPONTANEOUS ATYPICAL BSE EVENTS HAVE SURE BEEN HAPPENING A LOT LATELY...terry

TUESDAY, MAY 26, 2020 

Ireland OIE Atypical BSE H-type 


Atypical BSE cases in Ireland: neurological signs, brain histopathology and Tissue distribution of PrPres

Sebas6an Alessandro Mignacca, Ann Sharpe, Emma Curley, Semsa Omerovic, Cisca Kimbembe, Máire McElroy Department of Agriculture, Food and the Marine - Pathology Division, Celbridge, Co. Kildare, Ireland

Aims:

In Ireland, six atypical BSE cases, five H-type (H-1 to -5) and one L-type, have been confirmed up to May 2023. Herein, the neurological characteris6cs, brain histopathology, topographical distribu6on, and signal intensity of PrPres are described.

Material and Methods:

All cases were iden6fied through ac6ve surveillance. Clinical history was retrieved from the Department of Agriculture, Food and the Marine archives. Whole brains/brainstems of H-type animals, and the L-type, and selected peripheral 6ssues of L-type were further studied by histopathology, immunohistochemistry (IHC - MAb F89) and immunoblotting (APHA BioRad TeSeE Hybrid). Investigations on PrPres distribution on the H-5 are in progress.

Results:

All animals were beef-breed females, aged between 11 – 18 years-old. They had vague clinical histories of depression, inappetence, incoordination, and recumbency, lasting 2-4 days. In the L-type and in H-5 intermittent signs lasted 2 and 6 weeks, respectively. H-2 was a healthy slaughtered animal. 

Among the suitable obices for histopathology (H-1, -2 and -5), and the whole brain of H-5, vacuolation was only detected in H-5. Positive immunostaining was detected at the obex for H-1, in medulla, thalamus, cerebellum for H-2, and at all levels of the brain for H-3 and H-5. In the fallen H-type cases, immunoblot and Idexx EIA were consistently strong in all brain levels. In the healthy slaughter animal, PrPres levels were lower in cerebellum and cerebral cortex. 

L-type showed inconclusive histopathological changes at obex, whilst neuropil vacuolation was most marked in thalamus and midbrain. PrPres was detected by IHC, immunoblotting and Idexx EIA at all levels of the brain and spinal cord, and immunoblotting only in the op6c nerve and re6na.

Conclusions:

Clinical courses were short and non-specific. PrPres intensity in all cases were generally high at all levels of the brain tested including the obex, the official target area for BSE surveillance. A

Acknowledgements: Colleagues in Regional Veterinary Laboratories for collec6ng the brain material. Colleagues in TSE Division and DVOs for clinical information on cases


Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *

Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department 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 (CBSE) 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 PrPSc 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 PrPSc 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 PrPSc 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


'Spontaneous mutation'

***> PLEASE NOTE!

spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;

***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.


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.


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.

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


The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion


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


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.


Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate

Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata

Affiliations expand

PMID: 21266763

Abstract

A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.


see full text;


''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.


Wednesday, May 24, 2023 

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


FRIDAY, MAY 19, 2023 

USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection

 
https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa

SATURDAY, MAY 20, 2023 

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



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

Switzerland Bovine Spongiform Encephalopathy Atypical L-Type

Switzerland - Bovine spongiform encephalopathy - Immediate notification


BRAZIL BSE START DATE 2023/01/18

BRAZIL BSE CONFIRMATION DATE 2023/02/22

BRAZIL BSE END DATE 2023/03/03


SPAIN BSE START DATE 2023/01/21

SPAIN BSE CONFIRMATION DATE 2023/02/03

SPAIN BSE END DATE 2023/02/06


NETHERLANDS BSE START DATE 2023/02/01

NETHERLANDS BSE CONFIRMATION DATE 2023/02/01

NETHERLANDS BSE END DATE 2023/03/13


The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2021

Published:

30 November 2022

Approved: 3 November 2022

Metadata

EFSA Journal 2022;20(11):7655


Keywords: TSE, BSE, CWD, scrapie, classical, atypical, surveillance

On request from: European Commission Question Number: EFSA‐Q‐2021‐00765

Contact: zoonoses@efsa.europa.eu

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 2021 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland) (XI), and eight other non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Turkey. 

In total, 1,021,252 cattle were tested by EU27 and XI (−9%, compared with 2020 when data from the United Kingdom were not restricted to Northern Ireland), and 66,121 cattle by eight non‐EU reporting countries, 

with two cases of H‐BSE in France and Spain, and four L‐BSE in France (2), Germany and Spain. 

In total, 311,174 sheep and 118,457 goats were tested in the EU27 and XI (−6.4% and −1.8%, respectively, compared to 2020 when data from the whole United Kingdom were considered). 

In sheep, 551 cases of scrapie were reported by 17 MS and XI: 448 classical scrapie (CS) by six MS [80 index cases (IC) with genotypes of susceptible groups in 97% of the cases], 103 atypical scrapie (AS) (96 IC) by 13 MS and XI. 

In the other non‐EU reporting countries, 27,594 sheep were tested with 55 CS and 1 AS in Iceland and 8 AS in Norway. 

Ovine random genotyping was reported by nine MS and genotypes of susceptible groups accounted for 7.9%. 

In goats, 224 cases of scrapie were reported by six EU MS: 219 CS (30 IC) by six MS, and five AS (5 IC) by three MS. 

In total, 5,854 cervids were tested for chronic wasting disease by eight MS; all resulted negative. Norway tested 21,670 cervids with two moose and one red deer positive. In total, 149 animals from four other species tested negative in Finland and Turkey.

© European Food Safety Authority


see full text;


Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle 

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department 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 (CBSE) 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 PrPSc 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 PrPSc 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 PrPSc 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

Acknowledgement: TSE unit NCAD, Lethbridge (Jianmin Yang, Sarah Bogart, Rachana Muley, Yuanmu Fang, Keri Colwell, Renee Anderson, John Gray, Rakhi Katoch) (CFIA, Canada), Dr. Catherine Graham (NSDA, Canada), Dr. Michel Levy (UCVM, Canada), Dr. Martin Groschup (FLI, Germany), Dr. Christine Fast (FLI, Germany), Dr. Bob Hills (Health Canada, Canada) Theme: Animal prion diseases

"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 PrPSc in C-BSE. "

=====end


Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE). 

Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA 

Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME). 

Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97). 

Results: Approximately 16.6 months post-inoculation, Steer 6 (EK211 L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (EK211 L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME. 

Conclusions: Further study of L-BSE in EK211 cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE. 

Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE. 

Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases

=====end

PRION 2023 CONTINUED;


Monday, November 13, 2023

Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023


Professor John Collinge on tackling prion diseases, sCJD accounts for around 1 in 5000 deaths worldwide

MONDAY, SEPTEMBER 11, 2023 

Professor John Collinge on tackling prion diseases “The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.” There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.



Terry S. Singeltary Sr.

posted by Terry S. Singeltary Sr. at 11:33 AM 0 Comments

Wednesday, November 08, 2023

Ireland Atypical BSE confirmed November 3 2023

Ireland Atypical BSE confirmed November 3 2023

Beef exports to China suspended after case of Atypical BSE discovered in Irish cow

BSE, commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

BEEF EXPORTS TO China have been suspended after a case of Atypical BSE was detected during the testing of a cow in Ireland last Friday, the Department of Agriculture has said. 

The Department said that the decision was made by China and that the lifting of the suspension was at the discretion of the Chinese authorities. 

BSE (Bovine Spongiform Encephalopathy), commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

Classical BSE occurs when cows eat contaminated feed while the Atypical form is thought to occur spontaneously in bovine herds, according to the World Organisation for Animal Health This was the first Atypical BSE case detected in Ireland since 2020, the Department told The Journal. 

In statement the Department said the animal did not enter the food or feed chain and that no health risks were associated with the detected case. 

“Tests carried out at the Department’s Central Veterinary Research Laboratory confirmed a case of “atypical BSE” on 3 November. The animal in question was a 10 and a half year old cow and was identified during the Department’s on-going systematic surveillance of ‘fallen’ animals at ‘knackeries’. “The animal did not enter the food or feed chain and there are no public health risks associated with this occurrence. Atypical BSE is a rare spontaneous event that may occur in any bovine population. It is not related to feed contamination.”

The Department also said that the detection of such a case “does not impact on trade generally” but that Chinese protocols mean exports have been paused. 

“The protocol with China requires exports to be suspended pending submission and assessment of the epidemiological report,” the Department said.

“Therefore exports of beef to China are now temporarily suspended. The timeframe for resumption is a matter for the Chinese authorities.” 

The Department also said that the detection of this case is an example of the testing system working.

“Ireland’s BSE controls are robust and effective and consistent with legal requirements and best international practice.

“The discovery of this case exemplifies the strength of Ireland’s controls and surveillance system; demonstrable proof that our food and feed safety controls are effective,” the statement read. 

Beef is a major Irish export. Over 90% of Irish beef is sold abroad and the Government has just conducted a visit to South Korea, during which beef trade was a key topic on the agenda.

Reacting to the announcement this evening, the Irish Farmers’ Association’s Livestock Chair Brendan Golden said suspension of access to the Chinese market was disappointing but it must be resolved quickly.

Golden said: “Given that we had only recently regained access, it’s a setback that we could do without.”

Golden added that he hopes, once the case is reviewed by Chinese Authorities, there will be no delay in getting exports to the region restored. 

Beef exports to China suspended after case of Atypical BSE discovered in Irish cow

BSE, commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

BEEF EXPORTS TO China have been suspended after a case of Atypical BSE was detected during the testing of a cow in Ireland last Friday, the Department of Agriculture has said. 

The Department said that the decision was made by China and that the lifting of the suspension was at the discretion of the Chinese authorities. 

BSE (Bovine Spongiform Encephalopathy), commonly called Mad Cow Disease, comes in two forms, Classical BSE and Atypical BSE.

Classical BSE occurs when cows eat contaminated feed while the Atypical form is thought to occur spontaneously in bovine herds, according to the World Organisation for Animal Health This was the first Atypical BSE case detected in Ireland since 2020, the Department told The Journal. 

In statement the Department said the animal did not enter the food or feed chain and that no health risks were associated with the detected case. 

“Tests carried out at the Department’s Central Veterinary Research Laboratory confirmed a case of “atypical BSE” on 3 November. The animal in question was a 10 and a half year old cow and was identified during the Department’s on-going systematic surveillance of ‘fallen’ animals at ‘knackeries’. “The animal did not enter the food or feed chain and there are no public health risks associated with this occurrence. Atypical BSE is a rare spontaneous event that may occur in any bovine population. It is not related to feed contamination.”

The Department also said that the detection of such a case “does not impact on trade generally” but that Chinese protocols mean exports have been paused. 

“The protocol with China requires exports to be suspended pending submission and assessment of the epidemiological report,” the Department said.

“Therefore exports of beef to China are now temporarily suspended. The timeframe for resumption is a matter for the Chinese authorities.” 

The Department also said that the detection of this case is an example of the testing system working.

“Ireland’s BSE controls are robust and effective and consistent with legal requirements and best international practice.

“The discovery of this case exemplifies the strength of Ireland’s controls and surveillance system; demonstrable proof that our food and feed safety controls are effective,” the statement read. 

Beef is a major Irish export. Over 90% of Irish beef is sold abroad and the Government has just conducted a visit to South Korea, during which beef trade was a key topic on the agenda.

Reacting to the announcement this evening, the Irish Farmers’ Association’s Livestock Chair Brendan Golden said suspension of access to the Chinese market was disappointing but it must be resolved quickly.

Golden said: “Given that we had only recently regained access, it’s a setback that we could do without.”

Golden added that he hopes, once the case is reviewed by Chinese Authorities, there will be no delay in getting exports to the region restored. 


“Classical BSE occurs when cows eat contaminated feed while the Atypical form is thought to occur spontaneously in bovine herds, according to the World Organisation for Animal Health”


Ministers McConalogue and Heydon announce the resumption of beef exports to China From Department of Agriculture, Food and the Marine 

Published on 5 January 2023

Last updated on 10 January 2023

 Minister for Agriculture, Food and the Marine, Charlie McConalogue, and Minister of State Martin Heydon today announced the resumption of Irish beef exports to China.

Beef shipments to China were suspended following the confirmation in May 2020 by the Department of Agriculture, Food and the Marine of an isolated case of atypical BSE. This isolated case was detected by the department’s surveillance programme, did not enter the food chain and posed no risk to human health. Atypical BSE occurs naturally and sporadically in all cattle populations at a very low rate and is not considered a public health risk.

Nevertheless, beef exports were immediately suspended as a precautionary measure in line with the bilateral protocol on the trade agreed with the General Administration of Customs of China (GACC).

Minister McConalogue stated:

“Negotiating the resumption of beef access has been a top priority for my department over the last two and a half years. Together with colleagues in the Embassy of Ireland in Beijing, my officials have been in ongoing contact with the Chinese authorities since the start of the suspension. They provided the necessary technical information for evaluation by Chinese experts. Earlier this year, as a result of my communication with my counterpart in charge of GACC, our officials engaged in further bilateral talks to finalise the restoration of beef access based on scientific principles.

“Ireland’s reputation as an exporter of high-quality beef worldwide hinges on its compliance with stringent animal health and food safety standards. As a major food exporter, we are always very conscious of the concerns of our customers. China’s decision to resume Irish beef imports on the same conditions as before represents a clear vote of confidence in the output of our beef sector.

“This decision is a great vote of confidence in our beef sector and our committed suckler and beef farmers who produce a world class product. I look forward to seeing the value of the Chinese market grow in the time ahead to reward the efforts of everyone involved.”

Minister of State Martin Heydon, whose responsibilities include market development, hailed today’s news as a positive development for the Irish beef sector.

Minister Heydon said:

“We will continue to work with the Chinese authorities to maintain and enhance our access to the Chinese market for Irish food and drink. I know that Bord Bia will soon set in motion a programme of promotions to allow exporters capitalise on the opportunities offered by this announcement. Prior to the suspension, overall Irish beef exports to China were on an upward trajectory and I am confident that we can quickly regain momentum and market share there.

“I want to recognise the role of Ireland’s Ambassador to China, Dr Ann Derwin, the current and previous agricultural attachés, and all the team at the Embassy of Ireland in Beijing for their efforts in presenting at every opportunity a reasoned case on the safety of Irish beef exports. I also want to acknowledge the work of Bord Bia Shanghai in communicating the quality and sustainability of Irish beef to key trade customers in China.”

Notes There are two types of BSE: classical BSE which occurs through the consumption of contaminated feed, and atypical BSE which is believed to occur in all cattle populations at a very low rate and which have only been identified in older cattle. Ireland is internationally recognised as having the lowest possible risk status for BSE. The negligible risk designation in May 2021 by the World Organisation of Animal Health (WOAH), which sets trading standards for animals and animal products, provided independent verification of the effectiveness of Ireland’s control system for BSE.

After several years of negotiations, Ireland secured access for the export of frozen boneless beef to China in April 2018. Exports grew steadily until May 2020 when shipments were suspended in line with the sanitary agreement governing the beef trade with China. Irish beef exports to China, including Hong Kong which operates as a special administrative region with different market access rules, were worth €45 million in 2021, down from exports of €96 million in 2019.


TUESDAY, MAY 26, 2020 

Ireland OIE Atypical BSE H-type 


Atypical BSE cases in Ireland: neurological signs, brain histopathology and Tissue distribution of PrPres

Sebas6an Alessandro Mignacca, Ann Sharpe, Emma Curley, Semsa Omerovic, Cisca Kimbembe, Máire McElroy Department of Agriculture, Food and the Marine - Pathology Division, Celbridge, Co. Kildare, Ireland

Aims:

In Ireland, six atypical BSE cases, five H-type (H-1 to -5) and one L-type, have been confirmed up to May 2023. Herein, the neurological characteristics, brain histopathology, topographical distribution, and signal intensity of PrPres are described.

Material and Methods:

All cases were identified through active surveillance. Clinical history was retrieved from the Department of Agriculture, Food and the Marine archives. Whole brains/brainstems of H-type animals, and the L-type, and selected peripheral tissues of L-type were further studied by histopathology, immunohistochemistry (IHC - MAb F89) and immunoblotting (APHA BioRad TeSeE Hybrid). Investigations on PrPres distribution on the H-5 are in progress.

Results:

All animals were beef-breed females, aged between 11 – 18 years-old. They had vague clinical histories of depression, inappetence, incoordination, and recumbency, lasting 2-4 days. In the L-type and in H-5 intermittent signs lasted 2 and 6 weeks, respectively. H-2 was a healthy slaughtered animal. 

Among the suitable obices for histopathology (H-1, -2 and -5), and the whole brain of H-5, vacuolation was only detected in H-5. Positive immunostaining was detected at the obex for H-1, in medulla, thalamus, cerebellum for H-2, and at all levels of the brain for H-3 and H-5. In the fallen H-type cases, immunoblot and Idexx EIA were consistently strong in all brain levels. In the healthy slaughter animal, PrPres levels were lower in cerebellum and cerebral cortex. 

L-type showed inconclusive histopathological changes at obex, whilst neuropil vacuolation was most marked in thalamus and midbrain. PrPres was detected by IHC, immunoblotting and Idexx EIA at all levels of the brain and spinal cord, and immunoblotting only in the optic nerve and retina.

Conclusions:

Clinical courses were short and non-specific. PrPres intensity in all cases were generally high at all levels of the brain tested including the obex, the official target area for BSE surveillance. 

Acknowledgements: Colleagues in Regional Veterinary Laboratories for collec6ng the brain material. Colleagues in TSE Division and DVOs for clinical information on cases


Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *

Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department 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 (CBSE) 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 PrPSc 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 PrPSc 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 PrPSc 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


'Spontaneous mutation'

***> PLEASE NOTE!

spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;

***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.


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.


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.

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


The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion


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


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.


Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate

Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata

Affiliations expand

PMID: 21266763

Abstract

A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.


see full text;


''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.


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.




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.


Wednesday, May 24, 2023 

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


FRIDAY, MAY 19, 2023 

USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection

 
https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa

SATURDAY, MAY 20, 2023 

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



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

Switzerland Bovine Spongiform Encephalopathy Atypical L-Type

Switzerland - Bovine spongiform encephalopathy - Immediate notification


BRAZIL BSE START DATE 2023/01/18

BRAZIL BSE CONFIRMATION DATE 2023/02/22

BRAZIL BSE END DATE 2023/03/03


SPAIN BSE START DATE 2023/01/21

SPAIN BSE CONFIRMATION DATE 2023/02/03

SPAIN BSE END DATE 2023/02/06


NETHERLANDS BSE START DATE 2023/02/01

NETHERLANDS BSE CONFIRMATION DATE 2023/02/01

NETHERLANDS BSE END DATE 2023/03/13


The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2021

Published:

30 November 2022

Approved: 3 November 2022

Metadata

EFSA Journal 2022;20(11):7655


Keywords: TSE, BSE, CWD, scrapie, classical, atypical, surveillance

On request from: European Commission Question Number: EFSA‐Q‐2021‐00765

Contact: zoonoses@efsa.europa.eu

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 2021 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland) (XI), and eight other non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Turkey. 

In total, 1,021,252 cattle were tested by EU27 and XI (−9%, compared with 2020 when data from the United Kingdom were not restricted to Northern Ireland), and 66,121 cattle by eight non‐EU reporting countries, 

with two cases of H‐BSE in France and Spain, and four L‐BSE in France (2), Germany and Spain. 

In total, 311,174 sheep and 118,457 goats were tested in the EU27 and XI (−6.4% and −1.8%, respectively, compared to 2020 when data from the whole United Kingdom were considered). 

In sheep, 551 cases of scrapie were reported by 17 MS and XI: 448 classical scrapie (CS) by six MS [80 index cases (IC) with genotypes of susceptible groups in 97% of the cases], 103 atypical scrapie (AS) (96 IC) by 13 MS and XI. 

In the other non‐EU reporting countries, 27,594 sheep were tested with 55 CS and 1 AS in Iceland and 8 AS in Norway. 

Ovine random genotyping was reported by nine MS and genotypes of susceptible groups accounted for 7.9%. 

In goats, 224 cases of scrapie were reported by six EU MS: 219 CS (30 IC) by six MS, and five AS (5 IC) by three MS. 

In total, 5,854 cervids were tested for chronic wasting disease by eight MS; all resulted negative. Norway tested 21,670 cervids with two moose and one red deer positive. In total, 149 animals from four other species tested negative in Finland and Turkey.

© European Food Safety Authority


see full text;


Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle 

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department 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 (CBSE) 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 PrPSc 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 PrPSc 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 PrPSc 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

Acknowledgement: TSE unit NCAD, Lethbridge (Jianmin Yang, Sarah Bogart, Rachana Muley, Yuanmu Fang, Keri Colwell, Renee Anderson, John Gray, Rakhi Katoch) (CFIA, Canada), Dr. Catherine Graham (NSDA, Canada), Dr. Michel Levy (UCVM, Canada), Dr. Martin Groschup (FLI, Germany), Dr. Christine Fast (FLI, Germany), Dr. Bob Hills (Health Canada, Canada) Theme: Animal prion diseases

"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 PrPSc in C-BSE. "

=====end


Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE). 

Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA 

Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME). 

Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97). 

Results: Approximately 16.6 months post-inoculation, Steer 6 (EK211 L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (EK211 L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME. 

Conclusions: Further study of L-BSE in EK211 cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE. 

Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE. 

Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases

=====end

PRION 2023 CONTINUED;


Professor John Collinge on tackling prion diseases, sCJD accounts for around 1 in 5000 deaths worldwide

MONDAY, SEPTEMBER 11, 2023 

Professor John Collinge on tackling prion diseases “The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.” There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.




Terry S. Singeltary Sr.


posted by Terry S. Singeltary Sr. at 10:21 AM 0 Comments