Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, CJD, TSE prion Update September 2026
***> Bovine Spongiform Encephalopathy BSE, Scrapie, CJD, TSE prion in South America 2026 Review
Risk management strategies for bovine spongiform encephalopathy in South America
September 2010
International Journal of Risk Assessment and Management 14(3/4):254 DOI: 10.1504/IJRAM.2010.035270 Margaret A. WilsonShalu DarshanShalu DarshanDaniel KrewskiMichael G. TyshenkoMichael G. Tyshenko
Abstract and figures
Bovine spongiform encephalopathy (BSE) has never been reported in South America. As BSE began to be detected in other parts of the world, Argentina and Brazil were able to gain a larger share of the global demand for beef, with these two countries currently controlling one-third of the world export beef market. In both countries, the practice of pasturing cattle rather than feeding MBM has served to minimise the opportunity for BSE cycling, and early bans on the import of UK cattle coupled with minimal meat and bone meal (MBM) imports resulted in a very low external challenge. Specified risk material (SRM) regulations vary between countries in South America. Argentina enacted a ban on SRM in the feed chain in 2002; Brazil implemented a similar ban in early 2007. South American countries as a region had a lower risk of BSE entry, but higher cattle system instability than European Union countries.
Risk management strategies for BSE in South America
Conclusions
The implementation of preventive strategies for BSE in South America coupled with theadvantage of having a wealth of large natural grass plains used extensively for cattle grazing has played a major role in shielding the region from the influx of the disease. Argentina and especially Brazil enjoyed a boom in its beef export market as a result of BSE, with Brazil experiencing a 39% increase in beef exports in 2004 (USDA, 2005).The two countries benefited immensely from their favourable OIE and EFSA risk assessments at the time when BSE was being reported in many other countries. Other South American countries have begun to follow suit: Uruguay now exports as much beef as does Argentina, roughly 500 MT (US Department of Agriculture, 2008), and Paraguay shipped 183 MT in 2005 (Foreign Agriculture Service, USDA, 2006e).
Prior to the year 2007 the most evident difference between Argentina and Brazil in relation to BSE management was their respective policies for dealing with SRMs. Since January 2007 Brazil has implemented SRM controls. Argentina has excluded SRM from the feed chain since 2002. Brazil and Argentina recognised that there was considerable production of MBM in both respective countries intended mainly for domestic pig and poultry production; however, accidental cross-contamination could provide a means for amplification of BSE in cattle if the BSE agent were to gain entry into the national herd. This was true for Brazil prior to 2007, which, in addition to rendering SRM, imported more cattle from BSE-risk countries than did other South American countries prior to the import bans (Table 1). It must be emphasised that the predominance of pasturing in the Brazilian cattle industry and the rarity of co-farming act to minimise the risk of recycling of the infectious agent, even if it were present in imported cattle. Abetted by its willingness to provide beef raised without artificial hormones, Brazil has maintained a large share of the European beef market.
Both Argentina and Brazil were advised by EFSA to improve surveillance byincluding more at-risk animals in the testing program, such as fallen stock and emergency slaughters (European Food Safety Authority, 2005a, 2005b). The increasing orientation of the Brazilian beef industry towards the export market, prompted by a growing market share and shrinking domestic demand, makes the Brazilian industry vulnerable. The detection of a single case of BSE in the national herd could lead to catastrophic economici mpacts by limiting the beef trade in a reenactment of the situation that occurred in Canada after the detection of that country’s first BSE case in May 2003.
In the past, Argentina and Brazil have faced challenges to their beef export markets from foot and mouth disease (FMD) outbreaks. FMD outbreaks in the fall of 2005 in Brazil closed the borders of 58 markets, including Russia, Egypt, and the EU countries, to products from affected states (Meat and Livestock Australia, 2007b). Recently, the European Union banned Brazilian beef temporarily on grounds of unsatisfactory Brazilian cattle health checks for FMD (International Herald Tribune, 2008). Likewise in2001, Argentina dealt with an FMD crisis that severely challenged the beef export industry, resulting in intermittent border closures (World Organisation for Animal Health,2007b). The re-appearance of the disease after a protracted and intensive eradication program has led to a call for a ‘supranational’ surveillance system, possibly heralding broader harmonisation between countries of the region in the area of animal health (Can et al., 2004). Being mindful of the various means by which the beef export industry can be impacted negatively, regulatory bodies in these countries need to invest appropriate resources to control these emerging and reemerging cattle diseases.
(PDF) Risk management strategies for bovine spongiform encephalopathy in South America. Available from:
Argentina TSE Prion
Argentina - Scrapie - Follow up report 1 GENERAL INFORMATION COUNTRY/TERRITORY OR ZONE
ZONE ANIMAL TYPE
TERRESTRIAL DISEASE CATEGORY
Listed disease EVENT ID
7429 DISEASE
Scrapie CAUSAL AGENT
Scrapie prion protein GENOTYPE / SEROTYPE / SUBTYPE
- START DATE
2025/02/20 REASON FOR NOTIFICATION
First occurrence in the country/territory DATE OF LAST OCCURRENCE
- CONFIRMATION DATE
2026/04/07 EVENT STATUS
On-going END DATE
- SELF-DECLARATION
NO REPORT INFORMATION REPORT NUMBER
Follow-up report 1 REPORT ID
FUR_183036 REPORT REFERENCE
- REPORT DATE
2026/05/12 REPORT STATUS
Validated NO EVOLUTION REPORT
WHO WAHIS WOAH Argentina - Scrapie - Immediate notification
CONFIRMATION DATE
2026/04/07 EVENT STATUS
First detection of classical scrapie in sheep imported from Paraguay in Santa Fe and Entre Ríos provinces, Argentina
MON 13 APR 2026
Source Ministry of Economy. Agriculture, Livestock, and Fisheries. National Food Safety and Quality Service (SENASA). Press release: For the first time, outbreaks of classical scrapie were detected in imported sheep. 11 Apr 2026 (in Spanish)
Key findings
On 11 Apr 2026, Argentina's National Service for Food Safety and Quality (SENASA) confirmed the country's first-ever detection of classical scrapie in three imported breeding sheep (Dorper and Santa Inés breeds) that died naturally without showing symptoms at establishments in the provinces of Santa Fe and Entre Ríos.
The affected sheep were imported from Paraguay in 2021 and 2022, underwent annual clinical inspections by SENASA veterinarians without any health issues, and were detected through active surveillance screening using ELISA testing, with confirmation by the Western Blot technique at a reference laboratory in Spain.
SENASA implemented official control and monitoring measures, including movement restrictions on affected production units, intensified surveillance, and sanitary containment protocols to prevent the spread of disease.
The detection was officially reported to the World Organisation for Animal Health (WOAH) and to Paraguay's National Service for Quality and Animal Health (SENACSA), and both countries agreed to deepen the epidemiological investigation.
Argentina is adapting its export certification to WOAH recommendations to maintain access to international markets, with safe products including embryos, hides, skins, gelatin, collagen, tallow, wool, and sheep meat with risk tissues removed. It is also proposing specific mitigation measures for live animals, protein meals, and dairy products.
Epidemiological analysis and public health impact
This detection marks a fundamental shift in Argentina's animal health status, moving the country from scrapie-free to disease-present and requiring the establishment of new surveillance infrastructure and baseline monitoring systems. Identification through active surveillance screening, rather than clinical presentation, demonstrates the effectiveness of Argentina's post-import monitoring protocols, yet also reveals that subclinical infections can persist undetected for years despite annual veterinary inspections.
The vertical transmission pattern at birth poses a significant risk of within-flock amplification, particularly because the affected animals were breeding stock imported 4–5 years ago, which could have allowed multiple lambing cycles before detection. The absence of clinical signs until natural death suggests that other subclinical carriers may exist in the affected flocks and possibly in offspring, necessitating comprehensive herd testing and assessment of genetic susceptibility.
The geographic distribution across two provinces (Santa Fe and Entre Ríos) and the common Paraguayan origin of the imports indicate a point-source introduction with the potential for wider dissemination through breeding-stock sales or animal movements before detection. The restriction measures now in place will be critical to preventing establishment in Argentina's broader sheep population, which could have substantial economic implications for the country's wool and meat export industries.
snip…
Outbreaks of classical scrapie were detected in imported sheep for the first time.
SENASA reported the finding in breeding specimens belonging to establishments in the provinces of Santa Fe and Entre Ríos.
April 11, 2026
The National Service for Agrifood Health and Quality (SENASA) confirmed the positive result for classical scrapie in imported breeding sheep , after the natural death without associated symptoms of three animals was recorded in establishments in Santa Fe and Entre Ríos.
The sheep involved had been imported in 2021 and 2022 from Paraguay, in accordance with the sanitary requirements and the current International Veterinary Certificate model, passing the required post-entry controls.
FINAL REPORT OF AN AUDIT CARRIED OUT IN ARGENTINA FROM 21 NOVEMBER 2023 TO 1 DECEMBER 2023 IN ORDER TO EVALUATE THE IMPLEMENTATION OF OFFICIAL CONTROLS ON ANIMAL BY- PRODUCTS (ABP) AND DERIVED PRODUCTS (DP)
Executive Summary
This report describes the outcome of an audit in Argentina carried out from 21 November to 1 December 2023. The audit was part of the European Commission’s Directorate-General for Health and Food Safety’s published work programme.
The objective of the audit was to assess whether the official control system over the production of rendered fat (tallow) not intended for human consumption and destined for export to the European Union (EU), as well as related certification arrangements, are sufficient to allow for reliable attestation by the responsible competent authority that the EU requirements specified in the model EU health certificate in Chapter 10(B) of Annex XV to Commission Regulation (EU) No 142/2011 are complied with. This was the first time that the Commission services have carried out an audit on the subject of animal by-products (ABP) and derived products in Argentina. The report concludes that the official control system governing the production and certification of ABP for the EU market is not fit for its intended purpose. It results in the Argentinian competent authority not being in a position to reliably verify and certify compliance of tallow with the vast majority of requirements relevant for its production and export to the EU including identifying the category of the ABP used for its manufacture, the processing method used and the marking of the product with glycerol triheptanoate.
Due to the severity and scale of the non-compliances identified during the audit, the competent authority indicated its intention to revoke the EU export authorisations of all of the tallow processing plants and delist them from TRACES-NT, pending the implementation of corrective actions.
The report contains two recommendations to the Argentinian competent authority to address the identified gaps.
snip…
Conclusions on official certification
Given the gaps in the official controls performed and the absence of certifying officers’ direct knowledge of, or evidence for the category of the raw material used for the production of tallow, the processing method used, and the use of GTH and its content, the competent authority’s attestations of compliance with most of requirements referred to in the EU health certificate are unreliable.
6 OVERALL CONCLUSION
The official control system governing the production and certification of ABP for the EU market is not fit for its intended purpose. It results in the Argentinian competent authority not being in a position to reliably verify and certify compliance of tallow with the vast majority of requirements relevant for its production and export to the EU including identifying the category of the ABP used for its manufacture, the processing method used and the marking of the product with glycerol triheptanoate.
***> Brazil TSE Prion
Brazil 6 cases atypical BSE to date
Date: September 09, 2025 Report Number: BR2025-0024 Report Name: Livestock and Products Annual Country: Brazil Post: Brasilia Report Category: Livestock and Products Prepared By: Camila Aquino Approved By: Joseph Degreenia Report Highlights: Brazil is the second largest beef producing country and the largest beef exporting country in the world.
snip…
Animal Disease On September 9, 2024, MAPA published an Ordinance on norms of prevention and monitoring for bovine spongiform encephalopathy (BSE). Ordinance SDA/MAPA 1180/2024. To date, Brazil has only had six cases of BSE – all atypical. The country is considered as having insignificant risk for the disease per the World Organization for Animal Health (WOAH).
DIÁRIO OFICIAL DA UNIÃO Publicado em: 11/09/2024 | Edição: 176 | Seção: 1 | Página: 6 Órgão: Ministério da Agricultura e Pecuária/Secretaria de Defesa Agropecuária PORTARIA SDA/MAPA Nº 1.180, DE 9 DE SETEMBRO DE 2024 Estabelece as diretrizes do Programa Nacional de Encefalopatia Espongiforme Bovina para a aplicação de medidas oficiais de prevenção e vigilância.
BRAZIL BSE START DATE 2023/01/18
BRAZIL BSE CONFIRMATION DATE 2023/02/22
BRAZIL BSE END DATE 2023/03/03
Brazil TSE Prion
FRIDAY, MARCH 3, 2023
Brazil MAPA OMSA Confirms BSE TSE Prion atypical H-type
https://animalhealthreportpriontse.blogspot.com/2023/03/brazil-mapa-omsa-confirms-bse-tse-prion.html
THURSDAY, FEBRUARY 23, 2023
Brazil Confirms BSE Mad Cow Case, Strain not known yet
https://animalhealthreportpriontse.blogspot.com/2023/02/brazil-confirms-bse-mad-cow-case-strain.html
TUESDAY, FEBRUARY 21, 2023
Brazil Suspected case of Bovine Spongiform Encephalopathy
''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.
FRIDAY, MARCH 17, 2023
***> Are Brazilian cervids at risk of prion diseases?
THURSDAY, NOVEMBER 11, 2021
Brazil investigating two possible cases of mad cow disease in humans
SATURDAY, NOVEMBER 13, 2021
Brazil Creutzfeldt Jakob Disease CJD TSE Prion Update 2021
TUESDAY, SEPTEMBER 07, 2021
Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom
SATURDAY, SEPTEMBER 4, 2021
Brazil Confirms TWO More Cases of Mad Cow Disease BSE States of Mato Grosso and Minas Gerais
SATURDAY, JUNE 01, 2019
Brazil reports another cases of mad cow disease atypical BSE TSE Prion
TUESDAY, MARCH 26, 2019
Joint Statement from President Donald J. Trump USA and President Jair Bolsonaro Brazil FOREIGN POLICY BSE TSE Prion aka mad cow disease
FRIDAY, NOVEMBER 03, 2017
First case of V180I rare mutation in a Brazilian patient with Creutzfeldt-Jakob disease
WEDNESDAY, DECEMBER 14, 2016
English lab confirms that mad cow disease in PR Brazil was atypical 4 years ago
TUESDAY, SEPTEMBER 27, 2016
Classical Scrapie Diagnosis in ARR/ARR Sheep in Brazil
Acta Scientiae Veterinariae, 2015. 43(Suppl 1): 69.
MONDAY, AUGUST 1, 2016
USDA Announces Reopening of Brazilian Market to U.S. Beef Exports and the Potential for Transmissible Spongiform Encephalopathy TSE prion disease
Cattle in Cacoal are slaughtered after eating prohibited food Cities
Friday, December 18, 2015 - 10:21
Chicken litter
Cattle in Cacoal are slaughtered after eating prohibited food
The Agrosilvopastoral Health Defense Agency of the State of Rondônia (Idaron) in Cacoal received a complaint from a teacher at the Agricultural Family School (EFA) that three cattle from a neighboring property accidentally entered the institution and ate "chicken litter," a prohibited food under federal law. The ban is one of the main preventive measures against bovine spongiform encephalopathy (BSE), also known as mad cow disease. The animals were slaughtered last week.
Idaron inspectors collected material ingested by cattle for laboratory testing So-called "chicken litter" is the result of grinding pine sawdust (used to line the floors of farm aviaries) and poultry feces.
Normative Instruction 41/2009 of the Ministry of Agriculture, Livestock, and Supply (MAPA) prohibits feeding ruminants products containing animal proteins and fats. The Normative Instruction also provides for fines for producers who feed their herds this type of feed.
Following the complaint, Idaron's technical team collected a sample of the material consumed by the cattle and confirmed, through examination in an official MAPA laboratory, that it was "chicken litter." The animals were identified and remained on their original rural property, and their movement was prohibited.
Because it was an accident, the owner of the animals, who also notified Idaron of the incident, was not fined; he was only notified to send the cattle for slaughter. Ney Carlos Dias de Azevedo, coordinator of the EEB State Prevention and Surveillance Program, explained that mad cow disease can be transmitted to humans through the ingestion of meat from infected animals. "There is no risk of contamination through contact with the animal," he added.
The coordinator also explained that since 2012, 404 cattle have been slaughtered in Rondônia as a safety measure for having consumed feed containing animal protein, and that in 2014, 132 inspections were carried out on farms that provide supplemental feed for cattle. "Idaron is on alert. The goal is to prevent the use of prohibited foods in ruminant feed, such as meat and bone meal and chicken litter."
The disease has already caused enormous losses to cattle ranching in Europe, in addition to causing deaths from ingesting contaminated meat. In Brazil, there have been only two recorded cases of mad cow disease, which are considered an atypical form of the disease (which can occur naturally in older animals). There is no record of ingestion of prohibited food.
Friday, December 18, 2015 - 10:21
Poultry litter
Cacoal cattle are slaughtered after eating forbidden food
The Health Protection Agency Agrossilvopastoril the State of Rondônia (Idaron) Cacoal received a complaint from a professor at the Family Farm School (EFA) that three cattle of a neighboring property entered accidentally in the institution and were fed "poultry litter", food prohibited, according to federal law. The ban is one of the main measures to prevent bovine spongiform encephalopathy (BSE), known as mad cow disease. The animals were slaughtered last week.
snip...see full text;
Friday, October 30, 2015
Brazil Agriculture minister to visit Saudi Arabia over BSE mad cow trade
MONDAY, MAY 5, 2014
Brazil BSE Mad Cow disease confirmed OIE 02/05/2014
Monday, May 5, 2014
Brazil 2nd BSE Mad Cow disease confirmed OIE 02/05/2014
Thursday, April 24, 2014
Brazil investigates possible BSE mad cow case
WEDNESDAY, JANUARY 29, 2014
Another Suspect case of Creutzfeldt-Jakob disease investigated in Brazil
THURSDAY, SEPTEMBER 26, 2013
Brazil evaluate the implementation of health rules on animal by-products and derived products SRM BSE TSE PRION aka MAD COW DISEASE
Wednesday, December 19, 2012
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil
***> Friday, December 07, 2012
***> ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012
Updates on the WOAH activities in the field of TSEs
Natalie MOYEN Disease Status Officer
Status Department
May 14 th , 2024
Outline 1. Revised BSE standards (Terrestrial Code)
a) b) Transition process: where are we?
Official recognition of BSE risk status
i. Members/zones recognised as having a negligible or controlled BSE risk status
ii. New applications
iii. Annual reconfirmations
2. Revision of Scrapie standards (Terrestrial Code
Canada Trade Policy Monitoring Canada Bans Brazilian Beef Products 2001
Canada Livestock and Products Scientific Team to Assess BSE Status in Brazil 2001
***> A critical perspective of Prion Disease surveillance in Brazil <***
2025 PRION 2025
Proceedings of the Prion 2025 - Advancing the understanding and treatment of prion diseases ISBN: 978-65-80968-48-0
A critical perspective of Prion Disease surveillance in Brazil
BARBOSA, BRENO JOSE ALENCAR PIRES 1MONTENEGRO, MARIA LUIZA VASCONCELOS 2CUNHA, JOSE ERITON GOMES DA 3 Vol 1, 2025 - 326431 Abstract Prion 2025
Abstract
Introduction: Prion Diseases (PrD) are a group of rapidly progressive dementias. Among its subtypes, Creutzfeldt-Jakob Disease (CJD) is the most common, affecting around 1 to 2 individuals per million inhabitants yearly. In 2005, Brazil's Ministry of Health (MH) initiated a surveillance program for CJD, creating a protocol to report the cases. Despite advances, the MH still struggles to make a reliable database to determine PrD profile in Brazil.
Objectives: To understand the Brazilian PrD surveillance system.
Methods: This is a retrospective and descriptive study based on the epidemiological records of CJD surveillance from 2005 to 2021 in the Ministry of Health's Epidemiological Bulletin published in 2022.
Results/Discussion: 1.576 suspected cases of CJD were reported, concentrated in the Southeast, South and Northeast regions of Brazil. Among the notifications, the following age groups predominated: 55 to 74 years (60.2%), 45 to 54 years (15%), and 75 to 85 years (11.8%). Suspected cases were mainly represented by women (53.6%), white individuals (61.4%), and residents of urban areas (90%). In this period, the expected number of cases for the Brazilian population would be 3.200.
Additionally to underreporting, the data shared by MH are limited due to the use of a database destined to mainly observe epidemic outbreaks and mismatches between official documents. Conclusion: Despite some progress since 2005, PrD surveillance in Brazil faces significant problems, due to the inaccurate treatment of these data and the lack of a specific database for CJD.
SATURDAY, AUGUST 22, 2026
***> Brazil Creutzfeldt Jakob Disease CJD 547 confirmed cases in 16 years
***> EU TSE Prion
***> United Kingdom BSE
United Kingdom - Bovine spongiform encephalopathy - Immediate notification GENERAL INFORMATION COUNTRY/TERRITORY OR ZONE
2026/08/02 REASON FOR NOTIFICATION
Thursday, August 20, 2026
United Kingdom - Bovine spongiform encephalopathy - Immediate notification GENERAL INFORMATION COUNTRY/TERRITORY OR ZONE
WEDNESDAY, AUGUST 19, 2026
Bovine Spongiform Encephalopathy (BSE) has been confirmed in a cow on a farm in Dumfries and Galloway
FRIDAY, APRIL 17, 2026
Ireland Central Veterinary Research Laboratory confirmed a case of atypical BSE on April 9, 2026
FRIDAY, MARCH 27, 2026
Transmissible spongiform encephalopathies (TSE) Last reviewed date: 5 January 2026
FRIDAY, NOVEMBER 14, 2025
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2024
Published: 13 November 2025 Approved: 15 October 2025 EFSA Journal
SATURDAY, JUNE 14, 2025
1st meeting of the TSE subgroup of the Animal Health Network event date: 25 November 2025
THURSDAY, JUNE 5, 2025
World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?
FRIDAY, DECEMBER 06, 2024
Scotland Single Case of Atypical BSE disease confirmed in Dumfries and Galloway
Friday, October 4, 2024
atypical bovine spongiform encephalopathy (BSE) in Ireland
The UK's National CJD Research & Surveillance Unit (NCJDRSU) ceased to function on March 31, 2025, due to a decision by the National Institute for Health and Care Research (NIHR) not to renew its funding.
Active TSE surveillance in Great Britain and Northern Ireland 2025
Active TSE surveillance in Great Britain 2025
Active TSE Surveillance in Northern Ireland
Animal feed company convicted at Ballymena court
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.
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.
Scotland Single case of disease confirmed in Dumfries and Galloway
Published 06 December 2024 12:45
Topic Farming and rural
Single case of disease confirmed in Dumfries and Galloway.
A case of atypical Bovine Spongiform Encephalopathy (BSE) has been confirmed in a cow on a farm in Dumfries and Galloway.
Precautionary movement restrictions have been put in place at impacted premises and cover animals which have been in contact with the case. Further investigations to identify the origin of the disease are ongoing. This is standard procedure for a confirmed case of atypical BSE.
The case was identified as a result of our routine yet intensive BSE surveillance and stringent control measures are in place. Atypical BSE is not known to be a risk to public health and the animal did not enter the human food chain. Food Standards Scotland have confirmed there is no risk to human health as a result of this isolated case.
The owners of the affected animals are working with authorities on next steps.
Agriculture Minister Jim Fairlie said:
“Following confirmation of a case of atypical BSE in Dumfries and Galloway, the Scottish Government and other agencies took swift and robust action to protect the agriculture sector.
“The fact we identified this isolated case so quickly is proof that our surveillance system for detecting this type of disease is working effectively.
“I want to thank the animal’s owner for their diligence. Their decisive action has allowed us to identify and isolate the case at speed which has minimised its impact on the wider industry."
Chief Veterinary Officer Sheila Voas said:
“The fast detection of this case is proof that our surveillance system is doing its job.
“We are working closely with the Animal and Plant Health Agency, and other partners to identify where the disease came from.
“I want to reassure both farmers and the public that this is an isolated case and of the aytypical strain of BSE which is not transmissible and not connected to contaminated feed. But, if any farmers are concerned, I would urge them to seek veterinary advice."
Ian McWatt, Deputy Chief Executive of Food Standards Scotland said:
“There are strict controls in place to protect consumers from the risk of BSE and consumers can be reassured that these important protection measures remain in place and that Food Standards Scotland Official Veterinarians and Meat Hygiene Inspectors working in all abattoirs in Scotland will continue to ensure that in respect of BSE controls, the safety of consumers remains a priority.
“We will continue to work closely with Scottish Government, other agencies and industry at this time.”
Background
Bovine spongiform encephalopathy (BSE): how to spot and report the disease - gov.scot
The Animal Plant and Health Agency (APHA) is investigating the source of the disease.
All animals over four years of age that die on farm are routinely tested for BSE under our comprehensive surveillance system. Whilst the disease is not directly transmitted from animal to animal, its cohorts, including offspring, have been traced and isolated, and will be destroyed in line with our legal requirements.
In addition to the measures we have in place for fallen stock and animal feed, there is a strict control regime to protect consumers. This includes the removal of specified risk material such as the spinal column, brain and skull from carcasses destined for human consumption.
News BSE Published 10 May 2024 10:30 Topic Farming and rural Disease confirmed in Ayrshire.
A case of classical Bovine Spongiform Encephalopathy (BSE) has been confirmed on a farm in Ayrshire.
Precautionary movement restrictions have been put in place at impacted premises and cover animals which have been in contact with the case. Further investigations to identify the origin of the disease are ongoing. This is standard procedure for a confirmed case of classical BSE.
The case was identified as a result of routine surveillance and stringent control measures. The animal did not enter the human food chain. Food Standards Scotland have confirmed there is no risk to human health as a result of this isolated case.
The owners of the affected animals are working with authorities on next steps.
Read more: BSE: how to spot and report the disease. Agriculture Minister Jim Fairlie said:
“Following confirmation of a case of classical BSE in Ayrshire, the Scottish Government and other agencies took swift and robust action to protect the agriculture sector. This included establishing a precautionary movement ban on the farm.
“The fact we identified this isolated case so quickly is proof that our surveillance system for detecting this type of disease is working effectively.
“I want to thank the animal’s owner for their diligence. Their decisive action has allowed us to identify and isolate the case at speed which has minimised its impact on the wider industry."
Chief Veterinary Officer Sheila Voas said:
“The fast detection of this case is proof that our surveillance system is doing its job.
“We are working closely with the Animal and Plant Health Agency, and other partners to identify where the disease came from.
“I want to reassure both farmers and the public that the risk associated with this isolated case is minimal. But, if any farmers are concerned, I would urge them to seek veterinary advice."
Ian McWatt, Deputy Chief Executive of Food Standards Scotland said:
“There are strict controls in place to protect consumers from the risk of BSE, including controls on animal feed, and removal of the parts of cattle most likely to carry BSE infectivity.
“Consumers can be reassured that these important protection measures remain in place and that Food Standards Scotland Official Veterinarians and Meat Hygiene Inspectors working in all abattoirs in Scotland will continue to ensure that in respect of BSE controls, the safety of consumers remains a priority.
“We will continue to work closely with Scottish Government, other agencies and industry at this time.”
Background
The Animal Plant and Health Agency (APHA) is investigating the source of the outbreak.
All animals over four years of age that die on farm are routinely tested for BSE under our comprehensive surveillance system. Whilst the disease is not directly transmitted from animal to animal, its cohorts, including offspring, have been traced and isolated, and will be destroyed in line with our legal requirements.
In addition to the measures we have in place for fallen stock and animal feed, there is a strict control regime to protect consumers. This includes the removal of specified risk material such as the spinal column, brain and skull from carcasses destined for human consumption.
Movement restrictions have also been put in place at three further farms – the farm of the animal’s origin and two more holdings where animals that have had access to the same feed are.
Friday, October 4, 2024
another atypical bovine spongiform encephalopathy (BSE) in Ireland
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2024
Published: 13 November 2025 Approved: 15 October 2025 EFSA Journal
KEYWORDS atypical, BSE, classical, CWD, scrapie, surveillance, TSE
CONTACT biohaw@efsa.europa.eu
Abstract
This report presents results of surveillance on transmissible spongiform encephalopathies in bovines, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2024 by 27 EU Member States (EU27, MS), the UK (in respect of Northern Ireland, (XI)) and 8 non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye.
In total, 980,624 bovines were tested by EU27 and XI (+3.4% compared to 2023), with 3 atypical bovine spongiform encephalopathy (BSE) cases reported (2 H‐type: 1 in France, 1 in Ireland; 1 L‐type in Poland); and 41,397 bovines by 8 non‐EU reporting countries with no BSE cases reported.
Four additional BSE cases were reported by the UK (1 classical and 1 H‐type), the USA (1 L‐type), Brazil (1 H‐type).
In total, 277,064 sheep were tested in EU27 and XI (−2.7% compared to 2023).
In sheep, 458 scrapie cases were reported by 14 MS and XI: 380 classical scrapie (CS) by 6 MS (139 index cases (IC)) with genotypes of susceptible groups in 98.9% of the cases; 78 atypical scrapie (AS) (78 IC) by 13 MS.
In non‐EU reporting countries 25,337 sheep were tested, with Iceland reporting 61 CS and 2 AS cases, and Norway 9 AS cases.
Random genotyping was reported by five MS and susceptible genotypes accounted for 7.8%. In goats, out of 93,960 tested (−8.5% compared to 2023), 71 cases of scrapie were reported, all from EU27 and XI: 65 CS (14 IC) by five MS and 6 AS (6 IC) by three MS. None of the genotyped cases in goats carried polymorphisms at codon 146 and 222.
In total, 1761 cervids were tested for chronic wasting disease by 9 MS, none tested positive.
Norway tested 10,932 cervids with 2 European moose positive, Serbia tested 186 animals and Iceland 96 animals.
© 2025 European Food Safety Authority
Report on the epidemiological investigation of a BSE case in Scotland (RBSE24_00003) United Kingdom October 2024
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2023
Published: 28 November 2024
Adopted: 29 October 2024
KEYWORDS atypical, BSE, classical, CWD, scrapie, surveillance, TSE
CONTACT biohaw@efsa.europa.eu
Abstract
This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye.
In total, 948,165 cattle were tested by EU27 and XI (−3%, compared with 2022),
with five atypical BSE cases reported (four H‐type: two in Spain, one in France and one in Ireland; one L‐type in the Netherlands);
and 46,096 cattle by eight non‐EU reporting countries with two atypical BSE cases reported by Switzerland.
Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1).
In total, 284,686 sheep and 102,646 goats were tested in the EU27 and XI (−3.5% and −5.9%, respectively, compared to 2022).
In the other non‐EU reporting countries 26,047 sheep and 589 goats were tested.
In sheep, 538 cases of scrapie were reported by 14 MS and XI:
462 classical scrapie (CS) by 4 MS (104 index cases (IC) with genotypes of susceptible groups in 93.4% of the cases),
76 atypical scrapie (AS) (76 IC) by 12 MS.
In the other non‐EU reporting countries,
Iceland reported 70 cases of CS
while Norway reported 7 cases of ovine AS.
Ovine random genotyping was reported by six MS and genotypes of susceptible groups accounted for 6.9%.
In goats, 183 cases of scrapie were reported, all from EU MS:
176 CS (47 IC) by seven MS and 7 AS (7 IC) by five MS.
Three cases in Cyprus and one in Spain were reported in goats carrying heterozygous alleles at codon 146 and 222, respectively.
In total, 2096 cervids were tested for chronic wasting disease by ten MS, none tested positive.
Norway tested 14,224 cervids with one European moose positive.
© European Food Safety Authority
https://www.efsa.europa.eu/en/efsajournal/pub/9097
See full report;
MONDAY, MAY 13, 2024
UK Parliament UIN 24699, tabled on 2 May 2024 BSE Disease Control Singeltary Submission
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022
European Food Safety Authority (EFSA)
First published: 28 November 2023
Approved: 19 October 2023
Abstract
This report presents the results of surveillance on transmissible spongiform encephalopathies (TSE) in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2022 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland [XI]) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye.
In total, 977,008 cattle were tested by EU27 and XI (−4.3%, compared with 2021), and 52,395 cattle by eight non-EU reporting countries, with one case of H-BSE in France.
In total, 295,145 sheep and 109,074 goats were tested in the EU27 and XI (−5.2% and −7.9%, respectively, compared to 2021).
In the other non-EU reporting countries, 25,535 sheep and 633 goats were tested.
In sheep, 557 cases of scrapie were reported by 17 MS and XI: 480 classical scrapie (CS) by five MS (93 index cases [IC] with genotypes of susceptible groups in 97.6% of the cases), 77 atypical scrapie (AS) (76 IC) by 14 MS and XI. In the other non-EU reporting countries, Norway reported 16 cases of ovine AS.
Ovine random genotyping was reported by eight MS and genotypes of susceptible groups accounted for 7.3%.
In goats, 224 cases of scrapie were reported, all from EU MS: 216 CS (42 IC) by six MS, and 8 AS (8 IC) by four MS. In Cyprus, two cases of CS were reported in goats carrying the heterozygous DN146 allele.
In total, 3202 cervids were tested for chronic wasting disease by 10 MS.
One wild European moose tested positive in Finland.
Norway tested 17,583 cervids with two European moose, one reindeer and one red deer positive.
In total, 154 animals from four other species tested negative in Finland.
TUESDAY, NOVEMBER 28, 2023
EFSA TSE Report 2022 First published 28 November 2023 The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022
TUESDAY, NOVEMBER 28, 2023
EFSA TSE Report 2022 First published 28 November 2023
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022
European Food Safety Authority (EFSA)
First published: 28 November 2023
THURSDAY, NOVEMBER 9, 2023
EFSA Annual Report of the Scientific Network on BSE-TSE 2023
THURSDAY, NOVEMBER 10, 2022
Annual Report of the Scientific Network on BSE‐TSE 2022 Annual Report of the Scientific Network on BSE‐TSE 2022
Published: 10 November 2022
Approved: 27 October 2022
EFSA Journal 2022;19(11):EN-7656
On request from: EFSA Question Number: EFSA‐Q‐2021‐00660
Contact: biohaw@efsa.europa.eu
Abstract
snip...
2.3.3 EU TSE annual report 2020: preliminary results
Giuseppe Ru, Director of the EURL on TSE, presented the main findings that will be included in the EU annual report on TSE for 2021.
The report presents the results of surveillance of TSE in different animal species and is expected to be published by the end of November 2022 by EFSA.
It includes data from 27 Member States (MS), 8 non-European Reporting Countries (Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Turkey), and the United Kingdom (as non-MS from 1 February 2020).
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
snip...
2.3.4Update on the activities of WOAH in the TSE field
Aurelio Cabezas, Disease Status Officer of the Status Department of the WOAH, updated the Network on the TSE-related activities ongoing at WOAH since the previous Network meeting.
During 2021 there have been many activities in WOAH:
a) the official recognition of BSE risk status: France recognized as having a negligible BSE risk and Russia as having a controlled BSE risk. 53 members and 3 zones have negligible risk, 4 countries and 2 zones have controlled risk;
b) and revision of the BSE standards: the work continued with the latest revision by the Code Commission in September 2022 and possible submission of the new chapters in the Terrestrial Animal Health Code to the General Assembly on Delegates in May 2023 for approval;
c) development of BSE surveillance guidelines: under peer review at the moment; d) impact of revised BSE standards on member or zones currently having a BSE risk status: this work has finalized with the conclusion that the revised BSE standards won’t have any Annual report of the Scientific Network on BSE-TSE 2022 impact on the maintenance of status of the Members and zones currently having a BSE risk status.
An assessment of atypical BSE against the listing criteria in the Terrestrial Animal Health Code was conducted and the results can be consulted in the reports of the September 2022 meetings of the Scientific and Code Commissions soon to be available at the WOAH website.
Finally, following a meeting in September 2021, WOAH decided not to progress on the delisting of scrapie.
The Code Commission will start the revision of the Chapter 14.8 of the Terrestrial Animal Health Code soon.
Research and analysis Annual report to Parliament on the monitoring programme for transmissible spongiform encephalopathies (TSEs) – 2024 and 2025 Published 2 June 2026
https://efsaopinionbseanimalprotein.blogspot.com/2026/06/research-and-analysis-annual-report-to.html
MONDAY, MAY 13, 2024
UK Parliament UIN 24699, tabled on 2 May 2024 BSE Disease Control Singeltary Submission
WEDNESDAY, NOVEMBER 08, 2023
Ireland Atypical BSE confirmed November 3 2023
SUNDAY, JULY 16, 2023
Switzerland Atypical BSE detected in a cow in the canton of St. Gallen
WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type
Switzerland Bovine Spongiform Encephalopathy Atypical L-Type
Switzerland - Bovine spongiform encephalopathy - Immediate notification
Monday, March 20, 2023
WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type
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
THURSDAY, NOVEMBER 10, 2022
Annual Report of the Scientific Network on BSE‐TSE 2022
Published: 10 November 2022
https://efsaopinionbseanimalprotein.blogspot.com/2022/11/annual-report-of-scientific-network-on.html
UK BSE ARCHIVES
UK TSE STATISTICS
***> Russia BSE TSE PrP <***
FRIDAY, AUGUST 9, 2013
Evaluation of BSE status of the cattle population in Russia
AD.26: Evaluation of BSE status of the cattle population in Russia
Aleksander A. Yegorov, Sergey S. Rybakov, Asya V. Borisova and Andrey V. Pavlov
FGBI "Federal Centre for Animal Health"; Vladimir, Russia
Food safety and animal health problems associated with the international trade in animal products and farm animals are currently becoming more significant than several decades before. Evaluation of BSE status of the cattle population in accordance with the OIE recommendations after Russia's accession to the WTO in 2012 is one of the most important conditions potentially facilitating investments into animal production development and trade barrier elimination.
Cattle population in Russia has an undetermined BSE risk sta- tus currently and this fact is undermining the international trade in animal products and bioloigicals destined for veterinary and human medicine. One of the reasons for the situation that has developed is that after BSE epidemic Russia had to import several millions of tons of feeds and about one million of cattle from BSE affected countries. It will take several years for the country to be awarded with the BSE controlled risk status because the import of the abovementioned goods is a significant potential risk factor for the Russian cattle population. Taking into account the importance of this factor BSE anti-epidemic measures in Russia were predominantly aimed at the import prevention in regard to feeds contaminated with BSE agent and breeding animals from countries and regions where cattle subsequently affected by BSE had been born over the past 7 y_
Ban on feeding cattle, sheep goats, fur and zoo animals with meat and bone meal was imposed in Russia in August, 1990. BSE monitoring program including laboratory testing of cattle brain samples started in October, 1999. Test of imported feeds for contamination with ruminant tissue started in January, 2001.
BSE anti-epidemic measures in Russia were performed in accordance with the OIE recommendations based on the results of scientific research into BSE agent properties and the disease pathogenesis and epidemiology. Besides a great experience of several EU Veterinary Services in BSE control gained over the past two decades was taken into account for this purpose.
The most vital tasks at the moment are:
(1) Increase in number of tested brain samples from cattle at risk up to the level recommended by the OIE;
(2) Documentation of data on BSE risk factor control by veterinary services necessary for the evaluation of BSE status of the Russian cattle population performed by the OIE Scientific Commission for Animal Diseases.
These and other issues to be solved by Russia in order to improve BSE status of the cattle population will be detailed in the poster.
Tuesday, July 5, 2011
Risk Assessment of BSE Introduction in the Russian Federation in Connection with Importation of Cattle from the European Union in 2005–2010
Risk.40: Risk Assessment of BSE Introduction in the Russian Federation in Connection with Importation of Cattle from the European Union in 2005–2010
Sergey Rybakov† and Alexander Yegorov
FGI Federal Centre for Animal Health; Vladimir, Russia†Presenting author; Email: s.s.rybakov@mail.ru
The study is aimed at quantitative assessment of risk of BSE introduction from the EU countries into Russia with breeding animals imported during 2005–2010.
Within 2005–2010 importation of cattle born in 2003–2008 from the EU into Russia totally amounted to 363,000 animals. According to the data published in the EU reports, during 2003–2008 the BSE prevalence in the EU countries reduced from 125 cases per million bovine animals above 24 months of age in 2003 to 12 cases in 2008. If the imported subpopulation had proportionally represented all age-groups of cattle from EU25 countries, according to the calculation 19.4 animals in BSE incubation period should have been imported from 2005 until 2010.
The main suppliers of breeding cattle into Russia are the following EU countries: Austria, Denmark, France, Finland, Germany, Hungary and The Netherlands. Since 2007 import from these countries has amounted to 67.5% of the total import. The major suppliers—not the EU member states—are Canada (12.2%), Australia (12.1%) and the US (5.8%). As for the UK, Poland and Portugal, i.e. countries having the highest BSE incidence, currently export of live cattle from there is banned. Calculations demonstrated that limitation of live cattle importation from the countries with high BSE incidence allows to reduce the risk of BSE introduction into Russia in 14.5 times.
Risk assessment in relation to BSE in animals born after 2003 was performed by EFSA in 2009. The EFSA results demonstrated that estimated number of BSE cases in EU17 countries amounted totally to 32 cases within 2003–2008 and the highest 95% confidence limit constituted 65 cases. As for seven above mentioned live cattle importing countries the estimated number of BSE cases within 2003-2008 averaged to 1.8 and upper 95% confidence limit was 3.6.
Given that share of cattle annually exported from these seven countries into Russia averages to 0.293%, according to calculations, within 2005-2010 the mean probability of importation of an animal in which BSE can be detected amounts to 0.0064 and the highest probability amounts to 0.0129.
Results of the risk assessment of BSE introduction with cattle imported into Russia from the above mentioned seven countries within 2005-2010 demonstrate that the risk of BSE introduction amounts to 0.01 case in six years and implementation of measures recommended in the OIE Code is sufficient for BSE risk reduction. More detailed information will be provided in the poster.
Greetings,
IN my opinion, from the following risk factors i will post below, and the fact that the OIE and the USDA systematically did away with the BSE GBR system for the BSE MRR system, for the legal trading all strains of TSE globally, and the ramifications there from (BSE MRR), MY confidence level of any TSE regulatory risk assessment is 0...that is ZERO CONFIDENCE LEVEL IN ANY REGARDS TO THE TSE PRION DISEASES AKA MAD COW DISEASE. The BSE MRR regulations were set up to fail, and make legal the trading of all strains of TSE prion disease globally. the consumers were hung out to dry around the globe, and the ramifications there from will be long and costly thanks to the OIE and the USDA et al. ...TSS
SNIP...SEE FULL TEXT ;
Tuesday, July 5, 2011
Risk Assessment of BSE Introduction in the Russian Federation in Connection with Importation of Cattle from the European Union in 2005–2010
***> China TSE Prion <***
Transbound Emerg Dis. 2023 Jun 28;2023:2826256. doi: 10.1155/2023/2826256
Predicting Risk Areas of Classical Scrapie in China Based on Environmental Suitability
Hong Li 1, Haoju Pan 1, Le Xu 1, Suya Li 1, Shiyuan Li 1, Si Chen 1, Churiga Man 1, Li Du 1, Qiaoling Chen 1, Jianhua Xiao 2, Hongbin Wang 2, Fengyang Wang 1, Hongyan Gao 1,✉ Author information Article notes Copyright and License information PMCID: PMC12016686 PMID: 40303770
Abstract
Classical scrapie is a transmissible spongiform encephalopathy that attacks the central nervous system of sheep and goats. Since its discovery in the 18th century, the disease has caused enormous economic losses and public health impacts in continental Europe. In the late 20th century, classical scrapie began to spread to places, such as Asia and the Americas, becoming a disease of global concern. In this study, based on prion occurrence records and high-resolution environmental layers, a risk assessment of classical scrapie in China was performed using a maximum entropy model. The model achieved an area under the curve value of 0.906 (95% confidence interval, 0.0883–0.0929). Sheep distribution density, road density, goat distribution density, minimum temperature of the coldest month, port density, and precipitation of the driest quarter were identified as important factors affecting the occurrence of classical scrapie. The risk map showed that potential high-risk areas in China were mainly located in Northeast China, North China, and Northwest China. This study can provide a valuable reference for the prevention of classical scrapie in China. According to the environmental variables and risk areas of classical scrapie, implementing monitoring and early warning measures in these areas is recommended to reduce the possibility of classical scrapie occurrence and transmission.
snip…
3.2. Potential Risk Areas of Classical Scrapie in China
The predicted global risk areas of scrapie occurrence are shown in Figure 4. It can be seen from Figure 4 that the high-risk areas were mainly concentrated in the middle latitudes of the Eurasian continent, the Sudano–Sahelian zone, and coastal areas of southern Australia. Note that although there has been no scrapie outbreak in China, the predicted results showed that the risk of scrapie in China is widely distributed.
The global risk map of classical scrapie occurrence. The standard map was downloaded from the public standard map service of the National Surveying and Mapping Geographic Information Bureau, and the figure number is GS(2016)1666. The environmental suitabilities for global prion occurrence were marked on the map using ArcGIS, and the standard map has not been modified.
The potential risk map of scrapie in China is shown in Figure 5. The risk map showed that potential medium-to-high-risk areas in China were widely distributed in various subnational regions of Northeast China, North China, and Northwest China. Among them, high-risk areas were concentrated in Heilongjiang, eastern Inner Mongolia, and northern Xinjiang. Additionally, western Liaoning and the Beijing–Tianjin–Hebei region were also in higher risk areas.
Potential risk areas of classical scrapie in China. The standard map was downloaded from the public standard map service of the National Surveying and Mapping Geographic Information Bureau, and the figure number is GS(2019)1822. The potential risk map was marked on the map using ArcGIS, and the standard map has not been modified.
4. Discussion
In this study, a MaxEnt model was established using various environmental variables to identify risk areas of classical scrapie in China. According to the outputs of the model, sheep, and goat distribution density were important risk factors, and the scrapie epidemic may be closely related to flock management. Two large-scale surveys of scrapie in the United Kingdom showed that the risk of scrapie increased significantly with the denser distribution of sheep and the larger size of the flock, and the risk of scrapie was higher in grazing sheep than in captive sheep [46–48]. Our results also showed that the probability of scrapie occurrence increased with an increase in sheep and goat distribution density. China is a big sheep- and goat-breeding country; according to the statistics of the National Bureau of Statistics of China in 2021, the total number of sheep and goats in Northeast China, North China, and Northwest China was as high as 208 million. The total number of sheep and goats in Inner Mongolia, Xinjiang, and Gansu ranks among the top three in China. Performing risk-based surveillance in previous high-risk areas is recommended, and sampling sheep and goats in these areas is also necessary reasonably allocate the time, funds, and human resources required for investigation and provide an effective reference for local governments to prevent scrapie.
In the era of economic globalization, trade in live livestock and livestock products between countries is frequent. Previous incidents showed that some countries and regions, such as Australia, New Zealand, the Americas, and Oceania, have been threatened by the importation risk of scrapie [7, 8, 49]. Our response curve results also showed that transport and trade factors, such as roads and ports, are important factors affecting scrapie prevalence. However, according to a questionnaire survey, most Asian countries have not performed the import risk analyses of TSE [50], and even some countries imported by-products from cattle and ruminants from countries known to be infected with bovine spongiform encephalopathy, which brought greater challenges to the prevention and control of scrapie in Asia. The high-risk areas predicted in this study, such as the northern border region of China, border several countries such as Mongolia. The natural environment and social conditions in these areas are complex, and because of the lack of natural barriers, co-grazing often exists. A series of cross-border transmission events showed that the accidental invasion of a foreign animal disease would not only cause a huge blow and loss to the development of animal husbandry in China but also cause immeasurable damage to neighboring countries and even the entirety of Asia [51–53]. Therefore, implementing an extensive scrapie monitoring program along the northern border of China is particularly important.
This study also explored the effects of various climatic factors on scrapie occurrence, which helps deepen the understanding of the ecological needs of scrapie. Relevant studies have shown that temperature can induce prion to fold, and prion (PrPC) folds into a toxic scrapie form (PrPSC) [54]. In environmental transmission, the bioavailability of prion in soil may also be affected by temperature [29]. Furthermore, the climatic conditions with more precipitation are unsuitable for prion occurrence [55]. Our results showed that minimum temperature of coldest month and precipitation of coldest quarter were important factors affecting scrapie occurrence, which may be a reason why the risk of scrapie was higher in northern China than in southern China. Prion, similar to many other pathogens, readily spread as an interconnected system in the host and environment. According to the trophic cascade hypothesis, we speculate that under certain conditions, climate factors may also increase the risk of scrapie epidemic occurrence by affecting ecosystem productivity. These complexities highlight the importance of taking a “one health” approach when studying zoonotic diseases.
Although soil characteristic factors did not contribute much in the process of building the model, the distribution of prion, similar to that of Burkholderia pseudomallei, Bacillus anthracis, and other soil-dwelling bacteria, is influenced by the biochemical properties of soil [56]. In the early days, scientists have found that prion could remain infectious in soil for many years [57]. Various studies have shown that prion has a higher binding capacity with silt clay than with sand soil and is more easily adsorbed on soil with a larger clay fraction [55, 58]. Therefore, it is speculated that the increase in scrapie risk is related to the increase of in the clay fraction [59]. In contrast, the occurrence of scrapie is associated with the soil drainage capacity [48]. In detecting sand and clay rainwater filtrates in the environment, it was found that the amount of prion in clay filtrates was higher [48, 55]. More pieces of evidence also showed that prion can stably bind to soil minerals, and the ability of prion to spread through the mouth will be enhanced [60], and it is easy to survive in the rumen digestive tract of animals [61]. We speculate that the focus of scrapie may be hidden in a specific narrow range of conditions. Perhaps, soil characteristics affect the growth suitability of plants, or minerals affect the virulence of prion infection and thus determine the host immune homeostasis.
Classical scrapie is a serious threat to the healthy development of the sheep- and goat-breeding industry. Strengthening the attention to the prevention and control of disease, actively building an early warning and monitoring mechanism, prohibiting the import of ruminants and ruminant-derived feed from countries with relevant cases, and prohibiting illegal cross-border livestock trading, among others, are necessary. Future research can focus on the polymorphism of the prion protein gene and implement a breeding plan for scrapie resistance. Additionally, this study has limitations; although pseudo-absence data were generated to reduce the sampling bias, the accuracy of our results may be affected by any biases or missing data.
Acknowledgments This work was supported by the Academician Innovation Platform Project of Hainan Province (no. YSPTZX202013); Hainan Innovation Center for Academician of Jin Ningyi; Hainan Innovation Center for Academician of Zhang Yong; China Agriculture Research System of MOF and MARA: CARS-38; Special Funding Projects for Local Science and Technology Development Guided by the Central Committee (ZY2022HN09); and Priming Scientific Research Foundation of Hainan University (KYQD(ZR)22167).
Characteristics of Different Types of Prion Diseases - China's Surveillance.
Shi Q 1 , Chen C 1 , Xiao K 1 , Zhou W 1 , Gao C 1 , Gao L 1 , Han J 1 , Wang J 1 , Dong X 1
Author information China CDC Weekly, 01 Aug 2022, 4(33):723-728 https://doi.org/10.46234/ccdcw2022.151 PMID: 36285115 PMCID: PMC9547742
Review
Free full text in Europe PMC
Abstract
This report briefly described the establishment and implementation of national surveillance for human prion disease (PrD) in China. Reported cases came from Chinese surveillance network for PrD. Immunohistochemistry, Western blot, enzyme-linked immunosorbent assay (ELISA), Polymerase Chain Reaction (PCR), and real-time quaking-induced conversion (RT-QuIC) tests were used for the samples of brain, cerebrospinal fluid (CSF), and blood. Diagnosis standard for the PrDs is based on the National Commission of Health (WS/T 562-2017). The study summarized major epidemiological, clinical and laboratory features of more than 2,100 diagnosed different types of Chinese PrD cases. Sporadic Creutzfeldt-Jacob disease (sCJD) is the predominant type of PrD (88.7%). 19 different genotypes of genetic PrDs (gPrDs) were identified, accounting for about 11.3% of all PrDs, revealing ethno-relationships. No iatrogenic CJD (iCJD) and variant CJD (vCJD) was identified. The characteristics of different types of sCJD in China showed similar features as those reported globally, but gPrDs showed an obvious ethno-relationship.
WEDNESDAY, APRIL 22, 2026
Atypical Bovine Spongiform Encephalopathy, Should we be Worried? Hong Kong Government
***> Camel TSE Prion Disease <***
Camel Prion Disease TSE Prp Tataouine, Tunisia, 2019–2021 CDC Dispatch August 2026
Camel Prion Disease, Tataouine, Tunisia, 2019–2021 CDC Dispatch August 2026
***> United States APHIS USDA BSE TSE PrP <***
***> US Testing <25k annually for Bovine Spongiform Encephalopathy BSE TSE Prion
***> Scrapie, CWD, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026
***> USA Report, Scrapie, CWD, BSE, TSE, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026, OH MY!
April 2026
US BSE testing <25K annually…
PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...
Saturday, April 25, 2026
**> USDA Statement BSE Surveillance Information Center Update 2026
USA BSE, Scrapie, CWD, Pigs with CWD
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
ATYPICAL CASE OF BSE DETECTED IN SOUTH CAROLINA
May 19, 2023
By Meghan Grebner Filed Under: Beef, Human Interest, Livestock, News
The U.S. has reported an atypical case of Bovine Spongiform Encephalopathy (BSE) in a beef cow approximately five years old or older at a slaughter plant in South Carolina. The animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the U.S. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.
This is the nation’s 7th detection of BSE, of the six previous U.S. cases, the first, in 2003 was the only case of classical BSE, which was from a cow imported from Canada. The rest of the cases have been atypical BSE. The animal was tested as part of the USDA’s Animal and Plant Health Inspection Service’s routine surveillance of cattle that are deemed unsuitable for slaughter.
National Cattlemen’s Beef Association chief veterinarian Dr. Kathy Simmons says USDA’s ongoing BSE surveillance program has tested more than one million cattle since the program began, ensuring that the agency’s interlocking supply chain safety products are working. She says the incidence of BSE in the U.S. is extremely low and will remain so.
U.S. Cattlemen’s Association president Justin Tupper says the swift detection of this case proves that the systems and protocols put in place are working. He says the organization is grateful to the nationwide team of veterinarians, animal health officials, meat inspectors, and others who ensure the well-being of the U.S. cattle herd.
Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.
The World Organization for Animal Health (WOAH) recognizes the U.S. as negligible risk for BSE, the lowest possible risk in the world. Per WOAH guidelines in determining this status, atypical BSE cases do not impact official BSE risk status and this finding of an atypical case will not change the negligible risk status of the U.S., and should not lead to any trade issues.
Tags: Animal and Plant Health Inspection Service, animal health, bovine spongiform encephalopathy, BSE, cattle beef, US Cattlemen's Association, USCA, USDA
MAY 19, 2023
2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;
***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;
Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023
''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...
Mad cow disease: Could it be here? 2001 revisited 2026
***> U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book
Terry S. Singeltary Sr. Date: February 14, 2000 at 8:56 am PST
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene
Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur
Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933
ABSTRACT
Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation.
Snip…
Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.
Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene
KEYWORDS:
Atypical BSE Bovine Spongiform Encephalopathy central nervous systemE211K mutation prion diseases prion protein genesynonymous mutation
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE) The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)
Component 6: Transmissible Spongiform Encephalopathies (TSEs)
Problem Statement 6A: Determine pathobiology of prion strains.
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).
Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa
Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation
Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.
Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.
Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.
Interpretive Summary:
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
Highlights
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation
Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.
Frontiers in Veterinary Science. 3:78. Interpretive
Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.
***> E211K, sCJD, Atypical HG type BSE, what if?
***> Mortality of Individuals With PRNP Variants Associated With Prion Disease in the United States, 1998–2024 Singeltary Review 2026 August 26, 2026 <***
***> cwd transmits by oral routes to, cattle, pigs, sheep, primates <***
cwd to cattle
Prion Conference 2023
Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure
Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.
Strain characterization of chronic wasting disease in bovine-PrP transgenic mice
Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.
Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
***> US Scrapie TSE Prion <***
United States of America - Scrapie - Immediate notification WAHIS
USDA APHIS statement
Oregon statement
Scrapie Update
July 31, 2026
Classical Scrapie Confirmed in Oregon Sheep Through Routine Surveillance
Featured Image Post
Routine slaughter testing detected the case. There is no known risk to people, and most Oregon producers are unaffected.
The Oregon Department of Agriculture (ODA), in collaboration with the US Department of Agriculture's (USDA) Animal and Plant Health Inspection Service (APHIS), is announcing the confirmation of classical scrapie in a sheep slaughtered in a facility in Oregon. The animal was tested as part of USDA's ongoing national scrapie surveillance program, which is designed to identify classical scrapie in sheep and goats at slaughter facilities.
Classical scrapie is a fatal, degenerative prion disease of sheep and goats. The disease can be present in apparently healthy animals, making slaughter surveillance critical for early detection. Classical scrapie typically requires several years to produce clinical signs, and affected animals generally live only months after symptom onset.
Upon confirmation, ODA began an epidemiological investigation to identify the flock of origin and determine whether any potentially exposed animals are located within Oregon. This joint response supports the national effort to eradicate classical scrapie from the United States. Before this confirmation, the United States had gone more than five years without a case. The United States was positioned to meet WOAH's 7-year benchmark for self-declared scrapie freedom in January 2028. USDA says the confirmed detection could reset that timeline and may push the earliest possible date for national scrapie-free status to approximately 2033.
APHIS expects minimal impact on trade. Most countries restrict live animal exports only from affected flocks or premises, and products such as wool remain unaffected.
ODA asks Oregon sheep and goat producers to take four steps:
Keep official identification current. Sheep and goats need official ID before they leave the premises where they were born, so animals can be traced if a case is found. Watch for signs. Rubbing or scratching against fences and posts, tremors, an unsteady or high-stepping walk, changes in behavior, and weight loss in an animal that is still eating well. Call your veterinarian right away if you see those signs. Oregon law requires veterinarians to notify ODA by phone as soon as they suspect scrapie, before any lab testing. You can also call ODA's disease reporting line directly at 503-986-4711.
Ask your veterinarian about breeding for scrapie resistance. Some sheep carry genetics that make them highly resistant to classical scrapie, and breeding for those traits protects a flock over time.
For more information about Oregon's scrapie surveillance program, producers may visit ODA's scrapie website at https://oda.direct/scrapie. More information about scrapie and APHIS' eradication program is available at:www.aphis.usda.gov .
Producers or owners who suspect an animal disease should contact their veterinarian to evaluate the animal or herd. Scrapie is a reportable disease in Oregon. Veterinarians and diagnostic laboratories must immediately report suspected cases to ODA's State Veterinarian.
USDA Confirms Detection of Classical Scrapie in a Slaughtered Sheep
The U.S. Department of Agriculture’s (USDA) Animal and Plant Health Inspection Service (APHIS) has confirmed classical scrapie in a sheep slaughtered at a facility in Oregon. Final diagnostic results and genotype analysis were completed by the National Veterinary Services Laboratories (NVSL), with confirmatory testing indicating classical scrapie. As required, APHIS has notified the World Organisation for Animal Health (WOAH) of the confirmed detection.
Classical scrapie is a fatal, degenerative prion disease of sheep and goats. Prior to this confirmation, the United States had gone more than 5 years without a case. The United States was positioned to meet WOAH’s 7‑year benchmark for self‑declared scrapie freedom in January 2028. This confirmed detection could reset that timeline and may push the earliest possible date for national scrapie‑free status to approximately 2033.
APHIS and State animal health officials have initiated an epidemiological investigation to determine the flock of origin. The affected animal did not have an ear tag, which may limit traceability and highlights the critical importance of strong animal identification practices nationwide. To support the investigation, NVSL will conduct tissue‑matching and parentage testing on the affected sheep and two additional animals from the same accession.
APHIS expects minimal impact on trade. Most countries restrict live animal exports only from affected flocks or premises, and products such as wool remain unaffected.
USDA encourages producers to reinforce key practices that have advanced scrapie eradication efforts nationwide:
Strict compliance with ear tagging and traceability requirements to ensure exposed animals can be accurately tracked.
Robust surveillance participation, including submission of clinical suspects and routine monitoring.
Use of genetically resistant breeding stock, which significantly reduces disease susceptibility and depopulation needs.
Continued partnership with APHIS and State officials to support investigation and long‑term freedom efforts.
More information about scrapie and APHIS’ eradication program is available at www.aphis.usda.gov.
July 31, 2026
Classical Scrapie Confirmed in Oregon Sheep Through Routine Surveillance
Texas Scrapie Confirmed in a Hartley County Sheep
NEWS RELEASE
April 22, 2016
Scrapie Confirmed in a Hartley County Sheep AUSTIN - Texas Animal Health Commission (TAHC) officials have confirmed scrapie in a Hartley County ewe.
The ewe was tested by TAHC after the owner reported signs of weight loss and lack of coordination to their local veterinarian.
The premises was quarantined and a flock plan for monitoring is being developed by the TAHC and USDA.
"The TAHC is working closely with the flock owner, sharing all of the options for disease eradication," said Dr. David Finch, TAl-lC Region 1 Director.
‘We are thankful the producer was proactive in identifying a problem and seeking veterinary help immediately.”
Texas leads the nation in sheep and goat production.
Since 2003, there have been no confirmed cases of scrapie in Texas.
The last big spike in Texas scrapie cases was in 2006 when nine infected herds were identified and the last herd was released from restrictions in 2013. According to USDA regulations, Texas must conduct adequate scrapie surveillance by collecting a minimum of 598 sheep samples annually.
Since USDA slaughter surveillance started in FY 2003, the percent of cull sheep found positive for scrapie at slaughter (once adjusted for face color) has decreased 90 percent.
Scrapie is the oldest known transmissible spongiform encephalopathies, and under natural conditions only sheep and goats are known to be affected by scrapie.
It is a fatal disease that affects the central nervous system of sheep and goats.
It is not completely understood how scrapie is passed from one animal to the next and apparently healthy sheep infected with scrapie can spread the disease.
Sheep and goats are typically infected as young lambs or kids, though adult sheep and goats can become infected.
The most effective method of scrapie prevention is to maintain a closed flock.
Raising replacement ewes, purchasing genetically resistant rams and ewes, or buying from a certified-free scrapie flock are other options to reduce the risk of scrapie.
At this time the resistant genetic markers in goats have not been identified, therefore it is important to maintain your sheep and goat herds separately.
The incubation period for scrapie is typically two to five years.
Producers should record individual identification numbers and the seller's premise identification number on purchase and sales records.
These records must be maintained for a minimum of five years.
see more history on scrapie and the real risk factors to humans therefrom ;
Friday, April 22, 2016
Texas Scrapie Confirmed in a Hartley County Sheep where CWD was detected in a Mule Deer
atypical Nor-98 Scrapie Update 2025 Scrapie typical and atypical USA
FY 2023, 16,646 from sheep and 8,726 from goats.
* There have been 491 NVSL confirmed positive animals (474 classical cases – 471 sheep and 3 goats) and
19 Nor98- like cases since the beginning of RSSS. Figure 3 depicts RSSS collection sites in FY 2023.
In 2023, APHIS collected samples from more than 26,000 sheep and goats for scrapie testing. Out of the total number of animals tested in 2023, no animals tested positive for classical scrapie and
one sheep tested positive for non-classical scrapie (Nor98-like)...
In 2022, APHIS collected samples from more than 23,000 sheep and goats for scrapie testing. Out of the total number of animals tested in 2022, no animals tested positive for classical scrapie and
one sheep tested positive for non-classical scrapie (Nor98-like).
In FY 2021, APHIS collected samples from more than 30,000 sheep and goats for scrapie testing. Out of the total number of samples processed and reported in FY 2021,
one sheep tested positive for classical scrapie and one sheep tested positive for non-classical scrapie (Nor98-like).
In FY 2020, APHIS collected samples from 33,839 sheep and goats for scrapie testing. This number represents sample results reported by October 15, 2020 and is expected to slightly increase as the remaining results are processed and reported. No animals tested positive for classical scrapie.
Two sheep tested positive at slaughter for non-classical scrapie (Nor98-like).
In FY 2019, APHIS collected samples from 34,730 sheep and goats for scrapie testing, detecting 7 classical scrapie positive animals. Of these animals, five sheep and one goat were from a source flock in Pennsylvania that was found in August 2018, depopulated in October 2018, and tested for scrapie in November 2018. A second goat, which was from an Indiana herd, was sampled at slaughter in June 2019. The source flock completed a cleanup plan and was placed on a 5-year monitoring plan. The source herd of the positive Indiana goat no longer contained any exposed animals and was also placed on a 5-year monitoring plan. A trace-back investigation narrowed the goat’s birth herd to two possible herds. Animals in both herds tested negative for scrapie and were placed on 5-year monitoring plans. There were no classical scrapie cases detected in slaughter sheep in FY 2019.
Also in FY 2019, two sheep tested positive at slaughter for non-classical scrapie (Nor98-like) and were traced back to Colorado flocks.
In FY 2018, APHIS collected samples from 43,625 sheep and goats for scrapie testing, detecting three positive (0.0068%) cases. These figures are based on sample submissions and testing completed by September 30, 2018. FY 2018 values are expected to change when testing is completed for all animals sampled in FY 2018.
In October 2017, a non-classical scrapie case was detected in a sheep from Virginia sampled at slaughter. As a result, the non- classical scrapie affected flock was placed on a 5-year monitoring plan.
In FY 2016, the program identified one flock infected with classical scrapie and one infected with Nor98-like scrapie through slaughter surveillance, and two flocks infected with classical scrapie through on-farm surveillance.
An additional 10 sheep were confirmed with classical scrapie through testing of sheep depopulated from these infected flocks as part of flock clean-up activities conducted in FY 2016.
The Nor98-like scrapie affected flock will be placed on a 5-year monitoring plan.
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Nor98 cases Diagnosed in the US. To Date
Nor98 cases Diagnosed in the US.
Flock of Origin State FY
Wyoming 2007
Indiana 2007
Pennsylvania 2008
Oregon 2010
Ohio 2010
Pennsylvania 2010
Untraceable 2010
California 2011
Montana 2016
Utah 2017
Montana 2017
Virginia 2018
Colorado 2019
Colorado 2019
Wyoming 2020
Montana 2020
Pennsylvania 2021
Personal Communication from USDA et al Mon, Jan 4, 2021 11:37 am...terry
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
17 cases of the Nor98 in the USA to date, location, unknown...tss
17 Nor98-like cases since the beginning of RSSS.
17 Nor98-like cases since the beginning of RSSS. No animals have tested positive for classical scrapie in FY 2021.
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
MONDAY, JULY 27, 2020
APHIS USDA Nor98-like scrapie was confirmed in a sheep sampled at slaughter in May 2020
MONDAY, JULY 13, 2020
Efficient transmission of classical scrapie agent x124 by intralingual route to genetically susceptible sheep with a low dose inoculum
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
WEDNESDAY, MAY 29, 2019
***> Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures
THURSDAY, DECEMBER 31, 2020
Autoclave treatment of the classical scrapie agent US No. 13-7 and experimental inoculation to susceptible VRQ/ARQ sheep via the oral route results in decreased transmission efficiency
Snip…see full text;
Epidemiology of Scrapie in the United States
Snip…
Scrapie Field Trial was developed at Mission, Texas, what if?
Scrapie Field Trial was developed at Mission, Texas, on 450 acres of pastureland, part of the former Moore Air Force
EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES
Academic Preg
James Hourriganl, Albert Klingsporn2, Edited by » Peast
W. W. Clark3, and M, de Camp4
United States Department of Agriculture, Animal and Plant Health Inspection Service, Veterinary Services
snip...
METHODS
A Scrapie Field Trial was developed at Mission, Texas, to provide additional information for the eradication program on the epidemiology of natural scrapie. The Mission Field Trial Station is located on 450 acres of pastureland, part of the former Moore Air Force Base, near Mission,
Texas. It was designed to bring previously exposed, and later also unexposed, sheep or goats to the Station and maintain and breed them under close observation for extended periods
to determine which animals would develop scrapie and define more closely the natural spread and other epidemiological aspects of the disease.
The 547 previously exposed sheep brought to the Mission Station beginning in 1964 were of the Cheviot, Hampshire, Montadale, or Suffolk breeds. They were purchased as field outbreaks occurred, and represented 21 bloodlines in which scrapie had been diagnosed. Upon arrival at the Station, the sheep were maintained on pasture, with supplemental feeding as necessary. The station was divided into 2 areas:
snip...
RESULTS
Table 1 indicated that previously exposed sheep brought to the Station at various times and ages (1 to 89 months old) included 333 Suffolks at risk. Of these, 98 (29%) developed scrapie. This demonstrated the necessity to slaughter such sheep to prevent further Spread of the disease, These pre- viously exposed Suffolks were bred at the Station and produced 446 progeny at risk. Of these 153 (34%) developed scrapie.
Although the minimum and average ages when scnapied were similar for both groups, some of the previously exposed Suffolks brought to the Station developed scrapie when much older--ewes 60 to 142 months old and rams 67 to 102 months old. O£ the 153 Suffolks born at the Station, only 3 were more than 60 months of age (65, 66, and 69 months old).
This difference in age scrapied was attributed to the fact that the Suffolks born at the Station may have been subject to a greater exposure from birth.
It was also observed that when both dam and progeny were scrapied, the progeny nearly always developed clinical disease at a younger age than their respective dam. Thirty- two dams were scrapied at an average of 60 months of age. Forty-six of their progeny developed the disease at an average of 38 months (range 25 to 53 months). Thirty-seven of the 46 progeny were younger than the dam (average 20 months younger, range 2 to 99 months younger). Two were scrapied at the same age as their dams, and 8 were older (average 5 months, range 1 to 13 months older).
++. Although the incidence of scrapie was considerably Greater in the progeny of scrapied compared to free dams, the progeny of either scrapied or free dams manifested scrapie at the typical age and irrespective of the age their respective dams were scrapied. The differences in ages that dams and progeny were scrapied was believed due to difference of exposure, particularly whether they were exposed at an early age,
Table 2 summarized the data on exposed Suffolks and was Prepared so as to show scrapie incidence in the progeny of dams and sires of known Scrapie status. The scrapie incidence in the progeny of Free X Free parents was 25%, progeny of scrapied Sires 39%, and scrapied dams 42%. When both sire and dam were scrapied, the scrapie incidence in 18 Progeny at risk was 78%.
When the scrapie status of the sire was ignored, scrapie incidence in th- progeny of free dams was 34% and in pre y of scrapied da as 62%. When the scrapie status of the dam was ignored, scrapie incidence in the progeny of free sires was 26% and in the progeny of scrapied sires was 452.
Although the scrapie incidence was nearly double in the progeny of scrapied compared to free dams, the latter con- tributed a greater number of scrapied progeny, 116, compared to only 51 cases which had scrapied dams. This was because free dams made a considerably heavier contribution to the progeny at risk4-342 compared to 82. It was felt that in farm flocks a similar situation could exist.
It was possible that free dams could have been mis- classified; however, this was unlikely to have been significant, unless "nonclinical or carrier" dams exist. In this Suffolk group, the ages of 100 free dams of scrapied progeny ranged from 25 to 160 (average 97) months. These free dams did not show clinical signs of scrapie,”and there were no histopathological lesions suggesting scrapie in those which died, If one cannot classify as free, ewes which have reached 97 months (average) and did not develop the disease, from a practical standpoint, it is not possible to classify sheep as free, at least on the basis of clinical signs and histology. The free dams of 50% of the scrapied progeny were more than 100 months of age, averaging 126 months.
Upon arrival at the Mission Station at 3 to 9 months of age, the 140 previously unexposed sheep and goats were placed in infected pastures and corrals and were subjected to con- tact with a succession of natural cases of scrapie in sheep, and eventually also in goats. These animals were bred only within their respective groups and were not crossbred to other breeds of sheep or those brought to the Station from infected flocks or their progeny. The male or female animals mixed freely with animals of their respective sex of the infected Flock and were similarly identified and subjected to similar flock management and diagnostic procedures.
Table 3 indicated that natural scrapie had occurred in 5 of the 140 previously unexposed sheep. One case each occurred in Rambouillet, Targhee, and Hampshire ewes at 88, 89, and 89 months of age and in % Suffolk ewes at 73 and 102 months of age, and 85, 82, 80, 64, and 93 months following initial natural exposure. This represented a natural situation involving lateral spread, under the circumstances involved, when sheep were not exposed when very young. Scrapie was not detected clinicaliy or histologically in any of the dairy or Angora goats brought to the Station. The disease occurred in an average of 27% of the progeny of previously unexposed sheep or goats born at the Station and included cases in progeny of all breeds of sheep or goats taken there, The incidence in the progeny ranged from 14% in Rambouillet sheep to 61% in dairy goats. ~
These data showed that scrapie spread laterally, by contact exposure, from scrapied te previously free animals, but at an apparently lower rate when exposure was first received at the age of 3 to 9 months. These animals were presumed to be susceptible to the disease, as their progeny developed scrapie at rates and ages similar (on the average) to the progeny, pf previously exposed Suffolk sheep born and reared in the same environment.
It was suggested that the progeny of previously unexposed animals developed scrapie at a much higher rate than their parents, and at a younger age, because they were subjected to exposure from birth. The data did not rule out the possibility that the animals born at the Station could have also received the virus from their dams "vertically" prior te, at, or following birth.
Table 4 summarized the scrapie incidence in #he progeny, born at the Station, of previously unexposed dairy goats.
The data were prepared so as to show scrapie incidence in the progeny of dams and sires of known scrapie status.
The 58% incidence in the progeny (24 at risk) of Free X Free parents was more than twice the 25% seen in the Suffolk group (Table 2). Scrapied sires did not increase the incidence in goat progeny (it was 44%); scrapied dams increased the incidence to 71%. When both sire and dam were scrapied the incidence was 89%, with only 9 goat progeny at risk.
When the scrapie status of the sire was ignored, the scrapie incidence in the progeny of free dams was 56% and in the progeny of scrapied dams it was 74%.
Free dams contributed 34 progeny at risk and scrapied dams 31 progeny.
When the scrapie status of the dam was ignored, scrapie incidence was 64% in the progeny of free sires and a similar 66% in the progeny of scrapied sires.
A total of 244 sheep (127 Suffolk, 59 Rambouillet, and 58 Targhee) were removed from scrapie exposure within a few hours of birth or at 4, 9, or 20 months of age and placed in isolation pens. Removal of sheep from exposure at these ages was selected as being representative of usual flock operations when sheep might be sold from an infected flock at weaning, the first fall or the second fall after their birth.
Table 5 reflected the fate of such animals. Four of the 6 scrapied sheep which had been isolated at birth were Suffolks and the 2 older animals were Targhees. The first case in the group isolated at birth was a Targhee, progeny of a ewe that did not develop clinical scrapie. The scrapie incidence in 36 at risk Suffolks removed from exposure at birth was 11%, con- siderably less -“en that expected had these animals remz d in an infected en ment.
Table 6 reflected the status of 51 goats isolated from scrapie exposure at birth, and at 6, 8 to 10, 20, 32 to 59 and 60 to 82 months of age.
None of the goats removed at birth developed scrapie, although all 5 of those alive at 5 years of age had scrapied dams and 1 also had a scrapied sire. The sire of the remaining 4 had sired 7 scrapied progeny. Under such circumstances, had they remained in an infected environment nearly all of these goats would have been expected to develop scrapie. With the exception of the 20 month group, scrapie occurred at an incidence of 25 to 100% in ali other groups and at the expected age. A further observation was that 4 of the progeny of these dairy goats, born and kept apart from any sheep, developed scrapie which suggested that goats were not "dead- end hosts" insofar as scrapie was concerned.
Table 7 recorded the fate of progeny of certain selected scrapied or free Suffolk sheep or dairy goat dams.’
Suffolk ewe G298 was scrapied at 46 months of age. She had twin lambs in 1969 and 1 lamb in 1970. All 3 lambs developed scrapie. Suffolk ewe G27a was scrapied at 39 months. Her lamb born in 1966 was scrapied at 53 months; however, her lambs born in 1967 and 1968 remained free--lived to 102 months of age.
Suffolk ewe G25a died at 131] months of age and was nega- tive clinically and histologically. Mice remained negative following intracerebral inoculation of brain, spleen, and lymph nodes from this ewe. This ewe had 9 progeny at risk, of which 4 developed scrapie and 5 did not. There was no dis- cernible pattern to the cases. In two instances, 1 twin was scrapied and 1 remained free.
Goat B259 was scrapied when 43 months old. All of her 6 progeny at risk developed scrapie.
Goat B14a remained free and died at 101 months of age. Of her 11 progeny at risk, 7 were scrapied and 4 were not.
It was observed at the Station that when scrapied dams had several progeny at risk, 1 or more progeny usually developed the disease. However, many such scrapied dams also had progeny which lived, or are living, considerably beyond the age of their dams and beyond the age animals born at the Station manifested the disease.
It was also observed that individual free dams had free progeny in earlier years followed by scrapied progeny when they were older, or had scrapied progeny when young followed by free progeny when older, or scrapie and free progeny dis- persed throughout the dam's breeding life. The same situation occurred in progeny of scrapied dams; however, the pattern was less irregular due to the smaller number of progeny from each scrapied dam and the higher incidence of scrapie in such progeny. Circumstances prevented breeding all ewes ary year and, thus, many had only 1 progeny at risk. Scrapie developed in 100% of the single progeny at risk of 11 scrapied and 15 free dams. The 26 scrapied progeny were equally divided between ewes and rams.
Table 8 reflected the difference in age scrapied of - sheep brought to the Station compared to the age scrapied of those born there. Although the average age of previously exposed sheep (Suffolks) brought to the Station did not differ greatly from the overall average, several animals brought to the Station developed the disease at quite advanced ages. The previously unexposed scrapied animals brought to the Station were also considerably older than animals born there. Progeny of scrapied dams developed the disease at a slightly younger age than did progeny of free dams. The average age was nearly the same for males and females.
DISCUSSION
snip...see full text;
Scrapie Field Trial was developed at Mission, Texas, on 450 acres of pastureland, part of the former Moore Air Force Base
EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES
COLORADO THE ORIGIN OF CHRONIC WASTING DISEASE CWD TSE PRION?
*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
***> US WILD PIGS OUTBREAK OF CWD TSE PRION in pigs in Texas and Arkansas <***
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026 Department for Environment, Food and Rural Affairs
Preliminary Outbreak Assessment
Chronic wasting disease prions in cervids and wild pigs in North America
26 January 2026
Disease report
“They analysed over 300 brain and lymph node samples from 178 wild pigs living across Arkansas and Texas, USA…the researchers identified CWD prions in up to 37% of the lymph node samples and 15% of brain samples. The lowest detection rates were in the Texas samples (below 16%), matching the lower CWD prevalence in the state’s cervid population.”
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026 Department for Environment, Food and Rural Affairs
Preliminary Outbreak Assessment
Chronic wasting disease prions in cervids and wild pigs in North America
26 January 2026
Disease report
“They analysed over 300 brain and lymph node samples from 178 wild pigs living across Arkansas and Texas, USA…the researchers identified CWD prions in up to 37% of the lymph node samples and 15% of brain samples. The lowest detection rates were in the Texas samples (below 16%), matching the lower CWD prevalence in the state’s cervid population.”
Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United State Volume 31, Number 1—January 2025
***> USDA Chronic Wasting Disease CWD TSE Prion <***
TUESDAY, APRIL 07, 2026
APHIS USDA Captive CWD Herds Update by State March 2026
Distribution of Chronic Wasting Disease in North America APRIL 11, 2025
SUNDAY, AUGUST 09, 2026
Chronic Wasting Disease CWD and Population Declines of Cervid
Chronic Wasting Disease CWD TSE Prion, Economical, Environmental, Zoonotic, Risk Factors 2026
Cervid CWD TSE PrP Transmission to Humans, has it already happened and being masked as sporadic CJD, What if? July 2026 Review
Control of Chronic Wasting Disease OMB Control Number: 0579-0189APHIS-2021-0004 Singeltary Submission
Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed
PUBLIC SUBMISSION
Comment from Terry Singeltary Sr.
Posted by the Food and Drug Administration on May 17, 2016 Comment
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission
APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission
Comment from Singeltary Sr., Terry
Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022
***> Captive CWD Indemnity Program or Entitlement Program? <***
***> CWD money grab you say, who’s getting paid?
USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE 2025-2014 CHRONIC WASTING DISEASE CWD TSE CERVID
2025 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
In 2023, eight percent of the farmed cervids in the HCP were tested for CWD at APHIS and State laboratories.
Of the 303,242 farmed cervids tested in 2023, APHIS confirmed 22 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate one of the newly identified positive herds and approved an indemnity payment for a second positive herd which will be provided in 2024 once depopulation occurs. The remaining infected herds are under State quarantines.
2024 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
In 2022, 7 percent of the 285,589 farmed cervids in the HCP participating states were tested for CWD at State and APHIS laboratories.
APHIS confirmed 23 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate nine of the newly identified positive herds in 2022. The remaining infected herds are under State quarantines. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.
2023 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
Currently, 28 States participate in the national CWD HCP. In FY 2021, more than 20,502 farmed cervids were tested for CWD at State and APHIS laboratories.
As a result, APHIS identified 35 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate nine of the newly identified deer herds in FY 2021. The remaining infected herds are under State quarantines. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.
2022 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
Currently, 28 States participate in the national CWD HCP. In FY 2020, more than 11,182 farmed cervids were tested for CWD at State and APHIS laboratories.
As a result, APHIS identified 22 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate 15 of the 22 newly identified deer herds in FY 2020.
Four additional farmed cervid herds that were identified as CWD positive herds in FY 2019, were indemnified in FY 2020.
The remaining infected herds are under State quarantines.
2021 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
In FY 2019 APHIS tested more than 11,000 farmed cervids for CWD.
As a result, APHIS identified 17 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate 7 of the 17 newly identified deer herds in FY 2019. The remaining infected herds found in FY 2019 are under State quarantines.
2020 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
In FY 2018, APHIS identified 15 new CWD positive farmed cervid herds (14 deer herds and 1 reindeer herd).
The reindeer herd in Illinois was the first confirmed case of CWD in a reindeer in North America.
APHIS provided Federal indemnity to depopulate seven of the 15 newly identified deer herds in FY 2018.
The Agency also provided funding for the test and removal of 161 high risk animals that were in close proximity to reactors.
The remaining herds in FY 2018 are under State quarantines.
The Agency determines the use of Federal indemnities within the CWD program on a case-by-case basis. 20-59
2019 President’s Budget Animal and Plant Health Inspection Service
Cervids
APHIS’ voluntary national CWD Herd Certification Plan (HCP) helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds.
Currently, 28 States participate in the national CWD HCP and the program tested 23,053 farmed cervids for CWD.
In FY 2017, eight new CWD positive farmed corvid herds were identified– one white-tail deer in Iowa, one white-tail deer herd in Minnesota, one white-tail and mule deer herd in Minnesota, one white-tail and sika deer herd in Michigan, three white-tail deer herds in Pennsylvania, and one white-tail deer herd in Texas.
APHIS provided Federal indemnity to depopulate the Iowa herd, the white-tail deer herd in Minnesota, one herd in Pennsylvania and the Texas herd. The State depopulated the Michigan herd. The remaining herds are under State quarantines. One Texas herd used Federal indemnity to remove and test select, high-risk animals to inform the epidemiological investigation and to evaluate the performance of ante-mortem tests.
The Agency determines the use of Federal indemnities within the CWD program on a case-by-case basis.
2018 President’s Budget Animal and Plant Health Inspection Service
Cervids
APHIS’ voluntary national CWD Herd Certification Plan (HCP) helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds considered to be low risk.
Currently, 29 States participate in the national CWD HCP.
In FY 2016, the program tested 14,503 farmed cervids for CWD and identified seven new CWD positive farmed cervid herds – two white-tail deer herds in Texas, three white-tail deer herds in Wisconsin, one elk herd in Colorado and one elk herd in Iowa. The elk herd in Colorado was depopulated without Federal indemnity and the rest of the herds are under State quarantines. One Texas herd used Federal indemnity to remove and test select animals to inform the epidemiological investigation and to evaluate 20-72 the performance of ante-mortem tests.
The use of Federal indemnities within the CWD program is determined on a case-by-case basis. APHIS is also conducting several pilot projects related to new technologies. In FY 2016, the Agency
2017 Explanatory Notes Animal and Plant Health Inspection Service
Cervids
APHIS’ voluntary national CWD Herd Certification Plan (HCP) helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds considered to be low risk.
Currently, 30 States participate in the national CWD HCP: 29 have Approved Status and 1 has Provisional Approved Status. States that meet the CWD HCP requirements have Approved Status and States that do not meet CWD HCP program requirements but have developed a work plan and time frame with APHIS to complete those requirements have Provisional Approved Status.
In FY 2015, the program tested approximately 20,000 farmed cervids for CWD and identified eight new CWD positive farmed white-tailed deer herds – one in Utah, one in Pennsylvania, two in Ohio, two in Wisconsin, and two in Texas.
APHIS depopulated five of these herds (Pennsylvania, Utah, and one each in Wisconsin, Texas, and Ohio). Six elk herds in Colorado, four elk herds in Nebraska, one white-tailed deer herd in Wisconsin and one white-tailed deer herd in Texas remained in quarantine at the end of FY 2015.
APHIS also provided indemnity for and was the lead agency for the depopulation and disposal of four large CWD infected farmed cervid herds in Pennsylvania, Ohio, Utah, and Texas. In cooperation with the National Agricultural Statistics Service, APHIS conducted the first national study of the U.S. farmed-cervid industry in FY 2015. The study provides baseline industry statistics, a description of production practices and challenges, producer-reported disease occurrences, and an overview of health management and biosecurity practices.
2016 Explanatory Notes Animal and Plant Health Inspection Service
In FY 2014, the program tested approximately 20,000 farmed cervids for CWD.
Two new CWD positive farmed white-tailed deer herds were identified – one in Pennsylvania and one in Wisconsin.
The program depopulated the PA herd and two additional CWD positive herds in quarantine since FY 2012 in Iowa (white-tailed deer herd) and Minnesota (red deer herd).
Six elk herds in Colorado, four elk herds in Nebraska, and one white-tailed deer in Wisconsin remained in quarantine at the end of FY 2014.
There also are numerous CWD exposed herds that are epidemiologically linked to CWD positive herds that remain in State quarantine pending completion of the epidemiology investigations.
snip…
APHIS provided indemnity for and was the lead agency for the depopulation and disposal of two large CWD infected farmed cervid herds in Iowa and Minnesota.
APHIS also provided indemnity for and assisted with the 20-80 appraisal and depopulation of a CWD infected farmed cervid herd in Pennsylvania.
APHIS also provided assistance to States with outbreak investigation, assessment of risk posed by infected or exposed animals, development of herd plans and continues to develop strategies for the purpose of controlling and managing CWD in farmed cervids.
2015 Explanatory Notes Animal and Plant Health Inspection Service
In FY 2013, the program tested approximately 18,100 farmed cervids for CWD, a fatal, degenerative disease that affects the central nervous system and lymphoid system of cervids. Through this routine surveillance, no new CWD cases were reported in farmed cervids in FY 2013. The last CWD positive herd was reported in FY 2012 in an Iowa white tail deer herd. Twelve positive herds remain (seven elk herds in Colorado, three elk herds in Nebraska, one white tail deer herd in Iowa, and one red deer herd in Minnesota).
2014 Explanatory Notes Animal and Plant Health Inspection Service
In 2007, the cervid industry in the United States included 5,600 deer farms and 1,900 elk farms with an economic value of $894 million that supported nearly 30,000 jobs.
APHIS’ main cervid activities are testing approximately 15,000 captive cervids for tuberculosis each year and supporting the chronic wasting disease (CWD) herd certification program (HCP). The joint tuberculosis (TB) and brucellosis proposed rule will represent significant changes to TB activities in captive cervids when implemented; it proposes to bring cervids into the regulatory program for brucellosis, as requested by stakeholders. As a result, surveillance will be enhanced and the number of captive cervids that are tested for TB annually is expected to increase. In 2014, APHIS will implement a survey to evaluate the effectiveness and impact of new APHIS regulations on the industry. Additionally, approval for a new diagnostic test for TB in captive cervids is expected to occur by FY 2013. The CWD HCP allows participating States to enroll herd owners
18-26
to meet minimum Federal standards to achieve and maintain a herd certification status. APHIS approves State applications for the national voluntary CWD herd certification program, conducts periodic reviews to ensure compliance, and supports confirmatory testing of presumptive CWD cases.
snip…
Without continued program funding, there would be a reduced preparedness, surveillance, and response to equine/cervid health issues that could increase the likelihood of disease spread resulting in larger and more serious disease outbreaks, lack of national standards leading to a patchwork of State level requirements which diminish interstate commerce, and loss in international credibility regarding U.S. animal health status.
Approximately 59 percent of the Equine and Cervid Health funding will be used for salaries and benefits, less than 1 percent for cooperative agreements and programmatic contracts, and the remaining supports normal operating costs such as travel, supplies, rent and utilities.
Reduce lower priority program activities (-$1.295 million) APHIS will reduce lower priority equine and cervid program activities in 2014, including eliminating Federal contributions for addressing CWD.
CWD is a degenerative neurological illness affecting elk and deer (cervids) in North America.
***> APHIS has determined that continued efforts to manage CWD are not practical and therefore considers this to be a low priority for the Agency.
***> On August 13, 2012, the rule that established uniform standards for a voluntary Federal-State cooperative CWD HCP and interstate movement requirements became effective. Implementation of the interstate movement of cervids was implemented in December 2012.
***> Many States have herd certification programs in place, and the incidence of CWD detections in farmed cervids is decreasing.
***> With the regulatory framework in place, continued APHIS activity, while useful, is no longer essential.
Stakeholders can continue to carry on program activities. The success of the voluntary HCP is based upon cooperation and shared responsibility among the Federal government and State and local interests. However, since these are local or regional disease spread issues, State and local governments are better positioned to take a more active role and to better anticipate and plan for local or regional needs. APHIS will continue to conduct higher priority equine and cervid health activities and address concerns when identified. APHIS will reassign staff years to other Equine and Cervid Health activities as practical and reduce the overall staff years by eliminating the positions when vacancies arise. Reduction in Agency-level operating expenses (-$66,000) A reduction of $66,000 is requested for this line item related to Agency-level cost savings measures and operating efficiencies. Please refer to second paragraph on page 18-18.
18-27
Pay Increase (+$15,000)
An increase of $15,000 for pay costs which includes $3,000 for annualization of the FY 2013 pay raise and $12,000 for the anticipated FY 2014 pay raise.
***> APHIS has determined that continued efforts to manage CWD are not practical and therefore considers this to be a low priority for the Agency.
***> On August 13, 2012, the rule that established uniform standards for a voluntary Federal-State cooperative CWD HCP and interstate movement requirements became effective. Implementation of the interstate movement of cervids was implemented in December 2012.
***> Many States have herd certification programs in place, and the incidence of CWD detections in farmed cervids is decreasing.
***> With the regulatory framework in place, continued APHIS activity, while useful, is no longer essential.
***> The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA viewed it as a wildlife problem and consequently not their province!” page 26.
APHIS USDA Captive CWD Herds Update by State March 2026
Updated March 2026
Deer don’t die from CWD, it’s the insurance companies, or it's a Government conspiracy?
For all you …folks, that keep saying deer don’t die form CWD, or it's a Government conspiracy?
here’s your sign…
“The deer was found dead by a landowner and was severely emaciated.”
CWD-POSITIVE DETECTED IN CARBON COUNTY
February 12, 2025
HARRISBURG -- The Pennsylvania Game Commission today announced an additional CWD-positive deer has been detected in the northeastern part of the state.
The deer, an adult male, was detected in Packer Township, Carbon County. This detection is the first in Carbon County and is more than 10 miles from any other confirmed CWD-positive deer. The deer was found dead by a landowner and was severely emaciated.
Ever seen a dead deer from Cwd?
That dog don’t hunt!
First chronic wasting disease case confirmed in Spokane County
SPOKANE- Washington’s first case of chronic wasting disease (CWD) has been confirmed in an adult female white-tailed deer that was found dead in the Fairwood area of north Spokane.
Case of Chronic Wasting Disease found in dead deer at Breckinridge County farm Oct 14, 2024 Updated Oct 14, 2024 The Kentucky Department of Fish and Wildlife said Monday that a case of the disease was found in a dead deer from a Breckinridge County deer farm. This is Kentucky's first case of CWD in a captive group of deer, the department said in a news release.
Oklahoma CIMARRON COUNTY SSA ENLARGED AFTER CHRONIC WASTING DISEASE CONFIRMED IN DEAD DEER
MADISON, Wis. – The Wisconsin Department of Natural Resources (DNR) confirms the first positive test result for chronic wasting disease (CWD) in a wild deer in Waushara County. The deer was found dead in early February in the town of Wautoma and is within 10 miles of the Marquette and Portage county borders.
Ever see a dead deer from Cwd?
CWD, Seeing is believing Videos Part 1, Part 2
The below video series is provided by the Mississippi State Deer Lab with many contributing Partners. Click image to find all videos in the series.
Seeing is Believing is a two part documentary film that hopes to increase awareness about chornic wasting disease (CWD). Although most hunters and landowners may never witness a clinically ill animal in an area with high CWD prevalence, the documentary demonstrates how CWD is certainly present, explains why it is a major concern, and how stakeholders are key to managing the disease. Colorado Parks and Wildlife (CPW) developed these films in partnership with the Wyoming Game and Fish Department, Chronic Wasting Disease Alliance, Colorado Department of Agriculture, Animal and Plant Health Inspection Service, and multiple private conservation organizations.
CWD Seeing is believing part 1 Video
CWD Seeing is believing part 2 Video
The Rocky Mountain Elk Foundation - CWD Informational Video
Cwd videos
15 minute mark video shows sick deer with cwd, and this deer DIED FROM CWD, IT'S DOCUMENTED, commentator says ''so if anyone every tells you, that a deer has never died from CWD, think of this picture, because the Wisconsin Veterinary Lab told us, what when they looked at her sample under a microscope, she was the hottest animal they had ever seen, and that's in terms of the fluorescents that comes off the slide when the look at it, so, a lot of Prion in her system.''
''SCENTS AND LURES, we know that the Prion is shed in urine, and essentially the production of these products is unregulated, we have no idea, you can't tell where they come from, what species are in them, how many animals, how they are processed, there is really no rules about them, so we are concerned it is a way to bring the disease into new areas, and have us fighting on multiple fronts, AND there are zero risk synthetic options that are readily available in stores, so we have ask hunters to switch to zero risk options.''
see much more about 2 hours long...
TEXAS BREEDER DEER ESCAPEE WITH CWD IN THE WILD, or so the genetics would show?
OH NO, please tell me i heard this wrong, a potential Texas captive escapee with cwd in the wild, in an area with positive captive cwd herd?
apparently, no ID though. tell me it ain't so please...
23:00 minute mark
''Free Ranging Deer, Dr. Deyoung looked at Genetics of this free ranging deer and what he found was, that the genetics on this deer were more similar to captive deer, than the free ranging population, but he did not see a significant connection to any one captive facility that he analyzed, so we believe, Ahhhhhh, this animal had some captive ahhh, whatnot.''
Texas symposium Cwd
Arkansas Cwd
Wyoming Cwd 2022 test results
key takeaways ;
CWD substantially reduces deer survival rates and suppresses population growth.
Where CWD prevalence is high, deer populations are likely declining.
If CWD continues to spread, it will eventually impact deer populations elsewhere.
***> Captive CWD Indemnity Program or Entitlement Program? <***
***> CWD money grab you say, who’s getting paid?
The funding opportunity was open to State departments of agriculture; State animal health agencies; State departments of wildlife or natural resources; Federally Recognized Native American Tribal governments; Native American Tribal organizations or universities representing Federally Recognized Tribes; and research institutions and universities.
Eligible applicants were permitted to submit multiple proposals, with each proposal requesting up to $250,000 for activities aimed at controlling or preventing CWD in farmed cervids.
i These awards include indemnity funds for the removal of CWD-positive or -exposed animals.
ii Noncompetitively awarded using declined funds. $130,717ii $6
Chronic Wasting Disease in Cervids; Payment of Indemnity A Rule by the Animal and Plant Health Inspection Service on 02/08/2002
Amount of Indemnity Payments and Conditions for Receiving Indemnity
Subject to the availability of funding, the amount of indemnity payments for eligible animals will be determined by appraisal, with the indemnity payment set at 95 percent of the appraised value, with a cap on payments of $3,000 per animal. CWD positive herds will be appraised by an APHIS official appraiser and a State official appraiser jointly, or, if APHIS and State authorities agree that both appraisers are not needed for a given situation, by either a State official appraiser or an APHIS official appraiser alone. The appraised value of the cervids will be their fair market value as determined by the meat or breeding value of the animals. Animals may be appraised in groups, provided that where appraisal is by the head, each animal in the group is the same value per head, and where appraisal is by the pound, each animal in the group is the same value per pound.
Displaying title 9, up to date as of 8/13/2026. Title 9 was last amended 7/20/2026.
Subpart A—Chronic Wasting Disease Indemnification Program
§ 55.2 Payment of indemnity.
The Administrator is authorized to pay for the purchase and destruction of CWD positive animals, CWD exposed animals, and CWD suspect animals. Subject to available funding, the amount of the Federal payment for any such animals will be 95 percent of the appraised value established in accordance with § 55.3 of this part, but the Federal payment shall not exceed $3,000 per animal. If a non-Federal source makes a payment for an animal for which a Federal indemnity is paid, and the non-Federal payment exceeds 5 percent of the appraised value established in accordance with § 55.3 of this part, the amount of the Federal payment for any such animals will be reduced by the amount by which the non-Federal payment exceeds 5 percent of the appraised value. The Administrator is also authorized to reimburse State governments or State animal health agencies for payments they make for the purchase and destruction, on or after October 1, 2001, of CWD positive animals, CWD exposed animals, and CWD suspect animals, and for State expenditures for associated carcass disposal and cleaning and disinfection costs resulting from such purchase and destruction, in accordance with cooperative agreements signed by the Administrator and the duly authorized agent of the State.§ 55.3 Appraisal and destruction of captive cervids.
snip…see;
***> USDA atypical Scrapie
*** Grant Agreement number: 222887 ***
*** Project acronym: PRIORITY ***
*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***
Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014
Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es
Project website¡ Error! Marcador no definido. address: www.prionpriority.eu
PRIORITY, PROJECT FINAL REPORT
*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***
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Block D: Prion epidemiology
Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.
In parallel, studies in sheep have concluded that:
*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes
*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.
*** The combination of data from all our studies leads us to conclude that:
*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).
*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.
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We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:
a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:
• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;
• the uncertainties related to prion epidemiology in animal populations;
• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents; • the intrinsic capacity of prions to cross interspecies transmission barriers; • the lack of sensitive methodology for identifying cross contamination in food.
• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.
The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.
b. Atypical prion agents and surveillance
Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.
*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.
*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.
*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.
*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable
• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)
• to improve the sensitivity of detection assays that are applied in the field towards this type of agent
• to maintain a robust surveillance of both animal and human populations
c. The need for extended research on prions
Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.
Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.
Position of the Priority consortium
Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk
Origins of prion epidemies
Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.
In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.
The human prion problem
The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.
Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.
The animal prion problem
An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.
We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.
The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.
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Transmission of scrapie prions to primate after an extended silent incubation period
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
==============
PRION 2015 CONFERENCE
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X
WS-01: Prion diseases in animals and zoonotic potential
***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
Title: Transmission of scrapie prions to primate after an extended silent incubation period)
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014)
Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D 1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy.
(2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy.
Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure.
Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine).
Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased.
Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.
Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAFCOD_UO-P
''Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.''
Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
***> Chronic Wasting Disease CWD TSE PrP Outbreak Wild Pigs, Arkansas and Texas USA <***
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026 Department for Environment, Food and Rural Affairs
Preliminary Outbreak Assessment
Chronic wasting disease prions in cervids and wild pigs in North America
26 January 2026
Disease report
Chronic wasting disease (CWD) is a fatal neurodegenerative disease of cervids, such as deer, elk, moose and reindeer. It is caused by prions – infectious proteins that cause normal cellular prion proteins to misfold (CIDRAP, 2025). The disease is widespread in captive and free-ranging cervids in North America (Figure 1). For the first time, CWD prions have also been detected in the tissues of wild pigs (Sus scrofa) caught in CWD-affected areas of the USA (Soto et al. 2025). This discovery emerged from a study designed to investigate potential interactions between wild pigs and CWD prions, as wild pigs often coexist with cervids, which can shed prions into the environment. The following assessment discusses the epidemiology of CWD in North America and the detection of CWD prions in wild pigs. It also considers the potential implications for Great Britain.
Conclusion
CWD has continued to spread among captive and free-ranging cervids in North America since it was first detected in the 1960s. The finding of CWD prions in wild pigs in the USA suggests they could contribute towards transmission of the disease, influencing its epidemiology, geographic distribution and interspecies spread. However, further research is needed to confirm this. CWD has never been reported in Great Britain and the current risk of CWD prions being introduced into Great Britain’s wild pig or cervid population ranges from very low to negligible.
Readers are reminded to be vigilant for signs of CWD. Information on how to spot the disease can be found here. Suspected cases must be reported immediately to the Defra Rural Services Helpline on 03000 200 301. In Wales, call 0300 303 8268. In Scotland, contact your local Field Services Office. Failure to do so is an offence. We will continue to monitor the situation.
Authors • Lawrence Finn • Dr. Lauren Perrin • John Spiropoulos • Dr. Helen RobertsDepartment for Environment, Food and Rural Affairs
References
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WEDNESDAY, JANUARY 28, 2026
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak
***> USDA cwd to sheep
Chronic Wasting Disease CWD vs Scrapie TSE Prion
Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
Title: Differentiation of scrapie from chronic wasting disease in white-tailed deer
Accomplishments
1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.
Chronic Wasting Disease CWD vs Scrapie TSE Prion
Volume 30, Number 8—August 2024
Research
Scrapie Versus Chronic Wasting Disease in White-Tailed Deer
Zoe J. Lambert1, Jifeng Bian, Eric D. Cassmann, M. Heather West Greenlee, and Justin J. Greenlee
Author affiliations: Oak Ridge Institute for Science and Education, Oak Ridge, Tennessee, USA (Z.J. Lambert); US Department of Agriculture, Ames, Iowa, USA (Z.J. Lambert, J. Bian, E.D. Cassmann, J.J. Greenlee); Iowa State University, Ames (Z.J. Lambert, M.H. West Greenlee) Suggested citation for this article
Abstract
White-tailed deer are susceptible to scrapie (WTD scrapie) after oronasal inoculation with the classical scrapie agent from sheep. Deer affected by WTD scrapie are difficult to differentiate from deer infected with chronic wasting disease (CWD). To assess the transmissibility of the WTD scrapie agent and tissue phenotypes when further passaged in white-tailed deer, we oronasally inoculated wild-type white-tailed deer with WTD scrapie agent. We found that WTD scrapie and CWD agents were generally similar, although some differences were noted. The greatest differences were seen in bioassays of cervidized mice that exhibited significantly longer survival periods when inoculated with WTD scrapie agent than those inoculated with CWD agent. Our findings establish that white-tailed deer are susceptible to WTD scrapie and that the presence of WTD scrapie agent in the lymphoreticular system suggests the handling of suspected cases should be consistent with current CWD guidelines because environmental shedding may occur.
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The potential for zoonoses of cervid-derived PrPSc is still not well understood (6,18,45–47); however, interspecies transmission can increase host range and zoonotic potential (48–50). Therefore, to protect herds and the food supply, suspected cases of WTD scrapie should be handled the same as cases of CWD.
Western blots done on samples from the brainstem, cerebellum, and lymph nodes of scrapie-infected WTD have a molecular profile similar to CWD and distinct from western blots of samples from the cerebral cortex, retina, or the original sheep scrapie inoculum. WTD are susceptible to the agent of scrapie from sheep and differentiation from CWD may be difficult.
It has long been hypothesized that CWD arose through transmission of sheep scrapie to deer. ARS researchers in Ames, Iowa, conducted research to determine if scrapie derived from sheep could be transmitted to white-tailed deer. The deer inoculated with sheep scrapie developed clinical signs and the abnormal prion protein could be detected in a wide range of tissues. These results indicate that deer may be susceptible to sheep scrapie if exposed to the disease in natural or agricultural settings. In addition, several strong similarities between CWD in white-tailed deer and the experimental cases of scrapie in white-tailed deer suggests that it would be difficult to distinguish scrapie from CWD in deer or identify scrapie if a case occurs. This information should be considered by deer farmers for keeping their herds free from prion diseases.
https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/NP103%20FY2023%20Annual%20Report_Final.pdf
Additional studies in WTD established a minimum oral CWD infectious dose equivalent to 100–300 ng CWD-positive brain tissue (10)…
We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease.
ORIGIN OF CHRONIC WASTING DISEASE TSE PRION?COLORADO THE ORIGIN OF CHRONIC WASTING DISEASE CWD TSE PRION?
*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
IN CONFIDENCE, REPORT OF AN UNCONVENTIONAL SLOW VIRUS DISEASE IN ANIMALS IN THE USA 1989
The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA viewed it as a wildlife problem and consequently not their province!” page 26.
Friday, July 26, 2013
Voluntary Scrapie Program USA UPDATE July 26, 2013 increase in FY 2013 is not statistically meaningful due to the sample size
Thursday, May 30, 2013
World Organization for Animal Health (OIE) has upgraded the United States' risk classification for mad cow disease to "negligible" from "controlled", and risk further exposing the globe to the TSE prion mad cow type disease
U.S. gets top mad-cow rating from international group and risk further exposing the globe to the TSE prion mad cow type disease
Thursday, December 20, 2012
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE
***> Tuesday, June 9, 2026
***> Hazard identification for the risks to terrestrial animal health from the import of hay and straw, BSE, CWD, Scrapie, TSE, Prion <***
6.8. Bovine spongiform encephalopathy (Chapter 11.4.), Application for official recognition by the OIE of risk status for bovine spongiform encephalopathy (Chapter 1.8.) and Glossary definition for ‘protein meal’
Background
In February 2018, following preliminary work and scientific exchanges, the Code Commission and the Scientific Commission agreed to an in-depth review of Chapter 11.4. Bovine spongiform encephalopathy (BSE). The OIE convened three different ad hoc Groups between July 2018 and March 2019: i) an ad hoc Group on BSE risk assessment, which met twice, ii) an ad hoc Group on BSE surveillance, which met once, and iii) a joint ad hoc Group on BSE risk assessment and surveillance, which met once. The Code Commission, at its September 2019 meeting, reviewed the four ad hoc Group reports and the opinion of the Scientific Commission regarding the draft revised chapter and circulated a revised draft Chapter 11.4. for comments.
In February 2020, the Code Commission considered comments received on the revised draft Chapter 11.4. and requested that the joint ad hoc Group on BSE risk assessment and surveillance be reconvened to address comments of a technical nature. In June 2020, the joint ad hoc Group was convened to address relevant comments and was also requested to review Chapter 1.8. Application for official recognition by the OIE of risk status for bovine spongiform encephalopathy to ensure alignment with the proposed changes in Chapter 11.4.
In September 2020, the Code Commission reviewed the joint ad hoc Group report and the revised draft Chapters 11.4. and 1.8. and made some additional amendments and circulated the revised chapters for comments in its September 2020 report. In February 2021, the Commission considered comments received and amended the chapters, as appropriate, and circulated the revised chapters for a third round of comments.
In preparation for the September 2021 meetings, some members of the Code Commission and the Scientific Commission met to discuss key aspects of the revision of Chapters 11.4. and 1.8. to ensure a common understanding of the main concerns raised by Members, the decisions made on the revised chapters and their impact on the official status recognition, as well as on the adapted procedures that will be required. During this meeting, it was agreed that each Commission would address the issues relevant to its meeting and document discussions in their respective reports.
Discussion
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***> atypical Bovine Spongiform Encephalopathy BSE TSE Prion <***
Transmission of atypical BSE: a possible origin of Classical BSE in cattle
***> 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.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
***> 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.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
***> Atypical BSE TSE Prion in cattle <***
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
“According to the World Organisation for Animal Health (WOAH), Terrestrial Manual 2021, atypical BSE, caused by H- and L-type BSE agents, is rare and is believed to occur spontaneously in all bovine populations at a very low rate and has only been identified in older cattle.”
***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.
***> atypical Bovine Spongiform Encephalopathy BSE TSE Prion Madcow 2026 update <***
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
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
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.
'Spontaneous mutation'
***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.
Atypical BSE cases in Ireland: neurological signs, brain histopathology and Tissue distribution of PrPres
Sebastian 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
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
***> USA CJD Human TSE PrP <***
***> Mortality of Individuals With PRNP Variants Associated With Prion Disease in the United States, 1998–2024 Singeltary Review 2026 August 26, 2026 <***
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!!
Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
“As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture!”
END...TSS
Terry S. Singeltary Sr.
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