Friday, April 17, 2026

Ireland Central Veterinary Research Laboratory confirmed a case of atypical BSE on April 9, 2026

  Ireland Central Veterinary Research Laboratory confirmed a case of atypical BSE on April 9, 2026

 Single case of atypical bovine spongiform encephalopathy (BSE) detected through fallen animal surveillance in Ireland

WED 15 APR 2026

DAFM identifies case of atypical BSE in Ireland. Agriland. 13 Apr 2026

Key findings

On 09 Apr 2026, the Department of Agriculture, Food and the Marine (DAFM) confirmed one case of atypical bovine spongiform encephalopathy (BSE) in a fallen nine-year-old cow, identified through systematic surveillance at a fallen animal collection center.

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

Since 2015, Ireland has maintained a World Organisation for Animal Health (WOAH) negligible risk status for BSE, the lowest risk rating available. This status remains unaffected by this atypical case

On 10 Apr 2026, DAFM notified competent authorities in China, Japan, and Korea as required by trade agreements, and is awaiting their response regarding potential trade implications

This represents the second atypical BSE case detected in Ireland since October 2024, when a previous atypical case temporarily halted beef exports to China.

Epidemiological analysis and public health impact

Ireland’s systematic surveillance program, mandatory since the mid-2000s, requires testing of all fallen animals over 48 months of age, enabling detection of both clinical and subclinical cases before they can pose any risk to public or animal health. The detection of atypical BSE through this surveillance demonstrates the effectiveness of the country’s active monitoring system, while the immediate identification and exclusion of the affected animal from the food and feed chains reflect the standard control measures applied in all BSE cases.

This surveillance infrastructure enables early identification of spontaneous cases before they can enter the food chain, maintaining public health protection and supporting Ireland's negligible risk classification.

The rapid laboratory confirmation at DAFM's Central Veterinary Research Laboratory, within days of sample collection, reflects robust diagnostic capacity, which is essential for maintaining confidence in international trade.

Atypical BSE cases represent spontaneous, age-related prion protein misfolding events rather than feed-borne transmission, fundamentally distinguishing them from classical BSE. Ireland’s ability to detect such cases while maintaining a negligible risk status shows that surveillance exceeds international disease-control thresholds. The immediate notification to trading partners China, Japan, and Korea highlights the continued sensitivity of beef markets to BSE detection, even when cases pose no public health risk and do not affect official risk status.

The temporary halt of Chinese imports after the October 2024 atypical case—lasting over a year and lifted in January 2026—shows how single case notifications can trigger precautionary trade restrictions despite negligible scientific risk. The current case may lead to similar temporary market closures while authorities review Ireland’s compliance, potentially impacting a beef export sector worth hundreds of millions of euros annually. Comparative analysis and future outlook

The identification of sporadic atypical cases in 2017, 2020, 2023, 2024, and now 2026 reflects the expected baseline occurrence of spontaneous prion disease in aging cattle populations.

The future trajectory will likely continue to show rare, sporadic atypical BSE cases detected through ongoing surveillance of fallen animals.

The continued effectiveness of Ireland's surveillance program in detecting these rare events before animals enter the food chain provides confidence in the country's ability to maintain a negligible risk status and protect public health.

Byline: AMD, MCD

Copy editor: JM

BEACON is a program based at Boston University's Center on Emerging Infectious Diseases (CEID) and operated in partnership with the Hariri Institute for Computing and Data Sciences at Boston University and HealthMap at Boston Children's Hospital.

https://beaconbio.org/en/report/?reportid=f63793ee-dd12-4974-8b01-7a895226239c&eventid=f3d152a8-52ed-4ad2-9459-f2da4c36210b

Ireland Central Veterinary Research Laboratory confirms another case of atypical BSE on April 9, 2026 

Department confirms atypical BSE case

The animal with mad cow disease did not enter the food or feed chain and poses no public health risk

Department confirms atypical BSE case

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.

File picture TUE, 14 APR, 2026 - 16:30

ZOE GEARY

A case of atypical Bovine spongiform encephalopathy (BSE), commonly known as ‘mad cow disease’, has been confirmed by the department.

Tests carried out at the department’s Central Veterinary Research Laboratory confirmed a case of “atypical BSE” on April 9, 2026.

BSE is a fatal disease of the nervous system of bovines that is caused by the accumulation of an abnormal protein called ‘prion’ in nervous tissue.

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.

The manual highlights that a common feature amongst atypical BSE cases is that they are “almost exclusively detected in cattle older than eight years.”

Research has confirmed that L-type BSE may be transmitted orally to calves of an infected animal.

Signs of atypical BSE

Cattle with a ‘nervous form’ atypical BSE will likely display the same clinical signs as cattle with classical BSE, such as over-reactivity, unexpected startle response, and inco-ordination.

Alternatively, cattle with a ‘dull form’ of atypical BSE would show signs of dullness accompanied by low head carriage and compulsive behaviour such as licking, chewing or pacing in circles.

The disease usually progresses over weeks to several months, with death being the unavoidable end result.

There is no test to diagnose any form of BSE in live animals other than tentative diagnosis via observed clinical signs.

Diagnosis can only be identified after examination of the animal's brain post mortem.

Further tests must then be performed to distinguish which type of atypical BSE the animal had contracted.

Detection

The last reported case of atypical BSE was in May 2020. Classic BSE, caused by the C-type agent, occurs when an animal eats contaminated feed.

It is understood that the positive case was discovered during the department’s ongoing systematic surveillance of ‘fallen’ animals at a designated fallen animal collection centre.

A spokesperson for the department explained:

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

Ireland has a WOAH negligible risk status for BSE, which is the lowest risk rating available. The identification of this atypical BSE case does not jeopardise Ireland’s negligible risk status for BSE.

The department has reported that the detection of this atypical BSE case “does not impact on trade generally.”

Some agreements with some trading partners require notification of such cases, and the relevant competent authorities have been informed.

“Agreements with China, Japan and Korea require official notification of such cases to be issued to their competent authorities. The department notified their competent authorities on 10 April 2026 and awaits their response,” reports the spokesperson.

Reaction

Reacting to the news, Irish Farmers’ Association (IFA) health chair David Hall said the development was disappointing but demonstrated the effectiveness of Ireland’s BSE Surveillance Scheme.

IFA livestock chair Declan Hanrahan said the impact on trade would be limited and manageable.

“Exports will only be affected in a small number of markets, and our key export destinations will not be impacted by this development,” Mr Hanrahan said.

Mr Hanrahan also stressed that the situation must not be used to undermine farmer returns.

“Factories cannot and must not use this case as an excuse to lower beef prices… The fundamentals of the market remain strong, and farmers are entitled to a fair price for their stock,” concluded Mr Hanrahan.

https://www.irishexaminer.com/farming/arid-41827337.html

Ireland DAFM identifies case of atypical BSE

By Aisling O'Brien April 13, 2026 9:10 AM Share this article

The Department of Agriculture, Food and the Marine (DAFM) has confirmed a case of atypical bovine spongiform encephalopathy (BSE) in Ireland.

Agriland understands that tests carried out at the department’s Central Veterinary Research Laboratory confirmed a case of atypical BSE on Thursday evening (April 9).

The animal was identified during the department’s on-going systematic surveillance of ‘fallen’ animals at fallen animal collection centre.

A spokesperson for the department said that "the animal did not enter the food or feed chain and there are no public health risks associated with this occurrence".

Atypical BSE DAFM noted that "atypical BSE is a rare spontaneous event that may occur in any bovine population".

"Ireland has World Organisation for Animal Health (WOAH) negligible risk status for BSE, which is the lowest risk rating available.

"The identification of this atypical BSE case does not affect Ireland’s negligible risk status for BSE," the spokesperson said.

The department added that the identification of this atypical BSE case "does not impact on trade generally".

"However, agreements with some trading partners require notification of such cases, and the relevant competent authorities have been informed.

"Agreements with China, Japan and Korea require official notification of such cases to issue to their competent authorities," the spokesperson said.

The department notified the competent authorities in these countries on Friday and is awaiting their response.

In January, beef exports from Ireland to China were stopped due to the detection of bluetongue here.

The Chinese market only reopened to Irish beef two weeks previously having been stopped over a year earlier, in October 2024, after an atypical case of BSE was discovered in a cow here.

Ireland DAFM identifies case of atypical BSE

https://www.agriland.ie/farming-news/dafm-identifies-case-of-atypical-bse-in-ireland/

Friday, October 4, 2024

another atypical bovine spongiform encephalopathy (BSE) in Ireland

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

Statistics on the surveillance of transmissible spongiform encephalopathies (TSEs) in cattle in the United Kingdom updated 16 January 2025

Research and analysis

Cattle: TSE surveillance statistics

Statistics on the surveillance of transmissible spongiform encephalopathies (TSEs) in cattle in the United Kingdom.

From: Animal and Plant Health Agency

Published 11 February 2015

Last updated 16 January 2025

Documents

Overview of Great Britain statistics

MS Excel Spreadsheet, 33.9 KB

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General statistics

MS Excel Spreadsheet, 144 KB

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Age and related statistics

MS Excel Spreadsheet, 55.9 KB

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BSE cases born after the reinforced feed ban (BARB) in the UK

MS Excel Spreadsheet, 29.5 KB

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Details

These documents provide statistics on the number of cases of TSE disease found through the active and passive disease surveillance of cattle in the United Kingdom.

Cases of TSE disease identified in cattle from passive surveillance in United Kingdom have been recorded since 1986. The UK carried out limited active surveillance in cattle from 1999 to 2001. The European Union active surveillance programme started in July 2001.

The UK carries out active disease surveillance for bovine spongiform encephalopathy (BSE) in cattle. The testing programme includes cattle over 48 months of age which:

die or are killed other than for human consumption (fallen cattle)

are emergency slaughtered or show certain abnormalities at ante-mortem inspection

These age thresholds apply to cattle born in the United Kingdom or in other EU member states except Bulgaria and Romania. For cattle born elsewhere the age thresholds are 24 months for fallen cattle or emergency slaughtered cattle, and 30 months for healthy fallen cattle.

Passive disease surveillance takes place when an animal with clinical signs suspicious of a TSE disease is reported to Animal and Plant Health Agency (APHA), and further investigation determines whether the animal was affected by BSE or scrapie.

Published 11 February 2015

Last updated 16 January 2025

https://www.gov.uk/government/publications/cattle-tse-surveillance-statistics

Transmissible spongiform encephalopathies (TSE)

Last reviewed date: 5 January 2026. 

Transmissible spongiform encephalopathies (TSE) are a group of neurodegenerative diseases that affect humans and animals. They are always fatal. TSE are caused by agents called prion which are abnormal forms of proteins.

TSE include:

Classical and atypical bovine spongiform encephalopathy (BSE) in cattle; Classical and atypical scrapie in sheep and goats; Chronic wasting disease (CWD) in cervids; Transmissible mink encephalopathy (TME) in mink; Feline spongiform encephalopathy (FSE) in cats; Creutzfeldt-Jakob disease (CJD); variant Creutzfeldt-Jakob disease (vCJD); Gerstmann-Sträussler-Scheinker syndrome; Fatal Familial Insomnia; Kuru in humans. With the exception of the BSE agent, which can be transmitted to humans through consumption of contaminated meat causing vCJD, there is no scientific evidence that other animal TSE can be transmitted to humans.

Latest

In November 2025, EFSA published its latest Summary Report on the surveillance for the presence of TSE in the EU in 2024.

The report provides an overview of data collected by 27 EU Member States, the United Kingdom in respect of Northern Ireland, and a further eight non-EU countries (Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye). The data covers bovines, sheep, goats, cervids and other animal species , and genotyping in sheep and goats.

The main findings are:

Three cases of atypical BSE were reported in cattle across the EU. Three other cases were detected worldwide. One case of classical BSE was reported in the UK, the disease transmissible to humans. 601 cases of scrapie were detected in small ruminants in the reporting countries: 530 in sheep (615 in 2023) and 71 in goats (183 in 2023). Two cases of Chronic Wasting Disease were detected in wild European moose. Surveillance of TSE in cervids is voluntary in the EU. EFSA updated its storymap, which provides general information on TSE, including the history of the diseases and related control measures. A dashboard to search and visualise the surveillance data is also available.

Ongoing and completed assessments

snip…see full report;


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


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

Published: 10 November 2022


THURSDAY, NOVEMBER 9, 2023

EFSA Annual Report of the Scientific Network on BSE-TSE 2023


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 2024

Published: 13 November 2025 Approved: 15 October 2025 EFSA Journal

DOI https://doi.org/10.2903/j.efsa.2025.9732

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

 https://www.efsa.europa.eu/en/efsajournal/pub/9732

https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2025.9732

https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2025.9732

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.

https://cjdsupport.co.uk/10736-2/#:~:text=CJDSN,CJD%20and%20related%20prion%20diseases:

Active TSE surveillance in Great Britain and Northern Ireland 2025

Active TSE surveillance in Great Britain 2025

https://assets.publishing.service.gov.uk/media/68caac45c6df905ce77084cd/Active_TSE_surveillance_in_Great_Britian.xlsx

Active TSE Surveillance in Northern Ireland

https://assets.publishing.service.gov.uk/media/68caac5305e625519a6c9fe1/Active_TSE_Surveillance_in_Northern_Ireland.xlsx

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

Published: 28 November 2024

Adopted: 29 October 2024

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

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

CONTACT biohaw@efsa.europa.eu

Abstract

This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye.

In total, 948,165 cattle were tested by EU27 and XI (−3%, compared with 2022),

with five atypical BSE cases reported (four H‐type: two in Spain, one in France and one in Ireland; one L‐type in the Netherlands);

and 46,096 cattle by eight non‐EU reporting countries with two atypical BSE cases reported by Switzerland.

Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1).

In total, 284,686 sheep and 102,646 goats were tested in the EU27 and XI (−3.5% and −5.9%, respectively, compared to 2022).

In the other non‐EU reporting countries 26,047 sheep and 589 goats were tested.

In sheep, 538 cases of scrapie were reported by 14 MS and XI:

462 classical scrapie (CS) by 4 MS (104 index cases (IC) with genotypes of susceptible groups in 93.4% of the cases),

76 atypical scrapie (AS) (76 IC) by 12 MS.

In the other non‐EU reporting countries,

Iceland reported 70 cases of CS

while Norway reported 7 cases of ovine AS.

Ovine random genotyping was reported by six MS and genotypes of susceptible groups accounted for 6.9%.

In goats, 183 cases of scrapie were reported, all from EU MS:

176 CS (47 IC) by seven MS and 7 AS (7 IC) by five MS.

Three cases in Cyprus and one in Spain were reported in goats carrying heterozygous alleles at codon 146 and 222, respectively.

In total, 2096 cervids were tested for chronic wasting disease by ten MS, none tested positive.

Norway tested 14,224 cervids with one European moose positive.

© European Food Safety Authority

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

See full report;

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

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

European Food Safety Authority (EFSA)

First published: 28 November 2023

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

Approved: 19 October 2023 Abstract

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

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

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

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

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

https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-tse-report-2022-first-published-28.html

Scotland Single case of disease confirmed in Dumfries and Galloway

Published 06 December 2024 12:45

Topic Farming and rural

Single case of disease confirmed in Dumfries and Galloway.

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

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

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

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

Agriculture Minister Jim Fairlie said:

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

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

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

Chief Veterinary Officer Sheila Voas said:

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

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

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

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

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

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

Background

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

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

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

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

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

https://bse-atypical.blogspot.com/2024/12/scotland-single-case-of-atypical-bse.html

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

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

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

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

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

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

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

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

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

Chief Veterinary Officer Sheila Voas said:

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

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

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

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

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

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

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

Background

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

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

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

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

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

Friday, October 4, 2024

another atypical bovine spongiform encephalopathy (BSE) in Ireland

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

WEDNESDAY, NOVEMBER 08, 2023

Ireland Atypical BSE confirmed November 3 2023

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

TUESDAY, NOVEMBER 14, 2023

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

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

SUNDAY, JULY 16, 2023

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

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

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

Switzerland Bovine Spongiform Encephalopathy Atypical L-Type

Switzerland - Bovine spongiform encephalopathy - Immediate notification

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

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

Monday, March 20, 2023

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

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

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

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

BRAZIL BSE START DATE 2023/01/18

BRAZIL BSE CONFIRMATION DATE 2023/02/22

BRAZIL BSE END DATE 2023/03/03

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

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

SPAIN BSE START DATE 2023/01/21

SPAIN BSE CONFIRMATION DATE 2023/02/03

SPAIN BSE END DATE 2023/02/06

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

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

NETHERLANDS BSE START DATE 2023/02/01

NETHERLANDS BSE CONFIRMATION DATE 2023/02/01

NETHERLANDS BSE END DATE 2023/03/13

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

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

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

Wednesday, May 24, 2023

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

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

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

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

ATYPICAL CASE OF BSE DETECTED IN SOUTH CAROLINA

May 19, 2023 By Meghan Grebner Filed Under: Beef, Human Interest, Livestock, News

BeefPacker.jpg 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. 


https://usbiotechnologyregulation.mrp.usda.gov/wcm/connect/aphis_content_library/sa_newsroom/sa_stakeholders/sa_by_date/sa-2023/bse

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

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

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

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

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

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

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

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.

https://www.daera-ni.gov.uk/news/animal-feed-company-convicted-ballymena-court

“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.

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

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

Atypical BSE in cattle

THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.

Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.

Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.

Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous

VET RECORD | 29 March–12 April 2025

disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.

The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8

Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.

In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.

Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.

Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.

“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”

Timm Konold, TSE lead scientist

Brenda Rajanayagam, workgroup leader for the data systems group

APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk

Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX

References

1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)

Atypical BSE In Cattle

https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc

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

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

Aims:

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

Material and Methods:

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

Results:

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

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

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

Conclusions:

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

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

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.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.

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

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

34 Scientific Commission/September 2019

3. Atypical BSE

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

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

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

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

https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article

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

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

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

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

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.

https://pubmed.ncbi.nlm.nih.gov/21266763/

see full text;

https://www.niid.go.jp/niid/images/JJID/64/81.pdf

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

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

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

'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.

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

US Report, Scrapie, CWD, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026

*** 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 ***

snip...

http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf

see;

https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html

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.

snip...

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.

http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf

see;

https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html

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

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

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

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

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

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

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

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

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

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

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

PRION 2015 CONFERENCE

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

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.

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

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.

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

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

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 BSEBovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation

https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2511933#d1e1606

“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.

https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/Final%20NP103%20FY2024%20Annual%20Report.updated%205.30.25.pdf

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.

DOI: 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.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351

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.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351

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.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=326785

THURSDAY, JUNE 5, 2025

World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?

https://bovineprp.blogspot.com/2025/06/world-organisation-for-animal-health.html

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

Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada. *Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca

Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.

Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.

Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.

Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.

Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.

Presentation Type: Oral Presentation Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute

Grant Number: ALMA/APRI: 201400006, HC 414250

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

"After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE. "

=====end

PRION 2023 CONTINUED;

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

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

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

cwd to pigs

WEDNESDAY, JANUARY 28, 2026

Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak

https://journals.asm.org/doi/10.1128/mbio.01800-25

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

https://transmissiblespongiformencephalopathy.blogspot.com/2026/01/chronic-wasting-disease-prions-in.html

cwd to sheep

Chronic Wasting Disease CWD vs Scrapie TSE Prion

https://www.ars.usda.gov/research/publications/publication/?seqNo115=410511

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.

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

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

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

https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166

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.

https://www.ars.usda.gov/research/project/?accnNo=440677&fy=202

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.

snip…

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.

https://wwwnc.cdc.gov/eid/article/30/8/24-0007_article

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.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=336834

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.

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

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

http://webarchive.nationalarchives.gov.uk/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

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.

https://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

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

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

USA FDA PART 589 SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED, CWD, Scrapie, BSE, Oh My, 2026

https://prpsc.proboards.com/thread/202/usa-fda-589-feed-broken

https://madcowfeed.blogspot.com/2026/01/usa-fda-part-589-substances-prohibited.html

SATURDAY, APRIL 11, 2026

Chronic Wasting Disease CWD TSE PrP, Cervid, Genetic Manipulation, Unforeseen Circumstances

https://chronic-wasting-disease.blogspot.com/2026/04/chronic-wasting-disease-cwd-tse-prp.html

TUESDAY, APRIL 07, 2026

APHIS USDA Captive CWD Herds Update by State March 2026

https://chronic-wasting-disease.blogspot.com/2026/04/aphis-usda-captive-cwd-herds-update-by.html

Scrapie, CWD, BSE, CJD, TSE, PrP Update 2026

***> CWD Action Plan National Program 103 Animal Health 2022-2027 UPDATE JANUARY 2026

https://prpsc.proboards.com/thread/189/action-national-program-animal-health

https://chronic-wasting-disease.blogspot.com/2026/01/cwd-action-plan-national-program-103.html

***> SCRAPIE TSE Prion USA RAPID RESPONSE URGENT UPDATES DECEMBER 25, 2025

***> CWD vs Scrapie Urgent Update

https://scrapie-usa.blogspot.com/2025/12/scrapie-tse-prion-usa-rapid-response.html

https://prpsc.proboards.com/thread/186/scrapie-prion-response-urgent-updates

***> 2026 USDA EXPLANATORY NOTES, APHIS, CWD, BSE, Scrapie, TSE, Prion

https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/2026-usda-explanatory-notes-aphis-cwd.html

USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025 and history there from

https://www.researchgate.net/publication/396084947_USDA_National_Scrapie_Program_History_and_Bovine_Spongiform_Encephalopathy_BSE_TSE0AUpdate_2

TUESDAY, JANUARY 20, 2026

Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases Project Number 5P01AI077774-14 2025

https://chronic-wasting-disease.blogspot.com/2026/01/pathogenesis-transmission-and-detection.html

TUESDAY, SEPTEMBER 30, 2025

USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025

https://bovineprp.blogspot.com/2025/09/usda-national-scrapie-program-history.html

https://scrapie-usa.blogspot.com/2025/09/usda-national-scrapie-program-history.html

Cattle with the E211K polymorphism, and gCJD linked to a glutamic acid to lysine substitution at codon 200 (E200K) of PRNP, what if?

https://creutzfeldt-jakob-disease.blogspot.com/2026/01/cattle-with-e211k-polymorphism-and-gcjd.html

Cattle with the E211K vs Humans E200K of PRNP, what if?

https://prpsc.proboards.com/thread/195/cattle-e211k-humans-e200k-prnp

US Report, Scrapie, CWD, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026


SUNDAY, APRIL 12, 2026 

Chronic Wasting Disease in Farmed Cervids, South Korea, 2001–2024

https://chronic-wasting-disease.blogspot.com/2026/04/chronic-wasting-disease-in-farmed.html

Wednesday, April 1, 2026

First identification of camel prion disease in Tataouine, Tunisia: an emerging animal prion disease in North Africa

https://camelusprp.blogspot.com/2026/04/first-identification-of-camel-prion.html

SUNDAY, MARCH 8, 2026

Texas Creutzfeldt-Jakob Disease Deaths and Death Rates per Year (2013-2022) More Than Tripled, and case reporting has ceased since then

https://cjdtexas.blogspot.com/2026/03/texas-creutzfeldt-jakob-disease-deaths.html

https://prpsc.proboards.com/thread/209/texas-cases-more-triples-2013

WEDNESDAY, OCTOBER 15, 2025

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025

https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html

FRIDAY, NOVEMBER 21, 2025

While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists

https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html

SATURDAY, JANUARY 10, 2026

Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease, a review

https://creutzfeldt-jakob-disease.blogspot.com/2026/01/neuropsychiatric-symptoms-in-sporadic.html

https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review

SUNDAY, MARCH 23, 2025

Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update

https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html

FRIDAY, DECEMBER 13, 2024

Creutzfeldt Jacob Disease CJD, BSE, CWD, TSE Prion, December 14, 2024 Annual Update

https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html

Friendly Fire, unforeseen circumstances, iatrogenic Transmissible Spongiform Encephalopathy 

The Eyes are the windows to Our Souls, and a Potential Pathway for the TSE Prion disease, what if?



wasted days and wasted nights…Freddy Fender

Terry S. Singeltary Sr. Bacliff, Texas 77518 flounder9@verizon.net

posted by Terry S. Singeltary Sr. at 12:16 PM

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