Spain officially launches several animal disease programs for 2026 BSE and Scrapie
Spain officially launches several animal disease programs for 2026
The Ministry of Agriculture has published various national programs with measures for the surveillance, control and eradication of animal diseases in Spain during 2026.
BOVINE SPONGIFORM ENCEPHALOPATHY AND SCRAPIE
In addition, the MAPA has made public the programs corresponding to transmissible spongiform encephalopathies, such as the National Multiannual Program for Surveillance, Control and Eradication of Bovine Spongiform Encephalopathy (BSE) for the year 2026.
Along the same lines, the Ministry has published the multi-year program for the surveillance, control and eradication of scrapie for 2026, which indicates that since 2001 more than one million small ruminants have been analyzed and that, in total, 665 outbreaks have been detected from 2000 to 2024.
These programs are framed within the Community regulations on animal health and surveillance , with references in the documents themselves to Regulation (EU) 2016/429 and Delegated Regulation (EU) 2020/689, as well as to Regulation (EC) No 999/2001 in the case of transmissible spongiform encephalopathies.
https://www.animalshealth.es/ganaderia/newscastle-gripe-aviar-encefalopatias-espongiformes-espana-hace-oficiales-varios-programas-de-enfermedades-animales-2026
PROGRAMA NACIONAL PLURIANUAL DE VIGILANCIA CONTROL Y ERRADICACIÓN DE LA EEB (AÑO 2026)
https://www.animalshealth.es/fileuploads/user/PDF/2026/Enero/Programa%20de%20Vigilancia%20encefalopatia%20espongiforme%20bovina%202026.pdf
PROGRAMA NACIONAL PLURIANUAL DE VIGILANCIA CONTROL Y ERRADICACIÓN DE LA ENCEFALOPATÍA ESPONGIFORME DE LOS PEQUEÑOS RUMIANTES (SCRAPIE) (AÑO 2026)
https://www.animalshealth.es/fileuploads/user/PDF/2026/Enero/Programa%20de%20vigilancia%20scrapie%202026.pdf
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
MONDAY, APRIL 19, 2021
OIE WAHIS Spain confirm bovine spongiform encephalopathy (BSE)
https://bovineprp.blogspot.com/2021/04/oie-wahis-spain-confirm-bovine.html
TUESDAY, JULY 16, 2019
Spain Bovine Spongiform Encephalopathy BSE TSE Prion July 5 2019
https://bovineprp.blogspot.com/2019/07/spain-bovine-spongiform-encephalopathy.html
FRIDAY, MAY 12, 2017
SPAIN OIE Bovine Spongiform Encephalopathy atypical L-type Camargo, CANTABRIA
http://bse-atypical.blogspot.com/2017/05/spain-oie-bovine-spongiform.html
FRIDAY, MARCH 10, 2017
OIE Spain Prion (Atypical BSE type L) Bovine Spongiform Encephalopathy Mad Cow Disease
http://bse-atypical.blogspot.com/2017/03/oie-spain-prion-atypical-bse-type-l.html
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm
http://creutzfeldt-jakob-disease.blogspot.com/2009/01/mad-cow-disease-detected-on-madrid-farm.html
SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma) SEACAgenda 102nd Meeting on Wednesday 4 March 2009 Room 808, Nobel House, 17 Smith Square, Defra, London SW1P 3JR10.05 Approval of draft minutes from SEAC 101
snip...
ITEM 3 - CURRENT ISSUES 8. SEAC was informed about the following issues: . A mother and son in Spain had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. As both the mother and son lived in a region of Spain with a history of BSE, had frequently shared meals of cattle brain, and as no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure. Furthermore, the similar times of onset of disease of the cases did not suggest transmission had occurred from one to the other.
snip...
Thursday, February 26, 2009
SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)
http://seac992007.blogspot.com/2009/02/seac-102nd-meeting-on-wednesday-4-march.html
STATEMENT ON A VARIANT CJD FAMILY CLUSTER
Prof Robert G Will,NCJDSU 25th September2008r.g.will@ed.a c.uk
Three cases of pathologically confirmed variant CJD have been identified in Spain in recent years, including a man in his early 40s who died earlier this year. The clinical illness in this individual was typical of variant CJD, including the appearances on the MRI brain scan.
A few months ago his mother, who was in her 60s, developed a rapidly progressive neurological illness and died about 5 months from the onset of this illness. An MRI brain scan showed appearances suggestive of variant CJD and preliminary results from post-mortem examination suggest that the suspected diagnosis of variant CJD is correct. Further results, which may confirm this diagnosis, should be available within a few days.
Since 1994 there have been 167 cases of variant CJD in the UK, 23 cases in France and 15 cases in other countries, excluding Spain. The occurrence of variant CJD in more than one member of the same family has not been seen before and it has been the general view that family members of variant CJD cases are not themselves at greater risk of developing this condition. This raises the question as to why two cases of variant CJD have now been found in a family in Spain. There is no evidence of a genetic form of CJD in these Spanish cases and preliminary investigation has not shown any risk of CJD through medical or surgical treatment.
There is no evidence of any risk of transmission of CJD through direct personal contact. The mother and son lived in an area of Spain in which BSE has been found and it is possible that direct consumption of material with high levels of BSE infection may have been the source of the infection. In the UK and other countries it is believed that processed bovine tissues were the most likely source of BSE infection and it is possible that different forms of exposure to BSE infection may explain the occurrence of variant CJD in two family members in Spain and not elsewhere.
This, however, is uncertain and public health policies in relation to variant CJD may have to be reviewed in the light of these two cases in Spain.
http://www.scribd.com/doc/6224550/Memorandum-on-a-Variant-CJD-Family-Cluster
Monday, September 01, 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
http://creutzfeldt-jakob-disease.blogspot.com/2008/09/two-cases-of-variant-creutzfeldt-jakob.html
Emerg Infect Dis actions 2017 Sep;23(9):1593-1596. doi: 10.3201/eid2309.170159.
Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case
Abigail B Diack, Aileen Boyle, Diane Ritchie, Chris Plinston, Dorothy Kisielewski, Jesús de Pedro-Cuesta, Alberto Rábano, Robert G Will, Jean C Manson PMID: 28820380 PMCID: PMC5572887 DOI: 10.3201/eid2309.170159 Full text links Cite Abstract
We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears to be responsible for all vCJD cases to date.
Keywords: BSE; Spain; TSE; United Kingdom; mouse study; prions; strain; transmissible spongiform encephalopathy; vCJD; variant Creutzfeldt-Jakob disease.
Spanish woman and son could be first relatives to die of BSE
Submitted by Marina Dimova on Wed, 08/27/2008 - 11:53.
The Spanish health authorities are investigating the death of a woman whose son died earlier of Creutzfeldt-Jakob Disease (CJD), the human variant of bovine spongiform encephalopathy (BSE) or mad cow disease, experts said Wednesday in Madrid.
If it is confirmed that the woman and her son died from the same cause, they would be the first members of the same family in the world to succumb to CJD, neuropathologist Alberto Rabano said.
The woman, who was about 60-years-old, passed away last week in the northern city of Leon. The son died in February. Their identities were not given.
Veterinary experts said the son caught the disease by eating infected meat before 2001, when preventative measures were adopted.
http://www.braintalkcommunities.org/archives/06_11/showthread.php?t=36132
https://www.cnn.com/2008/HEALTH/conditions/09/24/mad.cow.spain/?iref=nextin
https://en.mercopress.com/2009/03/09/spain-confirms-fifth-human-death-of-mad-cow-disease
https://en.mercopress.com/2009/03/09/spain-confirms-fifth-human-death-of-mad-cow-disease
Terry S. Singeltary Sr. Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN. 21 Apr 2009. [Accessed 11 Apr 2012]. In: Monitoring the occurrence of emerging forms of CJD [blog]. Available from:
http://cjdusa.blogspot.com.es/2009/04/doctor-antonio-ruiz-villaespesa.html
snip...see full text ;
http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=20144
http://cjdusa.blogspot.com.es/2009/04/doctor-antonio-ruiz-villaespesa.html
snip...see full text ;
http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=20144
see archived url
MONDAY, SEPTEMBER 01, 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
http://creutzfeldt-jakob-disease.blogspot.com/2008/09/two-cases-of-variant-creutzfeldt-jakob.html
SUNDAY, MARCH 28, 2010
SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION
http://creutzfeldt-jakob-disease.blogspot.com/2010/03/spain-bse-nor-98-atypical-scrapie.html
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm Friday, January 9, 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/01/mad-cow-disease-detected-on-madrid-farm.html
http://creutzfeldt-jakob-disease.blogspot.ca/2009/01/mad-cow-disease-detected-on-madrid-farm.html
Eurosurveillance, Volume 10, Issue 31, 04 August 2005
Articles
Citation style for this article: Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(31):pii=2764. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2764 ;
First case of vCJD reported in Spain
Centro Nacional de Epidemiología, Instituto de Salud Carlos III, Madrid, Spain (http://cne.isciii.es/) ;
The Spanish Ministry of Health has reported the detection of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain [1].
The patient was a 26 year old woman in Madrid who died on 10 July 2005. She had no specific risk factors for vCJD (no prior blood transfusions or visits to the United Kingdom).
The patient’s symptoms began in November 2004, with rapid progression to dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal fluid. Other neurological manifestations including ataxia, dysarthria, apraxia and myoclonus appeared in early 2005. The first abnormal MRI was observed in April 2005. The patient was methionine homozygous at codon 129 of the prion protein gene (PRPN). There were no identified PRPN mutations or family history of CJD.
The case was notified to the Spanish CJD state registry in May 2005 and initially fitted the criteria for both probable sporadic CJD (sCJD) and possible vCJD; it was registered as probable sCJD. Brain neuropathology results of tests conducted at Fundación Hospital de Alcorcón at Madrid subsequently confirmed vCJD. Materials have been sent to the European reference centre at the United Kingdom’s National CJD Surveillance Unit in Edinburgh for further study, and results are expected later in August.
References:
Ministerio de Sanidad y Consumo. La Comunidad de Madrid comunica al Ministerio de Sanidad la probable detección del primer caso de la variante de la enfermedad de Creutzfeldt-Jakob en España. Press release 29 July 2005. (http://www.msc.es/gabinetePrensa/notaPrensa/desarrolloNotaPrensa.jsp?id=385)
http://www.eurosurveillance.org/ViewArticle.aspx?PublicationType=W&Volume=10&Issue=31&OrderNumber=1
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
https://efsaopinionbseanimalprotein.blogspot.com/2025/11/the-european-union-summary-report-on.html
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2023 European Food Safety Authority (EFSA) First published: 28 November 2024
https://doi.org/10.2903/j.efsa.2024.9097DigitalObject Identifier (DOI) Approved: 29 October 2024
The declarations of interest of all scientific experts active in EFSA’s work are available at https://open.efsa.europa.eu/experts
AMENDMENT: A number of minor editorial corrections have been included in the republished version. Amendments have been made to Tables 5, 12, 14, 32, and 33; Figures 1 and 5; and in the body of the text in sections 3.2 and 3.2.1.1. These editorial corrections do not materially affect the outcome or the conclusions of this scientific output. To avoid confusion, the original version of the output has been removed from the EFSA Journal, but is available upon request. Amended: 3 February 2025 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.
snip…
SUMMARY This report of the European Food Safety Authority (EFSA) presents the detailed results of surveillance activities on animal transmissible spongiform encephalopathies (TSE) carried out during 2023 in the European Union (EU) Member States (MS), in the United Kingdom (in respect of Northern Ireland) (hereafter: ‘XI’) and in other eight non-EU reporting countries: Bosnia and Herzegovina,1 Iceland, Montenegro, North Macedonia,2 Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye, as well as genotyping data in sheep and goats. Albania and Kosovo3 confirmed no TSE surveillance was conducted in 2023.
TSE monitoring data for cattle, sheep, goats, cervids and species other than domestic ruminants are reported by country according to Regulation (EC) 999/2001 (the TSE Regulation) and consist of testing and case data. Surveillance data were submitted through the EFSA TSE data reporting tool by 29 reporting countries. Seven MS submitted data directly as extensible markup language (XML) files by using their own system for the automatic upload of data into the EFSA Data Collection Framework (DCF). The electronically submitted data from the EFSA database were further processed, validated and extracted to draft the summary tables presented in the current EU summary report (EUSR).
As in 2022, the 2023 data of EU and XI have been compared with those of the previous years for the EU and the United Kingdom. That might introduce some bias, mainly in the 10-year trend analysis. In this report the 2023 EU27 data (i.e. data from the current 27 EU MS, referred to in the report as ‘EU27’) have been summed up with those provided by XI. However, all tables present separately the EU27 totals and those including EU27 data plus XI. Totals obtained from the three European Free Trade Association (EFTA) countries (Iceland, Norway and Switzerland (the data reported by Switzerland include those of Liechtenstein)) and the five non-EFTA IPA (Instrument for Pre-Accession Countries) (Bosnia and Herzegovina, Montenegro, North Macedonia, Serbia and Türkiye) were referred to as ‘non-EU reporting countries’ in the text and shortened in the Tables to ‘other non-EU’, for brevity of expression.
In total, 948,165 cattle were tested in 2023 in the EU27 and XI, with a decrease of 3% on the previous year. The 87.3% of all cattle tested in the EU27 and XI was reported to the group of risk animals (emergency slaughtered animals (ES), animals with clinical signs at ante-morteminspection (AM) and fallen stock (FS)), with FS being the largest contributor with 761,696 cattle tested in 2023 (92% of all cattle in the risk group). An additional 46,096 cattle were tested by the eight other non-EU reporting countries. Serbia (the main contributor with 14,031 cattle tested) reported mostly cattle from the healthy slaughtered (HS) target group while Switzerland with 11,376, including the data of Liechtenstein reported mostly cattle from animals in the risk group.
In the EU27 and XI, five atypical BSE cases in the FS testing group were reported in 2023. Four cases were H-type (two in Spain, one in France and one in Ireland) and one was L-type (in the Netherlands). Two atypical BSE cases (L-type) were reported by Switzerland. Three additional atypical cases were reported in the rest of the world in 2023: one L-type (one in the USA) and two H-type (one in the UK and one in Brazil).
In total, 387,332 small ruminants were tested in 2023 in the EU27 and XI: 284,686 sheep (a 3.5% decrease compared to 2022) and 102,646 goats (a 5.9% decrease). In addition, 26,047 sheep were tested by five of the eight other non-EU reporting countries: Iceland, North Macedonia, Norway, Serbia and Türkiye, and 589 goats were tested by Iceland, Norway, Serbia and Türkiye.
In sheep, 538 scrapie cases were reported in the EU27 (14 MS) and XI in 2023, 19 less cases than in 2022. In total, 462 ovine cases in the EU27 and XI were CS (85.9%), 76 cases were AS (14.1%). Among the five other non-EU reporting countries that tested sheep, Iceland reported 70 cases of classical scrapie (CS) while Norway reported 7 cases of atypical scrapie (AS). CS was reported only by four MS (Greece, Italy, Romania and Spain) and one non-EU country (Iceland). AS was reported by 12 EU reporting countries (Austria, Belgium, Finland, France, Germany, Hungary, Italy, Poland, Portugal, Slovenia, Spain and Sweden). Among non-EU reporting countries, only Norway reported the presence of AS.
In sheep, 180 (33.5%) of all cases in the EU27 and XI reported in 2023 were index cases (IC), with a much higher proportion in AS cases (100%) compared with CS cases (22.5%). In total, 93.4% of the CS cases in sheep reported in 2023 with known genotypes belonged to animals holding genotypes of the susceptible groups (NSP3, NSP3O, NSP4 or NSP5).
In 2023, the random genotyping of the national EU sheep populations was carried out by six MS: Belgium, France, Germany, Italy, the Netherlands and Poland. After excluding Cyprus, 6.9% of the randomly genotyped sheep with known genotypes still carried those of the susceptible groups, lower than the 7.3% in 2022. This percentage stands at 18.3% in Italy, one of the countries with high case load in 2023.
In goats, in total 183 scrapie cases were reported in the EU27 and XI: 176 CS (96.2%, with Cyprus accounting for 35% of these) and 7 were AS cases (3.8%). Seven MS (Bulgaria, Cyprus, Greece, Italy, Portugal, Romania and Spain) reported CS, whereas five MS (France, Germany, Italy, Portugal and Spain) reported AS. The four other non-EU reporting countries (Iceland, Norway, Serbia and Türkiye) that reported tested goats did not report any scrapie cases. In goats, 29.5% (54) of all cases reported in the EU27 and XI in 2023 were IC, representing an increase from 2022 (22.3%). The proportion of IC cases in AS (100%) was higher than in CS (26.7%).
Genotype of goat cases at either codon 146 or 222 were reported in 135 cases (4 AS, 131 CS), mostly by Cyprus (64), Greece (58), Italy (10) and Spain (31). Three cases of CS reported by Cyprus were heterozygous goats at codon 146: one DN and two NS, respectively. In Spain, one case was heterozygous at codon 222 (KQ).
With regard to long-term trends (cases per 10,000 tests), the analysis up to 2023 confirmed the 10-year statistically significant decrease in sheep for AS (4% annually) while a trend was not significant for CS. No detectable trend was found in goats for AS while a statistically significant increasing trend was shown for caprine CS.
In 2023 2096 cervids where tested for chronic wasting disease (CWD) by 10 MS (54.4% of them tested by Romania). No cases have been detected. The hunted/slaughtered fit for human consumption (HSHC) target group was the most tested group with 67.5% of all tested cervids. Norway tested 14,224 animals, leading to the detection of one case in a wild European moose. Additionally, Iceland and Serbia reported testing of 46 and 180 cervids respectively, which were all negative. In total, 142 animals of other species were TSE tested by Finland: 47 domestic cats, 49 American minks, 35 foxes and 11 raccoon dogs. None of them tested positive.
Two interactive communication tools on TSE – a story map (click_this_link), providing general information on TSEs, and a dashboard (click_this_link) to search and visualise the surveillance data from EU Member States and other reporting countries, have been updated with the data of the reporting year.
1 INTRODUCTION
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
http://creutzfeldt-jakob-disease.blogspot.com/2008/09/two-cases-of-variant-creutzfeldt-jakob.html
SUNDAY, MARCH 28, 2010
SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION
http://creutzfeldt-jakob-disease.blogspot.com/2010/03/spain-bse-nor-98-atypical-scrapie.html
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm Friday, January 9, 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/01/mad-cow-disease-detected-on-madrid-farm.html
http://creutzfeldt-jakob-disease.blogspot.ca/2009/01/mad-cow-disease-detected-on-madrid-farm.html
Eurosurveillance, Volume 10, Issue 31, 04 August 2005
Articles
Citation style for this article: Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(31):pii=2764. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2764 ;
First case of vCJD reported in Spain
Centro Nacional de Epidemiología, Instituto de Salud Carlos III, Madrid, Spain (http://cne.isciii.es/) ;
The Spanish Ministry of Health has reported the detection of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain [1].
The patient was a 26 year old woman in Madrid who died on 10 July 2005. She had no specific risk factors for vCJD (no prior blood transfusions or visits to the United Kingdom).
The patient’s symptoms began in November 2004, with rapid progression to dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal fluid. Other neurological manifestations including ataxia, dysarthria, apraxia and myoclonus appeared in early 2005. The first abnormal MRI was observed in April 2005. The patient was methionine homozygous at codon 129 of the prion protein gene (PRPN). There were no identified PRPN mutations or family history of CJD.
The case was notified to the Spanish CJD state registry in May 2005 and initially fitted the criteria for both probable sporadic CJD (sCJD) and possible vCJD; it was registered as probable sCJD. Brain neuropathology results of tests conducted at Fundación Hospital de Alcorcón at Madrid subsequently confirmed vCJD. Materials have been sent to the European reference centre at the United Kingdom’s National CJD Surveillance Unit in Edinburgh for further study, and results are expected later in August.
References:
Ministerio de Sanidad y Consumo. La Comunidad de Madrid comunica al Ministerio de Sanidad la probable detección del primer caso de la variante de la enfermedad de Creutzfeldt-Jakob en España. Press release 29 July 2005. (http://www.msc.es/gabinetePrensa/notaPrensa/desarrolloNotaPrensa.jsp?id=385)
http://www.eurosurveillance.org/ViewArticle.aspx?PublicationType=W&Volume=10&Issue=31&OrderNumber=1
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
https://efsaopinionbseanimalprotein.blogspot.com/2025/11/the-european-union-summary-report-on.html
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2023 European Food Safety Authority (EFSA) First published: 28 November 2024
https://doi.org/10.2903/j.efsa.2024.9097DigitalObject Identifier (DOI) Approved: 29 October 2024
The declarations of interest of all scientific experts active in EFSA’s work are available at https://open.efsa.europa.eu/experts
AMENDMENT: A number of minor editorial corrections have been included in the republished version. Amendments have been made to Tables 5, 12, 14, 32, and 33; Figures 1 and 5; and in the body of the text in sections 3.2 and 3.2.1.1. These editorial corrections do not materially affect the outcome or the conclusions of this scientific output. To avoid confusion, the original version of the output has been removed from the EFSA Journal, but is available upon request. Amended: 3 February 2025 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.
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SUMMARY This report of the European Food Safety Authority (EFSA) presents the detailed results of surveillance activities on animal transmissible spongiform encephalopathies (TSE) carried out during 2023 in the European Union (EU) Member States (MS), in the United Kingdom (in respect of Northern Ireland) (hereafter: ‘XI’) and in other eight non-EU reporting countries: Bosnia and Herzegovina,1 Iceland, Montenegro, North Macedonia,2 Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye, as well as genotyping data in sheep and goats. Albania and Kosovo3 confirmed no TSE surveillance was conducted in 2023.
TSE monitoring data for cattle, sheep, goats, cervids and species other than domestic ruminants are reported by country according to Regulation (EC) 999/2001 (the TSE Regulation) and consist of testing and case data. Surveillance data were submitted through the EFSA TSE data reporting tool by 29 reporting countries. Seven MS submitted data directly as extensible markup language (XML) files by using their own system for the automatic upload of data into the EFSA Data Collection Framework (DCF). The electronically submitted data from the EFSA database were further processed, validated and extracted to draft the summary tables presented in the current EU summary report (EUSR).
As in 2022, the 2023 data of EU and XI have been compared with those of the previous years for the EU and the United Kingdom. That might introduce some bias, mainly in the 10-year trend analysis. In this report the 2023 EU27 data (i.e. data from the current 27 EU MS, referred to in the report as ‘EU27’) have been summed up with those provided by XI. However, all tables present separately the EU27 totals and those including EU27 data plus XI. Totals obtained from the three European Free Trade Association (EFTA) countries (Iceland, Norway and Switzerland (the data reported by Switzerland include those of Liechtenstein)) and the five non-EFTA IPA (Instrument for Pre-Accession Countries) (Bosnia and Herzegovina, Montenegro, North Macedonia, Serbia and Türkiye) were referred to as ‘non-EU reporting countries’ in the text and shortened in the Tables to ‘other non-EU’, for brevity of expression.
In total, 948,165 cattle were tested in 2023 in the EU27 and XI, with a decrease of 3% on the previous year. The 87.3% of all cattle tested in the EU27 and XI was reported to the group of risk animals (emergency slaughtered animals (ES), animals with clinical signs at ante-morteminspection (AM) and fallen stock (FS)), with FS being the largest contributor with 761,696 cattle tested in 2023 (92% of all cattle in the risk group). An additional 46,096 cattle were tested by the eight other non-EU reporting countries. Serbia (the main contributor with 14,031 cattle tested) reported mostly cattle from the healthy slaughtered (HS) target group while Switzerland with 11,376, including the data of Liechtenstein reported mostly cattle from animals in the risk group.
In the EU27 and XI, five atypical BSE cases in the FS testing group were reported in 2023. Four cases were H-type (two in Spain, one in France and one in Ireland) and one was L-type (in the Netherlands). Two atypical BSE cases (L-type) were reported by Switzerland. Three additional atypical cases were reported in the rest of the world in 2023: one L-type (one in the USA) and two H-type (one in the UK and one in Brazil).
In total, 387,332 small ruminants were tested in 2023 in the EU27 and XI: 284,686 sheep (a 3.5% decrease compared to 2022) and 102,646 goats (a 5.9% decrease). In addition, 26,047 sheep were tested by five of the eight other non-EU reporting countries: Iceland, North Macedonia, Norway, Serbia and Türkiye, and 589 goats were tested by Iceland, Norway, Serbia and Türkiye.
In sheep, 538 scrapie cases were reported in the EU27 (14 MS) and XI in 2023, 19 less cases than in 2022. In total, 462 ovine cases in the EU27 and XI were CS (85.9%), 76 cases were AS (14.1%). Among the five other non-EU reporting countries that tested sheep, Iceland reported 70 cases of classical scrapie (CS) while Norway reported 7 cases of atypical scrapie (AS). CS was reported only by four MS (Greece, Italy, Romania and Spain) and one non-EU country (Iceland). AS was reported by 12 EU reporting countries (Austria, Belgium, Finland, France, Germany, Hungary, Italy, Poland, Portugal, Slovenia, Spain and Sweden). Among non-EU reporting countries, only Norway reported the presence of AS.
In sheep, 180 (33.5%) of all cases in the EU27 and XI reported in 2023 were index cases (IC), with a much higher proportion in AS cases (100%) compared with CS cases (22.5%). In total, 93.4% of the CS cases in sheep reported in 2023 with known genotypes belonged to animals holding genotypes of the susceptible groups (NSP3, NSP3O, NSP4 or NSP5).
In 2023, the random genotyping of the national EU sheep populations was carried out by six MS: Belgium, France, Germany, Italy, the Netherlands and Poland. After excluding Cyprus, 6.9% of the randomly genotyped sheep with known genotypes still carried those of the susceptible groups, lower than the 7.3% in 2022. This percentage stands at 18.3% in Italy, one of the countries with high case load in 2023.
In goats, in total 183 scrapie cases were reported in the EU27 and XI: 176 CS (96.2%, with Cyprus accounting for 35% of these) and 7 were AS cases (3.8%). Seven MS (Bulgaria, Cyprus, Greece, Italy, Portugal, Romania and Spain) reported CS, whereas five MS (France, Germany, Italy, Portugal and Spain) reported AS. The four other non-EU reporting countries (Iceland, Norway, Serbia and Türkiye) that reported tested goats did not report any scrapie cases. In goats, 29.5% (54) of all cases reported in the EU27 and XI in 2023 were IC, representing an increase from 2022 (22.3%). The proportion of IC cases in AS (100%) was higher than in CS (26.7%).
Genotype of goat cases at either codon 146 or 222 were reported in 135 cases (4 AS, 131 CS), mostly by Cyprus (64), Greece (58), Italy (10) and Spain (31). Three cases of CS reported by Cyprus were heterozygous goats at codon 146: one DN and two NS, respectively. In Spain, one case was heterozygous at codon 222 (KQ).
With regard to long-term trends (cases per 10,000 tests), the analysis up to 2023 confirmed the 10-year statistically significant decrease in sheep for AS (4% annually) while a trend was not significant for CS. No detectable trend was found in goats for AS while a statistically significant increasing trend was shown for caprine CS.
In 2023 2096 cervids where tested for chronic wasting disease (CWD) by 10 MS (54.4% of them tested by Romania). No cases have been detected. The hunted/slaughtered fit for human consumption (HSHC) target group was the most tested group with 67.5% of all tested cervids. Norway tested 14,224 animals, leading to the detection of one case in a wild European moose. Additionally, Iceland and Serbia reported testing of 46 and 180 cervids respectively, which were all negative. In total, 142 animals of other species were TSE tested by Finland: 47 domestic cats, 49 American minks, 35 foxes and 11 raccoon dogs. None of them tested positive.
Two interactive communication tools on TSE – a story map (click_this_link), providing general information on TSEs, and a dashboard (click_this_link) to search and visualise the surveillance data from EU Member States and other reporting countries, have been updated with the data of the reporting year.
1 INTRODUCTION
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https://efsa.onlinelibrary.wiley.com/doi/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
============================================
***> USA Testing ONLY Less Than 25k a year for BSE! <*** ============================================
***> SCRAPIE TSE Prion USA RAPID RESPONSE URGENT UPDATES DECEMBER 25, 2025
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
***> 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
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
2025 Annual Report
https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/ars-research-elucidating-pathobiology.html
APHIS USDA Captive CWD Herds Update by State December 2025 Update
https://chronic-wasting-disease.blogspot.com/2025/12/aphis-usda-captive-cwd-herds-update-by.html
https://prpsc.proboards.com/thread/187/aphis-captive-herds-update-december
TUESDAY, SEPTEMBER 30, 2025
USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE 2025-2014 CHRONIC WASTING DISEASE CWD TSE CERVID
https://chronic-wasting-disease.blogspot.com/2025/09/usda-explanatory-notes-animal-and-plant.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
TUESDAY, SEPTEMBER 30, 2025
USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025
https://scrapie-usa.blogspot.com/2025/09/usda-national-scrapie-program-history.html
White-tailed deer population declines in a high-prevalence chronic wasting disease region of Arkansas, USA
Heather E. Gaya , Marcelo H. Jorge, Lisa A. Jorge, Mark G. Ruder, Gino J. D’Angelo, Richard B. Chandler, Michael J. Chamberlain
Published: January 7, 2026
https://doi.org/10.1371/journal.pone.0340070
Abstract
Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy affecting cervids worldwide. CWD was first detected in Arkansas in 2015 and as of August 2025 has been detected in 24 counties across the state. Within the Tier 1 CWD management zone of northern Arkansas, average apparent CWD prevalence exceeded 25% at the onset of our study in 2021. We tested the hypothesis that high prevalence of CWD negatively affects white-tailed deer population viability. We collected data from 243 camera traps and deployed GPS-collars on 131 adult deer to monitor population dynamics. Using spatial mark-resight models, we estimated density of adult deer from 2021 to 2024 at three sites across a presumed CWD gradient to assess the impacts of high CWD prevalence on deer abundance. Deer densities declined at all three study sites, at an average 17% (95% CI: 8% − 24%) decline per year. Male densities declined by an average 23% (95% CI: 5% − 31%) per year compared to 15% (95% CI: 2% − 23%) yearly declines for females. These findings suggest that CWD can negatively impact deer populations through direct reductions in density, but additional research is needed to determine if additional factors contributed to these declines. Furthermore, our findings suggest the populations we studied are not sustainable under current harvest regulations.
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Conclusion
Our findings add to a growing body of evidence that CWD can greatly alter deer populations. Given that previous research has demonstrated reductions in survival due to CWD [5], the high prevalence in our study area likely contributed to the observed population declines. However, it is possible that factors other than CWD contributed to the observed trends in deer densities. Future work in this region should document the direct effects of CWD on vital rates such as survival and fecundity to confirm the role of CWD in population declines. At present, few management tools are available to control CWD once established on the landscape. We therefore suggest that wildlife managers focus on slowing or preventing the spread of CWD to uninfected populations, taking actions that aim to stabilize or lower CWD prevalence, and collect accurate data on deer abundance to monitor when harvest limits become unsustainable.
Supporting information
Estimates of encounter rate and spatial scale parameters. Showing 1/3: pone.0340070.s001.docx
S1 Table. Estimates of encounter rate and spatial scale parameters. https://doi.org/10.1371/journal.pone.0340070.s001 (DOCX) S1 Fig. Estimated sex ratio of adult white-tailed deer. https://doi.org/10.1371/journal.pone.0340070.s002
(DOCX) S2 Fig. CWD sample prevalence and hunter harvest in Newton County, Searcy County and Arkansas’s Zone 2 harvest area. https://doi.org/10.1371/journal.pone.0340070.s003
(DOCX)
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0340070
AGFC CWD-positive deer found in Grant, Sevier counties, 2,278 Confirmed Cases To Date
CWD-positive deer found in Grant, Sevier counties
BY Randy Zellers ON 12-19-2025 LAB DATA
AGFC CWD-positive deer found in Grant, Sevier counties, 2,278 Confirmed Cases To Date
https://www.agfc.com/news/cwd-positive-deer-found-in-grant-sevier-counties/
Arkansas CWD Deer Study Final 2025
4. Objective 4 and 6 - Infection rates and population modeling
a. In 2024, CWD sample prevalence was 40% across the study area, with higher rates seen in males (65%) than in females (34%).
b. Approximately 50% of males tested positive for CWD by the age of 2.5.
c. White-tailed deer abundance in the study area declined, driven by reduced lifespans and lower lifetime reproduction.
d. If survival does not increase, this population is expected to continue to decline.
https://drive.google.com/file/d/1jN5mtvXvz7IYFDQjv4Rasrw60dGe4KMJ/view
FRIDAY, NOVEMBER 21, 2025
Chronic Wasting Disease CWD TSE Prion Herd Declines
https://chronic-wasting-disease.blogspot.com/2025/11/chronic-wasting-disease-cwd-tse-prion.html
Texas TPWD Confirms 116 More Cases CWD, Total To Date 1,215 Positives
TPWD CWD Dashboard 1,215 Total Positive Samples
https://experience.arcgis.com/experience/8f6c27330c444a19b4b57beb7ffabb8b/page/Dashboard#data_s=id%3AdataSource_3-1966d773e34-layer-10%3A2
https://chronic-wasting-disease.blogspot.com/2025/12/texas-tpwd-confirms-116-more-cases-cwd.html
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
Sara Canoyra https://orcid.org/0000-0001-6371-8122, Alba Marín-Moreno https://orcid.org/0000-0002-4023-6398, Juan Carlos Espinosa https://orcid.org/0000-0002-6719-9902, +5 , and Juan María Torres https://orcid.org/0000-0003-0443-9232jmtorres@inia.csic.esAuthors Info & Affiliations
Edited by Byron Caughey, National Institute of Allergy and Infectious Diseases (National Institutes of Health), Hamilton, MT; received January 17, 2025; accepted June 7, 2025 by Editorial Board Member Lila M. Gierasch
July 15, 2025 122 (29) e2501104122
Classical bovine spongiform encephalopathy (c-BSE) is a fatal cattle prion disease transmissible to humans as variant Creutzfeldt–Jakob Disease (vCJD). Understanding how c-BSE emerges is crucial for preventing future outbreaks and protecting public health. Two main hypotheses explain prion adaptation during cross-species transmission: “conformational shift or deformed templating,” where the species barrier forces a change to a different pathological prion protein, and “conformational selection,” where the species barrier filters preexisting conformers. Our results demonstrate that the conformational shift mechanism explains the emergence of c-BSE when Nor98/atypical scrapie (AS) is transmitted to cattle. This is significant because AS, found in sheep and goats worldwide, can convert to c-BSE. Preventing AS from entering the food chain is crucial to reduce c-BSE/vCJD risks. Abstract
Prion diseases can manifest with distinct phenotypes in a single species, a phenomenon known as prion strains. Upon cross-species transmission, alterations in the disease phenotype can occur, interpreted as the emergence of a new strain. Two main and non–mutually exclusive evolutionary hypotheses have been proposed to explain this phenomenon: the “conformational shift” or “deformed templating” and the “conformational selection.” The conformational shift hypothesis proposes that the introduction of a new host prion protein (PrPC) forces a change in the conformation of the pathological prion protein (PrPSc), causing the new prion strain emergence. On the contrary, the conformational selection model postulates that prion isolates are a conglomerate of PrPSc conformations with relative distribution frequencies, wherein the species barrier acts as a filter selecting the one fittest for the new species environment. Previous studies reported the emergence of the classical bovine spongiform encephalopathy agent (c-BSE) upon transmission of Nor98/atypical scrapie (AS) onto a bovine PrP. This study investigates the evolutionary dichotomy of this c-BSE emergence by using prion strain thermostability combined with protein misfolding cyclic amplification to distinguish between both strains. Our results suggest that the conformational shift could be the principal mechanism responsible for the c-BSE emergence. Furthermore, the selection model was dismissed as the key mechanism based on the analysis of an artificial c-BSE and AS mixture. The ability of the AS conformers to shift conformation to a c-BSE one supports the hypothesis that the epidemic c-BSE prion may have originated from the transmission of AS in cattle.
https://www.pnas.org/doi/10.1073/pnas.2501104122
Six of the seven cases of BSE identified in the United States have been diagnosed as atypical BSE. In most cases, the animals were 10 years of age or older. Two of the six detections of atypical BSE involved animals aged approximately 5 years or older.
https://www.aphis.usda.gov/sites/default/files/fs-bse.pdf
USA BSE Testing and Surveillance?
Bottom line, USA is testing so few cows for BSE (<25k tested annually)
https://www.aphis.usda.gov/livestock-poultry-disease/cattle/bse/bse-surveillance-program
BUT, even at those low testing figures, the USA did just confirm another case of BSE just here recently. Feed ban has failed terribly, and CWD is spreading in the USA, at an alarming rate. Recent transmission studies show oral transmission of CWD of Cervid to cattle. Studies also show links of sporadic CJD to BSE, Scrapie, and CWD. It’s a Whole new game of Prion poker now$$$
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
https://wahis.woah.org/#/in-review/5067
https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html
https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html
https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed
MAY 19, 2023
https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse
2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;
***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;
Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023
''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...
https://www.regulations.gov/comment/APHIS-2023-0027-0002
https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf
Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?
Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues
Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019.
Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues.
Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: https://doi.org/10.3389/fvets.2019.00430
Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.
Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305
https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665
https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668
Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.
https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0
***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***
1985
Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf
https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf
Re-USA BSE Surveillance, BSE Testing, BSE Feed Regulation (21 CFR 589.2000), SRMs, and CWD
“Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.”
Abstract for Prion 2023
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas'
1, Samuel James Sharpe', Kristina Santiago-Mateo', Stefanie Czub', Waqas Tahirl,2, *
Affiliation: National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. ?Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.
*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (C-BSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined. Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of Prpso in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized.
Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for Prpsc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of Prpsc in their brains, having biochemical properties similar to that of Prps in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation
Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
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
============================================
***> USA Testing ONLY Less Than 25k a year for BSE! <*** ============================================
***> SCRAPIE TSE Prion USA RAPID RESPONSE URGENT UPDATES DECEMBER 25, 2025
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
***> 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
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
2025 Annual Report
https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/ars-research-elucidating-pathobiology.html
APHIS USDA Captive CWD Herds Update by State December 2025 Update
https://chronic-wasting-disease.blogspot.com/2025/12/aphis-usda-captive-cwd-herds-update-by.html
https://prpsc.proboards.com/thread/187/aphis-captive-herds-update-december
TUESDAY, SEPTEMBER 30, 2025
USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE 2025-2014 CHRONIC WASTING DISEASE CWD TSE CERVID
https://chronic-wasting-disease.blogspot.com/2025/09/usda-explanatory-notes-animal-and-plant.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
TUESDAY, SEPTEMBER 30, 2025
USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025
https://scrapie-usa.blogspot.com/2025/09/usda-national-scrapie-program-history.html
White-tailed deer population declines in a high-prevalence chronic wasting disease region of Arkansas, USA
Heather E. Gaya , Marcelo H. Jorge, Lisa A. Jorge, Mark G. Ruder, Gino J. D’Angelo, Richard B. Chandler, Michael J. Chamberlain
Published: January 7, 2026
https://doi.org/10.1371/journal.pone.0340070
Abstract
Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy affecting cervids worldwide. CWD was first detected in Arkansas in 2015 and as of August 2025 has been detected in 24 counties across the state. Within the Tier 1 CWD management zone of northern Arkansas, average apparent CWD prevalence exceeded 25% at the onset of our study in 2021. We tested the hypothesis that high prevalence of CWD negatively affects white-tailed deer population viability. We collected data from 243 camera traps and deployed GPS-collars on 131 adult deer to monitor population dynamics. Using spatial mark-resight models, we estimated density of adult deer from 2021 to 2024 at three sites across a presumed CWD gradient to assess the impacts of high CWD prevalence on deer abundance. Deer densities declined at all three study sites, at an average 17% (95% CI: 8% − 24%) decline per year. Male densities declined by an average 23% (95% CI: 5% − 31%) per year compared to 15% (95% CI: 2% − 23%) yearly declines for females. These findings suggest that CWD can negatively impact deer populations through direct reductions in density, but additional research is needed to determine if additional factors contributed to these declines. Furthermore, our findings suggest the populations we studied are not sustainable under current harvest regulations.
snip…
Conclusion
Our findings add to a growing body of evidence that CWD can greatly alter deer populations. Given that previous research has demonstrated reductions in survival due to CWD [5], the high prevalence in our study area likely contributed to the observed population declines. However, it is possible that factors other than CWD contributed to the observed trends in deer densities. Future work in this region should document the direct effects of CWD on vital rates such as survival and fecundity to confirm the role of CWD in population declines. At present, few management tools are available to control CWD once established on the landscape. We therefore suggest that wildlife managers focus on slowing or preventing the spread of CWD to uninfected populations, taking actions that aim to stabilize or lower CWD prevalence, and collect accurate data on deer abundance to monitor when harvest limits become unsustainable.
Supporting information
Estimates of encounter rate and spatial scale parameters. Showing 1/3: pone.0340070.s001.docx
S1 Table. Estimates of encounter rate and spatial scale parameters. https://doi.org/10.1371/journal.pone.0340070.s001 (DOCX) S1 Fig. Estimated sex ratio of adult white-tailed deer. https://doi.org/10.1371/journal.pone.0340070.s002
(DOCX) S2 Fig. CWD sample prevalence and hunter harvest in Newton County, Searcy County and Arkansas’s Zone 2 harvest area. https://doi.org/10.1371/journal.pone.0340070.s003
(DOCX)
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0340070
AGFC CWD-positive deer found in Grant, Sevier counties, 2,278 Confirmed Cases To Date
CWD-positive deer found in Grant, Sevier counties
BY Randy Zellers ON 12-19-2025 LAB DATA
AGFC CWD-positive deer found in Grant, Sevier counties, 2,278 Confirmed Cases To Date
https://www.agfc.com/news/cwd-positive-deer-found-in-grant-sevier-counties/
Arkansas CWD Deer Study Final 2025
4. Objective 4 and 6 - Infection rates and population modeling
a. In 2024, CWD sample prevalence was 40% across the study area, with higher rates seen in males (65%) than in females (34%).
b. Approximately 50% of males tested positive for CWD by the age of 2.5.
c. White-tailed deer abundance in the study area declined, driven by reduced lifespans and lower lifetime reproduction.
d. If survival does not increase, this population is expected to continue to decline.
https://drive.google.com/file/d/1jN5mtvXvz7IYFDQjv4Rasrw60dGe4KMJ/view
FRIDAY, NOVEMBER 21, 2025
Chronic Wasting Disease CWD TSE Prion Herd Declines
https://chronic-wasting-disease.blogspot.com/2025/11/chronic-wasting-disease-cwd-tse-prion.html
Texas TPWD Confirms 116 More Cases CWD, Total To Date 1,215 Positives
TPWD CWD Dashboard 1,215 Total Positive Samples
https://experience.arcgis.com/experience/8f6c27330c444a19b4b57beb7ffabb8b/page/Dashboard#data_s=id%3AdataSource_3-1966d773e34-layer-10%3A2
https://chronic-wasting-disease.blogspot.com/2025/12/texas-tpwd-confirms-116-more-cases-cwd.html
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
Sara Canoyra https://orcid.org/0000-0001-6371-8122, Alba Marín-Moreno https://orcid.org/0000-0002-4023-6398, Juan Carlos Espinosa https://orcid.org/0000-0002-6719-9902, +5 , and Juan María Torres https://orcid.org/0000-0003-0443-9232jmtorres@inia.csic.esAuthors Info & Affiliations
Edited by Byron Caughey, National Institute of Allergy and Infectious Diseases (National Institutes of Health), Hamilton, MT; received January 17, 2025; accepted June 7, 2025 by Editorial Board Member Lila M. Gierasch
July 15, 2025 122 (29) e2501104122
Classical bovine spongiform encephalopathy (c-BSE) is a fatal cattle prion disease transmissible to humans as variant Creutzfeldt–Jakob Disease (vCJD). Understanding how c-BSE emerges is crucial for preventing future outbreaks and protecting public health. Two main hypotheses explain prion adaptation during cross-species transmission: “conformational shift or deformed templating,” where the species barrier forces a change to a different pathological prion protein, and “conformational selection,” where the species barrier filters preexisting conformers. Our results demonstrate that the conformational shift mechanism explains the emergence of c-BSE when Nor98/atypical scrapie (AS) is transmitted to cattle. This is significant because AS, found in sheep and goats worldwide, can convert to c-BSE. Preventing AS from entering the food chain is crucial to reduce c-BSE/vCJD risks. Abstract
Prion diseases can manifest with distinct phenotypes in a single species, a phenomenon known as prion strains. Upon cross-species transmission, alterations in the disease phenotype can occur, interpreted as the emergence of a new strain. Two main and non–mutually exclusive evolutionary hypotheses have been proposed to explain this phenomenon: the “conformational shift” or “deformed templating” and the “conformational selection.” The conformational shift hypothesis proposes that the introduction of a new host prion protein (PrPC) forces a change in the conformation of the pathological prion protein (PrPSc), causing the new prion strain emergence. On the contrary, the conformational selection model postulates that prion isolates are a conglomerate of PrPSc conformations with relative distribution frequencies, wherein the species barrier acts as a filter selecting the one fittest for the new species environment. Previous studies reported the emergence of the classical bovine spongiform encephalopathy agent (c-BSE) upon transmission of Nor98/atypical scrapie (AS) onto a bovine PrP. This study investigates the evolutionary dichotomy of this c-BSE emergence by using prion strain thermostability combined with protein misfolding cyclic amplification to distinguish between both strains. Our results suggest that the conformational shift could be the principal mechanism responsible for the c-BSE emergence. Furthermore, the selection model was dismissed as the key mechanism based on the analysis of an artificial c-BSE and AS mixture. The ability of the AS conformers to shift conformation to a c-BSE one supports the hypothesis that the epidemic c-BSE prion may have originated from the transmission of AS in cattle.
https://www.pnas.org/doi/10.1073/pnas.2501104122
Six of the seven cases of BSE identified in the United States have been diagnosed as atypical BSE. In most cases, the animals were 10 years of age or older. Two of the six detections of atypical BSE involved animals aged approximately 5 years or older.
https://www.aphis.usda.gov/sites/default/files/fs-bse.pdf
USA BSE Testing and Surveillance?
Bottom line, USA is testing so few cows for BSE (<25k tested annually)
https://www.aphis.usda.gov/livestock-poultry-disease/cattle/bse/bse-surveillance-program
BUT, even at those low testing figures, the USA did just confirm another case of BSE just here recently. Feed ban has failed terribly, and CWD is spreading in the USA, at an alarming rate. Recent transmission studies show oral transmission of CWD of Cervid to cattle. Studies also show links of sporadic CJD to BSE, Scrapie, and CWD. It’s a Whole new game of Prion poker now$$$
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
https://wahis.woah.org/#/in-review/5067
https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html
https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html
https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed
MAY 19, 2023
https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse
2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;
***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;
Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023
''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...
https://www.regulations.gov/comment/APHIS-2023-0027-0002
https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf
Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?
Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues
Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019.
Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues.
Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: https://doi.org/10.3389/fvets.2019.00430
Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.
Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305
https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665
https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668
Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.
https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0
***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***
1985
Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf
https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf
Re-USA BSE Surveillance, BSE Testing, BSE Feed Regulation (21 CFR 589.2000), SRMs, and CWD
“Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.”
Abstract for Prion 2023
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas'
1, Samuel James Sharpe', Kristina Santiago-Mateo', Stefanie Czub', Waqas Tahirl,2, *
Affiliation: National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. ?Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.
*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (C-BSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined. Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of Prpso in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized.
Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for Prpsc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of Prpsc in their brains, having biochemical properties similar to that of Prps in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation
Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
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.
“Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.”
=====
Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE).
Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA
Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME).
Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97).
Results: Approximately 16.6 months post-inoculation, Steer 6 (EK211 L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (EK211 L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME.
Conclusions: Further study of L-BSE in EK211 cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE.
Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE.
Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases
=====end
=====
Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE).
Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA
Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME).
Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97).
Results: Approximately 16.6 months post-inoculation, Steer 6 (EK211 L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (EK211 L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME.
Conclusions: Further study of L-BSE in EK211 cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE.
Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE.
Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases
=====end
Prion 2023 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.
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
See also;
OIE 2019 atypical BSE
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
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/
''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
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Volume 31, Number 5—May 2025
Dispatch
Administration of L-Type Bovine Spongiform Encephalopathy to Macaques to Evaluate Zoonotic Potential
Morikazu Imamura1Comments to Author , Ken’ichi Hagiwara, Minoru Tobiume, Minako Ohno, Hiromi Iguchi, Hanae Takatsuki, Tsuyoshi Mori, Ryuichiro Atarashi, Hiroaki Shibata, and Fumiko Ono1 Author affiliation: University of Miyazaki, Miyazaki, Japan (M. Imamura, M. Ohno, H. Iguchi, H. Takatsuki, T. Mori, R. Atarashi); National Institute of Infectious Diseases, Tokyo, Japan (K. Hagiwara, M. Tobiume); The Corporation for Production and Research of Laboratory Primates, Tsukuba, Japan (H. Shibata); Okayama University of Science, Imabari, Japan (F. Ono) Cite This Article
Abstract
We administered L-type bovine spongiform encephalopathy prions to macaques to determine their potential for transmission to humans. After 75 months, no clinical symptoms appeared, and prions were undetectable in any tissue by Western blot or immunohistochemistry. Protein misfolding cyclic amplification, however, revealed prions in the nerve and lymphoid tissues.
Worldwide emergence of classical bovine spongiform encephalopathy (C-BSE) is associated with variant Creutzfeldt-Jakob disease in humans (1). Two other naturally occurring BSE variants have been identified, L-type (L-BSE) and H-type. Studies using transgenic mice expressing human normal prion protein (PrPC) (2) and primates (3–5) have demonstrated that L-BSE is more virulent than C-BSE. Although L-BSE is orally transmissible to minks (6), cattle (7), and mouse lemurs (5), transmissibility to cynomolgus macaques, a suitable model for investigating human susceptibility to prions, remains unclear. We orally inoculated cynomolgus macaques with L-BSE prions and explored the presence of abnormal prion proteins (PrPSc) in tissues using protein misfolding cyclic amplification (PMCA) along with Western blot (WB) and immunohistochemistry (IHC). PMCA markedly accelerates prion replication in vitro, and its products retain the biochemical properties and transmissibility of seed prion strains (8).
The Study
Two macaques orally inoculated with L-BSE prions remained asymptomatic and healthy but were euthanized and autopsied at 75 months postinoculation. WB showed no PrPSc accumulation in any tissue (Table), IHC revealed no PrPSc accumulation, hematoxylin and eosin staining revealed no spongiform changes in brain sections, and pathologic examination revealed no abnormalities.
Snip…
Conclusion We noted no detectable evidence of PrPSc by WB or IHC in any tissues of L-BSE orally inoculated macaques. Nevertheless, PMCA successfully amplified PrPres from lymphatic and neural tissues. The PrPres exhibited electrophoretic patterns distinct from those detected by PMCA using L-BSE–affected cattle BH as the seed (Figure 3, panel C), indicating that the PrPSc used as the template for PrPres amplification in orally inoculated macaques did not originate from the bovine L-BSE prions used as inoculum. Instead, PrPSc were newly generated by the conversion of macaque PrPC by bovine L-BSE prions. Our results provide strong evidence that L-BSE can infect macaques via the oral route.
We found no evidence that PrPSc reached the brain in orally inoculated macaques; however, the macaques euthanized 6 years postinoculation might have been in the preclinical period. At low infection levels, lymph nodes play a vital role in prion spread to the central nervous system (11). Therefore, had the macaques been maintained for a longer period, they might have developed prion disease. Retrospective surveillance studies using the appendix and tonsil tissues suggested a considerable number of humans harboring vCJD in a carrier state (12). Thus, we cannot exclude that L-BSE orally inoculated macaques could similarly remain in a potentially infectious state.
The brain of L-BSE intracerebrally inoculated macaque accumulated prions with biochemical properties resembling bovine L-BSE prions (Figure 3, panel C; Appendix Figure 2); however, we observed no PrPSc accumulation in lymphoid tissues by WB or IHC (4). In contrast, macaques orally inoculated with C-BSE prions showed PrPSc accumulation in lymphoid tissues, including the spleen, tonsils, and mesenteric lymph nodes by WB and IHC (13). In our study, L-BSE orally inoculated macaques harbored C-BSE–like prions in their lymphoid and neural tissues. Interspecies transmission of L-BSE prions to ovine PrP transgenic mice can result in a shift toward C-BSE–like properties (14,15). Our data suggest that L-BSE prions may alter biophysical and biochemical properties, depending on interspecies transmission and inoculation route, acquiring traits similar to those of C-BSE prions. This transformation might result from structural changes in the L-BSE prion to C-BSE–like prions and other lymphotropic prions within lymphoid tissues or from the selective propagation of low-level lymphotropic substrains within the L-BSE prion population.
The first limitation of our study is that the oral inoculation experiment involved only 2 macaques and tissues collected at 6 years postinoculation, before disease onset. Consequently, subsequent progression of prion disease symptoms remains speculative. A larger sample size and extended observation periods are required to conclusively establish infection in orally inoculated macaques. Furthermore, we performed no bioassays for PMCA-positive samples, leaving the relationship between PMCA results and infectious titers undefined. Considering that PrPres amplifications from tissues from the orally inoculated macaque tissues required 2 rounds of PMCA, the PrPSc levels in positive tissues might have been extremely low and undetectable in the bioassay.
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Dr. Imamura is an associate professor in the Division of Microbiology, Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan. His research interests are focused on elucidating the mechanisms underlying prion formation.
Acknowledgment This study was supported by the Health Labor Sciences Research Grant (H29-Shokuhin-Ippan-004, 20KA1003, and 23KA1004).
References…snip…end
https://wwwnc.cdc.gov/eid/article/31/5/24-1257_article#r5
Monday, May 22, 2023
BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?
https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html
THURSDAY, JANUARY 08, 2026
Confucius Ponders, what about Wild Pigs (Sus scrofa) and CWD TSE Prion, and the Environment, what if?
Confucius Ponders, what about Wild Pigs (Sus scrofa), they can cover some distance rather quickly, what about Wild Pigs (Sus scrofa) digging up the terrain, and as they do it, what if these Wild Pigs (Sus scrofa) were exposed to CWD TSE Prion, and then they go on exposing and saturating the land with CWD TSE Prion, then the soil becomes contaminated with CWD TSE Prion, then what about the plants that grow from that soil for the decades to come, what if???
https://prpsc.proboards.com/thread/190/confucius-ponders-wild-pigs-scrofa
https://chronic-wasting-disease.blogspot.com/2026/01/confucius-ponders-what-about-wild-pigs.html
FRIDAY, JULY 07, 2023
TME, 589.2000 (21 C.F.R. 589.2000), atypical L-BSE, who’s testing MINK for TSE?
https://bse-atypical.blogspot.com/2023/07/tme-5892000-21-cfr-5892000-atypical-l.html
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
Terry S. Singeltary Sr
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
See also;
OIE 2019 atypical BSE
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
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/
''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
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Volume 31, Number 5—May 2025
Dispatch
Administration of L-Type Bovine Spongiform Encephalopathy to Macaques to Evaluate Zoonotic Potential
Morikazu Imamura1Comments to Author , Ken’ichi Hagiwara, Minoru Tobiume, Minako Ohno, Hiromi Iguchi, Hanae Takatsuki, Tsuyoshi Mori, Ryuichiro Atarashi, Hiroaki Shibata, and Fumiko Ono1 Author affiliation: University of Miyazaki, Miyazaki, Japan (M. Imamura, M. Ohno, H. Iguchi, H. Takatsuki, T. Mori, R. Atarashi); National Institute of Infectious Diseases, Tokyo, Japan (K. Hagiwara, M. Tobiume); The Corporation for Production and Research of Laboratory Primates, Tsukuba, Japan (H. Shibata); Okayama University of Science, Imabari, Japan (F. Ono) Cite This Article
Abstract
We administered L-type bovine spongiform encephalopathy prions to macaques to determine their potential for transmission to humans. After 75 months, no clinical symptoms appeared, and prions were undetectable in any tissue by Western blot or immunohistochemistry. Protein misfolding cyclic amplification, however, revealed prions in the nerve and lymphoid tissues.
Worldwide emergence of classical bovine spongiform encephalopathy (C-BSE) is associated with variant Creutzfeldt-Jakob disease in humans (1). Two other naturally occurring BSE variants have been identified, L-type (L-BSE) and H-type. Studies using transgenic mice expressing human normal prion protein (PrPC) (2) and primates (3–5) have demonstrated that L-BSE is more virulent than C-BSE. Although L-BSE is orally transmissible to minks (6), cattle (7), and mouse lemurs (5), transmissibility to cynomolgus macaques, a suitable model for investigating human susceptibility to prions, remains unclear. We orally inoculated cynomolgus macaques with L-BSE prions and explored the presence of abnormal prion proteins (PrPSc) in tissues using protein misfolding cyclic amplification (PMCA) along with Western blot (WB) and immunohistochemistry (IHC). PMCA markedly accelerates prion replication in vitro, and its products retain the biochemical properties and transmissibility of seed prion strains (8).
The Study
Two macaques orally inoculated with L-BSE prions remained asymptomatic and healthy but were euthanized and autopsied at 75 months postinoculation. WB showed no PrPSc accumulation in any tissue (Table), IHC revealed no PrPSc accumulation, hematoxylin and eosin staining revealed no spongiform changes in brain sections, and pathologic examination revealed no abnormalities.
Snip…
Conclusion We noted no detectable evidence of PrPSc by WB or IHC in any tissues of L-BSE orally inoculated macaques. Nevertheless, PMCA successfully amplified PrPres from lymphatic and neural tissues. The PrPres exhibited electrophoretic patterns distinct from those detected by PMCA using L-BSE–affected cattle BH as the seed (Figure 3, panel C), indicating that the PrPSc used as the template for PrPres amplification in orally inoculated macaques did not originate from the bovine L-BSE prions used as inoculum. Instead, PrPSc were newly generated by the conversion of macaque PrPC by bovine L-BSE prions. Our results provide strong evidence that L-BSE can infect macaques via the oral route.
We found no evidence that PrPSc reached the brain in orally inoculated macaques; however, the macaques euthanized 6 years postinoculation might have been in the preclinical period. At low infection levels, lymph nodes play a vital role in prion spread to the central nervous system (11). Therefore, had the macaques been maintained for a longer period, they might have developed prion disease. Retrospective surveillance studies using the appendix and tonsil tissues suggested a considerable number of humans harboring vCJD in a carrier state (12). Thus, we cannot exclude that L-BSE orally inoculated macaques could similarly remain in a potentially infectious state.
The brain of L-BSE intracerebrally inoculated macaque accumulated prions with biochemical properties resembling bovine L-BSE prions (Figure 3, panel C; Appendix Figure 2); however, we observed no PrPSc accumulation in lymphoid tissues by WB or IHC (4). In contrast, macaques orally inoculated with C-BSE prions showed PrPSc accumulation in lymphoid tissues, including the spleen, tonsils, and mesenteric lymph nodes by WB and IHC (13). In our study, L-BSE orally inoculated macaques harbored C-BSE–like prions in their lymphoid and neural tissues. Interspecies transmission of L-BSE prions to ovine PrP transgenic mice can result in a shift toward C-BSE–like properties (14,15). Our data suggest that L-BSE prions may alter biophysical and biochemical properties, depending on interspecies transmission and inoculation route, acquiring traits similar to those of C-BSE prions. This transformation might result from structural changes in the L-BSE prion to C-BSE–like prions and other lymphotropic prions within lymphoid tissues or from the selective propagation of low-level lymphotropic substrains within the L-BSE prion population.
The first limitation of our study is that the oral inoculation experiment involved only 2 macaques and tissues collected at 6 years postinoculation, before disease onset. Consequently, subsequent progression of prion disease symptoms remains speculative. A larger sample size and extended observation periods are required to conclusively establish infection in orally inoculated macaques. Furthermore, we performed no bioassays for PMCA-positive samples, leaving the relationship between PMCA results and infectious titers undefined. Considering that PrPres amplifications from tissues from the orally inoculated macaque tissues required 2 rounds of PMCA, the PrPSc levels in positive tissues might have been extremely low and undetectable in the bioassay.
Previous studies have demonstrated that L-BSE can be orally transmitted to cattle (7) and might have caused prion disease in farm-raised minks (6), indicating that L-BSE could naturally affect various animal species. Our findings suggest that L-BSE can also be orally transmitted to macaques. Therefore, current control measures aimed at preventing primary C-BSE in cattle and humans may also need to consider the potential risk of spontaneous L-BSE transmission.
Dr. Imamura is an associate professor in the Division of Microbiology, Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan. His research interests are focused on elucidating the mechanisms underlying prion formation.
Acknowledgment This study was supported by the Health Labor Sciences Research Grant (H29-Shokuhin-Ippan-004, 20KA1003, and 23KA1004).
References…snip…end
https://wwwnc.cdc.gov/eid/article/31/5/24-1257_article#r5
Monday, May 22, 2023
BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?
https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html
THURSDAY, JANUARY 08, 2026
Confucius Ponders, what about Wild Pigs (Sus scrofa) and CWD TSE Prion, and the Environment, what if?
Confucius Ponders, what about Wild Pigs (Sus scrofa), they can cover some distance rather quickly, what about Wild Pigs (Sus scrofa) digging up the terrain, and as they do it, what if these Wild Pigs (Sus scrofa) were exposed to CWD TSE Prion, and then they go on exposing and saturating the land with CWD TSE Prion, then the soil becomes contaminated with CWD TSE Prion, then what about the plants that grow from that soil for the decades to come, what if???
https://prpsc.proboards.com/thread/190/confucius-ponders-wild-pigs-scrofa
https://chronic-wasting-disease.blogspot.com/2026/01/confucius-ponders-what-about-wild-pigs.html
FRIDAY, JULY 07, 2023
TME, 589.2000 (21 C.F.R. 589.2000), atypical L-BSE, who’s testing MINK for TSE?
https://bse-atypical.blogspot.com/2023/07/tme-5892000-21-cfr-5892000-atypical-l.html
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
Terry S. Singeltary Sr
