Transmissible Spongiform Encephalopathy TSE Prion Disease UPDATE AUGUST 2025
2025 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE TSE Prion Disease
2025 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
APHIS works with State agencies to encourage cervid owners to certify their herds by meeting the requirements in the CWD Herd Certification Program (HCP) Standards. APHIS' voluntary national CWD HCP helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds. Currently, 28 States participate in the national CWD HCP. In 2023, eight percent of the farmed cervids in the HCP were tested for CWD at APHIS and State laboratories. Of the 303,242 farmed cervids tested in 2023, APHIS confirmed 22 new CWD positive farmed cervid herds. APHIS provided Federal indemnity to depopulate one of the newly identified positive herds and approved an indemnity payment for a second positive herd which will be provided in 2024 once depopulation occurs. The remaining infected herds are under State quarantines. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.
In 2023, APHIS made approximately $12.3 million available for cooperative agreements with States and Tribal governments to further develop and implement CWD surveillance, testing, management, and response activities.
This includes the further development and evaluation of techniques and strategies to prevent or control CWD in farmed and wild cervid populations. APHIS funded cooperative agreement with 22 States, 15 universities, and 11 Tribes and Tribal Organizations for CWD projects.
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Bovine spongiform encephalopathy
BSE, widely referred to as "mad cow disease," is a progressive and fatal neurologic disease of cattle. The disease is caused by a transmissible abnormal prion protein. BSE is not a contagious disease and therefore is not spread through casual contact between cattle or with other species. BSE detections are separated into 2 distinct categories, classical and atypical. Classical BSE occurs through the consumption of contaminated feed. While classical BSE was identified as a significant threat in the 1990s, most years there are no detections made worldwide. This is a result of the successful implementation of effective control measures on an international scale. Atypical BSE refers to naturally and sporadically occurring forms, which are believed to occur in all bovine populations at a very low rate, and which have only been identified in older bovines when conducting surveillance. APHIS works with the USDA Food Safety and Inspection Service and the Food and Drug Administration to conduct ongoing BSE surveillance, allowing the United States to maintain BSE Negligible Risk status per the World Organisation for Animal Health's (WOAH) standards to facilitate trade.
The WOAH evaluates countries that submit a request for disease freedom and assigns a points-based risk status for BSE. The BSE surveillance program uses WOAH's weighted surveillance points system, which reflects that the best BSE surveillance programs focus on obtaining quality samples from targeted populations rather than looking at the entire adult cattle population. The WOAH's surveillance points system also incorporates a country's history with the disease, the implementation and enforcement of cattle feed regulations, and their overall BSE surveillance. In 2023, the Agency tested for BSE in 22,835 cattle, resulting in 239,648 points, exceeding the WOAH's international surveillance standards (21,429 points per year) by 11 times. No cases of classical BSE were detected in 2023; however, an atypical BSE case was detected at slaughter as part of the BSE surveillance program. An epidemiologic investigation was conducted in accordance with the BSE Response Plan.
BSE, widely referred to as "mad cow disease," is a progressive, fatal neurologic cattle disease which is primarily spread through contaminated feed. BSE detections are separated into 2 distinct categories, classical and atypical. Classical BSE occurs through the consumption of contaminated feed. Atypical BSE refers to naturally and sporadically occurring forms, which are believed to occur in all bovine populations at a very low rate, and which have only been identified in older bovines when conducting surveillance. The World Organisation for Animal Health evaluates countries that submit disease freedom requests and established official recognition of sanitary risk status through a transparent, science-based and impartial procedure. This system uses points to ensure the BSE surveillance programs obtain quality samples from targeted populations rather than the entire adult cattle population. The system also incorporates a country's BSE history, cattle feed regulations, and surveillance practices. APHIS samples approximately 25,000 animals each year and targets cattle populations where the disease is most likely to be found. The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including cattle that cannot walk, are low weight, injured, or dead. No cases of classical BSE were detected in 2023; however, an atypical BSE case was detected at slaughter as part of the BSE surveillance program. An epidemiologic investigation was conducted in accordance with the BSE Response Plan.
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Sheep and Goat
Scrapie is a fatal, degenerative disease affecting the central nervous system of sheep and goats. Infected flocks can experience significant production losses. The National Scrapie Eradication Program (NSEP) focuses on improving the health of domestic sheep and goats, reducing scrapie-associated economic losses, and increasing international marketing opportunities. APHIS and State animal health personnel implement NSEP standards to prevent, monitor, and eradicate classical scrapie throughout the United States. All 50 States maintain a Consistent State status under NSEP, where States must conduct an active scrapie control program which is verified through Consistent State reviews. In 2023, APHIS conducted Consistent State reviews in lowa, Louisiana, Mississippi, Montana, New York, and South Dakota.
Regulatory scrapie slaughter surveillance efforts began in 2003 and were designed to identify scrapie infected flocks and herds by sampling animals at slaughter. Since the surveillance program began in 2003, the program has collected samples from approximately 745,000 animals at slaughter, and only 471 sheep have tested positive for classical scrapie. There hasn't been a classical scrapie detection since 2021. In 2023, APHIS collected samples from more than 26,000 sheep and goats for scrapie testing. Out of the total number of animals tested in 2023, no animals tested positive for classical scrapie and one sheep tested positive for non-classical scrapie (Nor98-like). Unlike classical scrapie, non-classical scrapie is either not laterally transmissible or is transmissible at a very low rate. The WOAH and APHIS determined that it is not a disease of trade concern. NSEP has a voluntary flock certification component, the Scrapie Free Flock Certification Program (SFCP).
Participation in SFCP enables producers to enhance the marketability of their animals by monitoring them for scrapie and reducing the risk of introducing scrapie which provides participants an avenue to export sheep and goats.
In 2023, 179 flocks were enrolled in SFCP. Of these, 37 were export certified (scrapie-free), 28 were export monitored (working towards documenting scrapie freedom), and 114 were select monitored (reduced scrapie risk).
https://www.usda.gov/sites/default/files/documents/22-APHIS-2025-ExNotes.pdf
2024 Spending Plans for the Farmed Cervid Chronic Wasting Disease Management and Response Activities 2024 Cooperative Agreements
USDA APHIS Veterinary Services (VS) is awarding $6,045,215 through 30 Cooperative Agreements to 17 State departments of agriculture and 5 universities. The funded projects listed below will allow recipients to further develop and implement CWD management, response, and research activities in farmed cervids, including surveillance and testing, and include projects that propose to research the application of, or implement, whole genome predictive genetics CWD management plans.
Farmed Cervid Management Projects
Funding Project Title Entity Amount
Assessing Paramagnetic Nanoparticles for Improved CWD Detection
University of Minnesota $114,572
Establishing a Prion Reduction Plan For a CWD-Positive Deer Farm in Frio County
Texas Texas A&M $249,820
Establishing a Prion Reduction Plan For a CWD-Positive Deer Farm in Sutton County
Texas Texas A&M $205,913
Initiating Predictive Genetics for Chronic Wasting Disease Resistance in Mule Deer
Texas A&M $113,975
Screening of strain-specific anti CWD prion molecules
University of Texas Houston $244,977
Deploying Predictive Genetics for Chronic Wasting Disease Resistance in Farmed Elk
Texas A&M $222,335
Hapten-mediated and DNA-barcoded signal amplification for enhanced immunohistochemical detection of CWD prion protein
Louisiana State University $247,695
Further Expanding Outreach to Cervid Farmers and Veterinarians in States Participating in USDA’s CWD Herd Certification Program
Iowa State University $235,410
Increasing the efficacy of Illinois’ Chronic Wasting Disease (CWD) Certification Program and improving management and disease surveillance of farmed cervid herds by training producers in the collection and submission of satisfactory samples for CWD testing
Illinois Department of Agriculture $66,486
Detection and Decontamination of CWD Prions on Chemically Aged Steel Surfaces
University of Minnesota $118,925
Indiana farmed white-tailed deer herd management utilizing predictive genetics
Indiana Board of Animal Health $181,362
Iowa farmed white-tailed deer herd management utilizing predictive genetics
Iowa Department of Agriculture and Land Stewardship $82,470
North Dakota farmed white‐tailed deer herd management utilizing predictive genetics
North Dakota Department of Agriculture $100,357
West Virginia Farmed White-tailed Deer Herd Testing Utilizing Predictive Genetics
West Virginia Department of Agriculture $13,978
Assessment of CWD Risks to Cervid Farms from Wildlife Scavengers
University of Minnesota $170,877
Montana CWD Management and Response for CWD-Affected Alternative Livestock Herds
Montana Department of Livestock $205,616i
Enhancing herd health by leveraging predictive genetics in farmed white tail deer in Illinois
Illinois Department of Agriculture $249,952
CWD Information Management System
Pennsylvania Department of Agriculture $235,000
Genomic Predictions for Selective Breeding to Reduce Susceptibility to Chronic Wasting Disease (CWD) in Farmed White‐tailed Deer (Odocoileus virginianus) farms participating in the Alabama Department of Agriculture and Industry’s CWD Monitoring Program
Alabama Department of Agriculture and Industries $173,880
Kentucky farmed white-tailed deer herd management utilizing predictive genetics
Kentucky Department of Agriculture $186,682
Depopulation Activities and Indemnification as a Control Measure for CWD in Pennsylvania Captive Cervids
Pennsylvania Department of Agriculture $500,000i
South Dakota White Tailed Deer Herd Improvement Proposal Through Predictive Genetics
South Dakota Animal Industry Board $46,875
Genomic Predictions for Selective Breeding to Reduce Susceptibility to Chronic Wasting Disease (CWD) in Farmed White‐tailed Deer (Odocoileus virginianus) farms participating in the Louisiana Department of Agriculture and Forestry’s CWD Herd Certification Program (HCP)
Louisiana Department of Agriculture $52,500
Reducing Michigan farmed white-tailed deer herd CWD susceptibility using predictive genetics
Michigan Department of Agriculture and Rural Development $250,000
Depopulation of future CWD Infected herd/s in the state of Minnesota
Minnesota Board of Animal Health $443,586i
Optimizing herd management and CWD prevention through predictive genetics implementation
Missouri Department of Agriculture $215,930
North Carolina captive cervid white-tailed deer herd management utilizing predictive genetics
North Carolina Department of Agriculture and Consumer Services $122,908
CWD Epidemiology Support and Genetic Improvement Plan
Ohio Department of Agriculture $249,417
Michigan farmed white-tailed deer herd management utilizing predictive genetics in CWD affected herds
Michigan Department of Agriculture and Rural Development $613,000i
Oklahoma farmed white-tailed deer herd management utilizing predictive genetics
Oklahoma Department of Agriculture, Food, and Forestry $130,717ii
Total $6,045,215
i These awards include indemnity funds for the removal of CWD-positive or -exposed animals.
ii Noncompetitively awarded using declined funds
https://www.aphis.usda.gov/sites/default/files/2024-cwd-spending-plan.pdf
See;
https://www.aphis.usda.gov/funding/cwd
Bovine Spongiform Encephalopathy BSE TSE Prion
Revised Bovine Spongiform Encephalopathy BSE TSE Prion Standards
Revised BSE standards
Ad hoc Group on the evaluation of bovine spongiform encephalopathy (BSE) risk status of Members
Ad hoc Group on the evaluation of bovine spongiform encephalopathy (BSE) risk status of Members
Original: English (EN) 1-4 October 2024
1. Opening ............................................................ 2
2. Adoption of the agenda and appointment of Chairperson and Rapporteur ................................................................. 2
3. Evaluation of two applications from a Member for official recognition of zonal negligible BSE risk status .................. 2
4. Evaluation of the risk assessments of two Members already having an official negligible BSE risk status ................. 9
5. Review of the meeting and working procedure (summary tables) ............................................................................ 17
6. Annual reconfirmation forms: supportive documented evidence and internal screening guidance for maintenance of official BSE …….. 17
7. Any other business .................................................................................................................. 18
8. Adoption of the report .................................................................................................................. 18
List of Annexes
Appendix 1. Terms of reference
Appendix 2. Agenda
Appendix 3. List of Participants
Status Department
disease.status@woah.org
A meeting of the ad hoc Group on the evaluation of bovine spongiform encephalopathy (BSE) risk status of Members (hereafter the Group) was held from 1 to 4 October 2024.
Snip…see full text
Meeting of the AHG on the evaluation of BSE risk status of Members/October 2024
https://www.woah.org/app/uploads/2025/03/a-bse-ahg-report-2024-public-final.pdf
Canada
BSE Surveillance Program: Early participation and next steps
It’s been four months since we updated the National Bovine Spongiform Encephalopathy (BSE) Surveillance Program to align with new international standards. Early results are promising: we’ve already received 152 samples and have paid $102,000 to support clinical history recording and sampling – all from private veterinarians in Alberta.
To keep the momentum going, we’re encouraging participation from across the country.
Whether you’re a bovine vet, cattle producer, pathologist, abattoir staff or deadstock collector, the new tools make it easier to contribute:
Is the animal eligible? Take 2 minutes to check using our simple online questionnaire Need a refresher on sampling and packaging? Clear, practical guidance is available Want to claim for services provided? Use our online invoice form – submitting takes less than 2 minutes and payment rates have been updated Your participation matters: Market access depends on our ability to prove we’re actively looking for eligible cattle.
Visit the National BSE Surveillance Program webpage to learn more and get started.
Thanks for supporting disease surveillance in Canada.
Programme de surveillance de l’ESB : Participation initiale et prochaines étapes
Cela fait maintenant quatre mois que nous avons mis à jour le Programme national de surveillance de l’encéphalopathie spongiforme bovine (ESB) afin de l’aligner sur les nouvelles normes internationales. Les premiers résultats sont prometteurs : nous avons déjà reçu 152 échantillons et versé 102 000 $ pour soutenir la consignation de l’historique clinique et l’échantillonnage – tous provenant de vétérinaires privés en Alberta.
Pour maintenir cet élan, nous encourageons la participation à l’échelle du pays. Que vous soyez vétérinaire bovin, producteur de bovins, pathologiste, membre du personnel d’abattoir ou collecteur de cadavres d’animaux, les nouveaux outils facilitent votre contribution :
L’animal est-il admissible? Vérifiez-le en 2 minutes à l’aide de notre questionnaire en ligne Besoin d’un rappel sur l'échantillonnage et l'emballage? Des directives claires et pratiques sont disponibles Vous souhaitez réclamer des services fournis? Utilisez notre formulaire de facturation en ligne – l’envoi prend moins de 2 minutes, et les taux de rémunération ont été mis à jour Votre participation est essentielle : l’accès aux marchés dépend de notre capacité à démontrer que nous recherchons activement les bovins admissibles.
Consultez la page du Programme national de surveillance de l'ESB pour en savoir plus et commencer
Merci de soutenir la surveillance des maladies au Canada.
https://inspection.canada.ca/en/animal-health/terrestrial-animals/diseases/reportable/bovine-spongiform-encephalopathy/national-bse-surveillance-program
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene
Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur
Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933
ABSTRACT
Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation.
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Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.
Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene
KEYWORDS:
Atypical BSEBovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation
https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2511933#d1e1606
Updates on the WOAH activities in the field of TSEs
Natalie MOYEN Disease Status Officer
Status Department
May 14 th , 2024
2
Outline 1. Revised BSE standards (Terrestrial Code)
a) b) Transition process: where are we?
Official recognition of BSE risk status
i. Members/zones recognised as having a negligible or controlled BSE risk status
ii. New applications
iii. Annual reconfirmations
2. Revision of Scrapie standards (Terrestrial Code
https://www.eurl.craw.eu/wp-content/uploads/2024/06/07_WOAH_Updates-on-the-WOAH-activities-in-the-field-of-TSEs.pdf
WEDNESDAY, AUGUST 13, 2025
Revised Bovine Spongiform Encephalopathy BSE TSE Prion Standards
https://bovineprp.blogspot.com/2025/08/revised-bovine-spongiform.html
https://prpsc.proboards.com/thread/177/revised-bovine-spongiform-encephalopathy-standards
APHIS USDA BSE
6.8.Bovine spongiform encephalopathy (Chapter 11.4.), Application for official recognition by the OIE of risk status for bovine spongiform encephalopathy (Chapter 1.8.) and Glossary definition for ‘protein meal’
Background
In February 2018, following preliminary work and scientific exchanges, the Code Commission and the Scientific Commission agreed to an in-depth review of Chapter 11.4. Bovine spongiform encephalopathy (BSE). The OIE convened three different ad hoc Groups between July 2018 and March 2019: i) an ad hoc Group on BSE risk assessment, which met twice, ii) an ad hoc Group on BSE surveillance, which met once, and iii) a joint ad hoc Group on BSE risk assessment and surveillance, which met once. The Code Commission, at its September 2019 meeting, reviewed the four ad hoc Group reports and the opinion of the Scientific Commission regarding the draft revised chapter and circulated a revised draft Chapter 11.4. for comments.
In February 2020, the Code Commission considered comments received on the revised draft Chapter 11.4. and requested that the joint ad hoc Group on BSE risk assessment and surveillance be reconvened to address comments of a technical nature. In June 2020, the joint ad hoc Group was convened to address relevant comments and was also requested to review Chapter 1.8. Application for official recognition by the OIE of risk status for bovine spongiform encephalopathy to ensure alignment with the proposed changes in Chapter 11.4.
In September 2020, the Code Commission reviewed the joint ad hoc Group report and the revised draft Chapters 11.4. and 1.8. and made some additional amendments and circulated the revised chapters for comments in its September 2020 report. In February 2021, the Commission considered comments received and amended the chapters, as appropriate, and circulated the revised chapters for a third round of comments.
In preparation for the September 2021 meetings, some members of the Code Commission and the Scientific Commission met to discuss key aspects of the revision of Chapters 11.4. and 1.8. to ensure a common understanding of the main concerns raised by Members, the decisions made on the revised chapters and their impact on the official status recognition, as well as on the adapted procedures that will be required. During this meeting, it was agreed that each Commission would address the issues relevant to its meeting and document discussions in their respective reports.
Discussion
Snip…see;
https://www.aphis.usda.gov/sites/default/files/Sep-2021-Full-Comm-Report.docx
US BSE testing <25K annually…
Samples Tested by Fiscal Year
Fiscal Year v Total Samples Tested as Valid WOAH Points per Fiscal Year BSurvE Points
2024 22,848 207,808 468,946
2023 22,838 239,695 570,345
2022 21,816 360,553 842,517
2021 23,124 398,508 962,010
2020 21,441 331,561 795,183
2019 18,627 395,732 948,602
2018 21,809 540,960 1,305,585
2017 24,229 577,494 1,383,678
2016 26,564 547,959 1,314,579
2015 40,902 592,353 1,414,630
Updates on the WOAH activities in the field of TSEs
Natalie MOYEN Disease Status Officer
Status Department
May 14 th , 2024
2
Outline 1. Revised BSE standards (Terrestrial Code)
a) b) Transition process: where are we?
Official recognition of BSE risk status
i. Members/zones recognised as having a negligible or controlled BSE risk status
ii. New applications
iii. Annual reconfirmations
2. Revision of Scrapie standards (Terrestrial Code
https://www.eurl.craw.eu/wp-content/uploads/2024/06/07_WOAH_Updates-on-the-WOAH-activities-in-the-field-of-TSEs.pdf
Atypical BSE in cattle
THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.
Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.
Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.
Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous
VET RECORD | 29 March–12 April 2025
disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.
The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8
Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.
In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.
Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.
Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.
“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”
Timm Konold, TSE lead scientist
Brenda Rajanayagam, workgroup leader for the data systems group
APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk
Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX
References
1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)
Atypical BSE In Cattle
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2023
Published: 28 November 2024
Adopted: 29 October 2024
DOI https://doi.org/10.2903/j.efsa.2024.9097
KEYWORDS atypical, BSE, classical, CWD, scrapie, surveillance, TSE
CONTACT biohaw@efsa.europa.eu
Abstract
This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non‐EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye. In total, 948,165 cattle were tested by EU27 and XI (−3%, compared with 2022), with five atypical BSE cases reported (four H‐type: two in Spain, one in France and one in Ireland; one L‐type in the Netherlands); and 46,096 cattle by eight non‐EU reporting countries with two atypical BSE cases reported by Switzerland. Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1). In total, 284,686 sheep and 102,646 goats were tested in the EU27 and XI (−3.5% and −5.9%, respectively, compared to 2022). In the other non‐EU reporting countries 26,047 sheep and 589 goats were tested. In sheep, 538 cases of scrapie were reported by 14 MS and XI: 462 classical scrapie (CS) by 4 MS (104 index cases (IC) with genotypes of susceptible groups in 93.4% of the cases), 76 atypical scrapie (AS) (76 IC) by 12 MS. In the other non‐EU reporting countries, Iceland reported 70 cases of CS while Norway reported 7 cases of ovine AS. Ovine random genotyping was reported by six MS and genotypes of susceptible groups accounted for 6.9%. In goats, 183 cases of scrapie were reported, all from EU MS: 176 CS (47 IC) by seven MS and 7 AS (7 IC) by five MS. Three cases in Cyprus and one in Spain were reported in goats carrying heterozygous alleles at codon 146 and 222, respectively. In total, 2096 cervids were tested for chronic wasting disease by ten MS, none tested positive. Norway tested 14,224 cervids with one European moose positive.
© European Food Safety Authority
https://www.efsa.europa.eu/en/efsajournal/pub/9097
See full report;
https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2024.9097
The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022
European Food Safety Authority (EFSA)
First published: 28 November 2023
https://doi.org/10.2903/j.efsa.2023.8384
Approved: 19 October 2023 Abstract
This report presents the results of surveillance on transmissible spongiform encephalopathies (TSE) in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2022 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland [XI]) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye. In total, 977,008 cattle were tested by EU27 and XI (−4.3%, compared with 2021), and 52,395 cattle by eight non-EU reporting countries, with one case of H-BSE in France. In total, 295,145 sheep and 109,074 goats were tested in the EU27 and XI (−5.2% and −7.9%, respectively, compared to 2021). In the other non-EU reporting countries, 25,535 sheep and 633 goats were tested. In sheep, 557 cases of scrapie were reported by 17 MS and XI: 480 classical scrapie (CS) by five MS (93 index cases [IC] with genotypes of susceptible groups in 97.6% of the cases), 77 atypical scrapie (AS) (76 IC) by 14 MS and XI. In the other non-EU reporting countries, Norway reported 16 cases of ovine AS. Ovine random genotyping was reported by eight MS and genotypes of susceptible groups accounted for 7.3%. In goats, 224 cases of scrapie were reported, all from EU MS: 216 CS (42 IC) by six MS, and 8 AS (8 IC) by four MS. In Cyprus, two cases of CS were reported in goats carrying the heterozygous DN146 allele. In total, 3202 cervids were tested for chronic wasting disease by 10 MS. One wild European moose tested positive in Finland. Norway tested 17,583 cervids with two European moose, one reindeer and one red deer positive. In total, 154 animals from four other species tested negative in Finland.
https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2023.8384
https://www.efsa.europa.eu/en/search?s=Transmissible%20spongiform%20encephalopathy%20&sort=computed_sort_date&order=desc
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
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, Prpsc in the P2 animals acquired biochemical characteristics similar to that of Prps in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
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
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
https://wahis.woah.org/#/in-review/5067
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html
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
Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment
https://www.regulations.gov/comment/FSIS-2022-0027-0002
https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf
APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission
https://www.regulations.gov/comment/APHIS-2018-0087-0002
https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed PUBLIC SUBMISSION Comment from Terry Singeltary Sr.
Posted by the Food and Drug Administration on May 17, 2016
Comment Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission
https://www.regulations.gov/comment/FDA-2003-D-0432-0011
https://www.regulations.gov/docket/FDA-2003-D-0432
SCIENCE NEWS MAY 11, 2004 / 10:15 PM
USDA orders silence on mad cow in Texas
By STEVE MITCHELL, United Press International
https://www.upi.com/Science_News/2004/05/11/USDA-orders-silence-on-mad-cow-in-Texas/49171084328148/
Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II
and
Food Safety and Inspection Service Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
http://web.archive.org/web/20060210024116/http://www.usda.gov/oig/webdocs/50601-10-KC.pdf
Consumer Health
Inspector to file charges against USDA
By Steve Mitchell Sep 6, 2005, 22:46 GMT
http://web.archive.org/web/20050912185453/http://washingtontimes.com/upi/20050906-050340-6793r.htm
PAST US MAD COW CASES AND TRACEABILITY PROBLEMS, WHAT'S IT GOING TO TAKE?
AUG. 11, 2017
***>Assuming no other factors influenced the levels of correct diagnosis and that the numbers estimated for 1997 to 1999 were a true representation of the potential under-diagnosis of the entire epidemic up until 1999, then the total number of missed cases positive for BSE could have been in the region of 5,500.
***>As a result, using more sensitive diagnostic assays, we were able to diagnose BSE positive cattle from the years 1997-1999 inclusive that were originally negative by vacuolation. From these data we have estimated that approximately 3% of the total suspect cases submitted up until the year 1999 were mis-diagnosed.
YOU know, Confucius is confused again LOL, i seem to have remembered something in line with this here in the USA...
USDA did not test possible mad cows
By Steve Mitchell
United Press International
Published 6/8/2004 9:30 PM
WASHINGTON, June 8 (UPI) -- The U.S. Department of Agriculture claims ittested 500 cows with signs of a brain disorder for mad cow disease last year, but agency documents obtained by United Press International show the agency tested only half that number.
https://www.upi.com/Science_News/2004/06/08/USDA-did-not-test-possible-mad-cows/38651086744622/
http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html
http://www.usda.gov/oig/webdocs/50601-10-KC.pdf
"These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."
THIS WAS DONE FOR A REASON!
THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS
TEXAS 2ND MAD COW THAT WAS COVERED UP, AFTER AN ACT OF CONGRESS, AND CALLS FROM TSE PRION SCIENTIST AROUND THE GLOBE, THIS 2ND MAD COW IN TEXAS WAS CONFIRMED
THE USDA MAD COW FOLLIES POSITIVE TEST COVER UP
JOHANNS SECRET POSTIVE MAD COW TEST THAT WERE IGNORED
OIG AND THE HONORABLE FONG CONFIRMS TEXAS MAD AFTER AN ACT OF CONGRESS 7 MONTHS LATER
TEXAS MAD COW
THEY DID FINALLY TEST AFTER SITTING 7+ MONTHS ON A SHELF WHILE GW BORE THE BSE MRR POLICY, i.e. legal trading of all strains of TSE. now understand, i confirmed this case 7 months earlier to the TAHC, and then, only after i contacted the Honorable Phyllis Fong and after an act of Congress, this animal was finally confirmed ;
During the course of the investigation, USDA removed and tested a total of 67 animals of interest from the farm where the index animal's herd originated. All of these animals tested negative for BSE. 200 adult animals of interest were determined to have left the index farm. Of these 200, APHIS officials determined that 143 had gone to slaughter, two were found alive (one was determined not to be of interest because of its age and the other tested negative), 34 are presumed dead, one is known dead and 20 have been classified as untraceable. In addition to the adult animals, APHIS was looking for two calves born to the index animal. Due to record keeping and identification issues, APHIS had to trace 213 calves. Of these 213 calves, 208 entered feeding and slaughter channels, four are presumed to have entered feeding and slaughter channels and one calf was untraceable.
http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2005/08/0336.xml
NEW URL LINK;
http://web.archive.org/web/20090424121101/http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2005/08/0336.xml
Executive Summary In June 2005, an inconclusive bovine spongiform encephalopathy (BSE) sample from November 2004, that had originally been classified as negative on the immunohistochemistry test, was confirmed positive on SAF immunoblot (Western blot). The U.S. Department of Agriculture (USDA) identified the herd of origin for the index cow in Texas; that identification was confirmed by DNA analysis. USDA, in close cooperation with the Texas Animal Health Commission (TAHC), established an incident command post (ICP) and began response activities according to USDA’s BSE Response Plan of September 2004. Response personnel removed at-risk cattle and cattle of interest (COI) from the index herd, euthanized them, and tested them for BSE; all were negative. USDA and the State extensively traced all at-risk cattle and COI that left the index herd. The majority of these animals entered rendering and/or slaughter channels well before the investigation began. USDA’s response to the Texas finding was thorough and effective.
snip...
Trace Herd 3 The owner of Trace Herd 3 was identified as possibly having received an animal of interest. The herd was placed under hold order on 7/27/05. The herd inventory was conducted on 7/28/05. The animal of interest was not present within the herd, and the hold order was released on 7/28/05. The person who thought he sold the animal to the owner of Trace Herd 3 had no records and could not remember who else he might have sold the cow to. Additionally, a search of GDB for all cattle sold through the markets by that individual did not result in a match to the animal of interest. The animal of interest traced to this herd was classified as untraceable because all leads were exhausted.
Trace Herd 4 The owner of Trace Herd 4 was identified as having received one of the COI through an order buyer. Trace Herd 4 was placed under hold order on 7/29/05. A complete herd inventory was conducted on 8/22/05 and 8/23/05. There were 233 head of cattle that were examined individually by both State and Federal personnel for all man-made identification and brands. The animal of interest was not present within the herd. Several animals were reported to have died in the herd sometime after they arrived on the premises in April 2005. A final search of GDB records yielded no further results on the eartag of interest at either subsequent market sale or slaughter. With all leads having been exhausted, this animal of interest has been classified as untraceable. The hold order on Trace Herd 4 was released on 8/23/05.
Trace Herd 5 The owner of Trace Herd 5 was identified as having received two COI and was placed under hold order on 8/1/05. Trace Herd 5 is made up of 67 head of cattle in multiple pastures. During the course of the herd inventory, the owner located records that indicated that one of the COI, a known birth cohort, had been sold to Trace Herd 8 where she was subsequently found alive. Upon completion of the herd inventory, the other animal of interest was not found within the herd. A GDB search of all recorded herd tests conducted on Trace Herd 5 and all market sales by the owner failed to locate the identification tag of the animal of interest and she was subsequently classified as untraceable due to all leads having been exhausted. The hold order on Trace Herd 5 was released on 8/8/05.
Trace Herd 6 The owner of Trace Herd 6 was identified as possibly having received an animal of interest and was placed under hold order on 8/1/05. This herd is made up of 58 head of cattle on two pastures. A herd inventory was conducted and the animal of interest was not present within the herd. The owner of Trace Herd 6 had very limited records and was unable to provide further information on where the cow might have gone after he purchased her from the livestock market. A search of GDB for all cattle sold through the markets by that individual did not result in a match to the animal of interest. Additionally, many of the animals presented for sale by the owner of the herd had been re-tagged at the market effectually losing the traceability of the history of that animal prior to re-tagging. The animal of interest traced to this herd was classified as untraceable due to all leads having been exhausted. The hold order on Trace Herd 6 was released on 8/3/05.
Trace Herd 7 The owner of Trace Herd 7 was identified as having received an animal of interest and was placed under hold order on 8/1/05. Trace Herd 7 contains 487 head of cattle on multiple pastures in multiple parts of the State, including a unit kept on an island. The island location is a particularly rough place to keep cattle and the owner claimed to have lost 22 head on the island in 2004 due to liver flukes. Upon completion of the herd inventory, the animal of interest was not found present within Trace Herd 7. A GDB search of all recorded herd tests conducted on Trace Herd 7 and all market sales by the owner failed to locate the identification tag of the animal of interest. The cow was subsequently classified as untraceable. It is quite possible though that she may have died within the herd, especially if she belonged to the island unit. The hold order on Trace Herd 7 was released on 8/8/05.
http://www.aphis.usda.gov/lpa/issues/bse/epi-updates/bse_final_epidemiology_report.pdf
NEW URL LINK;
http://web.archive.org/web/20060315165436/http://www.aphis.usda.gov/lpa/issues/bse/epi-updates/bse_final_epidemiology_report.pdf
NOT to forget ;
It should be noted that since the enhanced surveillance program began, USDA has also conducted approximately 9,200 routine IHC tests on samples that did not first undergo rapid testing. This was done to ensure that samples inappropriate for the rapid screen test were still tested, and also to monitor and improve upon IHC testing protocols. Of those 9,200 routine tests, one test returned a non-definitive result on July 27, 2005. That sample underwent additional testing at NVSL, as well as at the Veterinary Laboratories Agency in Weybridge, England, and results were negative. ......
http://www.aphis.usda.gov/lpa/issues/bse_testing/test_results.html
https://www.fsc.go.jp/fsciis/attachedFile/download?retrievalId=kai20050824pr1&fileId=142
r i g h t ............
By Steve Mitchell
United Press International
Published 2/9/2004 7:06 PM
WASHINGTON, Feb. 9 (UPI) -- The federal laboratory in Ames, Iowa, that conducts all of the nation's tests for mad cow disease has a history of producing ambiguous and conflicting results -- to the point where many federal meat inspectors have lost confidence in it, Department of Agriculture veterinarians and a deer rancher told United Press International.
The veterinarians also claim the facility -- part of the USDA and known as the National Veterinary Services Laboratories -- has refused to release testing results to them and has been so secretive some suspect it is covering up additional mad cow cases. ...
http://www.upi.com/view.cfm?StoryID=20040209-061848-3665r
-------- Original Message --------
Subject: re-USDA's surveillance plan for BSE aka mad cow disease
Date: Mon, 02 May 2005 16:59:07 -0500
From: "Terry S. Singeltary Sr."
To: paffairs@oig.hhs.gov, HHSTips@oig.hhs.gov, contactOIG@hhsc.state.tx.us
Greetings Honorable Paul Feeney, Keith Arnold, and William Busbyet al at OIG, ...............
snip...
There will be several more emails of my research to follow.
I respectfully request a full inquiry into the cover-up of TSEs in the United States of America over the past 30 years. I would be happy to testify...
Thank you,I
am sincerely,
Terry S. Singeltary Sr. P.O. Box Bacliff, Texas USA 77518 xxx xxx xxxx
Date: June 14, 2005 at 1:46 pm PST
In Reply to: Re: Transcript Ag. Secretary Mike Johanns and Dr. John Clifford, Regarding further analysis of BSE Inconclusive Test Results posted by TSS on June 13, 2005 at 7:33 pm:
Secretary of Agriculture Ann M. Veneman resigns Nov 15 2004, three days later inclusive Mad Cow is announced. June 7th 2005 Bill Hawks Under Secretary for Marketing and Regulatory Programs resigns. Three days later same mad cow found in November turns out to be positive. Both resignations are unexpected. just pondering...TSS
-------- Original Message --------
Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ???
Date: Mon, 22 Nov 2004 17:12:15 -0600
From: "Terry S. Singeltary Sr."
To: Carla Everett References: <[log in to unmask]><[log in to unmask] us>
Greetings Carla,
still hear a rumor;
Texas single beef cow not born in Canada no beef entered the food chain?
and i see the TEXAS department of animal health is ramping up for something, but they forgot a url for update?
I HAVE NO ACTUAL CONFIRMATION YET...
can you confirm???
terry
snip...see full transmission;
http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html
UNITED STATES DEPARTMENT OF AGRICULTURE OFFICE OF INSPECTOR GENERAL STATEMENT OF THE HONORABLE PHYLLIS K. FONG INSPECTOR GENERAL Before the HOUSE APPROPRIATIONS SUBCOMMITTEE ON AGRICULTURE, RURAL DEVELOPMENT, FOOD AND DRUG ADMINISTRATION, AND RELATED AGENCIES March 1, 2006
snip...
For release only by the House Committee on Appropriations Good morning, Mr. Chairman and Members of the Subcommittee. I thank you for inviting me to testify before you today to discuss the activities of the Office of Inspector General (OIG) and to provide information about our oversight of the Department of Agriculture’s (USDA) programs and operations. I would like to introduce the members of the OIG senior management team who are here with me today: Kathy Tighe, our new Deputy Inspector General; Robert Young, Assistant Inspector General for Audit; Mark Woods, Assistant Inspector General for Investigations; and Suzanne Murrin, Assistant Inspector General for Policy Development and Resources Management. I welcome this opportunity to provide the Subcommittee with an overview of the highlights of our audit and investigative activity over the past year. Fiscal Year 2005 presented many difficult challenges for the USDA and our country’s agricultural producers and consumers. In addition to administering programs relied upon by farmers and rural communities and managing the $128 billion in public resources entrusted to the Department, USDA assumed significant responsibilities responding to the hurricanes that ravaged the Gulf Coast in 2005 and addressing the threat of plant and animal disease. To best serve the Department, our Congressional oversight committees, and the general public, OIG has formally prioritized, organized, and planned our work according to three
2
central objectives. I will present my testimony to the Subcommittee according to the framework of these three objectives: supporting Safety, Security, and Public Health in USDA programs and operations; protecting Program Integrity as USDA provides assistance to individuals and entities; and improving the Department’s Management of Public Resources. I. Safety, Security, and Public Health The BSE Surveillance Program and SRM Controls We recently issued our second report focusing on the Department’s efforts to establish and enforce effective, interlocking safeguards to protect producers and consumers from Bovine Spongiform Encephalopathy (BSE), commonly referred to as “mad cow disease.” Our February 2006 report reviewed the Animal and Plant Health Inspection Service’s (APHIS) implementation of its expanded BSE surveillance program and the Food Safety and Inspection Service’s (FSIS) controls to prevent banned specified risk materials (SRM) from entering our Nation’s food supply. We found that USDA made significant efforts to implement and improve the expanded surveillance program. The Department faced many challenges in a short period of time to establish the necessary processes, controls, and infrastructure needed for this massive effort. In our recent report, we discuss specific areas where we believe corrective actions were not fully effective in addressing our prior findings and recommendations on issues such as obtaining representative samples of the U.S. herd, identifying and obtaining samples from high-risk surveillance streams, and ensuring the completeness/accuracy of data. The Department
3
has responded to our report with immediate actions. For example, at the Secretary’s direction, APHIS revised its testing protocols to provide for additional confirmatory procedures when inconclusive test results occur. Also, both APHIS and FSIS agreed with all OIG recommendations, and they have corrected, or have developed action plans to correct, the program weaknesses identified. APHIS’ Implementation of the Expanded Surveillance Plan APHIS obtained significantly more samples for testing than it originally anticipated would be needed to achieve its stated level of confidence in estimating the prevalence of BSE in the U.S. herd. The voluntary nature of the surveillance program, however, makes it difficult to determine how successful USDA was in obtaining a representative proportion of high-risk cattle for testing. OIG found that APHIS’ various statistical approaches to determining the prevalence of BSE mitigate some, but not all, of the limitations associated with its data and the agency’s underlying assumptions in the design and implementation of its surveillance program. The accuracy of the underlying data is critical to the development of a future maintenance surveillance program. We recommended that APHIS disclose the limitations in its surveillance program and underlying data when it makes its final assessment of the prevalence of BSE in the U.S. We also found that USDA needed to strengthen its processes to ensure the quality and capability of its BSE testing program, especially when inconclusive test results occur. We recommended that USDA re-evaluate and adjust its testing protocols based on its
4
evaluation of emerging science and strengthen its proficiency testing and quality assurance reviews at participating laboratories. Evaluation of FSIS Processes Regarding SRMs To examine FSIS’ inspection procedures to enforce regulations to prevent risk materials in meat products, OIG reviewed the SRM plans of several meat processing facilities, observed FSIS inspections, and evaluated the effectiveness of controls during the slaughter process. FSIS technical experts assisted us in these reviews. We did not identify SRMs entering the food supply during our plant visits. However, we could not determine whether required SRM procedures were followed or were adequate due to the lack of specificity in the plans. We found that the plants lacked documentation of compliance with SRM control procedures and FSIS actions to validate such compliance. In addition to the control issues we identified regarding SRM procedures at slaughter and processing establishments, we found that FSIS’ information system could not readily provide FSIS with the data it needed to identify trends in SRM violations. The expanded stage of USDA’s BSE surveillance program is now nearing its end. Accordingly, it is important that the issues we have raised be considered as USDA completes its BSE surveillance program and reports on the prevalence of BSE in the U.S. herd. The Department has responded to our report with immediate action and agreed to address all of our findings and recommendations.
5
Assessing USDA Controls for Beef Exported to Japan On January 20, 2006, Japanese officials announced that they had banned any further imports of beef products from the United States, based on the discovery that a U.S. plant had shipped a veal product containing vertebral column material that was prohibited by the terms of an agreement with Japan. On the same date, in response to Japan’s decision, the Secretary announced 12 actions USDA would undertake to facilitate resuming trade. These actions include delisting and investigating the plant that exported the ineligible product, requiring a second signature on export certificates, providing training to inspection personnel on export certification, and holding meetings with inspection officials and industry representatives to reaffirm program requirements. Shortly thereafter, the Secretary requested OIG to audit the adequacy of USDA’s coordination and control processes for the Beef Export Verification (BEV) program for Japan. OIG’s report, issued on February 16, 2006, concluded that the Agricultural Marketing Service (AMS) and FSIS could strengthen their controls over the BEV program by improving processes used to communicate BEV program requirements, clearly defining roles and responsibilities, and implementing additional oversight of FSIS inspection personnel. In response to our recommendations, the agencies agreed to an array of actions. AMS agreed to maintain a list of specific, export-eligible products for each facility with an approved BEV program; to systematically notify FSIS when any establishment is approved/delisted from a BEV program; and to review all establishments in the BEV program to ensure that they adhere to program requirements. FSIS agreed to
6
clarify the roles and responsibilities of FSIS personnel involved at each stage of the export verification process; expedite the development of export certification training; and increase supervisory oversight of the export certification process. OIG believes that the full implementation of these measures will strengthen and improve the Department’s compliance with BEV program requirements. Assessment of the Equivalence of the Canadian Beef Inspection System Last year, my testimony discussed OIG’s findings from our audit of APHIS’ oversight of the importation of beef products from Canada. Our work on that audit led us to conduct an evaluation of FSIS’ assessment of the equivalence of the Canadian food safety inspection system, which we issued in December 2005. The then FSIS Administrator and the Under Secretary for Food Safety had identified concerns with the Canadian inspection system in late 2003. Our audit determined that FSIS did not fully address the issues raised by USDA officials in a timely manner. For example, in July 2003 FSIS found that Canadian inspection officials were not enforcing certain pathogen reduction and HACCP system regulations. These same types of concerns were identified again in June 2005. At the time of our audit, FSIS did not have protocols for evaluating deficiencies in a foreign country’s inspection system which could be used to question the system’s equivalence to U.S. standards. In addition, FSIS had not instituted compensating controls (such as increased port-of-entry testing) to strengthen public health protections while
7
deficiencies were present. During the period of January 2003–May 2005, 4.4 billion pounds of Canadian processed product entered the U.S., even though FSIS officials questioned the equivalence of the Canadian inspection system. FSIS agreed with OIG’s five recommendations, which included implementing protocols to determine which deficiencies would lead FSIS to question whether a foreign country’s inspection system is equivalent to the U.S. system. In response to the report, FSIS committed to develop these protocols by March 2006 and to implement them immediately thereafter. Oversight of FSIS Recalls For the past several years we have testified about our continuing work regarding adulterated beef product recalls. In July 2004, a Pennsylvania firm initiated a recall of approximately 170,000 pounds of ground beef patties because of mislabeling. Approximately one-fourth of this product was made, in part, from beef trim from Canada which was not eligible for import to the U.S., following the detection of a Canadian cow with BSE. In May 2005, we reported on the adequacy of FSIS’ effectiveness checks and the agency’s oversight of the recall. Overall, we concluded that FSIS had strengthened its procedures regarding the agency’s oversight of recalls. However, we noted that FSIS personnel did not determine the amount of product purchased by firms on 26 of the 58 completed effectiveness checks. This resulted in reduced assurance that mislabeled product was completely retrieved from distribution. Agency officials concurred with the firms’ assertions that the product had been removed from the marketplace. In response to
8
our recommendations, FSIS agreed to provide more specific direction to its personnel on identifying and evaluating the amount of product purchased. The Subcommittee has been interested in OIG’s investigation of a Pennsylvania company’s recall of meat products. This remains an ongoing civil fraud investigation and we will be pleased to provide information on its resolution to the Subcommittee upon its conclusion.
snip. ...9 of 34 pages. ...tss
http://appropriations.house.gov/_files/FongTestimony.pdf
see archived url;
http://web.archive.org/web/20060330031103/http://appropriations.house.gov/_files/FongTestimony.pdf
Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program
An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.
snip...
4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half
http://www.usda.gov/oig/webdocs/sarc070619.pdf
NEW URL LINK;
http://web.archive.org/web/20080922232504/http://www.usda.gov/oig/webdocs/sarc070619.pdf
Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II and Food Safety and Inspection Service Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
UNITED STATES DEPARTMENT OF AGRICULTURE OFFICE OF INSPECTOR GENERAL Washington, D.C. 20250 January 25, 2006 REPLY TO ATTN OF: 50601-10-KC
TO: W. Ron DeHaven Administrator Animal and Plant Health Inspection Service Barbara Masters Administrator Food Safety and Inspection Service ATTN: William J. Hudnall Deputy Administrator Marketing Regulatory Program Business Services William C. Smith Assistant Administrator Office of Program Evaluation, Enforcement, and Review
FROM: Robert W. Young /s/ Assistant Inspector General for Audit
SUBJECT: Animal and Plant Health Inspection Service - Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service - Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
This report presents the results of our audit of the enhanced BSE surveillance program and controls over specified risk materials and advanced meat recovery products. Your written response to the official draft report, dated January 20, 2006, is included as exhibit G with excerpts of the response and the Office of Inspector General’s (OIG) position incorporated into the Findings and Recommendations section of the report, where applicable. We accept the management decisions for all recommendations. Please follow your agency’s internal procedures in forwarding documentation for final action to the Office of the Chief Financial Officer (OCFO). We are providing a separate memorandum to the agencies and OCFO that provides specific information on the actions to be completed to achieve final action. We appreciate your timely response and the cooperation and assistance provided to our staff during the audit USDA/OIG-A/50601-10-KC/ Page i
Executive Summary
Animal and Plant Health Inspection Service - Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service - Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
Results in Brief This report evaluates elements of the interlocking safeguards in place to protect United States (U.S.) beef from Bovine Spongiform Encephalopathy, widely known as BSE or "mad cow disease." Since 1990, the U.S. Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), has led a multi-agency effort to monitor and prevent BSE from entering the food supply. After discovering a BSE-positive cow in December 2003, APHIS expanded its BSE surveillance program. To further protect the food supply, USDA banned materials identified as being at risk of carrying BSE (specified risk materials (SRM)), such as central nervous system tissue. As part of this effort, USDA’s Food Safety and Inspection Service (FSIS) required beef slaughter and processing facilities to incorporate controls for handling such materials into their operational plans. Onsite FSIS inspectors also inspect cattle for clinical signs in order to prevent diseased animals from being slaughtered for human consumption. To evaluate the effectiveness of the safeguards, we assessed APHIS’ implementation of the expanded surveillance program, as well as FSIS’ controls to prevent banned SRMs from entering the food supply.
In June 2004, APHIS implemented its expanded surveillance program; participation by industry in this surveillance program is voluntary. As of May 2005, over 350,000 animals were sampled and tested for BSE. To date, two animals tested positive for BSE; one tested positive after implementation of the expanded surveillance program.
USDA made significant efforts to implement the expanded BSE surveillance program. Much needed to be done in a short period of time to establish the necessary processes, controls, infrastructure, and networks to assist in this effort. In addition, extensive outreach and coordination was undertaken with other Federal, State, and local entities, private industry, and laboratory and veterinary networks. This report provides an assessment as to the progress USDA made in expanding its surveillance effort and the effectiveness of its controls and processes. This report also discusses the limitations of its program and data in assessing the prevalence of BSE in the U.S. herd.
snip...
40 ELISA test procedures require two additional (duplicate) tests if the initial test is reactive, before final interpretation. If either of the duplicate tests is reactive, the test is deemed inconclusive.
41 Protocol for BSE Contract Laboratories to Receive and Test Bovine Brain Samples and Report Results for BSE Surveillance Standard Operating Procedure (SOP), dated October 26, 2004.
42 The NVSL conducted an ELISA test on the original material tested at the contract laboratory and on two new cuts from the sample tissue.
43 A visual examination of brain tissue by a microscope.
44 A localized pathological change in a bodily organ or tissue.
SNIP...
PLEASE SEE FLAMING EVIDENCE THAT THE USDA ET AL COVERED UP MAD COW DISEASE IN TEXAS ;
PAGE 43;
Section 2. Testing Protocols and Quality Assurance Controls
snip...
FULL TEXT 130 PAGES
http://www.usda.gov/oig/webdocs/50601-10-KC.pdf
NEW URL LINK;
http://web.archive.org/web/20080920075242/http://www.usda.gov/oig/webdocs/50601-10-KC.pdf
Panel questions Homeland Security inspection of food By Jerry Hagstrom, CongressDailyPM
Agriculture Department Inspector General Phyllis Fong and several members of the House Agriculture Appropriations Subcommittee expressed concern Wednesday about whether the Homeland Security Department is properly inspecting food and other agricultural items when they arrive in the United States. Neither Fong nor the committee members provided any hard evidence of problems, but Fong said she and the Homeland Security Department's inspector general were conducting a joint review of inspection functions. Once the province of USDA's Animal and Plant Health Inspection Service, they were transferred to Homeland Security when that department was created.
"We are still very concerned about whether that broader inspection is being carried out," Fong said in testimony.
Fong said the joint inquiry was only in the stage of field investigation and had reached no conclusions.
Rep. Marcy Kaptur, D-Ohio, suggested the inspector general focus on the impact of the inspections on plants from other countries. House Agriculture Appropriations Subcommittee Chairman Henry Bonilla, R-Texas, said he had asked the Government Accountability Office to investigate the food inspection process. Rep. Tom Latham, R-Iowa, who noted he also sits on the House Homeland Security Appropriations Subcommittee, said, "Apparently there are still some unresolved issues about what the heck [the department] is doing."
Under questioning from Agriculture Appropriations ranking member Rosa DeLauro, D-Conn., Fong acknowledged it was APHIS Administrator Ron DeHaven who made the decision not to conduct further tests on a Texas cow whose initial test for bovine spongiform encephalopathy, or mad cow disease, was inconclusive. Further tests ordered by the inspector general several months later showed that the cow had the disease.
DeLauro noted that when Japan stopped importing U.S. beef in December, USDA moved faster to deal with that issue than it had when the Texas cow was tested, a sign, she said, that USDA was more concerned about trade than human health.
"We are taking years to deal with public health," DeLauro said. "If APHIS is in charge of avian influenza and we have the kinds of problems existing here, it doesn't bode well for public safety."
http://www.govexec.com/dailyfed/0306/030106cdpm2.htm
see archived url;
http://web.archive.org/web/20060311070356/http://www.govexec.com/dailyfed/0306/030106cdpm2.htm
Comments on technical aspects of the risk assessment were then submitted to FSIS.
Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.
This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:
http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf
NEW URL LINK;
http://web.archive.org/web/20090626021608/http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf
Owens, Julie From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Monday, July 24, 2006 1:09 PM To: FSIS RegulationsComments
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98 8/3/2006
Greetings FSIS, I would kindly like to comment on the following ;
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
NEW URL LINK;
http://web.archive.org/web/20080921202102/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
Suppressed peer review of Harvard study October 31, 2002.
October 31, 2002 Review of the Evaluation of the Potential for Bovine Spongiform Encephalopathy in the United States Conducted by the Harvard Center for Risk Analysis, Harvard School of Public Health and Center for Computational Epidemiology, College of Veterinary Medicine, Tuskegee University Final Report Prepared for U.S. Department of Agriculture Food Safety and Inspection Service Office of Public Health and Science Prepared by RTI Health, Social, and Economics Research Research Triangle Park, NC 27709 RTI Project Number 07182.024
http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf
NEW URL LINK;
http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf
Sunday, February 14, 2010
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html
snip...SEE FULL TEXT;
BSE research project final report 2005 to 2008 SE1796 SID5
http://bovineprp.blogspot.com/2020/12/
BSE REDBOOK
Preliminary Notification
The director of NVSL is responsible for immediately notifying the APHIS, Veterinary Services (VS) deputy administrator when tests suggest a presumptive diagnosis of BSE. Once NVSL has made a presumptive diagnosis of BSE, APHIS and FSIS field activities will also be initiated. APHIS will receive notification (either confirming or not confirming NVSL's diagnosis) from the United Kingdom anywhere between 24 and 96 hours. (The international animal health community has recognized the United Kingdom's Central Veterinary Laboratory {CVL} as the world's reference laboratory for diagnosing BSE. Other countries, including Belgium, France, Ireland, Luxembourg, the Netherlands, Portugal, and Switzerland, have all sent samples to this lab to confirm their first case of BSE).
snip...
BSE Response Team
The BSE Response Team will complete the informational memorandum for the Secretary. The Team will prepare the letter to the Office of International Epizootics (OIE), the international animal health organization, for signature by the APHIS, VS Deputy Administrator. OIE requires that all countries submit official notification within 24 hours of confirming a diagnosis of BSE. The BSE Response Team and the office of the APHIS, VS Deputy Administrator would coordinate a teleconference to inform all APHIS regional directors and AVIC'S. The BSE Response Team and the office of the FSIS, OPHS Deputy Administrator would coordinate a teleconference to inform all regional and field FSIS offices. The BSE Response Team would coordinate a teleconference to notify other Federal agencies. The BSE Response Team would coordinate a teleconference to notify key industry/consumer representatives. The BSE Response Team and APHIS International Services would notify foreign embassies. The BSE Response Team would establish a toll-free 800 telephone line for industry representatives, reporters, and the public. The BSE Response Team would coordinate with APHIS Legislative and Public Affairs and USDA office of Communications to issue a press release the day the diagnosis is confirmed. The press release would announce a press conference to be held the morning after the diagnosis is confirmed......
THE END
From: Terry S. Singeltary Sr.
Subject: Hunkering down in the APHIS BSE Situation Room...
Date: February 14, 2000 at 9:04 am PST
Subject: hunkering down in the APHIS BSE Situation Room
Subject: hunkering down in the APHIS BSE Situation Room
Date: Wed, 12 May 1999 01:55:54 -0800
From: tom Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de
i am looking now a bizarre Oct 98 internal USDA publication describing a james bond-type US effort to control media should the long-anticipated first case of BSE in the US be admitted.
'Players' on the 27 member BSE Response Team are to be flown in from all over the country to a BSE Headquarters 'situation room' apparently an underground bunker in Riverdale, Maryland under the command of the Assistant Secretary of Marketing.
Authentic press releases are already prepared and ready to go out after a few specifics have been filled in. They are spelled out in a separate document, the BSE Red Book, aka BSE Emergency Disease Guidelines.
Aphis' National Veterinary Services Laboratories (NVSL) activates team assembly. From the time a bovine brain sample is submitted, it takes 14-18 days to confirm a diagnosis of BSE. In the first 10-13 days, NVSL have enough information to determine the need for additional tests. If a provisional BSE diagnosis is made, the sample is 'hand-carried' (are they going to tell the airline and customs?) to the Central Veterinary Laboratory in England for confirmation, where they are expecting a 24 to 96 hour turn-around.
I guess that means we can get the white tiger brain analyzed by Friday despite the 22 year delay to date. Maybe we could throw in a few cougar brains from NE Colorado too.
A Team Member is designated to silently monitor this listserve and www.mad-cow.org(among others) -- for what, it doesn't say. The Freedom of Information Act request from the East Coast consumer group turned up numerous top-secret USDA downloads from that site and Dealler's.
After 24 hours of secret briefings for 'select industry and trading partners' (to allow them to take positions on the commodities markets opposite the 'non-select' industry and trading partners?), a press conference will be held the next day.
There are plans to trace the cow, its lineage, its herdmates, the renderer, traceout of product, buyout of herd, farm of origin, to get the state involved to quarantine the herd (pre-arranged for all 50 states), expectations for trade bans, notification of OIE within 24 hours, media 800 numbers, spokespersons and backups, notify CDC, FDA, NIH, and many other commendable activities. The Flow Chart is a sight to behold, I will try to scan it in tomorrow.
In short, that cow is going to be toast by the time the public first hears about it.
The Plan does not speak to the scenario in which the CVL says, yes, this is bovine spongiform encephalopathy all right but it is one of your strains, not ours. Invoking their Absence of Evidence is Evidence of Absence principle, there may be no perceived need for public disclosure in this case.
USDA is caught completely unprepared if BSE first turns up in a US zoo animal. These animals could easily be diagnosed outside the "system" and be the subject of a publicity-seeking lab press release. I think this is a more likely scenario because the US has likely imported many thousands of zoo animals with advanced infections from Britain and France and there has been zero monitoring. Unlike with downer cows, anyone with the right colleagues can get ahold of a fallen zoo animal. Zoo animals enter the food chain in some cases after being rendered.
Another scenario would be some stock market speculator obtaining the Red Book and issuing a flurry of bogus but authentic-looking press releases that included bogus 800 and hacked USDA web links. The press here is so lazy and so accustomed to putting out public relation handouts as news that the objectives would be accomplished for a few hour (or days, depending on the Response Team's paralysis vis-a-vis off-flow chart events). Some people think a practise run for this happened in the Indiana case a year or two back.
The first case of nvCJD in an American will also be a public relations fiasco. In the dim bulb of the public mind, any American with mad cow disease would have gotten it from eating meat here. USDA has no way to prove that the victim acquired it on a three week trip to England in 1987. This will sound lame even to the press. All CJD is synonymous with mad cow disease in the public perception; the more often the different kinds are explained, the more their suspicions are aroused. The first case of nvCJD in an American will simply validate what they already know and just be viewed as an overdue admission from the government.
tom
___________________________________________________________
From: Terry S. Singeltary Sr.
i am looking now a bizarre Oct 98 internal USDA publication describing a james bond-type US effort to control media should the long-anticipated first case of BSE in the US be admitted.
'Players' on the 27 member BSE Response Team are to be flown in from all over the country to a BSE Headquarters 'situation room' apparently an underground bunker in Riverdale, Maryland under the command of the Assistant Secretary of Marketing.
Authentic press releases are already prepared and ready to go out after a few specifics have been filled in. They are spelled out in a separate document, the BSE Red Book, aka BSE Emergency Disease Guidelines.
Aphis' National Veterinary Services Laboratories (NVSL) activates team assembly. From the time a bovine brain sample is submitted, it takes 14-18 days to confirm a diagnosis of BSE. In the first 10-13 days, NVSL have enough information to determine the need for additional tests. If a provisional BSE diagnosis is made, the sample is 'hand-carried' (are they going to tell the airline and customs?) to the Central Veterinary Laboratory in England for confirmation, where they are expecting a 24 to 96 hour turn-around.
I guess that means we can get the white tiger brain analyzed by Friday despite the 22 year delay to date. Maybe we could throw in a few cougar brains from NE Colorado too.
A Team Member is designated to silently monitor this listserve and www.mad-cow.org(among others) -- for what, it doesn't say. The Freedom of Information Act request from the East Coast consumer group turned up numerous top-secret USDA downloads from that site and Dealler's.
After 24 hours of secret briefings for 'select industry and trading partners' (to allow them to take positions on the commodities markets opposite the 'non-select' industry and trading partners?), a press conference will be held the next day.
There are plans to trace the cow, its lineage, its herdmates, the renderer, traceout of product, buyout of herd, farm of origin, to get the state involved to quarantine the herd (pre-arranged for all 50 states), expectations for trade bans, notification of OIE within 24 hours, media 800 numbers, spokespersons and backups, notify CDC, FDA, NIH, and many other commendable activities. The Flow Chart is a sight to behold, I will try to scan it in tomorrow.
In short, that cow is going to be toast by the time the public first hears about it.
The Plan does not speak to the scenario in which the CVL says, yes, this is bovine spongiform encephalopathy all right but it is one of your strains, not ours. Invoking their Absence of Evidence is Evidence of Absence principle, there may be no perceived need for public disclosure in this case.
USDA is caught completely unprepared if BSE first turns up in a US zoo animal. These animals could easily be diagnosed outside the "system" and be the subject of a publicity-seeking lab press release. I think this is a more likely scenario because the US has likely imported many thousands of zoo animals with advanced infections from Britain and France and there has been zero monitoring. Unlike with downer cows, anyone with the right colleagues can get ahold of a fallen zoo animal. Zoo animals enter the food chain in some cases after being rendered.
Another scenario would be some stock market speculator obtaining the Red Book and issuing a flurry of bogus but authentic-looking press releases that included bogus 800 and hacked USDA web links. The press here is so lazy and so accustomed to putting out public relation handouts as news that the objectives would be accomplished for a few hour (or days, depending on the Response Team's paralysis vis-a-vis off-flow chart events). Some people think a practise run for this happened in the Indiana case a year or two back.
The first case of nvCJD in an American will also be a public relations fiasco. In the dim bulb of the public mind, any American with mad cow disease would have gotten it from eating meat here. USDA has no way to prove that the victim acquired it on a three week trip to England in 1987. This will sound lame even to the press. All CJD is synonymous with mad cow disease in the public perception; the more often the different kinds are explained, the more their suspicions are aroused. The first case of nvCJD in an American will simply validate what they already know and just be viewed as an overdue admission from the government.
tom
___________________________________________________________
From: Terry S. Singeltary Sr.
Subject: When a case of B.S.E. is found in the U.S/Response to Disease outbreak...'redbook'
Date: March 13, 2000 at 10:13 am PST
BSE Red Book 2.1-26
5.0 Response to Disease Outbreak
snip...see full report of
From: Terry S. Singeltary Sr.
Subject: When a case of B.S.E. is found in the U.S/Response to Disease outbreak...'redbook'
Date: March 13, 2000 at 10:13 am PST
https://bseusa.blogspot.com/2008/08/qualitative-analysis-of-bse-risk.html
Thursday, April 6, 2023
WOAH OIE CHAPTER 11.4 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 11.4.1.
https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html
BSE TESTING ONLY 25K ANNUALLY WILL NOT FIND BSE CIRCULATING IN THE USA CATTLE HERDS, UNLESS IT'S REAL BAD...THAT'S WHEN YOU HAVE PROBLEMS, AND YOUR JUST TRYING TO HOLD THE NUMBERS DOWN...IMO...terry
Sample Size Estimate for BSE Ongoing Surveillance
July 20, 2006
snip...
In addition, we aim to meet the objective of conducting ongoing surveillance at a level that meets or exceeds OIE surveillance recommendations. We believe this objective is reached by the following sampling strategy, which is sufficient to detect BSE at 1 infected animal per 1,000,000 adult cattle in the population with a high degree of confidence.
Sample Size to Meet OIE Surveillance Recommendations
APHIS is committed to maintaining BSE surveillance that at least meets OIE guidelines. The OIE surveillance guidelines for BSE recommend a target number of surveillance points for Type A surveillance based on the size of a country’s cattle population. These points are accrued over 7 consecutive years, and are weighted according to the surveillance stream and age of the animal sampled. For a large cattle population, using the design prevalence of 1 case per 100,000 adult cattle and 95 percent confidence, 300,000 total points over 7 years, or 42,857 points per year, are required for Type A surveillance (OIE 2005).
http://web.archive.org/web/20090109193041/http://www.aphis.usda.gov/peer_review/downloads/BSE_sample_size_ongoing_surv_after.pdf
KEY POINTS In addition to a stringent feed ban imposed by the Food and Drug Administration in 1997 as well as the removal of all specified risk material which could harbor BSE, USDA has a strong surveillance program in place to detect signs of BSE in cattle in the United States. In fact, we test for BSE at levels greater than World Animal Health Organization standards. The program samples approximately 25,000 animals each year and targets cattle populations where the disease is most likely to be found. The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including emaciation or injury, and dead cattle, as well as non-ambulatory animals. Samples from the targeted population are taken at farms, veterinary diagnostic laboratories, public health laboratories, slaughter facilities, veterinary clinics, and livestock markets.
https://www.usda.gov/topics/animals/bse-surveillance-information-center
THURSDAY, AUGUST 20, 2020
Why is USDA "only" BSE TSE Prion testing 25,000 samples a year?
https://animalhealthreportpriontse.blogspot.com/2020/08/why-is-usda-only-bse-tse-prion-testing.html
Comment from Singeltary Sr., Terry Posted by the Animal and Plant Health Inspection Service on Mar 22, 2023
Submission
Greetings again APHIS et al,
I would kindly comment and voice my concerns again about the lack of a NAIS/COOL mandatory policy, and how important it is.
https://www.regulations.gov/comment/APHIS-2021-0020-0999
https://downloads.regulations.gov/APHIS-2021-0020-0999/attachment_1.pdf
FSIS [Docket No. FSIS-2019-0021] Notice of Request To Renew an Approved Information Collection: Specified Risk Materials Singeltary Submission
Food Safety and Inspection Service [Docket No. FSIS-2019-0021] Notice of Request To Renew an Approved Information Collection: Specified Risk Materials
Greetings FSIS et al,
I would kindly like to comment on the following docket;
[Docket No. FSIS-2019-0021] Notice
http://web.archive.org/web/20210720163644/https://downloads.regulations.gov/FSIS-2019-0021-0002/attachment_1.pdf
APHIS-2018-0012 Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy Singeltary…
https://beta.regulations.gov/comment/APHIS-2018-0012-0002
https://downloads.regulations.gov/APHIS-2018-0012-0002/attachment_1.pdf
Molecular, Biochemical and Genetic Characteristics of BSE in Canada
* Published: May 14, 2010 * DOI: 10.1371/journal.pone.0010638
Reader Comments (1)
RE-Molecular, Biochemical and Genetic Characteristics of BSE in Canada
Posted by flounder on 19 May 2010 at 21:21 GMT
Greetings,
>>>The occurrence of atypical cases of BSE in countries such as Canada with low BSE prevalence and transmission risk argues for the occurrence of sporadic forms of BSE worldwide. <<<
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0010638
In my opinion ;
THE statement above is about as non-scientific as a statement can be. There is no proof what-so-ever that any of the atypical BSE cases or atypical scrapie cases anywhere on the globe was a spontaneous case without any route and source of the TSE agent. This is a myth. The USDA and the OIE are trying to make the atypical BSE cases and they have already made the atypical Scrapie cases a legal trading commodity, without any transmission studies first confirming that in fact these atypical TSE will not transmit via feed. I suppose it is a human transmission study in progress…
Snip…see my full comment ;
http://web.archive.org/web/20140912011905/http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/4f9be886-69fe-4c7c-922b-85b0ecbe6d53
IBNC Tauopathy or TSE Prion disease, it appears, no one is sure
Posted by flounder on 03 Jul 2015 at 16:53 GMT
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/5adef4ac-a7e4-46a4-8806-c8533d5c862c
Susceptibility of European Red Deer (Cervus elaphus elaphus) to Alimentary Challenge with Bovine Spongiform Encephalopathy
Mark P. Dagleish mail,
Stuart Martin, Philip Steele, Jeanie Finlayson, Samantha L. Eaton, Sílvia Sisó
Published: January 23, 2015DOI: 10.1371/journal.pone.0116094
Singeltary comments;
31 Jan 2015 at 20:14 GMT
Ruminant feed ban for cervids in the United States?
https://web.archive.org/web/20150722000538/http://www.plosone.org/annotation/listThread.action?root=85351
SATURDAY, NOVEMBER 9, 2019
[Docket No. APHIS-2018-0087] Concurrence With OIE Risk Designations for Bovine Spongiform Nicaragua being of negligible risk for BSE
https://animalhealthreportpriontse.blogspot.com/2019/11/docket-no-aphis-2018-0087-concurrence.html
MONDAY, AUGUST 4, 2025
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
See;
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
https://bovineprp.blogspot.com/2025/08/canadian-2021-h-type-bovine-spongiform.html
WEDNESDAY, JULY 16, 2025
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
https://bse-atypical.blogspot.com/2025/07/classical-bse-emergence-from.html
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE), Singeltary Review
https://bse-atypical.blogspot.com/2025/06/the-naturally-occurring-lysine-to.html
Tuesday, August 12, 2025
Ad hoc Group on the evaluation of bovine spongiform encephalopathy (BSE) risk status of Members Singeltary Review August 2025
https://woahoie.blogspot.com/2025/08/ad-hoc-group-on-evaluation-of-bovine.html
https://prpsc.proboards.com/thread/176/group-evaluation-risk-singeltary-review
MONDAY, AUGUST 4, 2025
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
See;
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
https://bovineprp.blogspot.com/2025/08/canadian-2021-h-type-bovine-spongiform.html
WEDNESDAY, JULY 16, 2025
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
https://bse-atypical.blogspot.com/2025/07/classical-bse-emergence-from.html
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE), Singeltary Review
https://bse-atypical.blogspot.com/2025/06/the-naturally-occurring-lysine-to.html
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
THURSDAY, MAY 29, 2025
Animal feed company convicted at Ballymena court EU Regulation No.999/2001 TSE Regulations
https://www.daera-ni.gov.uk/news/animal-feed-company-convicted-ballymena-court
https://bovineprp.blogspot.com/2025/05/animal-feed-company-convicted-at.html
THURSDAY, JANUARY 9, 2025
UK ministers may lift BSE-era ban on animal remains in chicken and pig feed
https://efsaopinionbseanimalprotein.blogspot.com/2025/01/uk-ministers-may-lift-bse-era-ban-on.html
An isolated case of classical scrapie has been confirmed in a 2-year-old Welsh Mountain Badger Face female sheep. United Kingdom
https://wahis.woah.org/#/in-review/6524
THURSDAY, MAY 22, 2025
Single case of atypical BSE confirmed on a farm in Essex
https://www.gov.uk/government/news/single-case-of-atypical-bseconfirmed-on-a-farm-in-essex
https://bse-atypical.blogspot.com/2025/05/single-case-of-atypical-bseconfirmed-on.html
FRIDAY, MAY 23, 2025
Epidemiological investigation of a single atypical BSE case in Dumfries and Galloway, Scotland (RBSE 24/00006)
https://www.gov.uk/government/publications/bse-in-scotland-epidemiology-report-2025/epidemiological-investigation-of-a-single-atypical-bse-case-in-dumfries-and-galloway-scotland-rbse-2400006
https://bse-atypical.blogspot.com/2025/05/epidemiological-investigation-of-single.html
Tuesday, June 3, 2025
World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible
“Those that fail to learn from history are doomed to repeat it.”
https://woahoie.blogspot.com/2025/06/world-organisation-for-animal-health.html
Friday, October 4, 2024
another atypical bovine spongiform encephalopathy (BSE) in Ireland
https://woahoie.blogspot.com/2024/10/another-atypical-bovine-spongiform.html
WEDNESDAY, NOVEMBER 08, 2023
Ireland Atypical BSE confirmed November 3 2023
https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html
TUESDAY, NOVEMBER 14, 2023
Ireland Atypical BSE case, 3 progeny of case cow to be culled
https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html
SUNDAY, JULY 16, 2023
Switzerland Atypical BSE detected in a cow in the canton of St. Gallen
https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html
WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type
Switzerland Bovine Spongiform Encephalopathy Atypical L-Type
Switzerland - Bovine spongiform encephalopathy - Immediate notification
https://wahis.woah.org/#/in-review/4962
https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html
Monday, March 20, 2023
WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type
https://wahis.woah.org/#/in-review/4977
https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall
https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html
BRAZIL BSE START DATE 2023/01/18
BRAZIL BSE CONFIRMATION DATE 2023/02/22
BRAZIL BSE END DATE 2023/03/03
https://wahis.woah.org/#/in-review/4918
https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html
SPAIN BSE START DATE 2023/01/21
SPAIN BSE CONFIRMATION DATE 2023/02/03
SPAIN BSE END DATE 2023/02/06
https://wahis.woah.org/#/in-review/4888
https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html
NETHERLANDS BSE START DATE 2023/02/01
NETHERLANDS BSE CONFIRMATION DATE 2023/02/01
NETHERLANDS BSE END DATE 2023/03/13
https://wahis.woah.org/#/in-review/4876
https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html
Scrapie TSE Prion
NVAP Reference Guide - Scrapie (Control and Eradication)
Last Modified: July 30, 2025
There are two types of scrapie; classical and nonclassical. Nonclassical scrapie is also referred to as atypical, Nor98, or Nor98-like scrapie. Nonclassical scrapie appears to occur sporadically and has occurred in sheep of all the common genotypes and goats. It is either not transmissible or poorly transmissible under natural conditions. Given this the scrapie eradication program focuses on classical scrapie. Here after where “scrapie” is used it is intended to mean “classical scrapie”.
Scrapie is a fatal, degenerative TSE disease affecting the central nervous system of sheep and goats. First recognized as a disease of sheep in Great Britain and other countries of Western Europe more than 250 years ago, scrapie has been reported throughout the world. In the United States, scrapie has primarily been reported in the black-face meat breeds and their crosses. It also has been diagnosed in numerous other breeds and crossbreeds including wool and hair sheep, and in goats. At the end of FY 2016, the percent of cull sheep found positive at slaughter and adjusted for face color was 0.001 percent with an upper confidence limit of 0.009 percent. This measure of prevalence has decreased by 99 percent since slaughter surveillance started in FY 2003. Based on all goats sampled at slaughter through FY 2016, the prevalence of scrapie in U.S. cull goats is 0.002 percent with an upper 95 percent confidence limit of 0.004 percent.
BSE Red Book 2.1-26
5.0 Response to Disease Outbreak
snip...see full report of
From: Terry S. Singeltary Sr.
Subject: When a case of B.S.E. is found in the U.S/Response to Disease outbreak...'redbook'
Date: March 13, 2000 at 10:13 am PST
https://bseusa.blogspot.com/2008/08/qualitative-analysis-of-bse-risk.html
Thursday, April 6, 2023
WOAH OIE CHAPTER 11.4 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 11.4.1.
https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html
BSE TESTING ONLY 25K ANNUALLY WILL NOT FIND BSE CIRCULATING IN THE USA CATTLE HERDS, UNLESS IT'S REAL BAD...THAT'S WHEN YOU HAVE PROBLEMS, AND YOUR JUST TRYING TO HOLD THE NUMBERS DOWN...IMO...terry
Sample Size Estimate for BSE Ongoing Surveillance
July 20, 2006
snip...
In addition, we aim to meet the objective of conducting ongoing surveillance at a level that meets or exceeds OIE surveillance recommendations. We believe this objective is reached by the following sampling strategy, which is sufficient to detect BSE at 1 infected animal per 1,000,000 adult cattle in the population with a high degree of confidence.
Sample Size to Meet OIE Surveillance Recommendations
APHIS is committed to maintaining BSE surveillance that at least meets OIE guidelines. The OIE surveillance guidelines for BSE recommend a target number of surveillance points for Type A surveillance based on the size of a country’s cattle population. These points are accrued over 7 consecutive years, and are weighted according to the surveillance stream and age of the animal sampled. For a large cattle population, using the design prevalence of 1 case per 100,000 adult cattle and 95 percent confidence, 300,000 total points over 7 years, or 42,857 points per year, are required for Type A surveillance (OIE 2005).
http://web.archive.org/web/20090109193041/http://www.aphis.usda.gov/peer_review/downloads/BSE_sample_size_ongoing_surv_after.pdf
KEY POINTS In addition to a stringent feed ban imposed by the Food and Drug Administration in 1997 as well as the removal of all specified risk material which could harbor BSE, USDA has a strong surveillance program in place to detect signs of BSE in cattle in the United States. In fact, we test for BSE at levels greater than World Animal Health Organization standards. The program samples approximately 25,000 animals each year and targets cattle populations where the disease is most likely to be found. The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including emaciation or injury, and dead cattle, as well as non-ambulatory animals. Samples from the targeted population are taken at farms, veterinary diagnostic laboratories, public health laboratories, slaughter facilities, veterinary clinics, and livestock markets.
https://www.usda.gov/topics/animals/bse-surveillance-information-center
THURSDAY, AUGUST 20, 2020
Why is USDA "only" BSE TSE Prion testing 25,000 samples a year?
https://animalhealthreportpriontse.blogspot.com/2020/08/why-is-usda-only-bse-tse-prion-testing.html
Comment from Singeltary Sr., Terry Posted by the Animal and Plant Health Inspection Service on Mar 22, 2023
Submission
Greetings again APHIS et al,
I would kindly comment and voice my concerns again about the lack of a NAIS/COOL mandatory policy, and how important it is.
https://www.regulations.gov/comment/APHIS-2021-0020-0999
https://downloads.regulations.gov/APHIS-2021-0020-0999/attachment_1.pdf
FSIS [Docket No. FSIS-2019-0021] Notice of Request To Renew an Approved Information Collection: Specified Risk Materials Singeltary Submission
Food Safety and Inspection Service [Docket No. FSIS-2019-0021] Notice of Request To Renew an Approved Information Collection: Specified Risk Materials
Greetings FSIS et al,
I would kindly like to comment on the following docket;
[Docket No. FSIS-2019-0021] Notice
http://web.archive.org/web/20210720163644/https://downloads.regulations.gov/FSIS-2019-0021-0002/attachment_1.pdf
APHIS-2018-0012 Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy Singeltary…
https://beta.regulations.gov/comment/APHIS-2018-0012-0002
https://downloads.regulations.gov/APHIS-2018-0012-0002/attachment_1.pdf
Molecular, Biochemical and Genetic Characteristics of BSE in Canada
* Published: May 14, 2010 * DOI: 10.1371/journal.pone.0010638
Reader Comments (1)
RE-Molecular, Biochemical and Genetic Characteristics of BSE in Canada
Posted by flounder on 19 May 2010 at 21:21 GMT
Greetings,
>>>The occurrence of atypical cases of BSE in countries such as Canada with low BSE prevalence and transmission risk argues for the occurrence of sporadic forms of BSE worldwide. <<<
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0010638
In my opinion ;
THE statement above is about as non-scientific as a statement can be. There is no proof what-so-ever that any of the atypical BSE cases or atypical scrapie cases anywhere on the globe was a spontaneous case without any route and source of the TSE agent. This is a myth. The USDA and the OIE are trying to make the atypical BSE cases and they have already made the atypical Scrapie cases a legal trading commodity, without any transmission studies first confirming that in fact these atypical TSE will not transmit via feed. I suppose it is a human transmission study in progress…
Snip…see my full comment ;
http://web.archive.org/web/20140912011905/http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/4f9be886-69fe-4c7c-922b-85b0ecbe6d53
IBNC Tauopathy or TSE Prion disease, it appears, no one is sure
Posted by flounder on 03 Jul 2015 at 16:53 GMT
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/5adef4ac-a7e4-46a4-8806-c8533d5c862c
Susceptibility of European Red Deer (Cervus elaphus elaphus) to Alimentary Challenge with Bovine Spongiform Encephalopathy
Mark P. Dagleish mail,
Stuart Martin, Philip Steele, Jeanie Finlayson, Samantha L. Eaton, Sílvia Sisó
Published: January 23, 2015DOI: 10.1371/journal.pone.0116094
Singeltary comments;
31 Jan 2015 at 20:14 GMT
Ruminant feed ban for cervids in the United States?
https://web.archive.org/web/20150722000538/http://www.plosone.org/annotation/listThread.action?root=85351
SATURDAY, NOVEMBER 9, 2019
[Docket No. APHIS-2018-0087] Concurrence With OIE Risk Designations for Bovine Spongiform Nicaragua being of negligible risk for BSE
https://animalhealthreportpriontse.blogspot.com/2019/11/docket-no-aphis-2018-0087-concurrence.html
MONDAY, AUGUST 4, 2025
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
See;
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
https://bovineprp.blogspot.com/2025/08/canadian-2021-h-type-bovine-spongiform.html
WEDNESDAY, JULY 16, 2025
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
https://bse-atypical.blogspot.com/2025/07/classical-bse-emergence-from.html
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE), Singeltary Review
https://bse-atypical.blogspot.com/2025/06/the-naturally-occurring-lysine-to.html
Tuesday, August 12, 2025
Ad hoc Group on the evaluation of bovine spongiform encephalopathy (BSE) risk status of Members Singeltary Review August 2025
https://woahoie.blogspot.com/2025/08/ad-hoc-group-on-evaluation-of-bovine.html
https://prpsc.proboards.com/thread/176/group-evaluation-risk-singeltary-review
MONDAY, AUGUST 4, 2025
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
See;
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene, Singeltary Review
https://bovineprp.blogspot.com/2025/08/canadian-2021-h-type-bovine-spongiform.html
WEDNESDAY, JULY 16, 2025
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
https://bse-atypical.blogspot.com/2025/07/classical-bse-emergence-from.html
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE), Singeltary Review
https://bse-atypical.blogspot.com/2025/06/the-naturally-occurring-lysine-to.html
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
THURSDAY, MAY 29, 2025
Animal feed company convicted at Ballymena court EU Regulation No.999/2001 TSE Regulations
https://www.daera-ni.gov.uk/news/animal-feed-company-convicted-ballymena-court
https://bovineprp.blogspot.com/2025/05/animal-feed-company-convicted-at.html
THURSDAY, JANUARY 9, 2025
UK ministers may lift BSE-era ban on animal remains in chicken and pig feed
https://efsaopinionbseanimalprotein.blogspot.com/2025/01/uk-ministers-may-lift-bse-era-ban-on.html
An isolated case of classical scrapie has been confirmed in a 2-year-old Welsh Mountain Badger Face female sheep. United Kingdom
https://wahis.woah.org/#/in-review/6524
THURSDAY, MAY 22, 2025
Single case of atypical BSE confirmed on a farm in Essex
https://www.gov.uk/government/news/single-case-of-atypical-bseconfirmed-on-a-farm-in-essex
https://bse-atypical.blogspot.com/2025/05/single-case-of-atypical-bseconfirmed-on.html
FRIDAY, MAY 23, 2025
Epidemiological investigation of a single atypical BSE case in Dumfries and Galloway, Scotland (RBSE 24/00006)
https://www.gov.uk/government/publications/bse-in-scotland-epidemiology-report-2025/epidemiological-investigation-of-a-single-atypical-bse-case-in-dumfries-and-galloway-scotland-rbse-2400006
https://bse-atypical.blogspot.com/2025/05/epidemiological-investigation-of-single.html
Tuesday, June 3, 2025
World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible
“Those that fail to learn from history are doomed to repeat it.”
https://woahoie.blogspot.com/2025/06/world-organisation-for-animal-health.html
Friday, October 4, 2024
another atypical bovine spongiform encephalopathy (BSE) in Ireland
https://woahoie.blogspot.com/2024/10/another-atypical-bovine-spongiform.html
WEDNESDAY, NOVEMBER 08, 2023
Ireland Atypical BSE confirmed November 3 2023
https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html
TUESDAY, NOVEMBER 14, 2023
Ireland Atypical BSE case, 3 progeny of case cow to be culled
https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html
SUNDAY, JULY 16, 2023
Switzerland Atypical BSE detected in a cow in the canton of St. Gallen
https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html
WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type
Switzerland Bovine Spongiform Encephalopathy Atypical L-Type
Switzerland - Bovine spongiform encephalopathy - Immediate notification
https://wahis.woah.org/#/in-review/4962
https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html
Monday, March 20, 2023
WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type
https://wahis.woah.org/#/in-review/4977
https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall
https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html
BRAZIL BSE START DATE 2023/01/18
BRAZIL BSE CONFIRMATION DATE 2023/02/22
BRAZIL BSE END DATE 2023/03/03
https://wahis.woah.org/#/in-review/4918
https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html
SPAIN BSE START DATE 2023/01/21
SPAIN BSE CONFIRMATION DATE 2023/02/03
SPAIN BSE END DATE 2023/02/06
https://wahis.woah.org/#/in-review/4888
https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html
NETHERLANDS BSE START DATE 2023/02/01
NETHERLANDS BSE CONFIRMATION DATE 2023/02/01
NETHERLANDS BSE END DATE 2023/03/13
https://wahis.woah.org/#/in-review/4876
https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html
Scrapie TSE Prion
NVAP Reference Guide - Scrapie (Control and Eradication)
Last Modified: July 30, 2025
There are two types of scrapie; classical and nonclassical. Nonclassical scrapie is also referred to as atypical, Nor98, or Nor98-like scrapie. Nonclassical scrapie appears to occur sporadically and has occurred in sheep of all the common genotypes and goats. It is either not transmissible or poorly transmissible under natural conditions. Given this the scrapie eradication program focuses on classical scrapie. Here after where “scrapie” is used it is intended to mean “classical scrapie”.
Scrapie is a fatal, degenerative TSE disease affecting the central nervous system of sheep and goats. First recognized as a disease of sheep in Great Britain and other countries of Western Europe more than 250 years ago, scrapie has been reported throughout the world. In the United States, scrapie has primarily been reported in the black-face meat breeds and their crosses. It also has been diagnosed in numerous other breeds and crossbreeds including wool and hair sheep, and in goats. At the end of FY 2016, the percent of cull sheep found positive at slaughter and adjusted for face color was 0.001 percent with an upper confidence limit of 0.009 percent. This measure of prevalence has decreased by 99 percent since slaughter surveillance started in FY 2003. Based on all goats sampled at slaughter through FY 2016, the prevalence of scrapie in U.S. cull goats is 0.002 percent with an upper 95 percent confidence limit of 0.004 percent.
The agent responsible for scrapie and other TSEs is smaller than the smallest known virus and has not been completely characterized. There are a variety of theories regarding the nature of the agent. The most widely accepted is that disease is caused by an infectious protein, or prion, that causes the normal cellular version of the protein to change shape such that it can no longer be degraded by the cell, causing the protein to accumulate and damage the cell.
The scrapie agent is extremely resistant to heat and to normal sterilization processes. It does not evoke any detectable immune response or inflammatory reaction in host animals. The scrapie agent is thought to be spread most commonly from the ewe to her offspring and to other lambs in contemporary lambing groups through contact with the placenta and placental fluids and through milk and colostrum. Signs or effects of the disease usually appear 2 to 5 years after the animal is infected but may take longer to appear. Sheep usually live 1 to 6 months after the onset of clinical signs and in some cases longer, but death is inevitable.
On the farm, veterinarians identify scrapie suspects based on the appearance of its signs combined with knowledge of the animal’s history and signalment. Signs of scrapie vary widely among individual animals and develop very slowly. As the result of nerve cell damage, affected animals usually show behavioral changes, tremor (especially of the head and neck), pruritus, and locomotor incoordination, which progresses to recumbency and death. Early signs include subtle changes in behavior or temperament. These changes may be followed by scratching and rubbing against fixed objects, apparently to relieve itching. Other signs are loss of coordination, weight loss despite retention of appetite, biting of feet and limbs, lip smacking, and gait abnormalities, including high-stepping of the forelegs, hopping like a rabbit, and swaying of the back end.
An infected animal may appear normal if left undisturbed at rest. However, when stimulated by a sudden noise, excessive movement, or the stress of handling, the animal may tremble or fall down in a convulsive like state. Several other problems can cause clinical signs similar to scrapie in sheep, including the diseases ovine progressive pneumonia, listeriosis, and rabies; the presence of external parasites (lice and mites); pregnancy toxemia; and toxins.
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https://www.aphis.usda.gov/nvap/reference-guide/control-eradication/scrapie
“USA, AS of 2023, A total of 491 NVSL confirmed positive animals have been identified, including 474 classical cases (471 sheep and 3 goats) and 19 Nor98-like cases.”
https://www.aphis.usda.gov/sites/default/files/scrapie-annual-report.pdf
“Given this the scrapie eradication program focuses on classical scrapie. Here after where “scrapie” is used it is intended to mean “classical scrapie”.”
Atypical Nor98 Scrapie, Atypical BSE, CWD, Can Emerge As Different TSE PrP In Cross Species Transmission, A Volatile Situation For Human and Animal Health
Research Project: Genetic Impact and Improved Diagnostics for Sheep and Goat Transmissible Spongiform Encephalopathies Location: Animal Disease Research
Title: PrP-res in placental tissue following experimental transmission of atypical scrapie in ARR/ARR sheep is not infectious by Tg338 mouse bioassay
Author item Piel Iii, Robert item MCELLIOTT, VALERIE - University Of Georgia item STANTON, JAMES - University Of Georgia item ZHUANG, DONGYUE - Retired ARS Employee item MADSEN-BOUTERSE, SALLY - Washington State University item Hamburg, Linda item HARRINGTON, ROBERT - Retired ARS Employee item Schneider, David Submitted to: PLoS ONE Publication Type: Peer Reviewed Journal Publication Acceptance Date: 1/4/2022 Publication Date: 1/21/2022 Citation: Piel Iii, R., Mcelliott, V.R., Stanton, J.B., Zhuang, D., Madsen-Bouterse, S.A., Hamburg, L.K., Harrington, R.D., Schneider, D.A. 2022.
PrP-res in placental tissue following experimental transmission of atypical scrapie in ARR/ARR sheep is not infectious by Tg338 mouse bioassay.
PLoS ONE. PLoS ONE 17(1): e0262766.
https://doi.org/10.1371/journal.pone.0262766.
DOI: https://doi.org/10.1371/journal.pone.0262766
Interpretive Summary: Transmissible spongiform encephalopathies or prion diseases in sheep and goats are classified into two main categories: classical scrapie and Nor98-like/atypical scrapie. Classical scrapie is naturally transmissible and, along with surveillance and depopulation, breeding programs selecting for genetic resistance have been a major strategy for the control of classical scrapie in domestic ruminants. Nor98-like scrapie is generally considered non-transmissible in natural conditions and is currently exempt from control measures. Genotypes that confer resistance to classical scrapie remain susceptible to Nor98-like scrapie. ARR/ARR is a primary genotype target for resistance breeding programs and represents an increasing proportion of domestic sheep in the US. This study reports the experimental transmission of Nor98-like scrapie between ARR/ARR sheep, examines the pattern of disease in the recipient animals, and evaluates the potential for natural transmission from the recipient ewes to lambs. Inoculation of breeding ewes with Nor98-like scrapie was successful. Surprisingly, some protein with characteristics similar to prions was observed to accumulate in the placentas as recipient ewes aged; however, this material was determined to be non-infectious in a transgenic mouse model susceptible to scrapie.
Technical Abstract: Nor98-like atypical scrapie, isolated from a US sheep with the classical scrapie resistant ARR/ARR genotype, was transmitted to four ARR/ARR ewes via intracerebral inoculation of brain homogenate. These ewes were followed and observed to 8 years of age, remained non-clinical but exhibited progression of infection broadly consistent with Nor98-like scrapie, including characteristic patterns of PrP-Sc accumulation in the brain and a lack of accumulation in peripheral lymphoid tissues as detected by conventional methods. Immunoblots of placental tissues from the infected ewes revealed accumulation of a distinct conformation of PrP-res, particularly as the animals aged. These tissues showed no infectivity when analyzed via ovinized mouse bioassay. Taken together, these results support a low risk for natural transmission of Nor98-like scrapie in ARR/ARR sheep.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=388184
Volume 17, Number 5—May 2011
Research
Experimental Oral Transmission of Atypical Scrapie to Sheep
Marion M. SimmonsComments to Author , S. Jo Moore1, Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos
Author affiliations: Author affiliation: Veterinary Laboratories Agency–Weybridge, Addlestone, UK Cite This Article
Abstract
To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specific prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These findings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.
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Discussion This study is still ongoing and will not be completed until 2012. However, the current interim report documents the successful oral transmission of atypical scrapie, confirms that the disease phenotype is retained following transmission by this route in AHQ/AHQ sheep, and indicates that infectivity can be demonstrated in the gut in the absence of detectable PrPSc at least as early as 12 months after exposure.
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How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantified, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confirmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected.
https://wwwnc.cdc.gov/eid/article/17/5/10-1654_article
Experimental transmission of ovine atypical scrapie to cattle
Timm Konold, John Spiropoulos, Janet Hills, Hasina Abdul, Saira Cawthraw, Laura Phelan, Amy McKenna, Lauren Read, Sara Canoyra, Alba Marín-Moreno & Juan María Torres
Veterinary Research volume 54, Article number: 98 (2023)
Abstract
Classical bovine spongiform encephalopathy (BSE) in cattle was caused by the recycling and feeding of meat and bone meal contaminated with a transmissible spongiform encephalopathy (TSE) agent but its origin remains unknown. This study aimed to determine whether atypical scrapie could cause disease in cattle and to compare it with other known TSEs in cattle. Two groups of calves (five and two) were intracerebrally inoculated with atypical scrapie brain homogenate from two sheep with atypical scrapie. Controls were five calves intracerebrally inoculated with saline solution and one non-inoculated animal. Cattle were clinically monitored until clinical end-stage or at least 96 months post-inoculation (mpi). After euthanasia, tissues were collected for TSE diagnosis and potential transgenic mouse bioassay. One animal was culled with BSE-like clinical signs at 48 mpi. The other cattle either developed intercurrent diseases leading to cull or remained clinical unremarkable at study endpoint, including control cattle. None of the animals tested positive for TSEs by Western immunoblot and immunohistochemistry. Bioassay of brain samples from the clinical suspect in Ov-Tg338 and Bov-Tg110 mice was also negative. By contrast, protein misfolding cyclic amplification detected prions in the examined brains from atypical scrapie-challenged cattle, which had a classical BSE-like phenotype. This study demonstrates for the first time that a TSE agent with BSE-like properties can be amplified in cattle inoculated with atypical scrapie brain homogenate.
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This is the first study in cattle inoculated with naturally occurring scrapie isolates that found the presence of prions resembling classical BSE in bovine brain although this was limited to detection by the ultrasensitive PMCA. The results from thermostability assay confirmed that the isolates were as thermoresistant as the BSE agent as proven in other studies [36, 48]. Previous PMCA studies with various British atypical scrapie isolates did not find any evidence of amplification [49, 50]. This may be explained by the use of ovine brain as substrate rather than brain from Bov-Tg110 mice, which may facilitate conversion to classical BSE prions.
Two hypotheses for prion strain propagation in cross-species transmission experiments have been proposed: conformational selection favours a particular strain conformation out of a mixture of conformations in a scrapie isolate whilst mutation results in the conformational shift of one conformation into another [51]. Following on from the study in mice [17], it has been subsequently suggested that classical BSE properties that arise in atypical scrapie isolates transmitted to cattle may be due to conformational mutation in a new host [52]. It does not confirm that the atypical scrapie agent is the origin of the classical BSE epidemic and further transmission studies would be required to see whether classical BSE can be generated.
Would PMCA applied to brains from cattle exposed to TSE agents other than classical BSE and atypical scrapie also produce a classical BSE-like molecular phenotype? The PMCA product obtained in the thermostability test using a thermosensitive classical scrapie control showed a profile unlike classical BSE. Atypical BSE has been linked to the origin of classical BSE because of its conversion into classical BSE following serial passages in wild-type mice (L-type BSE [11]) and bovine transgenic mice (H-type BSE [53]). Although we have not tested PMCA products of atypical BSE isolates as part of this study, there is no evidence that PMCA products from atypical BSE convert into classical BSE, at least for H-type BSE using bovine brain as substrate [54]. In fact, we were unable to propagate H-type BSE using the same methodology (S Canoyra, A Marín-Moreno, JM Torres, unpublished observation).
The study results support the decision to maintain the current ban on animal meal in feedstuffs for ruminants, particularly as atypical scrapie occurs world-wide, and eradication is unlikely for a sporadic disease.
In summary, experimental inoculation of cattle with the atypical scrapie agent may produce clinical disease indistinguishable from classical BSE, which cannot be diagnosed by conventional diagnostic tests, but prions can be amplified by ultrasensitive tests in both clinically affected and clinically unremarkable cattle, which reveal classical BSE-like characteristics. Further studies are required to assess whether a BSE-like disease can be confirmed by conventional tests, which may initially include a second passage in cattle.
https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3
THURSDAY, JANUARY 7, 2021
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Nor98 cases Diagnosed in the US. To Date
Nor98 cases Diagnosed in the US.
Flock of Origin State FY
Wyoming 2007
Indiana 2007
Pennsylvania 2008
Oregon 2010
Ohio 2010
Pennsylvania 2010
Untraceable 2010
California 2011
Montana 2016
Utah 2017
Montana 2017
Virginia 2018
Colorado 2019
Colorado 2019
Wyoming 2020
Montana 2020
Pennsylvania 2021
Personal Communication from USDA et al Mon, Jan 4, 2021 11:37 am...terry
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
17 cases of the Nor98 in the USA to date, location, unknown...tss
17 Nor98-like cases since the beginning of RSSS.
https://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_report.pdf
17 Nor98-like cases since the beginning of RSSS. No animals have tested positive for classical scrapie in FY 2021.
https://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_report.pdf
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
https://scrapie-usa.blogspot.com/2020/09/aphis-usda-more-scrapie-atypical-nor-98.html
MONDAY, JULY 27, 2020
APHIS USDA Nor98-like scrapie was confirmed in a sheep sampled at slaughter in May 2020
https://nor-98.blogspot.com/2020/07/aphis-usda-nor98-like-scrapie-was.html
MONDAY, JULY 13, 2020
Efficient transmission of classical scrapie agent x124 by intralingual route to genetically susceptible sheep with a low dose inoculum
https://scrapie-usa.blogspot.com/2020/07/efficient-transmission-of-classical.html
WEDNESDAY, MAY 29, 2019
***> Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures
https://nor-98.blogspot.com/2019/05/incomplete-inactivation-of-atypical.html
THURSDAY, DECEMBER 31, 2020
Autoclave treatment of the classical scrapie agent US No. 13-7 and experimental inoculation to susceptible VRQ/ARQ sheep via the oral route results in decreased transmission efficiency
https://scrapie-usa.blogspot.com/2020/12/autoclave-treatment-of-classical.html
Snip…see full text;
https://nor-98.blogspot.com/2021/01/atypical-nor-98-scrapie-tse-prion-usa.html
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
==============
PRION 2015 CONFERENCE
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 1933-690X
WS-01: Prion diseases in animals and zoonotic potential
“Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.”
These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
Increased Atypical Scrapie Detections
Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.
http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf
Published: 04 October 2023
Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie
Marina Betancor, Belén Marín, Alicia Otero, Carlos Hedman, Antonio Romero, Tomás Barrio, Eloisa Sevilla, Jean-Yves Douet, Alvina Huor, Juan José Badiola, Olivier Andréoletti & Rosa Bolea Veterinary Research volume 54, Article number: 89 (2023) Cite this article
Abstract
The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently observed upon experimental transmission to rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle. Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease and tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prion seeding activity was detected during in vitro propagation of brain samples from the inoculated animals. These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.
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Discussion
Previous studies have demonstrated that C-BSE prions can be present as a minor variant in ovine atypical scrapie isolates and that C-BSE can emerge during the passage of these isolates to pigs and bovine PrP mice [7, 8]. These results pointed to atypical scrapie as a possible origin of C-BSE. Therefore, this study was meant to assess the link between atypical scrapie and C-BSE in the natural host of C-BSE, cattle. Although the intracerebral challenge has some limitations and does not reflect the natural transmission process of prions, bioassays using experimental prion inoculation have allowed to identify and describe the transmission mechanisms of these pathogens. Therefore, we decided to challenge cattle with an atypical scrapie isolate.
It is important to note that none of the animals in this study showed any clinical signs of TSE after inoculation with atypical scrapie, according to the results previously obtained in pigs [8]. In addition, the absence of spongiform changes in brain sections, as well as the absence of PrPSc accumulation by conventional techniques in brain areas from the atypical scrapie-inoculated cows, further highlights the need for highly sensitive techniques such as PMCA to detect low levels of prions. After the in vitro propagation of brain samples from the cows included in this study, seeding activity was detected in reactions seeded with brain material from three out of the four cows, in the areas of frontal cortex, thalamus, and/or cerebellum. Interestingly, none of the samples from the obex, which is one of the most affected areas in prion diseases [14], showed seeding activity. Importantly, the observed glycosylation pattern of the positive PMCA reactions was indistinguishable from that of C-BSE prions and PMCA products from reactions seeded with C-BSE prions. To check whether C-BSE-like prions were present in the original atypical scrapie isolate or if they emerged in the brain of the cows after the inoculation, we performed PMCA of the original inoculum in TgBov substrate, following the same conditions described above. The in vitro amplification of the atypical scrapie inoculum resulted in the propagation of BSE-like seeding activity, biochemically indistinguishable from C-BSE or positive PMCA reactions seeded with brain samples from the inoculated cows, suggesting that, as described before, certain atypical scrapie isolates contain low levels of C-BSE prions [9].
Moreover, in order to rule out a spontaneous in vitro misfolding of bovine PrP during PMCA, we included, as a control for the technique, brain samples from non-inoculated age-matching cows that were also subjected to serial in vitro propagation in TgBov substrate. No positivity was observed in PMCA reactions seeded with samples from these animals, suggesting a true C-BSE-like prion seeding activity and not a spontaneous in vitro misfolding of PrP.
All these results suggest the amplification of C-BSE-like prions during the transmission of ovine atypical scrapie to cows. It is true that, in order to confirm the presence of infectious BSE prions in the challenged cows, strain typing experiments of the PMCA products should be carried out in established mouse lines. Therefore, studies involving a bioassay in bovine and ovine PrP-expressing mice have been started.
Interestingly, the time after inoculation and the BSE-like prion seeding activity were not correlated. As previously stated, the emergence of C-BSE from atypical scrapie has been associated with the presence of low levels of C-BSE prions in the atypical scrapie isolates and our results after the in vitro amplification of the PS152 inoculum support this theory. Therefore, the number of C-BSE conformers contained in the used atypical scrapie isolates may be reduced and not homogeneously distributed, making cows receiving different amounts of C-BSE-like prions. It is true that the emergence of C-BSE-like PMCA seeding activity from the brains of cows could be related to the persistence of prions from the original atypical scrapie inoculum. Previous studies, in which prion seeding activity was detected in the brain of intracerebrally inoculated PrP0/0 mice have highlighted the capacity of prions to persist in non-replicative environments [15]. Nevertheless, cows were intracerebrally challenged in the frontal cortex, and seeding activity was detected in caudal regions of their brains but not in more rostral areas such as the frontal cortex. If these positive PMCA reactions were not a bona fide propagation of C-BSE-like prions but associated to inoculum persistence, it would be expected to detect such amplification in the most rostral areas of the brain. Although all these results support a bona fide propagation of C-BSE-like prions, the possibility of PMCA detecting remaining prions of the inoculum, would be definitely ruled out after in vivo bioassays in mouse lines, which are currently being carried out.
The lack of clinical signs of prion disease in cows after inoculation with atypical scrapie contrasts with results from a previous study in which bovine PrP mice (TgBov) were challenged with atypical scrapie isolates and displayed signs of clinical prion disease, developing neuropathological characteristics of C-BSE [7]. In addition, in the mentioned study, after the first passage, signs of clinical prion disease were only observed in a low proportion of the inoculated mice, and several of the inoculated isolates did not lead to PrPSc accumulation. Three serial passages of atypical scrapie were needed to observe complete attack rates in TgBov mice. Moreover, mice from the first passage that developed clinical signs showed long incubation periods considering the lifespan of a mouse. The cows in this study were also euthanized after a long post-inoculation period (between ~7 and ~11 years). However, the number of C-BSE-like prions present in the original atypical scrapie inoculum was probably too low to produce disease in the cows upon first passage. We also need to consider that TgBov mice overexpress ~8 times bovine PrPC, making them more susceptible to develop disease after the inoculation of C-BSE prions.
Further in vivo experiments challenging different mouse lines have been started in order to confirm the infectivity of the PMCA products obtained in this study. However, in conclusion, our findings show that the propagation of atypical scrapie in cattle leads to the emergence of BSE-like seeding activity. This is a concerning issue with far-reaching implications for public health and food safety. The possibility of interspecies transmission of prion diseases and the emergence of new prion strains highlight the critical need for continued surveillance and monitoring of these diseases in both animal and human populations. Early detection of prion diseases is crucial, and highly sensitive detection techniques such as PMCA can play an important role in this regard.
https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
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The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
12/10/76
AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note CHAIRMAN: PROFESSOR PETER WILDY
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A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates.
One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.
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76/10.12/4.6
http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html
Wednesday, February 16, 2011
IN CONFIDENCE
SCRAPIE TRANSMISSION TO CHIMPANZEES
IN CONFIDENCE
http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html
why do we not want to do TSE transmission studies on chimpanzees $
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5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
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R. BRADLEY
http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
BSE: TIME TO TAKE H.B. PARRY SERIOUSLY
If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1988/06/08004001.pdf
Scrapie: The flock identified in April 2016 remains under quarantine in Hartley County.
https://www.tahc.texas.gov/agency/meetings/minutes/SummaryMinutes_CommMtg_2020-09-22
http://web.archive.org/web/20201017124040/https://www.tahc.texas.gov/agency/meetings/minutes/SummaryMinutes_CommMtg_2020-09-22.pdf
A Space in Time, Scrapie USA, 2002!
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."
Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly infected flocks 2002) & CWD IN USA
As of September 30, 2002, there were 45 scrapie infected and source flocks (figure 3).
There were 105 newly infected flocks, reported in FY2002 (figure 4).
In addition, 379 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2002 (figure 5) and (figure 6).
Five cases of scrapie in goats were reported in FY 2002 (figure 7), the last of which was confirmed in August 2002.
New infected and source flocks numbers and the number of these flocks released in FY 2002 are depicted in chart 4.
One hundred (100) flocks which is 67 percent of the scrapie infected and source flocks present in FY 2002 were released or put on clean-up plans in FY2002. Slaughter Surveillance Slaughter Surveillance is currently in Phase II which is intended to determine the prevalence of scrapie in the US culled sheep population.
Through September 2002 samples from 3,269 sheep were submitted to NVSL for testing. Samples from a total of 6,795 sheep have been submitted since the beginning of Phase II on April 1, 2002. Surveillance regions are depicted in (figure 8).
Scrapie Testing During FY 2002 11,751 animals have been tested for scrapie which includes: 2,711 regular necropsy cases, 1,343 third eyelid biopsies for the test validation project, 546 third eyelid biopsies for the regulatory program, and approximately 7,151 animals for Phase I & II of SOSS (chart 5).
Laboratory testing has been taking 10 - 11 days on average with a range of 3 - 34 days. Ear Tag Orders During FY 2002 9.9 million plastic and 6.0 million metal tags were distributed by APHIS (chart 6).
http://www.aphis.usda.gov/vs/nahps/scrapie/annual_report/annual-report.html
Epidemiology of Scrapie in the United States
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Scrapie Field Trial was developed at Mission, Texas, what if?
Scrapie Field Trial was developed at Mission, Texas, on 450 acres of pastureland, part of the former Moore Air Force
EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES
Academic Preg
James Hourriganl, Albert Klingsporn2, Edited by » Peast
W. W. Clark3, and M, de Camp4
United States Department of Agriculture, Animal and Plant Health Inspection Service, Veterinary Services
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METHODS
A Scrapie Field Trial was developed at Mission, Texas, to provide additional information for the eradication program on the epidemiology of natural scrapie. The Mission Field Trial Station is located on 450 acres of pastureland, part of the former Moore Air Force Base, near Mission,
Texas. It was designed to bring previously exposed, and later also unexposed, sheep or goats to the Station and maintain and breed them under close observation for extended periods
to determine which animals would develop scrapie and define more closely the natural spread and other epidemiological aspects of the disease.
The 547 previously exposed sheep brought to the Mission Station beginning in 1964 were of the Cheviot, Hampshire, Montadale, or Suffolk breeds. They were purchased as field outbreaks occurred, and represented 21 bloodlines in which scrapie had been diagnosed. Upon arrival at the Station, the sheep were maintained on pasture, with supplemental feeding as necessary. The station was divided into 2 areas:
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RESULTS
Table 1 indicated that previously exposed sheep brought to the Station at various times and ages (1 to 89 months old) included 333 Suffolks at risk. Of these, 98 (29%) developed scrapie. This demonstrated the necessity to slaughter such sheep to prevent further Spread of the disease, These pre- viously exposed Suffolks were bred at the Station and produced 446 progeny at risk. Of these 153 (34%) developed scrapie.
Although the minimum and average ages when scnapied were similar for both groups, some of the previously exposed Suffolks brought to the Station developed scrapie when much older--ewes 60 to 142 months old and rams 67 to 102 months old. O£ the 153 Suffolks born at the Station, only 3 were more than 60 months of age (65, 66, and 69 months old).
This difference in age scrapied was attributed to the fact that the Suffolks born at the Station may have been sub- ject to a greater exposure from birth.
It was also observed that when both dam and progeny were scrapied, the progeny nearly always developed clinical disease at a younger age than their respective dam. Thirty- two dams were scrapied at an average of 60 months of age. Forty-six of their progeny developed the disease at an average of 38 months (range 25 to 53 months). Thirty-seven of the 46 progeny were younger than the dam (average 20 months younger, range 2 to 99 months younger). Two were scrapied at the same age as their dams, and 8 were older (average 5 months, range 1 to 13 months older).
++. Although the incidence of scrapie was considerably Greater in the progeny of scrapied compared to free dams, the progeny of either scrapied or free dams manifested scrapie at the typical age and irrespective of the age their respective dams were scrapied. The differences in ages that dams and progeny were scrapied was believed due to difference of exposure, particularly whether they were exposed at an early age,
Table 2 summarized the data on exposed Suffolks and was Prepared so as to show scrapie incidence in the progeny of dams and sires of known Scrapie status. The scrapie incidence in the progeny of Free X Free parents was 25%, progeny of scrapied Sires 39%, and scrapied dams 42%. When both sire and dam were scrapied, the scrapie incidence in 18 Progeny at risk was 78%.
When the scrapie status of the sire was ignored, scrapie incidence in th- progeny of free dams was 34% and in pre y of scrapied da as 62%. When the scrapie status of the dam was ignored, scrapie incidence in the progeny of free sires was 26% and in the progeny of scrapied sires was 452.
Although the scrapie incidence was nearly double in the progeny of scrapied compared to free dams, the latter con- tributed a greater number of scrapied progeny, 116, compared to only 51 cases which had scrapied dams. This was because free dams made a considerably heavier contribution to the progeny at risk4-342 compared to 82. It was felt that in farm flocks a similar situation could exist.
It was possible that free dams could have been mis- classified; however, this was unlikely to have been significant, unless "nonclinical or carrier" dams exist. In this Suffolk group, the ages of 100 free dams of scrapied progeny ranged from 25 to 160 (average 97) months. These free dams did not show clinical signs of scrapie,”and there were no histopathological lesions suggesting scrapie in those which died, If one cannot classify as free, ewes which have reached 97 months (average) and did not develop the disease, from a practical standpoint, it is not possible to classify sheep as free, at least on the basis of clinical signs and histology. The free dams of 50% of the scrapied progeny were more than 100 months of age, averaging 126 months.
Upon arrival at the Mission Station at 3 to 9 months of age, the 140 previously unexposed sheep and goats were placed in infected pastures and corrals and were subjected to con- tact with a succession of natural cases of scrapie in sheep, and eventually also in goats. These animals were bred only within their respective groups and were not crossbred to other breeds of sheep or those brought to the Station from infected flocks or their progeny. The male or female animals mixed freely with animals of their respective sex of the infected Flock and were similarly identified and subjected to similar flock management and diagnostic procedures.
Table 3 indicated that natural scrapie had occurred in 5 of the 140 previously unexposed sheep. One case each occurred in Rambouillet, Targhee, and Hampshire ewes at 88, 89, and 89 months of age and in % Suffolk ewes at 73 and 102 months of age, and 85, 82, 80, 64, and 93 months following initial natural exposure. This represented a natural situation involving lateral spread, under the circumstances involved, when sheep were not exposed when very young. Scrapie was not detected clinicaliy or histologically in any of the dairy or Angora goats brought to the Station. The disease occurred in an average of 27% of the progeny of previously unexposed sheep or goats born at the Station and included cases in progeny of all breeds of sheep or goats taken there, The incidence in the progeny ranged from 14% in Rambouillet sheep to 61% in dairy goats. ~
These data showed that scrapie spread laterally, by contact exposure, from scrapied te previously free animals, but at an apparently lower rate when exposure was first received at the age of 3 to 9 months. These animals were presumed to be susceptible to the disease, as their progeny developed scrapie at rates and ages similar (on the average) to the progeny, pf previously exposed Suffolk sheep born and reared in the same environment.
It was suggested that the progeny of previously unexposed animals developed scrapie at a much higher rate than their parents, and at a younger age, because they were subjected to exposure from birth. The data did not rule out the possibility that the animals born at the Station could have also received the virus from their dams "vertically" prior te, at, or following birth.
Table 4 summarized the scrapie incidence in #he progeny, born at the Station, of previously unexposed dairy goats.
The data were prepared so as to show scrapie incidence in the progeny of dams and sires of known scrapie status.
The 58% incidence in the progeny (24 at risk) of Free X Free parents was more than twice the 25% seen in the Suffolk group (Table 2). Scrapied sires did not increase the incidence in goat progeny (it was 44%); scrapied dams increased the incidence to 71%. When both sire and dam were scrapied the incidence was 89%, with only 9 goat progeny at risk.
When the scrapie status of the sire was ignored, the scrapie incidence in the progeny of free dams was 56% and in the progeny of scrapied dams it was 74%.
Free dams contributed 34 progeny at risk and scrapied dams 31 progeny.
When the scrapie status of the dam was ignored, scrapie incidence was 64% in the progeny of free sires and a similar 66% in the progeny of scrapied sires.
A total of 244 sheep (127 Suffolk, 59 Rambouillet, and 58 Targhee) were removed from scrapie exposure within a few hours of birth or at 4, 9, or 20 months of age and placed in isolation pens. Removal of sheep from exposure at these ages was selected as being representative of usual flock operations when sheep might be sold from an infected flock at weaning, the first fall or the second fall after their birth.
Table 5 reflected the fate of such animals. Four of the 6 scrapied sheep which had been isolated at birth were Suffolks and the 2 older animals were Targhees. The first case in the group isolated at birth was a Targhee, progeny of a ewe that did not develop clinical scrapie. The scrapie incidence in 36 at risk Suffolks removed from exposure at birth was 11%, con- siderably less -“en that expected had these animals remz d in an infected en ment.
Table 6 reflected the status of 51 goats isolated from scrapie exposure at birth, and at 6, 8 to 10, 20, 32 to 59 and 60 to 82 months of age.
None of the goats removed at birth developed scrapie, although all 5 of those alive at 5 years of age had scrapied dams and 1 also had a scrapied sire. The sire of the remaining 4 had sired 7 scrapied progeny. Under such circumstances, had they remained in an infected environment nearly all of these goats would have been expected to develop scrapie. With the exception of the 20 month group, scrapie occurred at an incidence of 25 to 100% in ali other groups and at the expected age. A further observation was that 4 of the progeny of these dairy goats, born and kept apart from any sheep, developed scrapie which suggested that goats were not "dead- end hosts" insofar as scrapie was concerned.
Table 7 recorded the fate of progeny of certain selected scrapied or free Suffolk sheep or dairy goat dams.’
Suffolk ewe G298 was scrapied at 46 months of age. She had twin lambs in 1969 and 1 lamb in 1970. All 3 lambs developed scrapie. Suffolk ewe G27a was scrapied at 39 months. Her lamb born in 1966 was scrapied at 53 months; however, her lambs born in 1967 and 1968 remained free--lived to 102 months of age.
Suffolk ewe G25a died at 131] months of age and was nega- tive clinically and histologically. Mice remained negative following intracerebral inoculation of brain, spleen, and lymph nodes from this ewe. This ewe had 9 progeny at risk, of which 4 developed scrapie and 5 did not. There was no dis- cernible pattern to the cases. In two instances, 1 twin was scrapied and 1 remained free.
Goat B259 was scrapied when 43 months old. All of her 6 progeny at risk developed scrapie.
Goat B14a remained free and died at 101 months of age. Of her 11 progeny at risk, 7 were scrapied and 4 were not.
It was observed at the Station that when scrapied dams had several progeny at risk, 1 or more progeny usually developed the disease. However, many such scrapied dams also had progeny which lived, or are living, considerably beyond the age of their dams and beyond the age animals born at the Station manifested the disease.
It was also observed that individual free dams had free progeny in earlier years followed by scrapied progeny when they were older, or had scrapied progeny when young followed by free progeny when older, or scrapie and free progeny dis- persed throughout the dam's breeding life. The same situation occurred in progeny of scrapied dams; however, the pattern was less irregular due to the smaller number of progeny from each scrapied dam and the higher incidence of scrapie in such progeny. Circumstances prevented breeding all ewes ary year and, thus, many had only 1 progeny at risk. Scrapie developed in 100% of the single progeny at risk of 11 scrapied and 15 free dams. The 26 scrapied progeny were equally divided between ewes and rams.
Table 8 reflected the difference in age scrapied of - sheep brought to the Station compared to the age scrapied of those born there. Although the average age of previously exposed sheep (Suffolks) brought to the Station did not differ greatly from the overall average, several animals brought to the Station developed the disease at quite advanced ages. The previously unexposed scrapied animals brought to the Station were also considerably older than animals born there. Progeny of scrapied dams developed the disease at a slightly younger age than did progeny of free dams. The average age was nearly the same for males and females.
DISCUSSION
snip...see full text;
http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf
Scrapie Field Trial was developed at Mission, Texas, on 450 acres of pastureland, part of the former Moore Air Force Base
EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES
http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf
Porcine Spongiform Encephalopathy or Pig CWD or Pig BSE?
Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States
Volume 31, Number 1—January 2025
Abstract
Using a prion amplification assay, we identified prions in tissues from wild pigs (Sus scrofa) living in areas of the United States with variable chronic wasting disease (CWD) epidemiology. Our findings indicate that scavenging swine could play a role in disseminating CWD and could therefore influence its epidemiology, geographic distribution, and interspecies spread.
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Conclusions
In summary, results from this study showed that wild pigs are exposed to cervid prions, although the pigs seem to display some resistance to infection via natural exposure. Future studies should address the susceptibility of this invasive animal species to the multiple prion strains circulating in the environment. Nonetheless, identification of CWD prions in wild pig tissues indicated the potential for pigs to move prions across the landscape, which may, in turn, influence the epidemiology and geographic spread of CWD.
https://wwwnc.cdc.gov/eid/article/31/1/24-0401_article
Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.
2. Determined that pigs naturally exposed to chronic wasting disease (CWD) may act as a reservoir of CWD infectivity. Chronic wasting disease is a naturally occurring, fatal, neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of CWD disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Pigs were assigned to 1 of 3 groups: intracranially inoculated; orally inoculated; or non-inoculated. At market weight age, half of the pigs in each group were tested ('market weight' groups). The remaining pigs ('aged' groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by multiple diagnostic methods. Brain samples from selected pigs were bioassayed in mice expressing porcine prion protein. Some pigs from each inoculated group were positive by one or more tests. Bioassay was positive in 4 out of 5 pigs assayed. Although only small amounts of PrPSc were detected using sensitive methods, this study demonstrates that pigs can serve as hosts for CWD. Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091
https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017
https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105
https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166
Infectious prions in brains and muscles of domestic pigs experimentally challenged with the BSE, scrapie, and CWD agents
Authors: Francisca Bravo-Risi, Fraser Brydon, Angela Chong, Kane Spicker, Justin J. Greenlee, Glenn Telling, Claudio Soto, Sandra Pritzkow, Marcelo A. Barria, Rodrigo Morales rodrigo.moralesloyola@uth.tmc.edu
ABSTRACT
Experimental studies suggest that animal species not previously described as naturally infected by prions are susceptible to prion diseases affecting sheep, cattle, and deer. These interspecies transmissions may generate prions with unknown host ranges. Pigs are susceptible to prions from different origins, including deer chronic wasting disease (CWD), sheep scrapie, and bovine spongiform encephalopathy (BSE). Here, we studied prions in brains and muscles from pigs previously infected with these different prion sources. Specifically, we measured the total prion protein (PrP) and PK-resistant PrP by western blot. Seeding activity in these tissues was evaluated using the protein misfolding cyclic amplification (PMCA) technique. We found that BSE-infected pigs contained substantially more seeding competent prions compared with those infected with CWD and scrapie. Moreover, the zoonotic potential of porcine-BSE prions seems to be relevant, as both brains and muscles from BSE-infected pigs induced the misfolding of the human prion protein in vitro. This study helps to understand the potential fate of naturally existing prion strains in a relevant host and calls for caution considering the co-existence between feral swine and other prion-susceptible animal species.
IMPORTANCE
Prions (PrPSc) are proteinaceous, infectious pathogens responsible for prion diseases. Some livestock are highly susceptible to prion diseases. These include cattle (bovine spongiform encephalopathy, BSE), sheep and goat (scrapie), and cervids (chronic wasting disease, CWD). Unfortunately, BSE has been reported to be naturally transmitted to humans and other animal species. Domestic pigs, a relevant livestock animal, have not been reported to be naturally affected by prions; however, they are susceptible to the experimental exposure to BSE, scrapie, and CWD prions. Given the widespread consumption of porcine food products by humans, we aimed to evaluate the levels of pig-derived BSE, scrapie, and CWD prions from experimentally challenged domestic pigs in brain and meat cuts (leg, cheek meat, skirt meat, and tenderloin). We detected pig-adapted prions in the brains and some muscles of these animals. Additionally, we evaluated the in vitro compatibility between pig prions and the human prion protein (as a surrogate of zoonosis). Our results show that only pig-derived BSE prions were able to induce the misfolding of the cellular human prion protein. This data highlights the consequences of prion spillovers to other animal species and their potential availability to humans.
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DISCUSSION
In this study, we evaluated the presence and relative quantities of prions in brains and muscles from pigs infected with three different prion isolates: C-type BSE, scrapie, and CWD. Our findings confirm previous reports by demonstrating the presence of disease-associated prions in the brains of these animals (20, 56, 61). In addition, we evaluated the replication capacity of the prions present in these samples using the PMCA technique. The presence of prions in the brains of these prion-infected pigs appears to be variable depending on the original prion source. Although prion seeding activity was identified in the brains of pigs from all three groups, we found that those from animals infected with BSE contained the most active seeds. It is relevant to mention that most of the pigs infected with either scrapie or CWD did not display neurological signs linked with prion disease, yet prion seeding activity was still detected in their brains. The Moore et al. publication (61) states the presence of sparse PK-resistant PrP accumulation in the brain of CWD-infected pigs, suggesting a low amount of PrPSc in these samples. To improve the detection, the authors treated brain extracts with a sarkosyl/ultracentrifugation protocol and loaded at 100 mg of brain tissue for detection via western blots. Due to the limited amount of sample available for the present study, we did not enrich the sample and loaded 2 mg of brain tissue on western blots. This might have affected the detection of the PrP signals using this method (Fig. 2; Fig. S2). The low amount of PrPSc in these samples was further confirmed by PMCA, as seeding activity was present in just some replicates (Fig. 4), mostly at low dilutions. Nevertheless, the identification of subclinical prion infection in these brains is consistent with previous studies suggesting the same when bioassays in transgenic mice expressing the porcine PrPC were used in the BSE and CWD transmissions (26, 56). The fact that subclinical prion infection was also identified in pigs orally exposed to BSE and CWD (56, 61, 62) suggests that transmissions in natural scenarios are possible and an eventual adaptation of the agent in pigs (e.g., considering the cannibalistic behavior of wild pigs) is plausible.
In addition to the prion content in brains, we evaluated the anatomical distribution of PrPSc in several skeletal muscles with the goal of understanding the tropism of the adapted prions in these tissues. The specific muscles tested included semitendinosus (leg), masseter (cheek meat), diaphragm (skirt meat), and psoas major (tenderloin). These pork cuts are commonly consumed by people and are commercially available. Unfortunately, the muscle tested for the BSE-infected group (semitendinosus) differed from those analyzed in the scrapie and CWD groups (masseter, diaphragm, and psoas) due to the availability of archived materials. Regardless, these samples are useful to understand whether prions in pigs accumulate in muscles that might be available for human consumption. Our results indicate that the muscles collected from the BSE-infected pigs contain higher levels of seeding-competent prions compared with those in other groups. Nevertheless, the same cautions regarding the routes of administration of BSE materials in pigs explained above must be considered when interpreting this data. The comparison between scrapie and CWD inoculated pig groups, that were inoculated with the same quantities of infectious materials and the same route, and where the same tissues were collected, allows us to make more accurate associations. In summary, our findings demonstrate that muscles closer to the animal head (masseter) supported a better seeding activity compared to those located at the thorax (diaphragm) and hind levels (psoas major). One explanation for this could be explained by the centrifugal spreading of prions, which agrees with the findings by Headman and colleagues (58) describing the detection of PrPSc in upper muscles of clinically affected pigs inoculated intracerebrally with sheep-BSE prions. In that study, two swine harbored PrPSc in muscles associated with the eyes (oculomotor muscle), while prions were identified in the semitendinosus muscle of just one animal. Recently, the same group analyzed the peripheral tissues of these pigs by PMCA, detecting seeding activity in the oculomotor muscle in all the animals exposed to the BSE-derived agent in dilutions as low as 10−4 (67). Due to the limited amount of muscle samples from the pigs inoculated with BSE, we were unable to perform a detailed anatomical analysis. However, we showed that the semitendinosus muscle, which is located at the hind limbs, contained relevant levels of seeding competent PrPSc. The relevantly abundant presence of prions in this particular group of pigs could be due to the fact that they were peripherally exposed to the agent, or by the peripheral tropism of BSE prions as it has been observed for the transmission of this particular prion strain to multiple other animal species (62, 68–80). The data describing higher quantities of seeding-competent prions in the brains of BSE-infected pigs needs to be interpreted with caution. As described, BSE-infected pigs received infectious materials at higher doses and through three simultaneous routes of administration compared to the animals exposed to the scrapie and CWD agents (20, 56, 61). If we restrict these comparisons to the latter two groups (scrapie- and CWD-infected pigs) that were exposed to the same prion doses (750 µL of 10% [w/v] pooled sheep and cervid brain homogenate, respectively), and through the same route of inoculation (intracerebral), we found that pig-adapted scrapie prions exhibited a higher amount of seeding-competent PrPSc in both brain and muscle tissues compared with their CWD-exposed counterparts. The differences in seeding capacities and tropisms between these newly generated porcine prions can be explained in the generation of different prion strains in each case. Nevertheless, it is also relevant to consider that these studies used unique scrapie and CWD strains from the many described in experimental and natural settings (31, 33, 40, 41). Along this line, future studies should focus on the differential susceptibilities experienced by pigs when exposed to different variants associated with these animal prionopathies.
Importantly, our studies evaluating zoonotic potentials showed that pig-adapted BSE prions were able to induce the misfolding of the human prion protein in vitro. These results were confirmed in two different laboratories, a fact that provides rigor to this study. Importantly, these analyses also show that muscles from these BSE-infected pigs may present a concern if introduced in the human food chain. Importantly, the zoonotic risk of these materials seems to be restricted to the 129M polymorphic version of the human prion protein, in line with the higher susceptibility of BSE to the population carrying this specific protein. Previous reports have shown that mice expressing the 129M human PrPC variant (HuPrP-tg 650 and 340) exhibited a species barrier to BSE prions that was overcome in a first transmission passage (81). Similar outcomes were observed when pig-adapted BSE prions were inoculated in HuPrP-tg340 mice, although with incomplete attack rates (50%) in a first passage. Serial transmissions of this infectious material in the same mice resulted in complete attack rates and reduction in the incubation periods, suggesting that the infectious material successfully adapted in the new host (60). On the contrary, extensive evidence demonstrates that the species barrier between scrapie and CWD prions and humans is strong, if not absolute (39, 42, 82). However, the existence of different prion strains associated with these animal prionopathies urges for a systematic analysis including the whole pathogenic spectrum associated with them. In our study, scrapie- and CWD-adapted prions were not able to induce the misfolding of the human prion protein. However, considering the data from the porcine-BSE prions, we cannot conclude whether the lower load of prions in the CWD- and scrapie-porcine tissues was responsible for these results.
Some of the limitations of the current study have been already discussed. One of the issues involves the unknown prion infectivity titers of the BSE-, scrapie-, and CWD-bearing tissues used to infect pigs. This importantly limits comparisons between the groups. Prion infectivity titers can affect tissue tropisms, especially in subclinical transmissions (as prions may need additional time to reach target tissues). This could be considered an additional source of variability for the dissimilar presence of prions in muscles for the different animal groups included in this study. Another limitation involves the uneven testing of muscle tissues across the three groups and the lack of analysis of other muscles representing different anatomical distributions within the body. The latter may be relevant for understanding the tropism of prions induced by each injectate. Finally, the scrapie and CWD groups were treated with specific prion strains from many that have been described. This is relevant considering that different strains may adapt with different efficiencies into new hosts and result in infectious particles with unique host ranges. Future studies should focus on the above-mentioned limitations. However, we believe that the current study provides a solid background to justify these analyses.
In summary, our data shows the dynamic of animal prions when exposed to infectious pigs, as well as their distributions and zoonotic potentials. The data presented here may be relevant to understanding the fate of naturally existing prions in a sympatric animal species relevant for human consumption. This acquires importance considering a recent report describing the interaction between CWD and wild pigs in natural settings.
https://journals.asm.org/doi/10.1128/mbio.01800-25
Experimental transmission of the chronic wasting disease agent to swine after oral or intracranial inoculation
Running Title: The chronic wasting disease agent transmits to swine
JVI Accepted Manuscript Posted Online 12 July 2017 J. Virol. doi:10.1128/JVI.00926-17
Running Title: The chronic wasting disease agent transmits to swine
JVI Accepted Manuscript Posted Online 12 July 2017 J. Virol. doi:10.1128/JVI.00926-17
Abstract
Chronic wasting disease (CWD) is a naturally occurring, fatal neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation . Crossbred piglets were assigned to one of three groups: intracranially inoculated (n=20), orally inoculated (n=19), or non -inoculated (n=9). At approximately the age at which commercial pigs reach market weight, half of the pigs in each group were culled (‘market weight’ groups). The remaining pigs (‘aged’ groups) were allowed to incubate for up to 73 months post inoculation (MPI ). Tissues collected at necropsy were examined for disease -associated prion protein (PrPSc) by western blotting (WB), antigen -capture immunoassay (EIA), immunohistochemistry (IHC) and in vitro real -time quaking induced conversion (RT -QuIC). Brain samples from selected pigs were also bioassayed in mice expressing porcine prion protein. Four intracranially inoculated aged pigs and one orally inoculated aged pig were positive by EIA, IHC and/or WB. Using RT -QuIC, PrPSc was detected in lymphoid and/or brain tissue from one or more pigs in each inoculated group. Bioassay was positive in 4 out of 5 pigs assayed.
This study demonstrates that pigs can support low -level amplification of CWD prions, although the species barrier to CWD infection is relatively high. However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.
https://journals.asm.org/doi/10.1128/jvi.00926-17
“However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.”
CWD TSE Prion, Cattle, Sheep, Pigs, Felines, Coyotes, Raccoons, Rodents, Primates, Oh My!
Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry
Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure
Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA
Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.
Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).
Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.
Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.
"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons
https://www.ars.usda.gov/research/publications/publication/?seqNo115=400777
Distribution of chronic wasting disease (CWD) prions in tissues from experimentally exposed coyotes (Canis latrans)
Published: July 9, 2025
Abstract
Cervids susceptible to chronic wasting disease (CWD) are sympatric with multiple other animal species that can interact with infectious prions. Several reports have described the susceptibility of other species to CWD prions, or their potential to transport them. One of these species is the coyote (Canis latrans), which has been previously shown to pass transmission-relevant prion titers in their feces for at least three days after ingesting prion-positive brain material. The current study followed up on these findings and evaluated the distribution of prions in multiple tissues from the same coyotes. Our results show that prions persist in the digestive tract of prion-exposed coyotes five days after exposure. Moreover, prion seeding activity was identified in other tissues, including lymph nodes and lungs. These results provide additional information about the dynamics of CWD prions in the environment and show the initial fate of prions after ingestion by a canid species that is a carnivorous predator and scavenger.
Snip…
Despite the strong species barriers, predators and scavengers may still be relevant as spreaders of infectious prion particles. In fact, both experimental research and natural observations indicate that insects, parasites, and various wild and domestic animals that co-exist with cervids can act as passive carriers of CWD prions [16–23]. For example, coyotes (Canis latrans) are relevant deer predators and scavengers. Previous studies showed that coyotes fed with an elk brain infected with CWD passed prions through their feces for at least three days after ingestion [23]. Similar observations were reported for cougars (Puma concolor) [21]. In contrast, crows (Corvus brachyrhynchos) passed prions for only few hours after ingesting infected materials [22]. Importantly, previous research in coyotes and cougars suggest that excreted prions contain decreased infectivity titers compared with the ingested material, suggesting that infectious particles are being retained in tissues within these animals, or degraded. In natural settings, prions have been identified in scat from multiple species of sympatric animals [20]. Overall, data indicates that predators and scavengers can excrete infectious prions after ingesting contaminated tissues. This, in turn, may contribute to the environmental spread of prions.
Snip…
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0327485
PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)
Davis Seelig, Amy Nalls, Maryanne Flasik, Victoria Frank, Candace Mathiason, Edward Hoover Colorado State University; Fort Collins, CO USA
Background and Introduction. Chronic wasting disease (CWD) is an efficiently transmitted prion disease of cervids with an as yet to be fully defined host range. Moreover, the risk that CWD poses to feline predators and scavangers, through crossspecies consumption and subsequent transmission, is unknown. Previous and ongoing studies in our laboratory evaluating the susceptibility of domestic cats (Felis catus) to CWD (Mathiason et. al., NeuroPrion 2011, Nalls et. al., NeuroPrion 2012) have documented the susceptibility of domestic cats to CWD following intracerebral (IC) inoculation. However, many of the pathologic features of feline-adapted CWD, including the neural and systemic patterns of PrPCWD accumulation and neuropathology, remain unknown. The chief objectives of this work were:
(1) to design a sensitive, enhanced immunohistochemical (E-IHC) protocol for the detection of CWD prions (PrPCWD) in feline tissues;
(2) to document the systemic distribution of PrPCWD in CWD-infected cats through E-IHC;
(3) to utilize single and multiple-label immunostaining and laser scanning confocal microscopy (LSCM) to provide insights into the subcellular patterns of PrPCWD accumulation and neuropathologic features of CWD-infected cats; and
(4) to compare feline CWD to the other known feline TSE
Materials and Methods. Periodate-lysine-paraformaldehyde (PLP)-fixed, paraffin-embedded (PLP-PE) from terminal, IC-inoculated (n = 9) and sham-inoculated (n = 2), 1st and 2nd passage, CWD-infected cats were examined by E-IHC for the presence of PrPCWD and its association with markers of cell phenotype and organelles. Results. The most sensitive E-IHC technique for the detection of PrPCWD in feline tissues incorporated a combination of slide pretreatment with proteinase-K (PK) in concert with tyramide signal amplification (TSA). With this protocol, we identified PrPCWD deposits throughout the CNS, which, in the 1st passage cats was primarily restricted to the obex, but increased in distribution and severity upon 2nd passage to include a number of midbrain nuclei, cortical gray matter, the thalamus and hypothalamus, and the hippocampus. Peripheral PrPCWD deposits were detected only in the 2nd passage cats, and included the enteric nervous system, the Peyer’s patches, and the retropharyngeal and mesenteric lymph nodes. PrPCWD was not detected in the sham-inoculated cats. Moreover, using multi-label analysis, intracellular PrPCWD aggregates were seen in association with neurofilament heavy chain (NFH)-positive neurons and GFAP-positive astrocytes. In addition, large aggregates of intracellular PrPCWD were identified within LAMP1-positive lysosomes. Conclusions. Feline PrPCWD is present in CNS neurons, astrocytes and LAMP-1-positive lysosomes. The morphologic overlap between the PrPCWD deposits in feline CWD and BSE-origin feline spongiform encephalopathy (FSE), implicates the importance of the host as a key determinant in the development of prion neuropathology and suggest a signature for detection of potential spontaneous feline prion disease.
http://www.landesbioscience.com/journals/prion/04-Prion6-2-Pathogenesis-and-pathology.pdf
https://web.archive.org/web/20140514022931/http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf
PO-041: Susceptibility of domestic cats to CWD infection
Amy Nalls, Jeanette Hayes-Klug, Kelly Anderson, Davis Seelig, Kevin Carnes, Susan Kraft, Edward Hoover, Candace Mathiason Colorado State University; Fort Collins, CO USA
Domestic and non-domestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE); very likely due to consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain homogenate. Between 40 and 43 months two IC-inoculated cats developed slowly progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors, and ataxia”’ultimately mandating euthanasia. PrPCWD was detected in the brains of these animals by western blot, immunohistochemistry (IHC), and quaking-induced conversion (RT-QuIC) assays. No clinical signs of TSE were detected in the remaining primary passage cats at 86 months pi. Feline-adapted CWD (FelCWD) was sub-passaged into groups (n = 4 or 5) of cats by IC, PO, and IP/SQ routes. All 5 IC inoculated cats developed symptoms of disease 20–24 months pi (approximately half the incubation period of primary passage). Additional symptoms in these animals included increasing aggressiveness and hyper responsiveness. FelCWD was demonstrated in the brains of all the affected cats by western blot and IHC. Currently, 3 of 4 IP/SQ, and 1 of 4 PO inoculated cats have developed abnormal behavior patterns consistent with the early stage of feline CWD. Magnetic resonance imaging (MRI) has been performed on 11 cats (6 clinically ill, 2 asymptomatic, and 3 age-matched negative controls).
Abnormalities were detected in 4 of 6 clinically ill cats and included multifocal signal changes consistent with inflammation, ventricular size increases, more prominent sulci, and white matter tract cavitation.
These results demonstrate that CWD can be transmitted and adapted to the domestic cat, and raise the potential for cervid-to-feline transmission in nature.
https://web.archive.org/web/20140514022931/http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf
MONDAY, AUGUST 8, 2011
Susceptibility of Domestic Cats to CWD Infection Oral.29: Susceptibility of Domestic Cats to CWD Infection
http://felinespongiformencephalopathyfse.blogspot.com/2011/08/susceptibility-of-domestic-cats-to-cwd.html
THURSDAY, JULY 10, 2025
Distribution of chronic wasting disease (CWD) prions in tissues from experimentally exposed coyotes (Canis latrans) Published: July 9, 2025
https://chronic-wasting-disease.blogspot.com/2025/07/distribution-of-chronic-wasting-disease.html
MONDAY, JANUARY 08, 2024
CWD TSE Prion, using canine and feline species as a tool, as scavengers to contain disease, is a bad idea, here's why
https://chronic-wasting-disease.blogspot.com/2024/01/cwd-tse-prion-using-canine-and-feline.html
FRIDAY, MARCH 24, 2023
Mountain lions, Wolves, Coyotes, could help stop the spread of CWD TSE Prion in deer, WHERE STUPID MEETS THE ROAD!
https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/mountain-lions-wolves-coyotes-could.html
Camel Prion Disease
Friday, May 12, 2023
Camel prion disease, a new emerging disease in North Africa, Lymphoid Tropism, Neuropathological Characterization Update 2023
11th Iberian Congress on Prions Barcelona 2023
http://prioncongressbcn.ppmclab.com/book_abstracts_v4.pdf
https://camelusprp.blogspot.com/2023/05/camel-prion-disease-new-emerging.html
A Camelid Anti-PrP Antibody Abrogates PrPSc Replication in Prion-Permissive Neuroblastoma Cell Lines
Daryl Rhys Jones,William Alexander Taylor,Clive Bate,Monique David,Mourad Tayebi
Published: March 22, 2010
https://doi.org/10.1371/journal.pone.0009804
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0009804
15 Apr 2018 23:13 GMT MOST RECENT
Prion Disease in Dromedary Camels, Algeria
Posted by flounder on 15 Apr 2018 at 23:13 GMT
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/fea97a95-2600-42a6-b289-0f490896a3aa
https://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0009804
https://wwwnc.cdc.gov/eid/article/24/6/17-2007_article
Chronic Wasting Disease CWD TSE Prion
Captive CHRONIC WASTING DISEASE CASES Update August 2025 CHRONIC WASTING DISEASE CASES
Expanding Distribution of CWD
https://www.usgs.gov/centers/nwhc/science/expanding-distribution-chronic-wasting-disease#overview
Date of Index Case Confirmation Index Case State County Species Herd Type HCP Enrolled HCP Certified Number of Animals Herd Status
8/6/2025 Adult Female PA Fulton WTD Breeder No No 14 Quarantine
7/21/2025 4 YR Female PA Bedford WTD Breeder No No 34 Quarantine
6/3/2025 11 YR Female PA Blair WTD Breeder No No 45 Quarantine
6/3/2025 8 YR Female PA Bedford WTD Breeder No No 6 Quarantine
5/16/2025 5.5 YR Female WI Rock WTD Breeder No No ~46 Quarantine
5/14/2025 3 YR Female UT Weber Elk Hunt No No ukn Quarantine
4/30/2025 4.5 YR Male PA Jefferson WTD Hunt No No 36 Depopulated
4/18/2025 10+ YR Ukn TX Zavala WTD Hunt No No 190 Quarantine
Updated August 2025
See full list;
Captive CHRONIC WASTING DISEASE CASES Update August 2025, Pennsylvania, Wisconsin, Utah, and Texas
https://www.aphis.usda.gov/sites/default/files/status-of-captive-herds.pdf
Texas Game Wardens Near Conclusion of ‘Ghost Deer’ Case with 24 Suspects, 1,400 Charges Filed Statewide
Aug. 14, 2025
Media Contact: TPWD News, Business Hours, 512-389-8030
AUSTIN – The Texas Game Warden investigation known as "Ghost Deer" has reached a possible conclusion after two additional suspects turned themselves in on felony charges. This brings the total number of individuals implicated in the case to 24, with approximately 1,400 charges filed across 11 Texas counties.
Ken Schlaudt, 64, of San Antonio, the owner of four deer breeding facilities and one release site, along with facility manager Bill Bowers, 55, of San Angelo, surrendered to the Travis County District Attorney’s Office on charges of felony tampering with a governmental record. Both men allegedly entered false information into the Texas Wildlife Information Management System (TWIMS) to facilitate illegal smuggling of white-tailed breeder deer. They also face more than 100 misdemeanor charges related to unlawful breeder deer activities in Tom Green County.
The "Ghost Deer" investigation has uncovered widespread, coordinated deer breeding violations including, but not limited to: smuggling captive breeder deer and free-range whitetail deer between breeder facilities and ranches, Chronic Wasting Disease (CWD) testing violations, license violations and misdemeanor and felony drug charges relating to the possession and mishandling of prescribed sedation drugs classified as controlled substances.
The suspects charged in the case include:
Evan Bircher, 59, San Antonio Vernon Carr, 55, Corpus Christi Jarrod Croaker, 47, Corpus Christi Terry Edwards, 54, Angleton Joshua Jurecek, 41, Alice Justin Leinneweber, 36, Orange Grove James Mann, 53, Odem Gage McKinzie, 28, Normanna Herbert “Tim” McKinzie, 47, Normanna Eric Olivares, 47, Corpus Christi Bruce Pipkin, 57, Beaumont Dustin Reynolds, 38, Robstown Kevin Soto, 55, Hockley Jared Utter, 52, Pipe Creek Reed Vollmering, 32, Orange Grove Clint West, 56, Beaumont James Whaley, 49, Sevierville, Tenn. Ryder Whitstine, 19, Rockport Ryker Whitstine, 21, Rockport Claude Wilhelm, 52, Orange Cases are pending adjudication in Bandera, Bee, Brazoria, Duval, Edwards, Jim Wells, Live Oak, Montgomery, Tom Green, Travis and Webb counties.
The investigation began in March 2024 when game wardens discovered the first violations during a traffic stop.
https://tpwd.texas.gov/newsmedia/releases/?req=20250206a
That incident led wardens to the much larger network of violations,
https://tpwd.texas.gov/newsmedia/releases/?req=20250227b
resulting in one of the largest deer smuggling operations in Texas history.
About Texas Game Wardens
Texas Game Wardens, within the Law Enforcement Division of Texas Parks and Wildlife Department, are responsible for enforcing laws related to the conservation and management of natural resources and public safety through community-based law enforcement. Their mission is to provide hunting, fishing and outdoor recreation opportunities for the use and enjoyment of present and future generations. Additionally, they play a crucial role in search and rescue operations during natural disasters, exemplifying their commitment to protecting both the environment and the people of Texas.
If you witness a fishing, wildlife or boating violation in progress, please call 1-800-792-GAME(4263) immediately and report it to Operation Game Thief (OGT), Texas’ Wildlife Crime-Stoppers Program. You can also text your tip by sending the keyword TXOGT plus your tip to 847411 or through the Texas OGT App, available for iOS and Android devices. Dispatchers are available 24/7.
https://tpwd.texas.gov/newsmedia/releases/?req=20250814c
Texas Chronic Wasting Disease CWD TSE Prion Dashboard Update August 2025
https://experience.arcgis.com/experience/8f6c27330c444a19b4b57beb7ffabb8b/page/Dashboard#data_s=id%3AdataSource_3-1966d773e34-layer-10%3A29
WEDNESDAY, MAY 14, 2025
Texas CWD TSE Prion Cases Rises to 1099 Confirmed Cases To Date
Entries CWD Positives
Positive Number CWD Positive Confirmation Date Free Range Captive County Source Species Sex Age
Snip…
https://www.aphis.usda.gov/nvap/reference-guide/control-eradication/scrapie
“USA, AS of 2023, A total of 491 NVSL confirmed positive animals have been identified, including 474 classical cases (471 sheep and 3 goats) and 19 Nor98-like cases.”
https://www.aphis.usda.gov/sites/default/files/scrapie-annual-report.pdf
“Given this the scrapie eradication program focuses on classical scrapie. Here after where “scrapie” is used it is intended to mean “classical scrapie”.”
Atypical Nor98 Scrapie, Atypical BSE, CWD, Can Emerge As Different TSE PrP In Cross Species Transmission, A Volatile Situation For Human and Animal Health
Research Project: Genetic Impact and Improved Diagnostics for Sheep and Goat Transmissible Spongiform Encephalopathies Location: Animal Disease Research
Title: PrP-res in placental tissue following experimental transmission of atypical scrapie in ARR/ARR sheep is not infectious by Tg338 mouse bioassay
Author item Piel Iii, Robert item MCELLIOTT, VALERIE - University Of Georgia item STANTON, JAMES - University Of Georgia item ZHUANG, DONGYUE - Retired ARS Employee item MADSEN-BOUTERSE, SALLY - Washington State University item Hamburg, Linda item HARRINGTON, ROBERT - Retired ARS Employee item Schneider, David Submitted to: PLoS ONE Publication Type: Peer Reviewed Journal Publication Acceptance Date: 1/4/2022 Publication Date: 1/21/2022 Citation: Piel Iii, R., Mcelliott, V.R., Stanton, J.B., Zhuang, D., Madsen-Bouterse, S.A., Hamburg, L.K., Harrington, R.D., Schneider, D.A. 2022.
PrP-res in placental tissue following experimental transmission of atypical scrapie in ARR/ARR sheep is not infectious by Tg338 mouse bioassay.
PLoS ONE. PLoS ONE 17(1): e0262766.
https://doi.org/10.1371/journal.pone.0262766.
DOI: https://doi.org/10.1371/journal.pone.0262766
Interpretive Summary: Transmissible spongiform encephalopathies or prion diseases in sheep and goats are classified into two main categories: classical scrapie and Nor98-like/atypical scrapie. Classical scrapie is naturally transmissible and, along with surveillance and depopulation, breeding programs selecting for genetic resistance have been a major strategy for the control of classical scrapie in domestic ruminants. Nor98-like scrapie is generally considered non-transmissible in natural conditions and is currently exempt from control measures. Genotypes that confer resistance to classical scrapie remain susceptible to Nor98-like scrapie. ARR/ARR is a primary genotype target for resistance breeding programs and represents an increasing proportion of domestic sheep in the US. This study reports the experimental transmission of Nor98-like scrapie between ARR/ARR sheep, examines the pattern of disease in the recipient animals, and evaluates the potential for natural transmission from the recipient ewes to lambs. Inoculation of breeding ewes with Nor98-like scrapie was successful. Surprisingly, some protein with characteristics similar to prions was observed to accumulate in the placentas as recipient ewes aged; however, this material was determined to be non-infectious in a transgenic mouse model susceptible to scrapie.
Technical Abstract: Nor98-like atypical scrapie, isolated from a US sheep with the classical scrapie resistant ARR/ARR genotype, was transmitted to four ARR/ARR ewes via intracerebral inoculation of brain homogenate. These ewes were followed and observed to 8 years of age, remained non-clinical but exhibited progression of infection broadly consistent with Nor98-like scrapie, including characteristic patterns of PrP-Sc accumulation in the brain and a lack of accumulation in peripheral lymphoid tissues as detected by conventional methods. Immunoblots of placental tissues from the infected ewes revealed accumulation of a distinct conformation of PrP-res, particularly as the animals aged. These tissues showed no infectivity when analyzed via ovinized mouse bioassay. Taken together, these results support a low risk for natural transmission of Nor98-like scrapie in ARR/ARR sheep.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=388184
Volume 17, Number 5—May 2011
Research
Experimental Oral Transmission of Atypical Scrapie to Sheep
Marion M. SimmonsComments to Author , S. Jo Moore1, Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos
Author affiliations: Author affiliation: Veterinary Laboratories Agency–Weybridge, Addlestone, UK Cite This Article
Abstract
To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specific prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These findings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.
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Discussion This study is still ongoing and will not be completed until 2012. However, the current interim report documents the successful oral transmission of atypical scrapie, confirms that the disease phenotype is retained following transmission by this route in AHQ/AHQ sheep, and indicates that infectivity can be demonstrated in the gut in the absence of detectable PrPSc at least as early as 12 months after exposure.
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How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantified, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confirmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected.
https://wwwnc.cdc.gov/eid/article/17/5/10-1654_article
Experimental transmission of ovine atypical scrapie to cattle
Timm Konold, John Spiropoulos, Janet Hills, Hasina Abdul, Saira Cawthraw, Laura Phelan, Amy McKenna, Lauren Read, Sara Canoyra, Alba Marín-Moreno & Juan María Torres
Veterinary Research volume 54, Article number: 98 (2023)
Abstract
Classical bovine spongiform encephalopathy (BSE) in cattle was caused by the recycling and feeding of meat and bone meal contaminated with a transmissible spongiform encephalopathy (TSE) agent but its origin remains unknown. This study aimed to determine whether atypical scrapie could cause disease in cattle and to compare it with other known TSEs in cattle. Two groups of calves (five and two) were intracerebrally inoculated with atypical scrapie brain homogenate from two sheep with atypical scrapie. Controls were five calves intracerebrally inoculated with saline solution and one non-inoculated animal. Cattle were clinically monitored until clinical end-stage or at least 96 months post-inoculation (mpi). After euthanasia, tissues were collected for TSE diagnosis and potential transgenic mouse bioassay. One animal was culled with BSE-like clinical signs at 48 mpi. The other cattle either developed intercurrent diseases leading to cull or remained clinical unremarkable at study endpoint, including control cattle. None of the animals tested positive for TSEs by Western immunoblot and immunohistochemistry. Bioassay of brain samples from the clinical suspect in Ov-Tg338 and Bov-Tg110 mice was also negative. By contrast, protein misfolding cyclic amplification detected prions in the examined brains from atypical scrapie-challenged cattle, which had a classical BSE-like phenotype. This study demonstrates for the first time that a TSE agent with BSE-like properties can be amplified in cattle inoculated with atypical scrapie brain homogenate.
snip...
This is the first study in cattle inoculated with naturally occurring scrapie isolates that found the presence of prions resembling classical BSE in bovine brain although this was limited to detection by the ultrasensitive PMCA. The results from thermostability assay confirmed that the isolates were as thermoresistant as the BSE agent as proven in other studies [36, 48]. Previous PMCA studies with various British atypical scrapie isolates did not find any evidence of amplification [49, 50]. This may be explained by the use of ovine brain as substrate rather than brain from Bov-Tg110 mice, which may facilitate conversion to classical BSE prions.
Two hypotheses for prion strain propagation in cross-species transmission experiments have been proposed: conformational selection favours a particular strain conformation out of a mixture of conformations in a scrapie isolate whilst mutation results in the conformational shift of one conformation into another [51]. Following on from the study in mice [17], it has been subsequently suggested that classical BSE properties that arise in atypical scrapie isolates transmitted to cattle may be due to conformational mutation in a new host [52]. It does not confirm that the atypical scrapie agent is the origin of the classical BSE epidemic and further transmission studies would be required to see whether classical BSE can be generated.
Would PMCA applied to brains from cattle exposed to TSE agents other than classical BSE and atypical scrapie also produce a classical BSE-like molecular phenotype? The PMCA product obtained in the thermostability test using a thermosensitive classical scrapie control showed a profile unlike classical BSE. Atypical BSE has been linked to the origin of classical BSE because of its conversion into classical BSE following serial passages in wild-type mice (L-type BSE [11]) and bovine transgenic mice (H-type BSE [53]). Although we have not tested PMCA products of atypical BSE isolates as part of this study, there is no evidence that PMCA products from atypical BSE convert into classical BSE, at least for H-type BSE using bovine brain as substrate [54]. In fact, we were unable to propagate H-type BSE using the same methodology (S Canoyra, A Marín-Moreno, JM Torres, unpublished observation).
The study results support the decision to maintain the current ban on animal meal in feedstuffs for ruminants, particularly as atypical scrapie occurs world-wide, and eradication is unlikely for a sporadic disease.
In summary, experimental inoculation of cattle with the atypical scrapie agent may produce clinical disease indistinguishable from classical BSE, which cannot be diagnosed by conventional diagnostic tests, but prions can be amplified by ultrasensitive tests in both clinically affected and clinically unremarkable cattle, which reveal classical BSE-like characteristics. Further studies are required to assess whether a BSE-like disease can be confirmed by conventional tests, which may initially include a second passage in cattle.
https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3
THURSDAY, JANUARY 7, 2021
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Nor98 cases Diagnosed in the US. To Date
Nor98 cases Diagnosed in the US.
Flock of Origin State FY
Wyoming 2007
Indiana 2007
Pennsylvania 2008
Oregon 2010
Ohio 2010
Pennsylvania 2010
Untraceable 2010
California 2011
Montana 2016
Utah 2017
Montana 2017
Virginia 2018
Colorado 2019
Colorado 2019
Wyoming 2020
Montana 2020
Pennsylvania 2021
Personal Communication from USDA et al Mon, Jan 4, 2021 11:37 am...terry
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
17 cases of the Nor98 in the USA to date, location, unknown...tss
17 Nor98-like cases since the beginning of RSSS.
https://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_report.pdf
17 Nor98-like cases since the beginning of RSSS. No animals have tested positive for classical scrapie in FY 2021.
https://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_report.pdf
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
https://scrapie-usa.blogspot.com/2020/09/aphis-usda-more-scrapie-atypical-nor-98.html
MONDAY, JULY 27, 2020
APHIS USDA Nor98-like scrapie was confirmed in a sheep sampled at slaughter in May 2020
https://nor-98.blogspot.com/2020/07/aphis-usda-nor98-like-scrapie-was.html
MONDAY, JULY 13, 2020
Efficient transmission of classical scrapie agent x124 by intralingual route to genetically susceptible sheep with a low dose inoculum
https://scrapie-usa.blogspot.com/2020/07/efficient-transmission-of-classical.html
WEDNESDAY, MAY 29, 2019
***> Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures
https://nor-98.blogspot.com/2019/05/incomplete-inactivation-of-atypical.html
THURSDAY, DECEMBER 31, 2020
Autoclave treatment of the classical scrapie agent US No. 13-7 and experimental inoculation to susceptible VRQ/ARQ sheep via the oral route results in decreased transmission efficiency
https://scrapie-usa.blogspot.com/2020/12/autoclave-treatment-of-classical.html
Snip…see full text;
https://nor-98.blogspot.com/2021/01/atypical-nor-98-scrapie-tse-prion-usa.html
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
==============
PRION 2015 CONFERENCE
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 1933-690X
WS-01: Prion diseases in animals and zoonotic potential
“Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.”
These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
Increased Atypical Scrapie Detections
Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.
http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf
Published: 04 October 2023
Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie
Marina Betancor, Belén Marín, Alicia Otero, Carlos Hedman, Antonio Romero, Tomás Barrio, Eloisa Sevilla, Jean-Yves Douet, Alvina Huor, Juan José Badiola, Olivier Andréoletti & Rosa Bolea Veterinary Research volume 54, Article number: 89 (2023) Cite this article
Abstract
The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently observed upon experimental transmission to rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle. Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease and tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prion seeding activity was detected during in vitro propagation of brain samples from the inoculated animals. These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.
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Discussion
Previous studies have demonstrated that C-BSE prions can be present as a minor variant in ovine atypical scrapie isolates and that C-BSE can emerge during the passage of these isolates to pigs and bovine PrP mice [7, 8]. These results pointed to atypical scrapie as a possible origin of C-BSE. Therefore, this study was meant to assess the link between atypical scrapie and C-BSE in the natural host of C-BSE, cattle. Although the intracerebral challenge has some limitations and does not reflect the natural transmission process of prions, bioassays using experimental prion inoculation have allowed to identify and describe the transmission mechanisms of these pathogens. Therefore, we decided to challenge cattle with an atypical scrapie isolate.
It is important to note that none of the animals in this study showed any clinical signs of TSE after inoculation with atypical scrapie, according to the results previously obtained in pigs [8]. In addition, the absence of spongiform changes in brain sections, as well as the absence of PrPSc accumulation by conventional techniques in brain areas from the atypical scrapie-inoculated cows, further highlights the need for highly sensitive techniques such as PMCA to detect low levels of prions. After the in vitro propagation of brain samples from the cows included in this study, seeding activity was detected in reactions seeded with brain material from three out of the four cows, in the areas of frontal cortex, thalamus, and/or cerebellum. Interestingly, none of the samples from the obex, which is one of the most affected areas in prion diseases [14], showed seeding activity. Importantly, the observed glycosylation pattern of the positive PMCA reactions was indistinguishable from that of C-BSE prions and PMCA products from reactions seeded with C-BSE prions. To check whether C-BSE-like prions were present in the original atypical scrapie isolate or if they emerged in the brain of the cows after the inoculation, we performed PMCA of the original inoculum in TgBov substrate, following the same conditions described above. The in vitro amplification of the atypical scrapie inoculum resulted in the propagation of BSE-like seeding activity, biochemically indistinguishable from C-BSE or positive PMCA reactions seeded with brain samples from the inoculated cows, suggesting that, as described before, certain atypical scrapie isolates contain low levels of C-BSE prions [9].
Moreover, in order to rule out a spontaneous in vitro misfolding of bovine PrP during PMCA, we included, as a control for the technique, brain samples from non-inoculated age-matching cows that were also subjected to serial in vitro propagation in TgBov substrate. No positivity was observed in PMCA reactions seeded with samples from these animals, suggesting a true C-BSE-like prion seeding activity and not a spontaneous in vitro misfolding of PrP.
All these results suggest the amplification of C-BSE-like prions during the transmission of ovine atypical scrapie to cows. It is true that, in order to confirm the presence of infectious BSE prions in the challenged cows, strain typing experiments of the PMCA products should be carried out in established mouse lines. Therefore, studies involving a bioassay in bovine and ovine PrP-expressing mice have been started.
Interestingly, the time after inoculation and the BSE-like prion seeding activity were not correlated. As previously stated, the emergence of C-BSE from atypical scrapie has been associated with the presence of low levels of C-BSE prions in the atypical scrapie isolates and our results after the in vitro amplification of the PS152 inoculum support this theory. Therefore, the number of C-BSE conformers contained in the used atypical scrapie isolates may be reduced and not homogeneously distributed, making cows receiving different amounts of C-BSE-like prions. It is true that the emergence of C-BSE-like PMCA seeding activity from the brains of cows could be related to the persistence of prions from the original atypical scrapie inoculum. Previous studies, in which prion seeding activity was detected in the brain of intracerebrally inoculated PrP0/0 mice have highlighted the capacity of prions to persist in non-replicative environments [15]. Nevertheless, cows were intracerebrally challenged in the frontal cortex, and seeding activity was detected in caudal regions of their brains but not in more rostral areas such as the frontal cortex. If these positive PMCA reactions were not a bona fide propagation of C-BSE-like prions but associated to inoculum persistence, it would be expected to detect such amplification in the most rostral areas of the brain. Although all these results support a bona fide propagation of C-BSE-like prions, the possibility of PMCA detecting remaining prions of the inoculum, would be definitely ruled out after in vivo bioassays in mouse lines, which are currently being carried out.
The lack of clinical signs of prion disease in cows after inoculation with atypical scrapie contrasts with results from a previous study in which bovine PrP mice (TgBov) were challenged with atypical scrapie isolates and displayed signs of clinical prion disease, developing neuropathological characteristics of C-BSE [7]. In addition, in the mentioned study, after the first passage, signs of clinical prion disease were only observed in a low proportion of the inoculated mice, and several of the inoculated isolates did not lead to PrPSc accumulation. Three serial passages of atypical scrapie were needed to observe complete attack rates in TgBov mice. Moreover, mice from the first passage that developed clinical signs showed long incubation periods considering the lifespan of a mouse. The cows in this study were also euthanized after a long post-inoculation period (between ~7 and ~11 years). However, the number of C-BSE-like prions present in the original atypical scrapie inoculum was probably too low to produce disease in the cows upon first passage. We also need to consider that TgBov mice overexpress ~8 times bovine PrPC, making them more susceptible to develop disease after the inoculation of C-BSE prions.
Further in vivo experiments challenging different mouse lines have been started in order to confirm the infectivity of the PMCA products obtained in this study. However, in conclusion, our findings show that the propagation of atypical scrapie in cattle leads to the emergence of BSE-like seeding activity. This is a concerning issue with far-reaching implications for public health and food safety. The possibility of interspecies transmission of prion diseases and the emergence of new prion strains highlight the critical need for continued surveillance and monitoring of these diseases in both animal and human populations. Early detection of prion diseases is crucial, and highly sensitive detection techniques such as PMCA can play an important role in this regard.
https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
12/10/76
AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note CHAIRMAN: PROFESSOR PETER WILDY
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A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates.
One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.
snip...
76/10.12/4.6
http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html
Wednesday, February 16, 2011
IN CONFIDENCE
SCRAPIE TRANSMISSION TO CHIMPANZEES
IN CONFIDENCE
http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html
why do we not want to do TSE transmission studies on chimpanzees $
snip...
5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
snip...
R. BRADLEY
http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
BSE: TIME TO TAKE H.B. PARRY SERIOUSLY
If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1988/06/08004001.pdf
Scrapie: The flock identified in April 2016 remains under quarantine in Hartley County.
https://www.tahc.texas.gov/agency/meetings/minutes/SummaryMinutes_CommMtg_2020-09-22
http://web.archive.org/web/20201017124040/https://www.tahc.texas.gov/agency/meetings/minutes/SummaryMinutes_CommMtg_2020-09-22.pdf
A Space in Time, Scrapie USA, 2002!
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."
Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly infected flocks 2002) & CWD IN USA
As of September 30, 2002, there were 45 scrapie infected and source flocks (figure 3).
There were 105 newly infected flocks, reported in FY2002 (figure 4).
In addition, 379 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2002 (figure 5) and (figure 6).
Five cases of scrapie in goats were reported in FY 2002 (figure 7), the last of which was confirmed in August 2002.
New infected and source flocks numbers and the number of these flocks released in FY 2002 are depicted in chart 4.
One hundred (100) flocks which is 67 percent of the scrapie infected and source flocks present in FY 2002 were released or put on clean-up plans in FY2002. Slaughter Surveillance Slaughter Surveillance is currently in Phase II which is intended to determine the prevalence of scrapie in the US culled sheep population.
Through September 2002 samples from 3,269 sheep were submitted to NVSL for testing. Samples from a total of 6,795 sheep have been submitted since the beginning of Phase II on April 1, 2002. Surveillance regions are depicted in (figure 8).
Scrapie Testing During FY 2002 11,751 animals have been tested for scrapie which includes: 2,711 regular necropsy cases, 1,343 third eyelid biopsies for the test validation project, 546 third eyelid biopsies for the regulatory program, and approximately 7,151 animals for Phase I & II of SOSS (chart 5).
Laboratory testing has been taking 10 - 11 days on average with a range of 3 - 34 days. Ear Tag Orders During FY 2002 9.9 million plastic and 6.0 million metal tags were distributed by APHIS (chart 6).
http://www.aphis.usda.gov/vs/nahps/scrapie/annual_report/annual-report.html
Epidemiology of Scrapie in the United States
Snip…
Scrapie Field Trial was developed at Mission, Texas, what if?
Scrapie Field Trial was developed at Mission, Texas, on 450 acres of pastureland, part of the former Moore Air Force
EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES
Academic Preg
James Hourriganl, Albert Klingsporn2, Edited by » Peast
W. W. Clark3, and M, de Camp4
United States Department of Agriculture, Animal and Plant Health Inspection Service, Veterinary Services
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METHODS
A Scrapie Field Trial was developed at Mission, Texas, to provide additional information for the eradication program on the epidemiology of natural scrapie. The Mission Field Trial Station is located on 450 acres of pastureland, part of the former Moore Air Force Base, near Mission,
Texas. It was designed to bring previously exposed, and later also unexposed, sheep or goats to the Station and maintain and breed them under close observation for extended periods
to determine which animals would develop scrapie and define more closely the natural spread and other epidemiological aspects of the disease.
The 547 previously exposed sheep brought to the Mission Station beginning in 1964 were of the Cheviot, Hampshire, Montadale, or Suffolk breeds. They were purchased as field outbreaks occurred, and represented 21 bloodlines in which scrapie had been diagnosed. Upon arrival at the Station, the sheep were maintained on pasture, with supplemental feeding as necessary. The station was divided into 2 areas:
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RESULTS
Table 1 indicated that previously exposed sheep brought to the Station at various times and ages (1 to 89 months old) included 333 Suffolks at risk. Of these, 98 (29%) developed scrapie. This demonstrated the necessity to slaughter such sheep to prevent further Spread of the disease, These pre- viously exposed Suffolks were bred at the Station and produced 446 progeny at risk. Of these 153 (34%) developed scrapie.
Although the minimum and average ages when scnapied were similar for both groups, some of the previously exposed Suffolks brought to the Station developed scrapie when much older--ewes 60 to 142 months old and rams 67 to 102 months old. O£ the 153 Suffolks born at the Station, only 3 were more than 60 months of age (65, 66, and 69 months old).
This difference in age scrapied was attributed to the fact that the Suffolks born at the Station may have been sub- ject to a greater exposure from birth.
It was also observed that when both dam and progeny were scrapied, the progeny nearly always developed clinical disease at a younger age than their respective dam. Thirty- two dams were scrapied at an average of 60 months of age. Forty-six of their progeny developed the disease at an average of 38 months (range 25 to 53 months). Thirty-seven of the 46 progeny were younger than the dam (average 20 months younger, range 2 to 99 months younger). Two were scrapied at the same age as their dams, and 8 were older (average 5 months, range 1 to 13 months older).
++. Although the incidence of scrapie was considerably Greater in the progeny of scrapied compared to free dams, the progeny of either scrapied or free dams manifested scrapie at the typical age and irrespective of the age their respective dams were scrapied. The differences in ages that dams and progeny were scrapied was believed due to difference of exposure, particularly whether they were exposed at an early age,
Table 2 summarized the data on exposed Suffolks and was Prepared so as to show scrapie incidence in the progeny of dams and sires of known Scrapie status. The scrapie incidence in the progeny of Free X Free parents was 25%, progeny of scrapied Sires 39%, and scrapied dams 42%. When both sire and dam were scrapied, the scrapie incidence in 18 Progeny at risk was 78%.
When the scrapie status of the sire was ignored, scrapie incidence in th- progeny of free dams was 34% and in pre y of scrapied da as 62%. When the scrapie status of the dam was ignored, scrapie incidence in the progeny of free sires was 26% and in the progeny of scrapied sires was 452.
Although the scrapie incidence was nearly double in the progeny of scrapied compared to free dams, the latter con- tributed a greater number of scrapied progeny, 116, compared to only 51 cases which had scrapied dams. This was because free dams made a considerably heavier contribution to the progeny at risk4-342 compared to 82. It was felt that in farm flocks a similar situation could exist.
It was possible that free dams could have been mis- classified; however, this was unlikely to have been significant, unless "nonclinical or carrier" dams exist. In this Suffolk group, the ages of 100 free dams of scrapied progeny ranged from 25 to 160 (average 97) months. These free dams did not show clinical signs of scrapie,”and there were no histopathological lesions suggesting scrapie in those which died, If one cannot classify as free, ewes which have reached 97 months (average) and did not develop the disease, from a practical standpoint, it is not possible to classify sheep as free, at least on the basis of clinical signs and histology. The free dams of 50% of the scrapied progeny were more than 100 months of age, averaging 126 months.
Upon arrival at the Mission Station at 3 to 9 months of age, the 140 previously unexposed sheep and goats were placed in infected pastures and corrals and were subjected to con- tact with a succession of natural cases of scrapie in sheep, and eventually also in goats. These animals were bred only within their respective groups and were not crossbred to other breeds of sheep or those brought to the Station from infected flocks or their progeny. The male or female animals mixed freely with animals of their respective sex of the infected Flock and were similarly identified and subjected to similar flock management and diagnostic procedures.
Table 3 indicated that natural scrapie had occurred in 5 of the 140 previously unexposed sheep. One case each occurred in Rambouillet, Targhee, and Hampshire ewes at 88, 89, and 89 months of age and in % Suffolk ewes at 73 and 102 months of age, and 85, 82, 80, 64, and 93 months following initial natural exposure. This represented a natural situation involving lateral spread, under the circumstances involved, when sheep were not exposed when very young. Scrapie was not detected clinicaliy or histologically in any of the dairy or Angora goats brought to the Station. The disease occurred in an average of 27% of the progeny of previously unexposed sheep or goats born at the Station and included cases in progeny of all breeds of sheep or goats taken there, The incidence in the progeny ranged from 14% in Rambouillet sheep to 61% in dairy goats. ~
These data showed that scrapie spread laterally, by contact exposure, from scrapied te previously free animals, but at an apparently lower rate when exposure was first received at the age of 3 to 9 months. These animals were presumed to be susceptible to the disease, as their progeny developed scrapie at rates and ages similar (on the average) to the progeny, pf previously exposed Suffolk sheep born and reared in the same environment.
It was suggested that the progeny of previously unexposed animals developed scrapie at a much higher rate than their parents, and at a younger age, because they were subjected to exposure from birth. The data did not rule out the possibility that the animals born at the Station could have also received the virus from their dams "vertically" prior te, at, or following birth.
Table 4 summarized the scrapie incidence in #he progeny, born at the Station, of previously unexposed dairy goats.
The data were prepared so as to show scrapie incidence in the progeny of dams and sires of known scrapie status.
The 58% incidence in the progeny (24 at risk) of Free X Free parents was more than twice the 25% seen in the Suffolk group (Table 2). Scrapied sires did not increase the incidence in goat progeny (it was 44%); scrapied dams increased the incidence to 71%. When both sire and dam were scrapied the incidence was 89%, with only 9 goat progeny at risk.
When the scrapie status of the sire was ignored, the scrapie incidence in the progeny of free dams was 56% and in the progeny of scrapied dams it was 74%.
Free dams contributed 34 progeny at risk and scrapied dams 31 progeny.
When the scrapie status of the dam was ignored, scrapie incidence was 64% in the progeny of free sires and a similar 66% in the progeny of scrapied sires.
A total of 244 sheep (127 Suffolk, 59 Rambouillet, and 58 Targhee) were removed from scrapie exposure within a few hours of birth or at 4, 9, or 20 months of age and placed in isolation pens. Removal of sheep from exposure at these ages was selected as being representative of usual flock operations when sheep might be sold from an infected flock at weaning, the first fall or the second fall after their birth.
Table 5 reflected the fate of such animals. Four of the 6 scrapied sheep which had been isolated at birth were Suffolks and the 2 older animals were Targhees. The first case in the group isolated at birth was a Targhee, progeny of a ewe that did not develop clinical scrapie. The scrapie incidence in 36 at risk Suffolks removed from exposure at birth was 11%, con- siderably less -“en that expected had these animals remz d in an infected en ment.
Table 6 reflected the status of 51 goats isolated from scrapie exposure at birth, and at 6, 8 to 10, 20, 32 to 59 and 60 to 82 months of age.
None of the goats removed at birth developed scrapie, although all 5 of those alive at 5 years of age had scrapied dams and 1 also had a scrapied sire. The sire of the remaining 4 had sired 7 scrapied progeny. Under such circumstances, had they remained in an infected environment nearly all of these goats would have been expected to develop scrapie. With the exception of the 20 month group, scrapie occurred at an incidence of 25 to 100% in ali other groups and at the expected age. A further observation was that 4 of the progeny of these dairy goats, born and kept apart from any sheep, developed scrapie which suggested that goats were not "dead- end hosts" insofar as scrapie was concerned.
Table 7 recorded the fate of progeny of certain selected scrapied or free Suffolk sheep or dairy goat dams.’
Suffolk ewe G298 was scrapied at 46 months of age. She had twin lambs in 1969 and 1 lamb in 1970. All 3 lambs developed scrapie. Suffolk ewe G27a was scrapied at 39 months. Her lamb born in 1966 was scrapied at 53 months; however, her lambs born in 1967 and 1968 remained free--lived to 102 months of age.
Suffolk ewe G25a died at 131] months of age and was nega- tive clinically and histologically. Mice remained negative following intracerebral inoculation of brain, spleen, and lymph nodes from this ewe. This ewe had 9 progeny at risk, of which 4 developed scrapie and 5 did not. There was no dis- cernible pattern to the cases. In two instances, 1 twin was scrapied and 1 remained free.
Goat B259 was scrapied when 43 months old. All of her 6 progeny at risk developed scrapie.
Goat B14a remained free and died at 101 months of age. Of her 11 progeny at risk, 7 were scrapied and 4 were not.
It was observed at the Station that when scrapied dams had several progeny at risk, 1 or more progeny usually developed the disease. However, many such scrapied dams also had progeny which lived, or are living, considerably beyond the age of their dams and beyond the age animals born at the Station manifested the disease.
It was also observed that individual free dams had free progeny in earlier years followed by scrapied progeny when they were older, or had scrapied progeny when young followed by free progeny when older, or scrapie and free progeny dis- persed throughout the dam's breeding life. The same situation occurred in progeny of scrapied dams; however, the pattern was less irregular due to the smaller number of progeny from each scrapied dam and the higher incidence of scrapie in such progeny. Circumstances prevented breeding all ewes ary year and, thus, many had only 1 progeny at risk. Scrapie developed in 100% of the single progeny at risk of 11 scrapied and 15 free dams. The 26 scrapied progeny were equally divided between ewes and rams.
Table 8 reflected the difference in age scrapied of - sheep brought to the Station compared to the age scrapied of those born there. Although the average age of previously exposed sheep (Suffolks) brought to the Station did not differ greatly from the overall average, several animals brought to the Station developed the disease at quite advanced ages. The previously unexposed scrapied animals brought to the Station were also considerably older than animals born there. Progeny of scrapied dams developed the disease at a slightly younger age than did progeny of free dams. The average age was nearly the same for males and females.
DISCUSSION
snip...see full text;
http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf
Scrapie Field Trial was developed at Mission, Texas, on 450 acres of pastureland, part of the former Moore Air Force Base
EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES
http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf
Porcine Spongiform Encephalopathy or Pig CWD or Pig BSE?
Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States
Volume 31, Number 1—January 2025
Abstract
Using a prion amplification assay, we identified prions in tissues from wild pigs (Sus scrofa) living in areas of the United States with variable chronic wasting disease (CWD) epidemiology. Our findings indicate that scavenging swine could play a role in disseminating CWD and could therefore influence its epidemiology, geographic distribution, and interspecies spread.
Snip…
Conclusions
In summary, results from this study showed that wild pigs are exposed to cervid prions, although the pigs seem to display some resistance to infection via natural exposure. Future studies should address the susceptibility of this invasive animal species to the multiple prion strains circulating in the environment. Nonetheless, identification of CWD prions in wild pig tissues indicated the potential for pigs to move prions across the landscape, which may, in turn, influence the epidemiology and geographic spread of CWD.
https://wwwnc.cdc.gov/eid/article/31/1/24-0401_article
Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.
2. Determined that pigs naturally exposed to chronic wasting disease (CWD) may act as a reservoir of CWD infectivity. Chronic wasting disease is a naturally occurring, fatal, neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of CWD disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Pigs were assigned to 1 of 3 groups: intracranially inoculated; orally inoculated; or non-inoculated. At market weight age, half of the pigs in each group were tested ('market weight' groups). The remaining pigs ('aged' groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by multiple diagnostic methods. Brain samples from selected pigs were bioassayed in mice expressing porcine prion protein. Some pigs from each inoculated group were positive by one or more tests. Bioassay was positive in 4 out of 5 pigs assayed. Although only small amounts of PrPSc were detected using sensitive methods, this study demonstrates that pigs can serve as hosts for CWD. Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091
https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017
https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105
https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166
Infectious prions in brains and muscles of domestic pigs experimentally challenged with the BSE, scrapie, and CWD agents
Authors: Francisca Bravo-Risi, Fraser Brydon, Angela Chong, Kane Spicker, Justin J. Greenlee, Glenn Telling, Claudio Soto, Sandra Pritzkow, Marcelo A. Barria, Rodrigo Morales rodrigo.moralesloyola@uth.tmc.edu
ABSTRACT
Experimental studies suggest that animal species not previously described as naturally infected by prions are susceptible to prion diseases affecting sheep, cattle, and deer. These interspecies transmissions may generate prions with unknown host ranges. Pigs are susceptible to prions from different origins, including deer chronic wasting disease (CWD), sheep scrapie, and bovine spongiform encephalopathy (BSE). Here, we studied prions in brains and muscles from pigs previously infected with these different prion sources. Specifically, we measured the total prion protein (PrP) and PK-resistant PrP by western blot. Seeding activity in these tissues was evaluated using the protein misfolding cyclic amplification (PMCA) technique. We found that BSE-infected pigs contained substantially more seeding competent prions compared with those infected with CWD and scrapie. Moreover, the zoonotic potential of porcine-BSE prions seems to be relevant, as both brains and muscles from BSE-infected pigs induced the misfolding of the human prion protein in vitro. This study helps to understand the potential fate of naturally existing prion strains in a relevant host and calls for caution considering the co-existence between feral swine and other prion-susceptible animal species.
IMPORTANCE
Prions (PrPSc) are proteinaceous, infectious pathogens responsible for prion diseases. Some livestock are highly susceptible to prion diseases. These include cattle (bovine spongiform encephalopathy, BSE), sheep and goat (scrapie), and cervids (chronic wasting disease, CWD). Unfortunately, BSE has been reported to be naturally transmitted to humans and other animal species. Domestic pigs, a relevant livestock animal, have not been reported to be naturally affected by prions; however, they are susceptible to the experimental exposure to BSE, scrapie, and CWD prions. Given the widespread consumption of porcine food products by humans, we aimed to evaluate the levels of pig-derived BSE, scrapie, and CWD prions from experimentally challenged domestic pigs in brain and meat cuts (leg, cheek meat, skirt meat, and tenderloin). We detected pig-adapted prions in the brains and some muscles of these animals. Additionally, we evaluated the in vitro compatibility between pig prions and the human prion protein (as a surrogate of zoonosis). Our results show that only pig-derived BSE prions were able to induce the misfolding of the cellular human prion protein. This data highlights the consequences of prion spillovers to other animal species and their potential availability to humans.
Snip…
DISCUSSION
In this study, we evaluated the presence and relative quantities of prions in brains and muscles from pigs infected with three different prion isolates: C-type BSE, scrapie, and CWD. Our findings confirm previous reports by demonstrating the presence of disease-associated prions in the brains of these animals (20, 56, 61). In addition, we evaluated the replication capacity of the prions present in these samples using the PMCA technique. The presence of prions in the brains of these prion-infected pigs appears to be variable depending on the original prion source. Although prion seeding activity was identified in the brains of pigs from all three groups, we found that those from animals infected with BSE contained the most active seeds. It is relevant to mention that most of the pigs infected with either scrapie or CWD did not display neurological signs linked with prion disease, yet prion seeding activity was still detected in their brains. The Moore et al. publication (61) states the presence of sparse PK-resistant PrP accumulation in the brain of CWD-infected pigs, suggesting a low amount of PrPSc in these samples. To improve the detection, the authors treated brain extracts with a sarkosyl/ultracentrifugation protocol and loaded at 100 mg of brain tissue for detection via western blots. Due to the limited amount of sample available for the present study, we did not enrich the sample and loaded 2 mg of brain tissue on western blots. This might have affected the detection of the PrP signals using this method (Fig. 2; Fig. S2). The low amount of PrPSc in these samples was further confirmed by PMCA, as seeding activity was present in just some replicates (Fig. 4), mostly at low dilutions. Nevertheless, the identification of subclinical prion infection in these brains is consistent with previous studies suggesting the same when bioassays in transgenic mice expressing the porcine PrPC were used in the BSE and CWD transmissions (26, 56). The fact that subclinical prion infection was also identified in pigs orally exposed to BSE and CWD (56, 61, 62) suggests that transmissions in natural scenarios are possible and an eventual adaptation of the agent in pigs (e.g., considering the cannibalistic behavior of wild pigs) is plausible.
In addition to the prion content in brains, we evaluated the anatomical distribution of PrPSc in several skeletal muscles with the goal of understanding the tropism of the adapted prions in these tissues. The specific muscles tested included semitendinosus (leg), masseter (cheek meat), diaphragm (skirt meat), and psoas major (tenderloin). These pork cuts are commonly consumed by people and are commercially available. Unfortunately, the muscle tested for the BSE-infected group (semitendinosus) differed from those analyzed in the scrapie and CWD groups (masseter, diaphragm, and psoas) due to the availability of archived materials. Regardless, these samples are useful to understand whether prions in pigs accumulate in muscles that might be available for human consumption. Our results indicate that the muscles collected from the BSE-infected pigs contain higher levels of seeding-competent prions compared with those in other groups. Nevertheless, the same cautions regarding the routes of administration of BSE materials in pigs explained above must be considered when interpreting this data. The comparison between scrapie and CWD inoculated pig groups, that were inoculated with the same quantities of infectious materials and the same route, and where the same tissues were collected, allows us to make more accurate associations. In summary, our findings demonstrate that muscles closer to the animal head (masseter) supported a better seeding activity compared to those located at the thorax (diaphragm) and hind levels (psoas major). One explanation for this could be explained by the centrifugal spreading of prions, which agrees with the findings by Headman and colleagues (58) describing the detection of PrPSc in upper muscles of clinically affected pigs inoculated intracerebrally with sheep-BSE prions. In that study, two swine harbored PrPSc in muscles associated with the eyes (oculomotor muscle), while prions were identified in the semitendinosus muscle of just one animal. Recently, the same group analyzed the peripheral tissues of these pigs by PMCA, detecting seeding activity in the oculomotor muscle in all the animals exposed to the BSE-derived agent in dilutions as low as 10−4 (67). Due to the limited amount of muscle samples from the pigs inoculated with BSE, we were unable to perform a detailed anatomical analysis. However, we showed that the semitendinosus muscle, which is located at the hind limbs, contained relevant levels of seeding competent PrPSc. The relevantly abundant presence of prions in this particular group of pigs could be due to the fact that they were peripherally exposed to the agent, or by the peripheral tropism of BSE prions as it has been observed for the transmission of this particular prion strain to multiple other animal species (62, 68–80). The data describing higher quantities of seeding-competent prions in the brains of BSE-infected pigs needs to be interpreted with caution. As described, BSE-infected pigs received infectious materials at higher doses and through three simultaneous routes of administration compared to the animals exposed to the scrapie and CWD agents (20, 56, 61). If we restrict these comparisons to the latter two groups (scrapie- and CWD-infected pigs) that were exposed to the same prion doses (750 µL of 10% [w/v] pooled sheep and cervid brain homogenate, respectively), and through the same route of inoculation (intracerebral), we found that pig-adapted scrapie prions exhibited a higher amount of seeding-competent PrPSc in both brain and muscle tissues compared with their CWD-exposed counterparts. The differences in seeding capacities and tropisms between these newly generated porcine prions can be explained in the generation of different prion strains in each case. Nevertheless, it is also relevant to consider that these studies used unique scrapie and CWD strains from the many described in experimental and natural settings (31, 33, 40, 41). Along this line, future studies should focus on the differential susceptibilities experienced by pigs when exposed to different variants associated with these animal prionopathies.
Importantly, our studies evaluating zoonotic potentials showed that pig-adapted BSE prions were able to induce the misfolding of the human prion protein in vitro. These results were confirmed in two different laboratories, a fact that provides rigor to this study. Importantly, these analyses also show that muscles from these BSE-infected pigs may present a concern if introduced in the human food chain. Importantly, the zoonotic risk of these materials seems to be restricted to the 129M polymorphic version of the human prion protein, in line with the higher susceptibility of BSE to the population carrying this specific protein. Previous reports have shown that mice expressing the 129M human PrPC variant (HuPrP-tg 650 and 340) exhibited a species barrier to BSE prions that was overcome in a first transmission passage (81). Similar outcomes were observed when pig-adapted BSE prions were inoculated in HuPrP-tg340 mice, although with incomplete attack rates (50%) in a first passage. Serial transmissions of this infectious material in the same mice resulted in complete attack rates and reduction in the incubation periods, suggesting that the infectious material successfully adapted in the new host (60). On the contrary, extensive evidence demonstrates that the species barrier between scrapie and CWD prions and humans is strong, if not absolute (39, 42, 82). However, the existence of different prion strains associated with these animal prionopathies urges for a systematic analysis including the whole pathogenic spectrum associated with them. In our study, scrapie- and CWD-adapted prions were not able to induce the misfolding of the human prion protein. However, considering the data from the porcine-BSE prions, we cannot conclude whether the lower load of prions in the CWD- and scrapie-porcine tissues was responsible for these results.
Some of the limitations of the current study have been already discussed. One of the issues involves the unknown prion infectivity titers of the BSE-, scrapie-, and CWD-bearing tissues used to infect pigs. This importantly limits comparisons between the groups. Prion infectivity titers can affect tissue tropisms, especially in subclinical transmissions (as prions may need additional time to reach target tissues). This could be considered an additional source of variability for the dissimilar presence of prions in muscles for the different animal groups included in this study. Another limitation involves the uneven testing of muscle tissues across the three groups and the lack of analysis of other muscles representing different anatomical distributions within the body. The latter may be relevant for understanding the tropism of prions induced by each injectate. Finally, the scrapie and CWD groups were treated with specific prion strains from many that have been described. This is relevant considering that different strains may adapt with different efficiencies into new hosts and result in infectious particles with unique host ranges. Future studies should focus on the above-mentioned limitations. However, we believe that the current study provides a solid background to justify these analyses.
In summary, our data shows the dynamic of animal prions when exposed to infectious pigs, as well as their distributions and zoonotic potentials. The data presented here may be relevant to understanding the fate of naturally existing prions in a sympatric animal species relevant for human consumption. This acquires importance considering a recent report describing the interaction between CWD and wild pigs in natural settings.
https://journals.asm.org/doi/10.1128/mbio.01800-25
Experimental transmission of the chronic wasting disease agent to swine after oral or intracranial inoculation
Running Title: The chronic wasting disease agent transmits to swine
JVI Accepted Manuscript Posted Online 12 July 2017 J. Virol. doi:10.1128/JVI.00926-17
Running Title: The chronic wasting disease agent transmits to swine
JVI Accepted Manuscript Posted Online 12 July 2017 J. Virol. doi:10.1128/JVI.00926-17
Abstract
Chronic wasting disease (CWD) is a naturally occurring, fatal neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation . Crossbred piglets were assigned to one of three groups: intracranially inoculated (n=20), orally inoculated (n=19), or non -inoculated (n=9). At approximately the age at which commercial pigs reach market weight, half of the pigs in each group were culled (‘market weight’ groups). The remaining pigs (‘aged’ groups) were allowed to incubate for up to 73 months post inoculation (MPI ). Tissues collected at necropsy were examined for disease -associated prion protein (PrPSc) by western blotting (WB), antigen -capture immunoassay (EIA), immunohistochemistry (IHC) and in vitro real -time quaking induced conversion (RT -QuIC). Brain samples from selected pigs were also bioassayed in mice expressing porcine prion protein. Four intracranially inoculated aged pigs and one orally inoculated aged pig were positive by EIA, IHC and/or WB. Using RT -QuIC, PrPSc was detected in lymphoid and/or brain tissue from one or more pigs in each inoculated group. Bioassay was positive in 4 out of 5 pigs assayed.
This study demonstrates that pigs can support low -level amplification of CWD prions, although the species barrier to CWD infection is relatively high. However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.
https://journals.asm.org/doi/10.1128/jvi.00926-17
“However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.”
CWD TSE Prion, Cattle, Sheep, Pigs, Felines, Coyotes, Raccoons, Rodents, Primates, Oh My!
Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry
Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure
Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA
Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.
Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).
Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.
Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.
"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons
https://www.ars.usda.gov/research/publications/publication/?seqNo115=400777
Distribution of chronic wasting disease (CWD) prions in tissues from experimentally exposed coyotes (Canis latrans)
Published: July 9, 2025
Abstract
Cervids susceptible to chronic wasting disease (CWD) are sympatric with multiple other animal species that can interact with infectious prions. Several reports have described the susceptibility of other species to CWD prions, or their potential to transport them. One of these species is the coyote (Canis latrans), which has been previously shown to pass transmission-relevant prion titers in their feces for at least three days after ingesting prion-positive brain material. The current study followed up on these findings and evaluated the distribution of prions in multiple tissues from the same coyotes. Our results show that prions persist in the digestive tract of prion-exposed coyotes five days after exposure. Moreover, prion seeding activity was identified in other tissues, including lymph nodes and lungs. These results provide additional information about the dynamics of CWD prions in the environment and show the initial fate of prions after ingestion by a canid species that is a carnivorous predator and scavenger.
Snip…
Despite the strong species barriers, predators and scavengers may still be relevant as spreaders of infectious prion particles. In fact, both experimental research and natural observations indicate that insects, parasites, and various wild and domestic animals that co-exist with cervids can act as passive carriers of CWD prions [16–23]. For example, coyotes (Canis latrans) are relevant deer predators and scavengers. Previous studies showed that coyotes fed with an elk brain infected with CWD passed prions through their feces for at least three days after ingestion [23]. Similar observations were reported for cougars (Puma concolor) [21]. In contrast, crows (Corvus brachyrhynchos) passed prions for only few hours after ingesting infected materials [22]. Importantly, previous research in coyotes and cougars suggest that excreted prions contain decreased infectivity titers compared with the ingested material, suggesting that infectious particles are being retained in tissues within these animals, or degraded. In natural settings, prions have been identified in scat from multiple species of sympatric animals [20]. Overall, data indicates that predators and scavengers can excrete infectious prions after ingesting contaminated tissues. This, in turn, may contribute to the environmental spread of prions.
Snip…
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0327485
PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)
Davis Seelig, Amy Nalls, Maryanne Flasik, Victoria Frank, Candace Mathiason, Edward Hoover Colorado State University; Fort Collins, CO USA
Background and Introduction. Chronic wasting disease (CWD) is an efficiently transmitted prion disease of cervids with an as yet to be fully defined host range. Moreover, the risk that CWD poses to feline predators and scavangers, through crossspecies consumption and subsequent transmission, is unknown. Previous and ongoing studies in our laboratory evaluating the susceptibility of domestic cats (Felis catus) to CWD (Mathiason et. al., NeuroPrion 2011, Nalls et. al., NeuroPrion 2012) have documented the susceptibility of domestic cats to CWD following intracerebral (IC) inoculation. However, many of the pathologic features of feline-adapted CWD, including the neural and systemic patterns of PrPCWD accumulation and neuropathology, remain unknown. The chief objectives of this work were:
(1) to design a sensitive, enhanced immunohistochemical (E-IHC) protocol for the detection of CWD prions (PrPCWD) in feline tissues;
(2) to document the systemic distribution of PrPCWD in CWD-infected cats through E-IHC;
(3) to utilize single and multiple-label immunostaining and laser scanning confocal microscopy (LSCM) to provide insights into the subcellular patterns of PrPCWD accumulation and neuropathologic features of CWD-infected cats; and
(4) to compare feline CWD to the other known feline TSE
Materials and Methods. Periodate-lysine-paraformaldehyde (PLP)-fixed, paraffin-embedded (PLP-PE) from terminal, IC-inoculated (n = 9) and sham-inoculated (n = 2), 1st and 2nd passage, CWD-infected cats were examined by E-IHC for the presence of PrPCWD and its association with markers of cell phenotype and organelles. Results. The most sensitive E-IHC technique for the detection of PrPCWD in feline tissues incorporated a combination of slide pretreatment with proteinase-K (PK) in concert with tyramide signal amplification (TSA). With this protocol, we identified PrPCWD deposits throughout the CNS, which, in the 1st passage cats was primarily restricted to the obex, but increased in distribution and severity upon 2nd passage to include a number of midbrain nuclei, cortical gray matter, the thalamus and hypothalamus, and the hippocampus. Peripheral PrPCWD deposits were detected only in the 2nd passage cats, and included the enteric nervous system, the Peyer’s patches, and the retropharyngeal and mesenteric lymph nodes. PrPCWD was not detected in the sham-inoculated cats. Moreover, using multi-label analysis, intracellular PrPCWD aggregates were seen in association with neurofilament heavy chain (NFH)-positive neurons and GFAP-positive astrocytes. In addition, large aggregates of intracellular PrPCWD were identified within LAMP1-positive lysosomes. Conclusions. Feline PrPCWD is present in CNS neurons, astrocytes and LAMP-1-positive lysosomes. The morphologic overlap between the PrPCWD deposits in feline CWD and BSE-origin feline spongiform encephalopathy (FSE), implicates the importance of the host as a key determinant in the development of prion neuropathology and suggest a signature for detection of potential spontaneous feline prion disease.
http://www.landesbioscience.com/journals/prion/04-Prion6-2-Pathogenesis-and-pathology.pdf
https://web.archive.org/web/20140514022931/http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf
PO-041: Susceptibility of domestic cats to CWD infection
Amy Nalls, Jeanette Hayes-Klug, Kelly Anderson, Davis Seelig, Kevin Carnes, Susan Kraft, Edward Hoover, Candace Mathiason Colorado State University; Fort Collins, CO USA
Domestic and non-domestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE); very likely due to consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain homogenate. Between 40 and 43 months two IC-inoculated cats developed slowly progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors, and ataxia”’ultimately mandating euthanasia. PrPCWD was detected in the brains of these animals by western blot, immunohistochemistry (IHC), and quaking-induced conversion (RT-QuIC) assays. No clinical signs of TSE were detected in the remaining primary passage cats at 86 months pi. Feline-adapted CWD (FelCWD) was sub-passaged into groups (n = 4 or 5) of cats by IC, PO, and IP/SQ routes. All 5 IC inoculated cats developed symptoms of disease 20–24 months pi (approximately half the incubation period of primary passage). Additional symptoms in these animals included increasing aggressiveness and hyper responsiveness. FelCWD was demonstrated in the brains of all the affected cats by western blot and IHC. Currently, 3 of 4 IP/SQ, and 1 of 4 PO inoculated cats have developed abnormal behavior patterns consistent with the early stage of feline CWD. Magnetic resonance imaging (MRI) has been performed on 11 cats (6 clinically ill, 2 asymptomatic, and 3 age-matched negative controls).
Abnormalities were detected in 4 of 6 clinically ill cats and included multifocal signal changes consistent with inflammation, ventricular size increases, more prominent sulci, and white matter tract cavitation.
These results demonstrate that CWD can be transmitted and adapted to the domestic cat, and raise the potential for cervid-to-feline transmission in nature.
https://web.archive.org/web/20140514022931/http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf
MONDAY, AUGUST 8, 2011
Susceptibility of Domestic Cats to CWD Infection Oral.29: Susceptibility of Domestic Cats to CWD Infection
http://felinespongiformencephalopathyfse.blogspot.com/2011/08/susceptibility-of-domestic-cats-to-cwd.html
THURSDAY, JULY 10, 2025
Distribution of chronic wasting disease (CWD) prions in tissues from experimentally exposed coyotes (Canis latrans) Published: July 9, 2025
https://chronic-wasting-disease.blogspot.com/2025/07/distribution-of-chronic-wasting-disease.html
MONDAY, JANUARY 08, 2024
CWD TSE Prion, using canine and feline species as a tool, as scavengers to contain disease, is a bad idea, here's why
https://chronic-wasting-disease.blogspot.com/2024/01/cwd-tse-prion-using-canine-and-feline.html
FRIDAY, MARCH 24, 2023
Mountain lions, Wolves, Coyotes, could help stop the spread of CWD TSE Prion in deer, WHERE STUPID MEETS THE ROAD!
https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/mountain-lions-wolves-coyotes-could.html
Camel Prion Disease
Friday, May 12, 2023
Camel prion disease, a new emerging disease in North Africa, Lymphoid Tropism, Neuropathological Characterization Update 2023
11th Iberian Congress on Prions Barcelona 2023
http://prioncongressbcn.ppmclab.com/book_abstracts_v4.pdf
https://camelusprp.blogspot.com/2023/05/camel-prion-disease-new-emerging.html
A Camelid Anti-PrP Antibody Abrogates PrPSc Replication in Prion-Permissive Neuroblastoma Cell Lines
Daryl Rhys Jones,William Alexander Taylor,Clive Bate,Monique David,Mourad Tayebi
Published: March 22, 2010
https://doi.org/10.1371/journal.pone.0009804
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0009804
15 Apr 2018 23:13 GMT MOST RECENT
Prion Disease in Dromedary Camels, Algeria
Posted by flounder on 15 Apr 2018 at 23:13 GMT
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/fea97a95-2600-42a6-b289-0f490896a3aa
https://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0009804
https://wwwnc.cdc.gov/eid/article/24/6/17-2007_article
Chronic Wasting Disease CWD TSE Prion
Captive CHRONIC WASTING DISEASE CASES Update August 2025 CHRONIC WASTING DISEASE CASES
Expanding Distribution of CWD
https://www.usgs.gov/centers/nwhc/science/expanding-distribution-chronic-wasting-disease#overview
Date of Index Case Confirmation Index Case State County Species Herd Type HCP Enrolled HCP Certified Number of Animals Herd Status
8/6/2025 Adult Female PA Fulton WTD Breeder No No 14 Quarantine
7/21/2025 4 YR Female PA Bedford WTD Breeder No No 34 Quarantine
6/3/2025 11 YR Female PA Blair WTD Breeder No No 45 Quarantine
6/3/2025 8 YR Female PA Bedford WTD Breeder No No 6 Quarantine
5/16/2025 5.5 YR Female WI Rock WTD Breeder No No ~46 Quarantine
5/14/2025 3 YR Female UT Weber Elk Hunt No No ukn Quarantine
4/30/2025 4.5 YR Male PA Jefferson WTD Hunt No No 36 Depopulated
4/18/2025 10+ YR Ukn TX Zavala WTD Hunt No No 190 Quarantine
Updated August 2025
See full list;
Captive CHRONIC WASTING DISEASE CASES Update August 2025, Pennsylvania, Wisconsin, Utah, and Texas
https://www.aphis.usda.gov/sites/default/files/status-of-captive-herds.pdf
Texas Game Wardens Near Conclusion of ‘Ghost Deer’ Case with 24 Suspects, 1,400 Charges Filed Statewide
Aug. 14, 2025
Media Contact: TPWD News, Business Hours, 512-389-8030
AUSTIN – The Texas Game Warden investigation known as "Ghost Deer" has reached a possible conclusion after two additional suspects turned themselves in on felony charges. This brings the total number of individuals implicated in the case to 24, with approximately 1,400 charges filed across 11 Texas counties.
Ken Schlaudt, 64, of San Antonio, the owner of four deer breeding facilities and one release site, along with facility manager Bill Bowers, 55, of San Angelo, surrendered to the Travis County District Attorney’s Office on charges of felony tampering with a governmental record. Both men allegedly entered false information into the Texas Wildlife Information Management System (TWIMS) to facilitate illegal smuggling of white-tailed breeder deer. They also face more than 100 misdemeanor charges related to unlawful breeder deer activities in Tom Green County.
The "Ghost Deer" investigation has uncovered widespread, coordinated deer breeding violations including, but not limited to: smuggling captive breeder deer and free-range whitetail deer between breeder facilities and ranches, Chronic Wasting Disease (CWD) testing violations, license violations and misdemeanor and felony drug charges relating to the possession and mishandling of prescribed sedation drugs classified as controlled substances.
The suspects charged in the case include:
Evan Bircher, 59, San Antonio Vernon Carr, 55, Corpus Christi Jarrod Croaker, 47, Corpus Christi Terry Edwards, 54, Angleton Joshua Jurecek, 41, Alice Justin Leinneweber, 36, Orange Grove James Mann, 53, Odem Gage McKinzie, 28, Normanna Herbert “Tim” McKinzie, 47, Normanna Eric Olivares, 47, Corpus Christi Bruce Pipkin, 57, Beaumont Dustin Reynolds, 38, Robstown Kevin Soto, 55, Hockley Jared Utter, 52, Pipe Creek Reed Vollmering, 32, Orange Grove Clint West, 56, Beaumont James Whaley, 49, Sevierville, Tenn. Ryder Whitstine, 19, Rockport Ryker Whitstine, 21, Rockport Claude Wilhelm, 52, Orange Cases are pending adjudication in Bandera, Bee, Brazoria, Duval, Edwards, Jim Wells, Live Oak, Montgomery, Tom Green, Travis and Webb counties.
The investigation began in March 2024 when game wardens discovered the first violations during a traffic stop.
https://tpwd.texas.gov/newsmedia/releases/?req=20250206a
That incident led wardens to the much larger network of violations,
https://tpwd.texas.gov/newsmedia/releases/?req=20250227b
resulting in one of the largest deer smuggling operations in Texas history.
About Texas Game Wardens
Texas Game Wardens, within the Law Enforcement Division of Texas Parks and Wildlife Department, are responsible for enforcing laws related to the conservation and management of natural resources and public safety through community-based law enforcement. Their mission is to provide hunting, fishing and outdoor recreation opportunities for the use and enjoyment of present and future generations. Additionally, they play a crucial role in search and rescue operations during natural disasters, exemplifying their commitment to protecting both the environment and the people of Texas.
If you witness a fishing, wildlife or boating violation in progress, please call 1-800-792-GAME(4263) immediately and report it to Operation Game Thief (OGT), Texas’ Wildlife Crime-Stoppers Program. You can also text your tip by sending the keyword TXOGT plus your tip to 847411 or through the Texas OGT App, available for iOS and Android devices. Dispatchers are available 24/7.
https://tpwd.texas.gov/newsmedia/releases/?req=20250814c
Texas Chronic Wasting Disease CWD TSE Prion Dashboard Update August 2025
https://experience.arcgis.com/experience/8f6c27330c444a19b4b57beb7ffabb8b/page/Dashboard#data_s=id%3AdataSource_3-1966d773e34-layer-10%3A29
WEDNESDAY, MAY 14, 2025
Texas CWD TSE Prion Cases Rises to 1099 Confirmed Cases To Date
Entries CWD Positives
Positive Number CWD Positive Confirmation Date Free Range Captive County Source Species Sex Age
1099 5/5/25 Breeder Deer Gillespie Facility #14 White-tailed Deer M 4.9
1098 4/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer F 7.8
1097 4/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer F 7.8
1096 4/17/25 Breeder Release Site Zavala N/A White-tailed Deer M 10.5
1095 4/3/25 Breeder Deer Kimble Facility #26 White-tailed Deer F 2.5
1094 4/3/25 Breeder Deer Kimble Facility #26 White-tailed Deer F 6.5
1093 4/3/25 Breeder Deer Kimble Facility #26 White-tailed Deer F 3.5
1092 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 1.7
1091 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 1.7
1090 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 3.7
1089 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 5.7
1088 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 5.7
1087 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 7.7
1086 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 3.7
1085 4/3/25 Breeder Deer Frio Facility #24 White-tailed Deer F 3.7
1084 3/19/25 Free Range El Paso N/A Mule Deer M 6.5
1083 3/14/25 Breeder Deer Frio Facility #24 White-tailed Deer M 1.7
1082 2/27/25 Breeder Deer Kaufman Facility #36 White-tailed Deer F 0.5
1081 2/27/25 Breeder Deer Kaufman Facility #36 White-tailed Deer M 1.5
1080 2/21/25 Breeder Deer Gillespie Facility #15 White-tailed Deer M 2.5
1079 2/19/25 Breeder Deer Frio Facility #24 White-tailed Deer M 1.4
1078 2/13/25 Breeder Release Site Medina Facility #3 Elk F 4
1077 1/14/25 Breeder Deer Frio Facility #24 White-tailed Deer F 2.5
1076 1/14/25 Breeder Deer Frio Facility #24 White-tailed Deer M 1.5
1075 1/14/25 Breeder Deer Frio Facility #24 White-tailed Deer M 1.5
1074 1/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer F 1.5
1073 1/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer F 4.5
1072 1/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer M 2.5
1071 1/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer M 2.5
1070 1/24/25 Breeder Deer Zavala Facility #23 White-tailed Deer M 3.5
1069 2/4/25 Breeder Release Site Brown N/A White-tailed Deer F 2.6
1068 1/23/25 Breeder Release Site Sutton N/A White-tailed Deer M 6.5
1067 1/23/25 Breeder Release Site Medina Facility #3 White-tailed Deer M 5.5
1066 1/24/25 Breeder Release Site Hunt N/A White-tailed Deer M 2.5
1065 1/14/25 Breeder Release Site Zavala N/A White-tailed Deer M 5.5
1064 1/14/25 Breeder Release Site Zavala N/A White-tailed Deer M 5.5
1063 1/16/25 Free Range Hudspeth N/A Mule Deer M 8.5
1062 1/7/25 Breeder Deer Real Facility #29 White-tailed Deer F 3.4
1061 12/26/24 Breeder Release Site Brown N/A White-tailed Deer F 3.5
Snip…see full list of CWD Positives;
https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml
WEDNESDAY, MAY 14, 2025
Texas CWD TSE Prion Cases Rises to 1099 Confirmed Cases To Date
https://chronic-wasting-disease.blogspot.com/2025/05/texas-cwd-tse-prion-cases-rises-to-1099.html
December 2024
***> TEXAS CWD TSE PRION POSITIVE SAMPLES BY CALENDAR YEAR JANUARY 1 TO DECEMBER 31 2024 TOTAL TO DATE 1061 CASES CONFIRMED
Texas CWD total by calendar years
https://chronic-wasting-disease.blogspot.com/2024/12/texas-cwd-tse-prion-positive-samples-by.html
May 2024
Texas TAHC TPWD Confirm 132 More Cases of CWD TSE PrP
Jumps from 663 in March, to 795 Positive In May 2024, wow!
https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD
https://chronic-wasting-disease.blogspot.com/2024/05/texas-tahc-tpwd-confirm-132-more-cases.html
TPWD CWD Tracking
https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD
Counties where CWD Exposed Deer were Released
https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf
Number of CWD Exposed Deer Released by County
https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf
Texas Kimble County Farm Chronic Wasting Disease CWD TSE Prion Approximate Herd Prevalence 12%
SUMMARY MINUTES OF THE 407th COMMISSION MEETING Texas Animal Health Commission
September 22, 2020
Chronic Wasting Disease (CWD):
A new CWD positive breeding herd was disclosed in February 2020 in Kimble County. This herd depopulation was completed in July 2020. Including the two index positive deer, an additional eight more positive deer were disclosed (approximate herd prevalence 12%). Since July 2015 and prior to this discovery, five positive captive breeder herds have been disclosed and four of those are in Medina County. One herd in Lavaca and three herds in Medina County were depopulated leaving one large herd in Medina County that is managed on a herd plan. A new zone was established in Val Verde County in December 2019 as a result of a positive free-ranging White-tailed Deer (WTD). A second positive WTD was also disclosed in February 2020 in the same area.
SUMMARY MINUTES OF THE 407th COMMISSION MEETING – 9/22/2020
Scrapie: The flock identified in April 2016 remains under quarantine in Hartley County.
https://www.tahc.texas.gov/agency/meetings/minutes/SummaryMinutes_CommMtg_2020-09-22
http://web.archive.org/web/20201017124040/https://www.tahc.texas.gov/agency/meetings/minutes/SummaryMinutes_CommMtg_2020-09-22.pdf
Trucking CWD TSE Prion
“CWD spreads among wild populations at a relatively slow rate, limited by the natural home range and dispersed nature of wild animals.”
NOW HOLD YOUR HORSES, Chronic Wasting Disease CWD of Cervid can spread rather swiftly, traveling around 50 MPH, from the back of truck and trailer, and Here in Texas, we call it ‘Trucking CWD’…
Preventive Veterinary Medicine Volume 234, January 2025, 106385
Use of biosecurity practices to prevent chronic wasting disease in Minnesota cervid herds
Vehicles or trailers that entered the farm were used to transport other live cervids, cervid carcasses, or cervid body parts in past 3 years in 64.3 % (95 % CI 46.3–82.3) of larger elk/reindeer herds compared to 13.6 % (95 % CI 4.7–22.4) of smaller deer herds.
Snip…
Identifying the exact pathway of initial CWD transmission to cervid herds is often not possible, in part due to many potential pathways of transmission for the infection, including both direct and indirect contact with infected farmed or wild cervids (Kincheloe et al., 2021). That study identified that transmissions from infected farmed cervids may occur from direct contact with the movement of cervids from one herd to another and from indirect contact with the sharing of equipment, vehicles, clothing, reproductive equipment, and potentially through semen or embryos.
https://www.sciencedirect.com/science/article/abs/pii/S016758772400271X
Snip…see;
Trucking CWD TSE Prion
https://chronic-wasting-disease.blogspot.com/2025/04/trucking-cwd-tse-prion.html
WEDNESDAY, MAY 14, 2025
Texas CWD TSE Prion Cases Rises to 1099 Confirmed Cases To Date
https://chronic-wasting-disease.blogspot.com/2025/05/texas-cwd-tse-prion-cases-rises-to-1099.html
Addressing chronic wasting disease in Korean farms: topsoil removal and 2N NaOH treatment before cervid restocking
Kyung-Je Park , Hoo-Chang Park , Yu-Ran Lee , In-Soon Roh , Gordon Mitchell , Young Pyo Choi …
Published online: 08 Jul 2025
Abstract
Chronic wasting disease (CWD) is a highly contagious prion disease occurring in free-ranging and farmed cervids. In the Republic of Korea, cases of CWD continue to be detected almost annually, on both new and occasionally previously infected farms. CWD-infected animals contaminate soil and other environmental components by shedding prions through their excreta. Since shed prions remain infectious for years in the environment, they can act as infectivity reservoirs facilitating horizontal transmission of CWD. To prevent the further spread of CWD and allow farms to resume operations, control measures on infected farms, including topsoil removal and thorough environmental treatment with 2N NaOH, have been implemented in the Republic of Korea. Restocking remediated farms with cervids was permitted after confirming the absence of prion seeding activity in soil samples using protein misfolding cyclic amplification (PMCA). A total of 215 samples from 18 remediated farms were collected and analysed using PMCA, with only 3 samples from 3 farms displaying prion seeding activity. While the disease control measures effectively eliminated prion seeding activity in CWD-affected farms, CWD recurred at two of the 18 remediated farms 4 to 5 years after restocking animals. It remains unclear whether the recurrence of CWD at the two farms was due to residual prions in the environment after the control measures, or the introduction of the infected animals from other farms. This uncertainty is heightened by the annual occurrence of CWD at multiple farms and the absence of a traceability system for farmed cervids.
Keywords: Chronic wasting disease (CWD); NaOH; Protein-misfolding cyclic amplification (PMCA); Republic of Korea; farm; prions; remediation; topsoil.
https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2527588
“While the disease control measures effectively eliminated prion seeding activity in CWD-affected farms, CWD recurred at two of the 18 remediated farms 4 to 5 years after restocking animals.”
I remember what “deep throat” told me about Scrapie back around 2001, during early days of my BSE investigation, after my Mom died from hvCJD, I never forgot, and it seems it’s come to pass;
***> Confidential!!!!
***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!
---end personal email---end...tss
and so it seems…
so, this is what we leave our children and grandchildren?
Rapid recontamination of a farm building occurs after attempted prion removal
First published: 19 January 2019 https://doi.org/10.1136/vr.105054
The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.
snip...
This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapie positive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054
***>This is very likely to have parallels with control efforts for CWD in cervids.
https://pubmed.ncbi.nlm.nih.gov/30602491/
Front. Vet. Sci., 14 September 2015 | https://doi.org/10.3389/fvets.2015.00032
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination.
http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full
"Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation."
15 YEARS!
Detection of prions in soils contaminated by multiple routes
Results: We are able to detect prion seeding activity at multiple types of environmental hotspots, including carcass sites, contaminated captive facilities, and scrapes (i.e. urine and saliva). Differences in relative prion concentration vary depending on the nature and source of the contamination. Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation.
Conclusions: Detection of prions in the environment is of the utmost importance for controlling chronic wasting disease spread. Here, we have demonstrated a viable method for detection of prions in complex environmental matrices. However, it is quite likely that this method underestimates the total infectious prion load in a contaminated sample, due to incomplete recovery of infectious prions. Further refinements are necessary for accurate quantification of prions in such samples, and to account for the intrinsic heterogeneities found in the broader environment.
Funded by: Wisconsin Department of Natural Resources
Prion 2023 Abstracts
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
SUNDAY, APRIL 06, 2025
Failure to prevent classical scrapie after repeated decontamination of a barn
https://scrapie-usa.blogspot.com/2025/04/failure-to-prevent-classical-scrapie.html
https://prpsc.proboards.com/thread/165/failure-prevent-scrapie-repeated-decontamination
CWD, So, this is what we leave our children and grandchildren?
Detection of chronic wasting disease prions in the farm soil of the Republic of Korea
Here, we show that prion seeding activity was detected in extracts from farm soil following 4 years of incubation with CWD-infected brain homogenate.
https://journals.asm.org/doi/10.1128/msphere.00866-24
=====***>
"Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation."
=====***>
Detection of prions in soils contaminated by multiple routes
Results: We are able to detect prion seeding activity at multiple types of environmental hotspots, including carcass sites, contaminated captive facilities, and scrapes (i.e. urine and saliva). Differences in relative prion concentration vary depending on the nature and source of the contamination. Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation.
Conclusions: Detection of prions in the environment is of the utmost importance for controlling chronic wasting disease spread. Here, we have demonstrated a viable method for detection of prions in complex environmental matrices. However, it is quite likely that this method underestimates the total infectious prion load in a contaminated sample, due to incomplete recovery of infectious prions. Further refinements are necessary for accurate quantification of prions in such samples, and to account for the intrinsic heterogeneities found in the broader environment.
Funded by: Wisconsin Department of Natural Resources
Prion 2023 Abstracts
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
Artificial mineral sites that pre-date endemic chronic wasting disease become prion hotspots
The detection of PrPCWD in soils at attractant sites within an endemic CWD zone significantly advances our understanding of environmental PrPCWD accumulation dynamics, providing valuable information for advancing adaptive CWD management approaches.
https://int-cwd-sympo.org/wp-content/uploads/2023/06/final-agenda-with-abstracts.pdf
Chronic wasting disease detection in environmental and biological samples from a taxidermy site
Results: The PMCA analysis demonstrated CWD seeding activity in some of the components of this facility, including insects involved in head processing, soils, and a trash dumpster.
Conclusions: Different areas of this property were used for various taxidermy procedures. We were able to detect the presence of prions in i) soils that were in contact with the heads of dead animals, ii) insects involved in the cleaning of skulls, and iii) an empty dumpster where animal carcasses were previously placed. This is the first report demonstrating that swabbing is a helpful method to screen for prion infectivity on surfaces potentially contaminated with CWD. These findings are relevant as this swabbing and amplification strategy may be used to evaluate the disease status of other free-ranging and captive settings where there is a concern for CWD transmissions, such as at feeders and water troughs with CWD-exposed properties. This approach could have substantial implications for free-ranging cervid surveillance as well as in epidemiological investigations of CWD.
Prion 2022 Conference abstracts: pushing the boundaries
https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286
https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true
***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years
***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded.
JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12
Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free
https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0
Rapid recontamination of a farm building occurs after attempted prion removal
First published: 19 January 2019 https://doi.org/10.1136/vr.105054
The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease. snip...
This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapie positive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054
***>This is very likely to have parallels with control efforts for CWD in cervids.
https://pubmed.ncbi.nlm.nih.gov/30602491/
Chronic Wasting Disease CWD TSE Prion
THE CWD TSE Prion aka mad cow type disease is not your normal pathogen.
The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.
you cannot cook the TSE prion disease out of meat.
you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.
the TSE prion agent also survives Simulated Wastewater Treatment Processes.
IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.
you can bury it and it will not go away.
The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.
it’s not your ordinary pathogen you can just cook it out and be done
New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication
http://www.pnas.org/content/97/7/3418.full
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/
March 13, 2025
Prion Partitioning and Persistence in Environmental Waters
https://pubs.acs.org/doi/epdf/10.1021/acs.est.4c11497?ref=article_openPDF
Prions in Waterways
https://vimeo.com/898941380?fbclid=IwAR3Di7tLuU-iagCetdt4-CVPrOPQQrv037QS1Uxz0tX3z7BuvPeYlwIp7IY
Detection of protease-resistant cervid prion protein in water from a CWD-endemic area
https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf
A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing
http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract
Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals
https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf
THURSDAY, FEBRUARY 28, 2019
BSE infectivity survives burial for five years with only limited spread
https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf
So, this is what we leave our children and grandchildren?
Aug 18, 2021
Oh, Deer
Heading Off a Wildlife Epidemic
CWD poses a significant threat to the future of hunting in Texas. Deer population declines of 45 and 50 percent have been documented in Colorado and Wyoming. A broad infection of Texas deer populations resulting in similar population impacts would inflict severe economic damage to rural communities and could negatively impact land markets. Specifically, those landowners seeking to establish a thriving herd of deer could avoid buying in areas with confirmed CWD infections. As they do with anthrax-susceptible properties, land brokers may find it advisable to inquire about the status of CWD infections on properties that they present for sale. Prospective buyers should also investigate the status of the wildlife on prospective properties. In addition, existing landowners should monitor developments as TPWD crafts management strategies to identify and contain this deadly disease.
Dr. Gilliland (c-gilliland@tamu.edu) is a research economist with the Texas Real Estate Research Center at Texas A&M University.
https://www.recenter.tamu.edu/articles/tierra-grande/oh-d
So, this is what we leave our children and grandchildren?
CDC CWD TSE Prion Update 2025
KEY POINTS
Chronic wasting disease affects deer, elk and similar animals in the United States and a few other countries.
The disease hasn't been shown to infect people.
However, it might be a risk to people if they have contact with or eat meat from animals infected with CWD.
https://www.cdc.gov/chronic-wasting/about/index.html
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Volume 31, Number 2—February 2025
Research
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Snip…
In summary, the results of our study indicate that prions are widely distributed in peripheral and edible tissues of cervids in Norway, including muscles. This finding highlights the risk of human exposure to small amounts of prions through handling and consuming infected cervids. Nevertheless, we note that this study did not investigate the zoonotic potential of the Norway CWD prions. In North America, humans have historically consumed meat from CWD-infected animals, which has been documented to harbor prions (35,44–47). Despite the potential exposure to prions, no epidemiologic evidence indicates a correlation between the occurrence of CWD cases in animals and the prevalence of human prion diseases (48). A recent bioassay study reported no transmissions from 3 Nordic isolates into transgenic mice expressing human PrP (49). Therefore, our findings should be interpreted with caution in terms of human health implications, and further research is required to determine the zoonotic potential of these CWD strains.
The presence of prions in peripheral tissues indicates that CWD may have a systemic nature in all Norwegian cervid species, challenging the view that prions are exclusively localized in the CNS in sporadic CWD of moose and red deer. Our findings expand the notion of just how widely distributed prions can be in cervids affected with CWD and call into question the capability of emerging CWD strains in terms of infectivity to other species, including humans.
Appendix
https://wwwnc.cdc.gov/eid/article/31/2/24-0903-app1.pdf
https://wwwnc.cdc.gov/eid/article/31/2/24-0903_article
Volume 31, Number 2—February 2025
Dispatch
Detection of Chronic Wasting Disease Prions in Raw, Processed, and Cooked Elk Meat, Texas, USA
Rebeca Benavente, Fraser Brydon, Francisca Bravo-Risi, Paulina Soto, J. Hunter Reed, Mitch Lockwood, Glenn Telling, Marcelo A. Barria, and Rodrigo MoralesComments to Author
Snip…
CWD prions have been detected in the muscle of both farmed and wild deer (10), and at concentrations relevant to sustain disease transmission (11). CWD prions have also been identified across several cervid species and in multiple tissues, including lymph nodes, spleen, tongue, intestines, adrenal gland, eyes, reproductive tissues, ears, lungs, and liver, among others (12–14). Those findings raise concerns about the safety of ingesting processed meats that contain tissues other than skeletal muscle (15) (Appendix). https://wwwnc.cdc.gov/eid/article/31/2/24-0906-app1.pdf .
In addition, those findings highlight the need for continued vigilance and research on the transmission risks of prion diseases and for development of new preventative and detection measures to ensure the safety of the human food supply.
Snip…
Overall, our study results confirm previous reports describing the presence of CWD prions in elk muscles (13). The data also demonstrated CWD prion persistence in food products even after processing through different procedures, including the addition of salts, spices, and other edible elements. Of note, our data show that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification. Considering the potential implications in food safety and public health, we believe that the findings described in this study warrant further research. Our results suggest that although the elk meat used in this study resisted different manipulations involved in subsequent consumption by humans, their zoonotic potential was limited. Nevertheless, even though no cases of CWD transmission to human have been reported, the potential for human infection is still unclear and continued monitoring for zoonotic potential is warranted.
https://wwwnc.cdc.gov/eid/article/31/2/24-0906_article
***> Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry <***
Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure
Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA
Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.
Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).
Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.
Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.
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.
*****>>> "Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material." <<<*****
=====end
Strain characterization of chronic wasting disease in bovine-PrP transgenic mice
Nuria Jerez-Garrido1, Sara Canoyra1, Natalia Fernández-Borges1, Alba Marín Moreno1, Sylvie L. Benestad2, Olivier Andreoletti3, Gordon Mitchell4, Aru Balachandran4, Juan María Torres1 and Juan Carlos Espinosa1. 1 Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Madrid, Spain. 2 Norwegian Veterinary Institute, Ås, Norway. 3 UMR Institut National de la Recherche Agronomique (INRA)/École Nationale Vétérinaire de Toulouse (ENVT), Interactions Hôtes Agents Pathogènes, Toulouse, France. 4 Canadian Food Inspection Agency, Ottawa, Canada.
Aims: Chronic wasting disease (CWD) is an infectious prion disease that affects cervids. Various CWD prion strains have been identified in different cervid species from North America and Europe. The properties of the infectious prion strains are influenced by amino acid changes and polymorphisms in the PrP sequences of different cervid species. This study, aimed to assess the ability of a panel of CWD prion isolates from diverse cervid species from North America and Europe to infect bovine species, as well as to investigate the properties of the prion strains following the adaptation to the bovine-PrP context.
Materials and Methods: BoPrP-Tg110 mice overexpressing the bovine-PrP sequence were inoculated by intracranial route with a panel of CWD prion isolates from both North America (two white-tailed deer and two elk) and Europe (one reindeer, one moose and one red deer).
Results: Our results show distinct behaviours in the transmission of the CWD isolates to the BoPrP-Tg110 mouse model. Some of these isolates did not transmit even after the second passage. Those able to transmit displayed differences in terms of attack rate, survival times, biochemical properties of brain PrPres, and histopathology.
Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.
Funded by: MCIN/AEI /10.13039/501100011033 and by European Union NextGeneration EU/PRTR
Grant number: PCI2020-120680-2 ICRAD
"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."
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https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
so, this is what we leave our children and grandchildren?
Redefining the zoonotic potential of chronic wasting disease
Project Number 5R01NS121016-04
Contact PI/Project Leader SCHATZL, HERMANN M
Other PIs
Awardee Organization
UNIVERSITY OF CALGARY
Description
Abstract Text
The rapid expansion of chronic wasting disease (CWD), a prion disease of free-ranging and farmed deer, elk and moose, is a major and ongoing threat in North America. Approximately 1 in 36 Americans hunt deer and elk and eat venison, and it is estimated that 7,000 – 15,000 CWD-infected cervids are consumed annually. This fuels growing concerns about the human health risks imposed by CWD. There are no documented cases of CWD transmission to humans, even though with the long incubation periods of all prion diseases and the unknown presentation of CWD in humans definite conclusions are not possible. The zoonotic potential of prion diseases has been exemplified by bovine spongiform encephalopathy (BSE, mad cow disease) which resulted in a new form of human prion disease (vCJD). BSE was transmissible to Cynomolgus macaques and transgenic mice expressing the human prion protein. Initial results of CWD transmission studies to the same non-human primate and mouse models of human prion disease were not successful, corroborating the conclusion that the zoonotic potential of CWD is low, if not absent. Our groups were part of a consortium that inoculated Cynomolgus macaques via different routes with CWD. Some animals exhibited subtle clinical signs reminiscent of prion disease, and upon euthanasia, weak signs of vacuolation, PrPSc deposition and astrocytosis in the brain were found, while no proteinase K (PK) resistant prion protein (PrP) was detectable. We have now demonstrated for the first time that CWD from macaques can transmit clinical prion disease to transgenic mouse models of CWD and human prion disease, albeit in the absence of detectable PK-resistant PrP. Bona fide PrPSc was only detected upon 3rd passage from mouse to bank vole models. Altogether, this is the first evidence that CWD very likely has zoonotic potential. The goal of the current proposal is to redefine the zoonotic potential of CWD by characterizing the biological properties of CWD prions emerging upon experimental transmission into macaques, for obtaining important information on how CWD could manifest in humans.
In Aim 1, we will study whether CWD from macaque (CWDmac) in bank voles represents a new prion strain, by comparing biochemical and biological properties to an array of known prion strains from different species.
Aim 2 addresses the question whether CWDmac represents an intermediate prion strain, adaptable to cervids or humans upon passage, and possessing an expanded host range. We will address this by in vivo passage in cervidized or humanized mouse models. In vitro, we will utilize serial PMCA and a newly generated PrP0/0 cell culture model for infection, upon reconstitution with PrP from different species.
In Aim 3, we will shed light on the observed dissociation between infectivity and the presence of bona fide PrPSc. We propose to identify atypical PrP fragments associated with CWDmac, and we will elucidate brain cell responses to CWDmac exposure by innovative single cell RNA sequencing. In summary, our studies will uncover the possible manifestation of CWD in humans, which is of critical importance for drawing definite conclusions about the zoonotic potential of CWD.
Public Health Relevance Statement
The zoonotic potential of chronic wasting disease (CWD) is unclear to date. We provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents. Our proposed studies will unravel the properties of these prions, how they will adapt to humans and which pathways are activated in brain cells and associated with clinical disease. Results from these studies uncover the potential manifestation of CWD in humans, which is highly relevant for human health.
https://eventos.galoa.com.br/prion-2025/page/5178-home
Project 3A: CWD Prion Shedding and Environmental Contamination: Role in Transmission and Zoonotic
Parent Project Number 5P01AI077774-14
Sub-Project ID 5512
Contact PI/Project Leader HOOVER, EDWARD ARTHUR
Awardee Organization UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON
Description
Abstract Text
Chronic wasting disease (CWD) is an emergent, highly transmissible, geographically expanding, prion disease of both wild and captive cervids. CWD is unique among prion diseases in its facile contagion and environmental persistence. Its expanding geographical range, combined with the increasing transport of animals and animal products, portend its continued expansion and diversification. The zoonotic potential of CWD remains poorly understood. CWD endemic areas interface cervids with livestock species and humans, posing obvious zoonotic risks that over time will increase. While it is known that strains of CWD exist, nothing is known about the zoonotic potential of these strains. Work from our applicant group has shown that CWD infected cervids continually shed prions into the environment and that previously unrecognized environmental factors can influence the emergence of a dominant strain from a mixture. The ability to recognize the zoonotic potential of CWD strains is central to mitigating CWD transmission risk. The central hypothesis for work described here is that CWD strains evolve continuously due to a combination of both host and environmental factors. We will test this hypothesis by:
i) determining the evolution and zoonotic impact of CWD strains in the native cervid species;
ii) leveraging our unique animal resources, expertise, and in vivo & in vitro methodologies to assess environmental factors that alter CWD strain selection and evolution and
iii) evaluate zoonotic potential of CWD strains by a complementary combination of in vitro amplification assays and animal transmission studies.
The results will provide new information about this emergent transmissible prion disease and the risk it poses to humans and other species.
https://reporter.nih.gov/project-details/11056111#description
Project 3B: Pathogenesis Transmission and Detection of Zoonotic Prion Diseases
Parent Project Number 5P01AI077774-14
Sub-Project ID 5513
Contact PI/Project Leader BARTZ, JASON C
Awardee Organization UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON
Description Abstract Text
Project Summary: Chronic wasting disease (CWD) is an emergent, highly transmissible, geographically expanding, prion disease of both wild and captive cervids. CWD is unique among prion diseases in its facile contagion and environmental persistence. Its expanding geographical range, combined with the increasing transport of animals and animal products, portend its continued expansion and diversification. The zoonotic potential of CWD remains poorly understood. CWD endemic areas interface cervids with livestock species and humans, posing obvious zoonotic risks that over time will increase. While it is known that strains of CWD exist, nothing is known about the zoonotic potential of these strains. Work from our applicant group has shown that CWD- infected cervids continually shed prions into the environment and that previously unrecognized environmental factors can influence the emergence of a dominant strain from a mixture. The ability to recognize the zoonotic potential of CWD strains is central to mitigating CWD transmission risk. The central hypothesis for work described here is that CWD strains evolve continuously due to a combination of both host and environmental factors. We will test this hypothesis by:
i) determining the evolution and zoonotic impact of CWD strains in the native cervid species;
ii) leveraging our unique animal resources, expertise, and in vivo & in vitro methodologies to assess environmental factors that alter CWD strain selection and evolution and
iii) evaluate zoonotic potential of CWD strains by a complementary combination of in vitro amplification assays and animal transmission studies.
The results will provide new information about this emergent transmissible prion disease and the risk it poses to humans and other species.
https://reporter.nih.gov/project-details/11056115#description
Project 1: Modeling the Mechanisms of Prion Transmission, Strain Selection, Mutation and Species Barrier in Transgenic Mice
Parent Project Number 5P01AI077774-14
Sub-Project ID 5510
Contact PI/Project Leader TELLING, GLENN C
Awardee Organization UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON
Description Abstract Text
Our broad, long-term objectives are to are to decipher the mechanisms by which infectious prions replicate, encode strain information, and evolve to acquire new properties. We propose four Specific Aims to address our central hypothesis that incompletely adapted prion strains are comprised of poorly optimized ensembles of PrPSc quasi species conformers that evolve under selective pressure towards states of enhanced stability and pathogenicity. Our particular focus is chronic wasting disease (CWD), an uncontrollable contagious epidemic of cervids of uncertain zoonotic potential. Using genetically engineered CWD-susceptible mice, cultured cells, cell free amplification, and antibodies recognizing defined conformation-dependent PrP epitopes,
Aim I will address the mechanism of adaptation of unstable emergent CWD prions in response to physical and chemical constraints.
In Aim II we will address the hypothesis that that residue 226 and other cervid PrP polymorphisms influence selection of distinct portfolios of CWD strain conformers with different adaptive potentials. Using gene targeted mice expressing physiologically controlled levels of PrP variants and in vitro systems for prion replication, we will characterize the properties of strains propagated in these backgrounds and explore whether interference between them affects selection and adaptation of CWD.
In Aim III, we will assess the properties of emergent Norwegian moose and reindeer CWD strains experimentally propagated in deer and compare with established North American CWD.
Aim IV will address an unmet need in the field of significant importance, namely the paucity of model systems and tools for studying human prions. Using newly generated gene targeted mice expressing physiological levels of human PrP and novel approaches to derive susceptible human neuroblastoma cells, we will assess the zoonotic potential of emergent CWD strains and their adapted derivatives propagated in different cervid PrP backgrounds. Our ultimate goal is to assess and manage the risk posed to humans from continually evolving prions, specifically those causing CWD, by understanding the means by which they propagate and exist as heritable strains with protean host range properties that adapt and evolve under selective pressure.
https://reporter.nih.gov/project-details/11056096#description
Project 2
Parent Project Number 5P01AI077774-14
Sub-Project ID 5511
Contact PI/Project Leader SOTO, CLAUDIO
Awardee Organization UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON
Description Abstract Text
ABSTRACT
Chronic wasting disease (CWD) affecting various species of cervids in North American and Northern Europe represents a serious problem, because it continues to propagate uncontrollably among wild and captive cervids. CWD appears to be very heterogeneous with multiple different strains and can be transmitted to other animal species. The risk of CWD transmission to humans is unknown which is a major concern because the number of sick animals and their geographical distribution is rapidly increasing. The mechanism by which CWD propagates so efficiently among cervids is also unknown. The main goal of this project is to utilize a set of highly innovative techniques to study the cellular, molecular and structural features of naturally occurring CWD strains and their potential for inter- species transmission, particularly focusing on the possibility that certain CWD strains may infect humans. We will also attempt to elucidate the atomic resolution structure of CWD prions using cryo-electron microscopy. The overarching hypothesis is that CWD exists as multiple strains in distinct individuals and even within the same individual in different brain cells and that inter- species transmission and zoonotic potential depend on the specific strain characteristics. The project is divided in the following specific aims:
(1) Study the structural and molecular diversity of natural CWD strains and the high resolution three-dimensional structure of CWD prions.
(2) Understand CWD prion strain diversity in single brain cells isolated by laser capture microdissection and subsequently amplified by PMCA.
(3) Evaluate CWD inter-species transmission spillover potential and its effect on zoonotic potential.
(4) Analyze the deer-human prion species barrier in vivo using chimeric mice harboring human and cervid neuronal cells.
The studies included in this projects will address some of the most pressing questions regarding CWD, including
(i) the CWD prion strain variability,
(ii) the zoonotic potential of different CWD prion strains,
(iii) the atomic resolution structure of infectious prions and the structural basis of prion strains,
(iv) the cellular distribution of CWD prion strains in the brain and its gene expression consequences,
(v) the spillover potential of CWD to other animal species,
(vi) the potential role of intermediate species in the transmission of CWD prions to humans.
The findings generated in this project will be essential to design measures to prevent further propagation of CWD, and to avoid the emergence of new diseases with potentially disastrous consequences.
https://reporter.nih.gov/search/fXQ8kEmVa0mNDk1JNnx3Cw/project-details/11056103#description
18. Zoonotic potential of moose-derived chronic wasting disease prions after adaptation in intermediate species
Tomás Barrioa, Jean-Yves Doueta, Alvina Huora, Séverine Lugana, Naïma Arona, Hervé Cassarda, Sylvie L. Benestadb, Juan Carlos Espinosac, Juan María Torresc, Olivier Andréolettia
aUnité Mixte de Recherche de l’Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement 1225 Interactions Hôtes-Agents Pathogènes, École Nationale Vétérinaire de Toulouse, 31076 Toulouse, France; bNorwegian Veterinary Institute, P.O. Box 64, NO-1431 Ås, Norway; cCentro de Investigación en Sanidad Animal (CISA-INIA), 28130, Valdeolmos, Madrid, Spain
Aims: Chronic wasting disease (CWD) is an emerging prion disease in Europe. To date, cases have been reported in three Nordic countries and in several species, including reindeer (Rangifer tarandus), moose (Alces alces) and red deer (Cervus elaphus). Cumulating data suggest that the prion strains responsible for the European cases are distinct from those circulating in North America. The biological properties of CWD prions are still poorly documented, in particular their spillover and zoonotic capacities. In this study, we aimed at characterizing the interspecies transmission potential of Norwegian moose CWD isolates.
Materials and Methods: For that purpose, we performed experimental transmissions in a panel of transgenic models expressing the PrPC sequence of various species.
Results: On first passage, one moose isolate propagated in the ovine PrPC-expressing model (Tg338). After adaptation in this host, moose CWD prions were able to transmit in mice expressing either bovine or human PrPC with high efficacy.
Conclusions: These results suggest that CWD prions can acquire enhanced zoonotic properties following adaptation in an intermediate species.
Funding
Grant number: AAPG2020 EU-CWD, ICRAD2020 TCWDE, NRC2022 NorCWD
Acknowledgement
https://www.tandfonline.com/doi/full/10.1080/19336896.2024.2424058
“ After adaptation in this host, moose CWD prions were able to transmit in mice expressing either bovine or human PrPC with high efficacy.”
regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
Date: Fri, 18 Oct 2002 23:12:22 +0100
From: Steve Dealler
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
To: BSE-L@ …
######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Dear Terry,
An excellent piece of review as this literature is desparately difficult to get back from Government sites.
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported.
Well, if you dont look adequately like they are in USA currenly then you wont find any!
Steve Dealler
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
Subject: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
From: "Terry S. Singeltary Sr." <flounder@WT.NET>
Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE>
Date: Thu, 17 Oct 2002 17:04:51 -0700
snip...
''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
Table 9 presents the results of an analysis of these data.
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
snip...
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
snip...
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
snip...
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
snip...see full report ;
http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf
http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf
Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk
BSE Inquiry Steve Dealler
Management In Confidence
BSE: Private Submission of Bovine Brain Dealler
snip...end
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
BSE INQUIRY
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane
BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended.. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
TSE in wild UK deer? The first case of BSE (as we now realise) was in a nyala in London zoo and the further zoo cases in ungulates were simply thought of as being interesting transmissions of scrapie initially. The big problem started to appear with animals in 1993-5 when it became clear that there was an increase in the CJD cases in people that had eaten deer although the statistics involved must have been questionable. The reason for this was that the CJD Surveillance was well funded to look into the diet of people dying of CJD. This effect is not clear with vCJD...if only because the numbers involved are much smaller and hence it is difficult to gain enough statistics. They found that many other foods did not appear to have much association at all but that deer certainly did and as years went by the association actually became clearer. The appearance of vCJD in 1996 made all this much more difficult in that it was suddenly clearer that the cases of sporadic CJD that they had been checking up until then probably had nothing to do with beef...and the study decreased. During the period there was an increasing worry that deer were involved with CJD..
see references:
DEER BRAIN SURVEY
https://web.archive.org/web/20090506025229/http://www.bseinquiry.gov.uk/files/yb/1991/11/20004001.pdf
CONFIDENTIAL AND IN CONFIDENCE TRANSMISSION TO CHIMPANZEES AND PIGS
IN CONFIDENCE
TRANSMISSION TO CHIMPANZEES
Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not.
We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of field isolates subsequently strain typed in mice been inoculated by the appropriate routes (i/c, i/p and i/v).
I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.
In view of Dr Gibbs' probable use of chimpazees Mr Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.
A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding mans ‘susceptibility. In the meantime no doubt the negativity would be used defensively. It would however be counterproductive if the experiment finally became positive. We may learn more about public reactions following next Monday's meeting.
R Bradley
CVO (+ Mr Wells’ commenters 23 September 1990 Dr T W A Little Dr B J Shreeve
90/9.23/1.1
https://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????
“Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091).
Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From:
Sent: Sunday, September 29, 2002 10:15 AM
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip... *** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
However, to date, no CWD infections have been reported in people.
sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven. if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;
sporadic = 54,983 hits
https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic
spontaneous = 325,650 hits
https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous
key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD.
SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
However, to date, no CWD infections have been reported in people. key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys
http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true
https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article
So, this is what we leave our children and grandchildren?
CDC CWD TSE Prion Update 2025
KEY POINTS
Chronic wasting disease affects deer, elk and similar animals in the United States and a few other countries.
The disease hasn't been shown to infect people.
However, it might be a risk to people if they have contact with or eat meat from animals infected with CWD.
https://www.cdc.gov/chronic-wasting/about/index.html
Volume 31, Number 4—April 2025
Research
Detection and Decontamination of Chronic Wasting Disease Prions during Venison Processing
https://wwwnc.cdc.gov/eid/article/31/4/24-1176_article
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Volume 31, Number 2—February 2025
Research
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Snip…
In summary, the results of our study indicate that prions are widely distributed in peripheral and edible tissues of cervids in Norway, including muscles. This finding highlights the risk of human exposure to small amounts of prions through handling and consuming infected cervids. Nevertheless, we note that this study did not investigate the zoonotic potential of the Norway CWD prions. In North America, humans have historically consumed meat from CWD-infected animals, which has been documented to harbor prions (35,44–47). Despite the potential exposure to prions, no epidemiologic evidence indicates a correlation between the occurrence of CWD cases in animals and the prevalence of human prion diseases (48). A recent bioassay study reported no transmissions from 3 Nordic isolates into transgenic mice expressing human PrP (49). Therefore, our findings should be interpreted with caution in terms of human health implications, and further research is required to determine the zoonotic potential of these CWD strains.
The presence of prions in peripheral tissues indicates that CWD may have a systemic nature in all Norwegian cervid species, challenging the view that prions are exclusively localized in the CNS in sporadic CWD of moose and red deer. Our findings expand the notion of just how widely distributed prions can be in cervids affected with CWD and call into question the capability of emerging CWD strains in terms of infectivity to other species, including humans.
Appendix
https://wwwnc.cdc.gov/eid/article/31/2/24-0903-app1.pdf
https://wwwnc.cdc.gov/eid/article/31/2/24-0903_article
Volume 31, Number 2—February 2025
Dispatch
Detection of Chronic Wasting Disease Prions in Raw, Processed, and Cooked Elk Meat, Texas, USA
Rebeca Benavente, Fraser Brydon, Francisca Bravo-Risi, Paulina Soto, J. Hunter Reed, Mitch Lockwood, Glenn Telling, Marcelo A. Barria, and Rodrigo MoralesComments to Author
Snip…
CWD prions have been detected in the muscle of both farmed and wild deer (10), and at concentrations relevant to sustain disease transmission (11). CWD prions have also been identified across several cervid species and in multiple tissues, including lymph nodes, spleen, tongue, intestines, adrenal gland, eyes, reproductive tissues, ears, lungs, and liver, among others (12–14). Those findings raise concerns about the safety of ingesting processed meats that contain tissues other than skeletal muscle (15) (Appendix). https://wwwnc.cdc.gov/eid/article/31/2/24-0906-app1.pdf .
In addition, those findings highlight the need for continued vigilance and research on the transmission risks of prion diseases and for development of new preventative and detection measures to ensure the safety of the human food supply.
Snip…
Overall, our study results confirm previous reports describing the presence of CWD prions in elk muscles (13). The data also demonstrated CWD prion persistence in food products even after processing through different procedures, including the addition of salts, spices, and other edible elements. Of note, our data show that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification. Considering the potential implications in food safety and public health, we believe that the findings described in this study warrant further research. Our results suggest that although the elk meat used in this study resisted different manipulations involved in subsequent consumption by humans, their zoonotic potential was limited. Nevertheless, even though no cases of CWD transmission to human have been reported, the potential for human infection is still unclear and continued monitoring for zoonotic potential is warranted.
https://wwwnc.cdc.gov/eid/article/31/2/24-0906_article
Volume 31, Number 1—January 2025
Dispatch
Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States
Abstract
Using a prion amplification assay, we identified prions in tissues from wild pigs (Sus scrofa) living in areas of the United States with variable chronic wasting disease (CWD) epidemiology. Our findings indicate that scavenging swine could play a role in disseminating CWD and could therefore influence its epidemiology, geographic distribution, and interspecies spread.
Snip…
Conclusions In summary, results from this study showed that wild pigs are exposed to cervid prions, although the pigs seem to display some resistance to infection via natural exposure. Future studies should address the susceptibility of this invasive animal species to the multiple prion strains circulating in the environment. Nonetheless, identification of CWD prions in wild pig tissues indicated the potential for pigs to move prions across the landscape, which may, in turn, influence the epidemiology and geographic spread of CWD.
https://wwwnc.cdc.gov/eid/article/%2031/1/24-0401_article
Detection of chronic wasting disease prions in processed meats
Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA
Aims: identify the presence of CWD prions in processed meats derived from elk.
Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures.
Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked.
Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated.
Funded by: NIH and USDA
Grant number: 1R01AI132695 and APP-20115 to RM
Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples
"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."
end...
PRION 2023 CONTINUED;
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The detection and decontamination of chronic wasting disease prions during venison processing
Aims: There is a growing concern that chronic wasting disease (CWD) prions in venison pose a risk to human health. CWD prions accumulate in infected deer tissues that commonly enter the human food chain through meat processing and consumption. The United States (US) Food and Drug Administration and US Department of Agriculture now formally consider CWD-positive venison unfit for human and animal consumption. Yet, the degree to which prion contamination occurs during routine venison processing is unknown. Here, we use environmental surface swab methods to:
a) experimentally test meat processing equipment (i.e., stainless steel knives and polyethylene cutting boards) before and after processing CWD-positive venison and
b) test the efficacy of five different disinfectant types (i.e., Dawn dish soap, Virkon-S, Briotech, 10% bleach, and 40% bleach) to determine prion decontamination efficacy.
Materials and Methods: We used a real-time quaking-induced conversion (RT-QuIC) assay to determine CWD infection status of venison and to detect CWD prions in the swabs. We collected three swabs per surface and ran eight technical replicates on RT-QuIC.
Results: CWD prions were detected on all cutting boards (n= 3; replicates= 8/8, 8/8, 8/8 and knives (n= 3; replicates= 8/8, 8/8, 8/8) used in processing CWD-positive venison, but not on those used for CWD-negative venison. After processing CWD-positive venison, allowing the surfaces to dry, and washing the cutting board with Dawn dish soap, we detected CWD prions on the cutting board surface (n= 3; replicates= 8/8, 8/8, 8/8) but not on the knife (n= 3, replicates = 0/8, 0/8, 0/8). Similar patterns were observed with Briotech (cutting board: n= 3; replicates= 7/8, 1/8, 0/8; knife: n= 3; replicates = 0/8, 0/8, 0/8). We did not detect CWD prions on the knives or cutting boards after disinfecting with Virkon-S, 10% bleach, and 40% bleach.
Conclusions: These preliminary results suggest that Dawn dish soap and Briotech do not reliably decontaminate CWD prions from these surfaces. Our data suggest that Virkon-S and various bleach concentrations are more effective in reducing prion contamination of meat processing surfaces; however, surface type may also influence the ability of prions to adsorb to surfaces, preventing complete decontamination. Our results will directly inform best practices to prevent the introduction of CWD prions into the human food chain during venison processing.
Prion 2023 Abstracts
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.
Abstract
The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.
***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.
***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.
***> Our results show positive prion detection in all products.
***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.
***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.
https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true
Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.
Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany
Snip…
***> Further passage to cervidized mice revealed transmission with a 100% attack rate.
***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.
****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.
***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease
=====
https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true
Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD
Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha
Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.
Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.
Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.
https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286
The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD
Acta Neuropathol 144, 767–784 (2022). https://doi.org/10.1007/s00401-022-02482-9
Published
22 August 2022
https://link.springer.com/article/10.1007/s00401-022-02482-9
Fortuitous generation of a zoonotic cervid prion strain
Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.
Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (Tg12), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the Tg12 mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice.
Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the Tg12 mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice.
Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD
Acta Neuropathol 144, 767–784 (2022). https://doi.org/10.1007/s00401-022-02482-9
Published
22 August 2022
https://link.springer.com/article/10.1007/s00401-022-02482-9
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD
Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1
Received: 24 May 2022 / Revised: 5 August 2022 / Accepted: 7 August 2022
© The Author(s) 2022
Abstract
Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model. Inoculation of these mice with deer CWD isolates resulted in atypical clinical manifestation with prion seeding activity and efficient transmissible infectivity in the brain and, remarkably, in feces, but without classical neuropathological or Western blot appearances of prion diseases. Intriguingly, the protease-resistant PrP in the brain resembled that found in a familial human prion disease and was transmissible upon second passage. Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.
Keywords Chronic wasting disease · CWD · Zoonotic potential · Prion strains · Zoonotic prions
HIGHLIGHTS OF THIS STUDY
================================
Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.
In this study, we evaluated the zoonotic potential of CWD using a transgenic mouse model overexpressing human M129-PrPC (tg650 [12]). We inoculated tg650 mice intracerebrally with two deer CWD isolates, Wisc-1 and 116AG [22, 23, 27, 29]. We demonstrate that this transgenic line was susceptible to infection with CWD prions and displayed a distinct leading clinical sign, an atypical PrPSc signature and unusual fecal shedding of infectious prions. Importantly, these prions generated by the human PrP transgenic mice were transmissible upon passage. Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute. The fact that its signature was not typical raises the questions whether CWD would manifest in humans as a subclinical infection, whether it would arise through direct or indirect transmission including an intermediate host, or a silent to uncovered human-to-human transmission, and whether current detection techniques will be suffcient to unveil its presence.
Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.
Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerstmann-Sträussler-Scheinker syndrome (GSS) is defined by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profile and the N-terminal cleavage site.
CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent passage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.
“suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.”
=================================
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/s00401-022-02482-9
snip...see full text;
https://link.springer.com/article/10.1007/s00401-022-02482-9
https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf
EFSA Panel on Biological Hazards (BIOHAZ) Antonia Ricci Ana Allende Declan Bolton Marianne Chemaly Robert Davies Pablo Salvador Fernández Escámez ...
First published: 17 January 2018 https://doi.org/10.2903/j.efsa.2018.5132
also, see;
8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data.
***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison.
The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers.. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.
https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132
2004
Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD
????: CBCnews
https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html
2004
April 22, 2004, 10:30 AM CDT Guests: Patrick Singh, Terry Schwan, Janet Skarbek, Bill Fielding (BEGIN VIDEOTAPE) ANNOUNCER: DEBORAH NORVILLE TONIGHT.
https://www.nbcnews.com/id/wbna4806886
1997-11-10: Panorama - The British disease
https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html
TUESDAY, MAY 11, 2021
A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet
Conclusion
We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.
Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.
https://thescipub.com/pdf/ajidsp.2021.43.48.pdf
''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''
https://thescipub.com/pdf/ajidsp.2021.43.48.pdf
CREUTZFELDT JAKOB DISEASE: A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet
i was warning England and the BSE Inquiry about just this, way back in 1998, and was ask to supply information to the BSE Inquiry. for anyone that might be interested, see;
Singeltary submission to the BSE Inquiry on CJD and Nutritional Supplements 1998
ABOUT that deer antler spray and CWD TSE PRION... I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease. just saying...
I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.
Sender: "Patricia Cantos"
To: "Terry S Singeltary Sr. (E-mail)"
Subject: Your submission to the Inquiry
Date: Fri, 3 Jul 1998 10:10:05 +0100 3 July 1998
Mr Terry S Singeltary Sr. E-Mail: Flounder at wt.net Ref: E2979
Dear Mr Singeltary, Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments. Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died. As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments. Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD.
I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;
http://www.bse.org.uk.
Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it? In the meantime, thank you for you comments. Please do not hesitate to contact me on... snip...end...tss
everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year previously and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. both cases confirmed. ...
kind regards, terry
TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS IPLEX, mad by standard process; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. also; what about potential mad cow candy bars ? see their potential mad cow candy bar list too... THESE are just a few of MANY of just this ONE COMPANY...TSS
https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article
http://www.spiegel.de/spiegel/print/d-18578755.html
http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html
http://creutzfeldt-jakob-disease.blogspot.com/2013/11/large-cjd-tse-prion-potential-case.html
http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html
Two Hunters from the Same Lodge Afflicted with Sporadic CJD: Is Chronic Wasting Disease to Blame?
(P7-13.002) Jonathan Trout, Matthew Roberts, Michel Tabet, Eithan Kotkowski, and Sarah HornAUTHORS INFO & AFFILIATIONS April 9, 2024 issue 102 (17_supplement_1) https://doi.org/10.1212/WNL.0000000000204407
Abstract Publication History Information & Authors Metrics & Citations Share Abstract
Objective:
This study presents a cluster of Creutzfeldt-Jakob disease (CJD) cases after exposure to chronic wasting disease (CWD)-infected deer, suggestive of potential prion transmission from CWD-infected deer to humans.
Background:
CJD is a rapidly progressive central nervous system disorder caused by misfolded prion proteins. CWD, a prion disease prevalent in North American deer, has raised concerns due to its possible link to CJD. Although no conclusive evidence of cross-species prion transmission exists, vigilance for such cases is crucial for public health.
Design/Methods:
Not applicable.
Results:
In 2022, a 72-year-old man with a history of consuming meat from a CWD-infected deer population presented with rapid-onset confusion and aggression. His friend, who had also eaten venison from the same deer population, recently died of CJD, raising concerns about a potential link between CWD and human prion disease. Despite aggressive symptomatic treatment of seizures and agitation, the patient’s condition deteriorated and he died within a month of initial presentation. The diagnosis was confirmed postmortem as sporadic CJD with homozygous methionine at codon 129 (sCJDMM1). The patient’s history, including a similar case in his social group, suggests a possible novel animal-to-human transmission of CWD. Based on non-human primate and mouse models, cross-species transmission of CJD is plausible. Due to the challenge of distinguishing sCJDMM1 from CWD without detailed prion protein characterization, it is not possible to definitively rule out CWD in these cases. Although causation remains unproven, this cluster emphasizes the need for further investigation into the potential risks of consuming CWD-infected deer and its implications for public health.
Conclusions:
Clusters of sporadic CJD cases may occur in regions with CWD-confirmed deer populations, hinting at potential cross-species prion transmission. Surveillance and further research are essential to better understand this possible association.
Disclosure: Mr. Trout has nothing to disclose. Dr. Roberts has nothing to disclose. Dr. Tabet has nothing to disclose. Dr. Kotkowski has nothing to disclose. Dr. Horn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cala Trio. The institution of Dr. Horn has received research support from Alzheimer's Association.
https://www.neurology.org/doi/abs/10.1212/WNL.0000000000204407
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update
October 2009 T. Singeltary
14th ICID ISE 114
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
T. Singeltary
Bacliff, TX, USA
Background:
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods:
12 years independent research of available data
Results:
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion:
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
https://web.archive.org/web/20110417201250/http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 Singeltary Journal of Neurology
26 MARCH 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary, retired (medically)
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6
Tracking spongiform encephalopathies in North America, Lancet, Singeltary, August 2003
Tracking spongiform encephalopathies in North America 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
Volume 3, Number 8
01 August 2003
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.
49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.
Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.
Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.
To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.
Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.
Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.
Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.
Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.
Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.
CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
Diagnosis and Reporting of Creutzfeldt-Jakob Disease JAMA 2001 Singeltary on CJD
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
https://jamanetwork.com/journals/jama/article-abstract/1031186
https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
US scientists develop a possible test for BSE
BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999) Cite this as: BMJ 1999;319:1312
Rapid Response: Terry S. Singeltary Sr.
Re: vCJD in the USA * BSE in U.S.
In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease…see;
https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us
Re: vCJD in the USA * BSE in U.S. BMJ 1999;319:1312 Singeltary
https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us
Saturday, June 13, 2009
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
***> Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024).
OPINION article
Front. Cell. Neurosci., 13 April 2025
Sec. Cellular Neuropathology
Volume 19 - 2025 | https://doi.org/10.3389/fncel.2025.1571662
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
Ilia V. Baskakov, .jpegIlia V. Baskakov1,2*
1Center for Biomedical Engineering and Technology, University of Maryland School of Medicine, Baltimore, MD, United States 2Department of Neurobiology, University of Maryland School of Medicine, Baltimore, MD, United States
Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024). Considering that the mean age of onset for sCJD is 67 years, these early onset cases are exceedingly rare. Both patients were methionine/valine heterozygous at codon 129 of the prion protein gene (PRNP) and were classified as the MV1 subtype of sCJD. Both individuals exhibited an atypical clinical presentation and unusual profile of PrPSc, notably lacking the diglycosylated glycoform. Following clinical onset, disease progression was remarkably slow in both cases, with disease durations of 119 and 39 months in the 15- and 21-year-old individuals, respectively.
Although sCJD in adolescents is very rare, such cases are not unprecedented. Two adolescent cases, in which patients succumbed to the disease at ages 16 and 20, were previously reported in the United Kingdom (Murray et al., 2008). In one of these cases, the diagnosis of sCJD was confirmed based on neuropathological findings, PrPScsubtype analysis, and transmission in mice. Additionally, a 19-year-old patient was identified in Germany among a cohort of 52 sCJD patients aged 50 or younger at symptom onset during 1993–2003 (Boesenberg et al., 2005). The clinical manifestations in younger sCJD patients differ from those in older individuals in terms of clinical signs, disease duration, and neuropathological lesion profiles (Pocchiari et al., 2004; Boesenberg et al., 2005), with a younger age at onset correlating with prolonged survival (Pocchiari et al., 2004). In a cohort of 2,304 sCJD cases identified in Western Europe between 1993 and 2000, two cases with onset between 11 and 20 years exhibited disease durations of 54 and 58 months, respectively (Pocchiari et al., 2004). Valine homozygosity at codon 129 was more frequent in the “young” cohort (patients younger than 50 at symptom onset) compared to the “old” cohort (patients older than 50; Boesenberg et al., 2005). The ratio of type 1 to type 2 PrPSc subtypes, determined based on glycoform ratios and the electrophoretic mobility of proteinase K-resistant core, did not differ between the two groups (Boesenberg et al., 2005).
In the case of the 21-year-old patient identified in the United States (Ahn et al., 2024), the absence of the diglycosylated isoform and the higher molecular weight of the mono- and unglycosylated isoforms, relative to those typically observed in type 1 or type 2 PrPSc subtypes, raise questions about whether the disease phenotype aligns with any known sCJD subtype. The possibility of CJD transmission was ruled out, as the patient had never undergone any medical procedures associated with a risk of prion transmission nor traveled to countries affected by bovine spongiform encephalopathy (BSE; Ahn et al., 2024). Nevertheless, although such an early age of onset is rare for sCJD, it is characteristic of variant CJD (vCJD), which has a median onset age of 28 years.
Unlike sCJD, vCJD is acquired through the consumption of beef or beef products contaminated with BSE, also known as mad cow disease—a fatal prion disorder in cattle (Prusiner, 1997). While prion diseases primarily affect the brain, the lymphoreticular system playing a crucial role in transmission of BSE to humans (Hilton et al., 2004b; Aguzzi et al., 2013). Shortly after exposure, lymphotropic prion strains, including BSE, colonize secondary lymphoid organs (SLOs), where they exploit follicular dendritic cells to replicate and accumulate before spreading to the central nervous system (CNS; Hilton et al., 1998; Brown et al., 1999; McCulloch et al., 2011; Mabbott, 2012; Aguzzi et al., 2013). Similar to BSE, chronic wasting disease (CWD), a prion disease affecting cervids, also exhibits strong lymphotropism (Sigurdson et al., 1999).
CWD, which affects deer, elk, and moose, has been rapidly expanding across Canada and the U.S. As of early 2025, CWD has been detected in 36 U.S. states (USGS, 2025). The disease is highly contagious and primarily transmitted horizontally among cervids. CWD prions are shed in bodily fluids such as urine, saliva, and feces, contributing to persistent environmental contamination, particularly in soil (Tamgüney et al., 2009; Bartelt-Hunt and Bartz, 2013; Henderson et al., 2015, 2017; Davenport et al., 2018; Denkers et al., 2020; Tennant et al., 2020; Hwang et al., 2021; Denkers et al., 2024; Kuznetsova et al., 2024). Prions can be taken up by plants from contaminated soil and accumulate at levels sufficient for transmission to animals (Pritzkow et al., 2015; Carlson et al., 2023). Additionally, ticks have been shown to carry lethal doses of CWD infectivity (Inzalaco et al., 2023). While a substantial portion of the U.S. and Canadian populations is exposed to CWD through environmental contamination, the risk of transmission to humans is considered very low due to a significant species barrier.
The species barrier of CWD transmission to humans has been extensively studied using mouse models expressing human prion protein (PrPC; Kong et al., 2005; Sandberg et al., 2010; Wilson et al., 2012; Race et al., 2019; Hannaoui et al., 2022; Race et al., 2022; Wadsworth et al., 2022). In nearly all studies, humanized mice inoculated with CWD prions showed no clinical or subclinical disease and no detectable prion infectivity, with one notable exception (Hannaoui et al., 2022). In that study, infected humanized mice exhibited atypical clinical signs, prion seeding activity, and transmissible prion infectivity (Hannaoui et al., 2022).
In all previous studies assessing this risk, the intracranial (ic) route was used to administer CWD prions to humanized mice (Kong et al., 2005; Sandberg et al., 2010; Wilson et al., 2012; Race et al., 2019; Hannaoui et al., 2022; Race et al., 2022; Wadsworth et al., 2022). Ic inoculation is the most effective method for transmitting prions both within and across species (Race et al., 2009). However, successful cross-species transmission of lymphotropic prion strains appears to depend on SLOs for adaptation to a new host. Among the aforementioned studies, only one examined PrPSc accumulation in the spleen following CWD transmission to humanized mice (Wilson et al., 2012). When prions, such as BSE and CWD, cross species barriers, SLOs consistently exhibit greater permissiveness to prion replication than the brain (Béringue et al., 2012). Moreover, even after ic inoculation, lymphoreticular tissues exhibit a higher capacity than the brain to sustain and replicate lymphotropic prion strains, particularly at low-dose exposure (Halliez et al., 2014). This may be attributed to differences in glycosylation and sialylation between prions residing in the lymphoreticular system and those in the brain (Srivastava et al., 2015; Wagner et al., 2022). In spleens and lymph nodes, prions exhibit increased sialylation, potentially enhancing their resistance to clearance by innate immune system (Srivastava et al., 2015, 2017). Consequently, SLOs may provide a more favorable environment than the brain for prion adaptation to a new species. Furthermore, prion isolates, including CWD, have been shown to produce divergent disease phenotypes when introduced via ic versus peripheral routes, suggesting that brain and lymphoreticular tissues preferentially support different variants of PrPSc present in natural prion isolates (Béringue et al., 2012; DeFranco et al., 2024). Whether peripheral exposure facilitates more efficient cross-species adaptation of CWD prions remains unclear. Nonetheless, assessing PrPSc accumulation in SLOs following ic inoculation of humanized mice could provide a more sensitive approach for evaluating the potential risk of CWD transmission across species.
Additionally, with one exception (Wilson et al., 2012), all previous studies assessing the zoonotic potential of CWD have employed humanized mice homozygous for either 129MM or 129VV PrP (Kong et al., 2005; Sandberg et al., 2010; Race et al., 2019; Hannaoui et al., 2022; Race et al., 2022; Wadsworth et al., 2022). In the study that employed heterozygous 129MV humanized mice, the risk of transmission was assessed using only one CWD isolate (Wilson et al., 2012). In 129MV hosts, PrPSc structures must accommodate both 129M and 129V PrP molecules, likely resulting in an alternating incorporation of these isoforms. The presence of both 129M and 129V PrPC substrates is expected to boost the conformational diversity of PrPSc variants. Whether the structure of 129MV PrPSc is more compatible with CWD strains than that of 129MM or 129VV PrPSc, and whether the 129MV genotype is more susceptible to CWD prions, remains to be investigated.
With the continuous geographical expansion of CWD into highly populated areas and its increasing prevalence, human exposure—including that of children—to high doses of CWD prions via the environment may become unavoidable. The decomposition of carcasses from free-ranging deer infected with CWD could create environmental hotspots containing high concentrations of prions, posing long-term risks to ecosystems.
The uptake of prions by plants raises the possibility of contamination in the food chain, including dairy products. Evidence from prion research suggests that prions can be present in the mammary glands and milk of sheep incubating scrapie, the prion disease of sheep (Ligios et al., 2005; Lacroux et al., 2008; Maddison et al., 2009). If CWD prions can be adsorbed by the digestive system of cattle without causing clinical disease, they may still be excreted into milk, thereby introducing an unrecognized route of human exposure. The potential for milk contamination in dairy cattle that are not infected but are persistently exposed to CWD prions in contaminated environments warrants investigation.
A number of species, including goats, sheep, swine, rodents, mink, ferrets, raccoons, and possibly wild pigs and cattle are susceptible to CWD (Hamir et al., 2005, 2006; Raymond Gregory et al., 2007; Sigurdson et al., 2008; Heisey et al., 2010; Greenlee et al., 2012; Kurt and Sigurdson, 2016; Moore et al., 2017, 2019, 2022; Soto et al., 2025). Prions are subject to evolution and adaptation (Li et al., 2010; Baskakov, 2014). Upon transmission to new hosts, prion replication in a novel molecular environment enhances the conformational diversity of PrPSc variants, accelerating evolution and generating new strains with altered transmission characteristics (Gonzalez-Montalban et al., 2013; Makarava and Baskakov, 2013; Katorcha et al., 2018). As such, interspecies passage of CWD through different hosts may serve as a breeding ground for novel prion strains to emerge.
To date, more than 10 distinct CWD strains have been identified in deer, elk, moose, and reindeer (Otero et al., 2023; Sun et al., 2023). Assessing the transmissibility of diverse CWD strains is crucial for evaluating the potential risk of transmission to humans. Due to prion protein gene polymorphisms, cross-species transmission of CWD strains among different cervid species can alter strain properties, potentially leading to the emergence of novel variants with modified transmission characteristics (Bian et al., 2019, 2021; Otero et al., 2023). Such adaptations could expand the range of hosts susceptible to CWD.
Given the scale of potential CWD exposure, rare instances of transmission to humans might be expected beyond those directly linked to hunting. Since the clinical presentation of CWD in humans has not been defined, it is challenging to determine whether individuals diagnosed with sporadic sCJD at young ages, such as 15 and 21 years old, may have been infected with CWD. Autopsy of SLOs, including the spleen, lymph nodes, and tonsils, could help differentiate between sporadic and acquired forms of CJD. In sCJD patients, prions are detected in SLOs at a low prevalence (Glatzel et al., 2003), whereas in vCJD cases, linked to consumption of BSE-contaminated products, prions have been found in lymphoreticular tissues at a 100% rate (Hill et al., 1999; Ironside et al., 2002). Similarly, if CWD were transmissible to humans, it is expected that prions would accumulate in SLOs. Therefore, histopathological examination and biochemical analysis of PrPSc in SLOs should be conducted for all young individuals succumbed to CJD, as well as older individuals presenting with atypical clinical or neuropathological features. Additionally, transmission studies in animal models could provide further insights into distinguishing between sCJD and zoonotic forms of CJD.
Lymphotropic prion strains, such as BSE, acquired through cross-species transmission, can persist stably and silently in SLOs for extended periods without neuroinvasion (Peden et al., 2010; Bishop et al., 2013). In fact, SLOs serve as silent reservoirs of prion infection, where prions may remain undetected while posing a potential risk of transmission (Hilton et al., 2004a; Peden et al., 2004; Wroe et al., 2006; Peden et al., 2010; Bishop et al., 2013; Gill et al., 2013). Screening human lymphoreticular tissues in regions with a long history of CWD could provide valuable insights into whether CWD prions are silently harbored within the human population.
In conclusion, the expanding scale of human exposure to the growing CWD epidemic necessitates urgent discussions on safeguarding public health. The implementation of lymphoid tissue autopsies could aid in differentiating between sCJD from CJD acquired via transmission. Furthermore, improved risk assessment for CWD transmission to humans could be achieved by analyzing PrPSc accumulation in both the spleen and brain following ic inoculation of humanized mice along with the use of humanized mouse models with the 129MV genotype.
Author contributions IB: Writing – original draft, Writing – review & editing.
Funding The author(s) declare that financial support was received for the research and/or publication of this article. Financial support for this study was provided by National Institute of Health Grants R01 NS045585 and R01 NS129502 to IB.
Conflict of interest The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.
Generative AI statement The author(s) declare that no Gen AI was used in the creation of this manuscript.
Publisher's note All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.
References…snip…end
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2025.1571662/full
Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease
Jennifer Zitser, Sven Forner, Katherine Wong, Jin Chengshi, John Neuhaus, Jennifer Martindale, Ben J Raudabaugh, Kendra Benisano, Kelly Goodman-O’Leary, Stacy Metcalf
Brain, awaf077, https://doi.org/10.1093/brain/awaf077 Published: 28 March 2025
Although no neuropsychiatric symptom is pathognomonic for sCJD, certain symptoms might help differentiate sCJD from some other neurodegenerative diseases. Our findings support the inclusion of behavioral symptoms in sCJD diagnostic criteria.
https://academic.oup.com/brain/advance-article-abstract/doi/10.1093/brain/awaf077/8098176?login=false
See further ;
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/neuropsychiatric-symptoms-in-sporadic.html
SUNDAY, MARCH 23, 2025
Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html
https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html
RESTRICTED – POLICY CJD IN ADOLESCENTS (16 year old Vickey Rimmer), FARMERS WITH BSE HERDS, AND FARMERS WIFE with Sporadic CJD
June 2015
https://creutzfeldt-jakob-disease.blogspot.com/2015/06/restricted-policy-cjd-in-adolescents-16.html
https://www.researchgate.net/publication/279942086_RESTRICTED_-_POLICY_CJD_IN_ADOLESCENTS_16_year_old_Vickey_Rimmer_FARMERS_WITH_BSE_HERDS_AND_FARMERS_WIFE_with_Sporadic_CJD
TUESDAY, APRIL 15, 2025
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024).
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2025.1571662/full
https://creutzfeldt-jakob-disease.blogspot.com/2025/04/cases-of-creutzfeldt-jakob-disease-in.html
CASE REPORT article
Front. Cell. Neurosci., 02 January 2025
Sec. Cellular Neuropathology
Volume 18 - 2024 | https://doi.org/10.3389/fncel.2024.1518542
Case report: Atypical young case of MV1 Creutzfeldt-Jakob disease with unusually long survival
Lucie Yeongran Ahn.jpegLucie Yeongran Ahn1Mark L. Cohen,.jpegMark L. Cohen2,3Ignazio Cali,.jpegIgnazio Cali2,3Tia Russell.jpegTia Russell3Jessica Ludwig.jpegJessica Ludwig3Xun Jia.jpegXun Jia3Alberto Bizzi.jpegAlberto Bizzi4Lawrence B. Schonberger.jpegLawrence B. Schonberger5Ryan A. Maddox.jpegRyan A. Maddox5Rohini Paul.jpegRohini Paul6Tania C. Ghazarian.jpegTania C. Ghazarian7Jaspreet Garcha.jpegJaspreet Garcha8Mostafa Hammoudi.jpegMostafa Hammoudi9Brian Stephen Appleby,,, .jpegBrian Stephen Appleby2,3,10,11*
1Medical Scientist Training Program, Case Western Reserve University, Cleveland, OH, United States
2Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, United States
3National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, Cleveland, OH, United States
4Neuroradiology Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Neurologico Carlo Besta, Milan, Italy
5Centers for Disease Control and Prevention (CDC), U.S. Department of Health and Human Services (USDHHS), Atlanta, GA, United States
6Department of Psychiatry, Kaiser Permanente San Jose Medical Center, Graduate Medical Education, San Jose, CA, United States
7Department of Psychiatry, Loma Linda University School of Medicine, Graduate Medical Education, Loma Linda, CA, United States
8Department of Internal Medicine, Community Memorial Health Systems, Ventura, CA, United States 9Department of Neurology, Community Memorial Health Systems, Ventura, CA, United States 10Department of Neurology, Case Western Reserve University, School of Medicine, Cleveland, OH, United States 11Department of Psychiatry, Case Western Reserve University, School of Medicine, Cleveland, OH, United States
Creutzfeldt-Jakob disease (CJD) is a rare, fatal, rapidly progressive neurodegenerative disease resulting from an accumulation of misfolded prion proteins (PrP). CJD affects 1–2 new individuals per million each year, and the sporadic type accounts for 90% of those cases. Though the median age at onset and disease duration vary depending on the subtype of sporadic CJD (sCJD), the disease typically affects middle-aged to elderly individuals with a median survival of 4–6 months. sCJD in younger individuals is extremely rare. Here, we present a 21-year-old female who died with a sporadic prion disease. She presented with psychiatric symptoms followed by a rapidly progressive neurocognitive and motor decline. EEG was negative for periodic sharp wave complexes; however, brain MRI was suggestive of prion disease. The cerebrospinal fluid (CSF) real-time quaking-induced conversion (RT-QuIC) assay was indeterminate. Neuropathologic examination at autopsy revealed severe neuronal loss and gliosis with secondary white matter degeneration but minimal spongiform changes and PrP deposits in the cerebellum and neocortex by immunohistochemistry. Absence of pathogenic mutations and methionine/valine heterozygosity at codon 129 of the prion protein gene (PRNP), atypical type 1 protease-resistant PrP that lacks or shows underrepresentation of the diglycosylated PrP isoform by western blot analysis, and no acquired prion disease risk factors resulted in a final diagnosis of atypical sCJD. Very young onset sCJD often has atypical clinical presentations and disease progression, neuropathological examination results, and/or laboratory test results that may confound diagnosis. It is critical to perform thorough, comprehensive evaluations to make an accurate diagnosis, which includes autopsy confirmation with histology, prion protein typing and prion gene sequencing.
Snip…
Conclusion
In summary, very young sCJD patients with long disease duration may present quite differently than typical sCJD patients. To our knowledge, our case is the second reported case of a very young onset sCJD MV1 subtype with an unusually long disease duration and PrP type. Our case's MRI findings are consistent with what we would expect from an MV1 subtype patient. However, the clinical presentation, disease course, the absence of PSWCs in EEG, and indeterminate RT-QuIC results is not characteristic of sCJD MV1. Ultimately, the neuropathological exam analyses performed at the NPDPSC combined with no known risk factors for acquired prion disease provided confidence that this young patient had sporadic prion disease. An extremely rare, very young onset sCJD with a long disease duration case may present differently from the characteristic clinical presentations of sCJD. Thus, it is critical to comprehensively evaluate clinical findings, but most importantly to verify the presence and type of prion disease in these younger cases though neuropathologic evaluation at autopsy, especially since acquired prion diseases historically occur in young individuals (Rhoads et al., 2020; Manix et al., 2015). Neuropathologic evaluation of young cases in the UK was crucial for identifying variant CJD in those exposed to bovine spongiform encephalopathy (Will et al., 1996). The U.S. is currently dealing with a massive spread of an animal prion disease in the cervid species called chronic wasting disease. As such, it is more important than ever to confirm the presence and type of prion disease is the US, especially in atypical cases of prion disease (Osterholm et al., 2019).
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2024.1518542/full
Research Letter
December 11, 2023
Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020
Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et al John A. Romley, PhD4; John C. Probasco, MD5
Author Affiliations Article Information JAMA Neurol. 2024;81(2):195-197. doi:10.1001/jamaneurol.2023.4678
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
Snip…
Discussion Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing. This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.
https://jamanetwork.com/journals/jamaneurology/fullarticle/2812784
Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023
The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis
Angéline Denouel1 · Jean-Philippe Brandel1,2 · Danielle Seilhean1 · Jean-Louis Laplanche3,4 · Alexis Elbaz5 · Stéphane Haik1,2
Abstract
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.
We included all cases aged 45–89 years old who died with a probable/definite sCJD diagnosis based on the French national surveillance network. We used age-period-cohort (APC) Poisson regression models to study variation in mortality rates by sex, age, period, and time.
A total of 2475 sCJD cases aged 45–89 years were included. Mortality rates increased with age, reached a peak between 75 and 79 years, and decreased thereafter. Mortality rates were higher in women than men at younger ages and lower at older ages. The full APC model with a sex×age interaction provided the best fit to the data, thus in favour of sex, age, period, and cohort effects on mortality rates. In particular, mortality rates increased progressively with successive birth cohorts.
Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.
Keywords Age-period-cohort model · Prion · Temporal trend · Sporadic Creutzfeldt-Jakob disease
snip...
Worldwide, the number of patients with sCJD appears to have progressively increased over time [13]. This increase can be partly explained by increasing life expectancy as well as by better case ascertainment due to improved diagnostic tests and awareness of the disease among clinicians. Indeed, a relationship between surveillance intensity and sCJD incidence has been shown [14]. It cannot be excluded, however, that an actual increase of sCJD cases has occurred, and this hypothesis can be examined using age-period-cohort (APC) models.
snip...
In this paper, we estimated mortality rates from sCJD in France over a 25-year period (1992–2016) based on data from the French national surveillance network.
snip...
The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1).
snip...
Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.
https://link.springer.com/article/10.1007/s10654-023-01004-5
3 cases of the rare CJD in Hood River County, over the last 8 months
PUBLIC SERVICE ANNOUNCEMENTS
For Immediate Release
Date: April 14, 2025
To: All Media
From: Hood River County Health Department
Subject: Update on Creutzfeldt-Jakob Disease (CJD) Investigation
The health department recently reported three cases of the rare brain disorder Creutzfeldt-Jakob Disease (CJD) in Hood River County. Over the last 8 months, there have been 1 confirmed and 2 probable cases. Of these three cases, two people are deceased.
Per state law, the health department investigates and monitors diseases that could be a risk to the public. At this time, there is no identifiable link between these three cases.
The risk of getting CJD is extremely low. It does not spread through the air, touch, social contact, or water. People with questions about their healthcare are encouraged to contact their primary medical team.
This is an active and ongoing investigation in partnership with Oregon Health Authority (OHA) and Centers for Disease Control and Prevention (CDC). The health department will continue to monitor the situation and keep you informed of any risk to public health.
For more information on CJD, please visit CJD Foundation or the CDC’s website.
###
https://www.hoodrivercounty.gov/index.asp?SEC=AF40862A-8B0E-4107-B2B4-956029C71941&DE=11E4744C-DDC6-4DBD-8F95-B86EFBB310E6
Hood River County, Oregon has a population of 23,977 according to the Census Bureau.
https://www.google.com/search?q=hood+river+county+population%3F&rlz=1C9BKJA_enUS1037US1037&oq=hood+river+county+population%3F&gs_lcrp=EgZjaHJvbWUyBggAEEUYOdIBCTE3MDIyajBqN6gCD7ACAeIDBBgBIF8&hl=en-US&sourceid=chrome-mobile&ie=UTF-8
Oregon Prion TSE CJD ???
https://public.tableau.com/app/profile/oregon.public.health.division.acute.and.communicable.disease.pre/viz/WeeklyCommunicableDiseaseReport/ACDPWeeklyReport
https://www.oregon.gov/oha/ph/diseasesconditions/communicabledisease/diseasesurveillancedata/weekly-monthlystatistics/pages/index.aspx
Annual CJD
https://www.oregon.gov/oha/PH/DISEASESCONDITIONS/COMMUNICABLEDISEASE/DISEASESURVEILLANCEDATA/ANNUALREPORTS/Pages/arpt.aspx
https://www.oregon.gov/oha/ph/diseasesconditions/diseasesaz/pages/prion-diseases.aspx
https://www.oregon.gov/oha/PH/DISEASESCONDITIONS/COMMUNICABLEDISEASE/REPORTINGCOMMUNICABLEDISEASE/REPORTINGGUIDELINES/Documents/cjd-prion.pdf
What’s going on with CJD in Maricopa, Arizona? Concerning…
Arizona CJD
Arizona CJD CONFIRMED & PROBABLE CASES 2024* (selected morbidities)
https://www.azdhs.gov/documents/preparedness/epidemiology-disease-control/disease-data-statistics-reports/data-statistics-archive/2024/weekly.pdf
Maricopa is a city in the Gila River Valley in Pinal County, Arizona, United States. With 66,290 residents as of 2022, Maricopa is the largest incorporated municipality in Pinal County.[4]
https://en.m.wikipedia.org/wiki/Maricopa,_Arizona
***> Creutzfeldt Jakob Disease CJD TSE Prion Cases Increasing March 2025
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html
***> Creutzfeldt Jakob Disease CJD, BSE, CWD, TSE, Prion, December 14, 2024 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html
(1) Study the structural and molecular diversity of natural CWD strains and the high resolution three-dimensional structure of CWD prions.
(2) Understand CWD prion strain diversity in single brain cells isolated by laser capture microdissection and subsequently amplified by PMCA.
(3) Evaluate CWD inter-species transmission spillover potential and its effect on zoonotic potential.
(4) Analyze the deer-human prion species barrier in vivo using chimeric mice harboring human and cervid neuronal cells.
The studies included in this projects will address some of the most pressing questions regarding CWD, including
(i) the CWD prion strain variability,
(ii) the zoonotic potential of different CWD prion strains,
(iii) the atomic resolution structure of infectious prions and the structural basis of prion strains,
(iv) the cellular distribution of CWD prion strains in the brain and its gene expression consequences,
(v) the spillover potential of CWD to other animal species,
(vi) the potential role of intermediate species in the transmission of CWD prions to humans.
The findings generated in this project will be essential to design measures to prevent further propagation of CWD, and to avoid the emergence of new diseases with potentially disastrous consequences.
https://reporter.nih.gov/search/fXQ8kEmVa0mNDk1JNnx3Cw/project-details/11056103#description
18. Zoonotic potential of moose-derived chronic wasting disease prions after adaptation in intermediate species
Tomás Barrioa, Jean-Yves Doueta, Alvina Huora, Séverine Lugana, Naïma Arona, Hervé Cassarda, Sylvie L. Benestadb, Juan Carlos Espinosac, Juan María Torresc, Olivier Andréolettia
aUnité Mixte de Recherche de l’Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement 1225 Interactions Hôtes-Agents Pathogènes, École Nationale Vétérinaire de Toulouse, 31076 Toulouse, France; bNorwegian Veterinary Institute, P.O. Box 64, NO-1431 Ås, Norway; cCentro de Investigación en Sanidad Animal (CISA-INIA), 28130, Valdeolmos, Madrid, Spain
Aims: Chronic wasting disease (CWD) is an emerging prion disease in Europe. To date, cases have been reported in three Nordic countries and in several species, including reindeer (Rangifer tarandus), moose (Alces alces) and red deer (Cervus elaphus). Cumulating data suggest that the prion strains responsible for the European cases are distinct from those circulating in North America. The biological properties of CWD prions are still poorly documented, in particular their spillover and zoonotic capacities. In this study, we aimed at characterizing the interspecies transmission potential of Norwegian moose CWD isolates.
Materials and Methods: For that purpose, we performed experimental transmissions in a panel of transgenic models expressing the PrPC sequence of various species.
Results: On first passage, one moose isolate propagated in the ovine PrPC-expressing model (Tg338). After adaptation in this host, moose CWD prions were able to transmit in mice expressing either bovine or human PrPC with high efficacy.
Conclusions: These results suggest that CWD prions can acquire enhanced zoonotic properties following adaptation in an intermediate species.
Funding
Grant number: AAPG2020 EU-CWD, ICRAD2020 TCWDE, NRC2022 NorCWD
Acknowledgement
https://www.tandfonline.com/doi/full/10.1080/19336896.2024.2424058
“ After adaptation in this host, moose CWD prions were able to transmit in mice expressing either bovine or human PrPC with high efficacy.”
regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
Date: Fri, 18 Oct 2002 23:12:22 +0100
From: Steve Dealler
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
To: BSE-L@ …
######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Dear Terry,
An excellent piece of review as this literature is desparately difficult to get back from Government sites.
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported.
Well, if you dont look adequately like they are in USA currenly then you wont find any!
Steve Dealler
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
Subject: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
From: "Terry S. Singeltary Sr." <flounder@WT.NET>
Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE>
Date: Thu, 17 Oct 2002 17:04:51 -0700
snip...
''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
Table 9 presents the results of an analysis of these data.
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
snip...
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
snip...
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
snip...
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
snip...see full report ;
http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf
http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf
Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk
BSE Inquiry Steve Dealler
Management In Confidence
BSE: Private Submission of Bovine Brain Dealler
snip...end
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
BSE INQUIRY
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane
BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended.. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
TSE in wild UK deer? The first case of BSE (as we now realise) was in a nyala in London zoo and the further zoo cases in ungulates were simply thought of as being interesting transmissions of scrapie initially. The big problem started to appear with animals in 1993-5 when it became clear that there was an increase in the CJD cases in people that had eaten deer although the statistics involved must have been questionable. The reason for this was that the CJD Surveillance was well funded to look into the diet of people dying of CJD. This effect is not clear with vCJD...if only because the numbers involved are much smaller and hence it is difficult to gain enough statistics. They found that many other foods did not appear to have much association at all but that deer certainly did and as years went by the association actually became clearer. The appearance of vCJD in 1996 made all this much more difficult in that it was suddenly clearer that the cases of sporadic CJD that they had been checking up until then probably had nothing to do with beef...and the study decreased. During the period there was an increasing worry that deer were involved with CJD..
see references:
DEER BRAIN SURVEY
https://web.archive.org/web/20090506025229/http://www.bseinquiry.gov.uk/files/yb/1991/11/20004001.pdf
CONFIDENTIAL AND IN CONFIDENCE TRANSMISSION TO CHIMPANZEES AND PIGS
IN CONFIDENCE
TRANSMISSION TO CHIMPANZEES
Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not.
We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of field isolates subsequently strain typed in mice been inoculated by the appropriate routes (i/c, i/p and i/v).
I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.
In view of Dr Gibbs' probable use of chimpazees Mr Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.
A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding mans ‘susceptibility. In the meantime no doubt the negativity would be used defensively. It would however be counterproductive if the experiment finally became positive. We may learn more about public reactions following next Monday's meeting.
R Bradley
CVO (+ Mr Wells’ commenters 23 September 1990 Dr T W A Little Dr B J Shreeve
90/9.23/1.1
https://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????
“Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091).
Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From:
Sent: Sunday, September 29, 2002 10:15 AM
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip... *** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
However, to date, no CWD infections have been reported in people.
sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven. if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;
sporadic = 54,983 hits
https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic
spontaneous = 325,650 hits
https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous
key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD.
SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
However, to date, no CWD infections have been reported in people. key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys
http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true
https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article
So, this is what we leave our children and grandchildren?
CDC CWD TSE Prion Update 2025
KEY POINTS
Chronic wasting disease affects deer, elk and similar animals in the United States and a few other countries.
The disease hasn't been shown to infect people.
However, it might be a risk to people if they have contact with or eat meat from animals infected with CWD.
https://www.cdc.gov/chronic-wasting/about/index.html
Volume 31, Number 4—April 2025
Research
Detection and Decontamination of Chronic Wasting Disease Prions during Venison Processing
https://wwwnc.cdc.gov/eid/article/31/4/24-1176_article
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Volume 31, Number 2—February 2025
Research
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Snip…
In summary, the results of our study indicate that prions are widely distributed in peripheral and edible tissues of cervids in Norway, including muscles. This finding highlights the risk of human exposure to small amounts of prions through handling and consuming infected cervids. Nevertheless, we note that this study did not investigate the zoonotic potential of the Norway CWD prions. In North America, humans have historically consumed meat from CWD-infected animals, which has been documented to harbor prions (35,44–47). Despite the potential exposure to prions, no epidemiologic evidence indicates a correlation between the occurrence of CWD cases in animals and the prevalence of human prion diseases (48). A recent bioassay study reported no transmissions from 3 Nordic isolates into transgenic mice expressing human PrP (49). Therefore, our findings should be interpreted with caution in terms of human health implications, and further research is required to determine the zoonotic potential of these CWD strains.
The presence of prions in peripheral tissues indicates that CWD may have a systemic nature in all Norwegian cervid species, challenging the view that prions are exclusively localized in the CNS in sporadic CWD of moose and red deer. Our findings expand the notion of just how widely distributed prions can be in cervids affected with CWD and call into question the capability of emerging CWD strains in terms of infectivity to other species, including humans.
Appendix
https://wwwnc.cdc.gov/eid/article/31/2/24-0903-app1.pdf
https://wwwnc.cdc.gov/eid/article/31/2/24-0903_article
Volume 31, Number 2—February 2025
Dispatch
Detection of Chronic Wasting Disease Prions in Raw, Processed, and Cooked Elk Meat, Texas, USA
Rebeca Benavente, Fraser Brydon, Francisca Bravo-Risi, Paulina Soto, J. Hunter Reed, Mitch Lockwood, Glenn Telling, Marcelo A. Barria, and Rodrigo MoralesComments to Author
Snip…
CWD prions have been detected in the muscle of both farmed and wild deer (10), and at concentrations relevant to sustain disease transmission (11). CWD prions have also been identified across several cervid species and in multiple tissues, including lymph nodes, spleen, tongue, intestines, adrenal gland, eyes, reproductive tissues, ears, lungs, and liver, among others (12–14). Those findings raise concerns about the safety of ingesting processed meats that contain tissues other than skeletal muscle (15) (Appendix). https://wwwnc.cdc.gov/eid/article/31/2/24-0906-app1.pdf .
In addition, those findings highlight the need for continued vigilance and research on the transmission risks of prion diseases and for development of new preventative and detection measures to ensure the safety of the human food supply.
Snip…
Overall, our study results confirm previous reports describing the presence of CWD prions in elk muscles (13). The data also demonstrated CWD prion persistence in food products even after processing through different procedures, including the addition of salts, spices, and other edible elements. Of note, our data show that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification. Considering the potential implications in food safety and public health, we believe that the findings described in this study warrant further research. Our results suggest that although the elk meat used in this study resisted different manipulations involved in subsequent consumption by humans, their zoonotic potential was limited. Nevertheless, even though no cases of CWD transmission to human have been reported, the potential for human infection is still unclear and continued monitoring for zoonotic potential is warranted.
https://wwwnc.cdc.gov/eid/article/31/2/24-0906_article
Volume 31, Number 1—January 2025
Dispatch
Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States
Abstract
Using a prion amplification assay, we identified prions in tissues from wild pigs (Sus scrofa) living in areas of the United States with variable chronic wasting disease (CWD) epidemiology. Our findings indicate that scavenging swine could play a role in disseminating CWD and could therefore influence its epidemiology, geographic distribution, and interspecies spread.
Snip…
Conclusions In summary, results from this study showed that wild pigs are exposed to cervid prions, although the pigs seem to display some resistance to infection via natural exposure. Future studies should address the susceptibility of this invasive animal species to the multiple prion strains circulating in the environment. Nonetheless, identification of CWD prions in wild pig tissues indicated the potential for pigs to move prions across the landscape, which may, in turn, influence the epidemiology and geographic spread of CWD.
https://wwwnc.cdc.gov/eid/article/%2031/1/24-0401_article
Detection of chronic wasting disease prions in processed meats
Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA
Aims: identify the presence of CWD prions in processed meats derived from elk.
Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures.
Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked.
Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated.
Funded by: NIH and USDA
Grant number: 1R01AI132695 and APP-20115 to RM
Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples
"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."
end...
PRION 2023 CONTINUED;
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The detection and decontamination of chronic wasting disease prions during venison processing
Aims: There is a growing concern that chronic wasting disease (CWD) prions in venison pose a risk to human health. CWD prions accumulate in infected deer tissues that commonly enter the human food chain through meat processing and consumption. The United States (US) Food and Drug Administration and US Department of Agriculture now formally consider CWD-positive venison unfit for human and animal consumption. Yet, the degree to which prion contamination occurs during routine venison processing is unknown. Here, we use environmental surface swab methods to:
a) experimentally test meat processing equipment (i.e., stainless steel knives and polyethylene cutting boards) before and after processing CWD-positive venison and
b) test the efficacy of five different disinfectant types (i.e., Dawn dish soap, Virkon-S, Briotech, 10% bleach, and 40% bleach) to determine prion decontamination efficacy.
Materials and Methods: We used a real-time quaking-induced conversion (RT-QuIC) assay to determine CWD infection status of venison and to detect CWD prions in the swabs. We collected three swabs per surface and ran eight technical replicates on RT-QuIC.
Results: CWD prions were detected on all cutting boards (n= 3; replicates= 8/8, 8/8, 8/8 and knives (n= 3; replicates= 8/8, 8/8, 8/8) used in processing CWD-positive venison, but not on those used for CWD-negative venison. After processing CWD-positive venison, allowing the surfaces to dry, and washing the cutting board with Dawn dish soap, we detected CWD prions on the cutting board surface (n= 3; replicates= 8/8, 8/8, 8/8) but not on the knife (n= 3, replicates = 0/8, 0/8, 0/8). Similar patterns were observed with Briotech (cutting board: n= 3; replicates= 7/8, 1/8, 0/8; knife: n= 3; replicates = 0/8, 0/8, 0/8). We did not detect CWD prions on the knives or cutting boards after disinfecting with Virkon-S, 10% bleach, and 40% bleach.
Conclusions: These preliminary results suggest that Dawn dish soap and Briotech do not reliably decontaminate CWD prions from these surfaces. Our data suggest that Virkon-S and various bleach concentrations are more effective in reducing prion contamination of meat processing surfaces; however, surface type may also influence the ability of prions to adsorb to surfaces, preventing complete decontamination. Our results will directly inform best practices to prevent the introduction of CWD prions into the human food chain during venison processing.
Prion 2023 Abstracts
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.
Abstract
The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.
***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.
***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.
***> Our results show positive prion detection in all products.
***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.
***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.
https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true
Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.
Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany
Snip…
***> Further passage to cervidized mice revealed transmission with a 100% attack rate.
***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.
****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.
***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease
=====
https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true
Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD
Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha
Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.
Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.
Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.
https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286
The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD
Acta Neuropathol 144, 767–784 (2022). https://doi.org/10.1007/s00401-022-02482-9
Published
22 August 2022
https://link.springer.com/article/10.1007/s00401-022-02482-9
Fortuitous generation of a zoonotic cervid prion strain
Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.
Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (Tg12), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the Tg12 mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice.
Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the Tg12 mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice.
Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD
Acta Neuropathol 144, 767–784 (2022). https://doi.org/10.1007/s00401-022-02482-9
Published
22 August 2022
https://link.springer.com/article/10.1007/s00401-022-02482-9
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD
Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1
Received: 24 May 2022 / Revised: 5 August 2022 / Accepted: 7 August 2022
© The Author(s) 2022
Abstract
Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model. Inoculation of these mice with deer CWD isolates resulted in atypical clinical manifestation with prion seeding activity and efficient transmissible infectivity in the brain and, remarkably, in feces, but without classical neuropathological or Western blot appearances of prion diseases. Intriguingly, the protease-resistant PrP in the brain resembled that found in a familial human prion disease and was transmissible upon second passage. Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.
Keywords Chronic wasting disease · CWD · Zoonotic potential · Prion strains · Zoonotic prions
HIGHLIGHTS OF THIS STUDY
================================
Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.
In this study, we evaluated the zoonotic potential of CWD using a transgenic mouse model overexpressing human M129-PrPC (tg650 [12]). We inoculated tg650 mice intracerebrally with two deer CWD isolates, Wisc-1 and 116AG [22, 23, 27, 29]. We demonstrate that this transgenic line was susceptible to infection with CWD prions and displayed a distinct leading clinical sign, an atypical PrPSc signature and unusual fecal shedding of infectious prions. Importantly, these prions generated by the human PrP transgenic mice were transmissible upon passage. Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute. The fact that its signature was not typical raises the questions whether CWD would manifest in humans as a subclinical infection, whether it would arise through direct or indirect transmission including an intermediate host, or a silent to uncovered human-to-human transmission, and whether current detection techniques will be suffcient to unveil its presence.
Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.
Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerstmann-Sträussler-Scheinker syndrome (GSS) is defined by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profile and the N-terminal cleavage site.
CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent passage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.
“suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.”
=================================
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/s00401-022-02482-9
snip...see full text;
https://link.springer.com/article/10.1007/s00401-022-02482-9
https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf
EFSA Panel on Biological Hazards (BIOHAZ) Antonia Ricci Ana Allende Declan Bolton Marianne Chemaly Robert Davies Pablo Salvador Fernández Escámez ...
First published: 17 January 2018 https://doi.org/10.2903/j.efsa.2018.5132
also, see;
8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data.
***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison.
The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers.. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.
https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132
2004
Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD
????: CBCnews
https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html
2004
April 22, 2004, 10:30 AM CDT Guests: Patrick Singh, Terry Schwan, Janet Skarbek, Bill Fielding (BEGIN VIDEOTAPE) ANNOUNCER: DEBORAH NORVILLE TONIGHT.
https://www.nbcnews.com/id/wbna4806886
1997-11-10: Panorama - The British disease
https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html
TUESDAY, MAY 11, 2021
A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet
Conclusion
We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.
Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.
https://thescipub.com/pdf/ajidsp.2021.43.48.pdf
''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''
https://thescipub.com/pdf/ajidsp.2021.43.48.pdf
CREUTZFELDT JAKOB DISEASE: A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet
i was warning England and the BSE Inquiry about just this, way back in 1998, and was ask to supply information to the BSE Inquiry. for anyone that might be interested, see;
Singeltary submission to the BSE Inquiry on CJD and Nutritional Supplements 1998
ABOUT that deer antler spray and CWD TSE PRION... I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease. just saying...
I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.
Sender: "Patricia Cantos"
To: "Terry S Singeltary Sr. (E-mail)"
Subject: Your submission to the Inquiry
Date: Fri, 3 Jul 1998 10:10:05 +0100 3 July 1998
Mr Terry S Singeltary Sr. E-Mail: Flounder at wt.net Ref: E2979
Dear Mr Singeltary, Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments. Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died. As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments. Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD.
I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;
http://www.bse.org.uk.
Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it? In the meantime, thank you for you comments. Please do not hesitate to contact me on... snip...end...tss
everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year previously and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. both cases confirmed. ...
kind regards, terry
TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS IPLEX, mad by standard process; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. also; what about potential mad cow candy bars ? see their potential mad cow candy bar list too... THESE are just a few of MANY of just this ONE COMPANY...TSS
https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article
http://www.spiegel.de/spiegel/print/d-18578755.html
http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html
http://creutzfeldt-jakob-disease.blogspot.com/2013/11/large-cjd-tse-prion-potential-case.html
http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html
Two Hunters from the Same Lodge Afflicted with Sporadic CJD: Is Chronic Wasting Disease to Blame?
(P7-13.002) Jonathan Trout, Matthew Roberts, Michel Tabet, Eithan Kotkowski, and Sarah HornAUTHORS INFO & AFFILIATIONS April 9, 2024 issue 102 (17_supplement_1) https://doi.org/10.1212/WNL.0000000000204407
Abstract Publication History Information & Authors Metrics & Citations Share Abstract
Objective:
This study presents a cluster of Creutzfeldt-Jakob disease (CJD) cases after exposure to chronic wasting disease (CWD)-infected deer, suggestive of potential prion transmission from CWD-infected deer to humans.
Background:
CJD is a rapidly progressive central nervous system disorder caused by misfolded prion proteins. CWD, a prion disease prevalent in North American deer, has raised concerns due to its possible link to CJD. Although no conclusive evidence of cross-species prion transmission exists, vigilance for such cases is crucial for public health.
Design/Methods:
Not applicable.
Results:
In 2022, a 72-year-old man with a history of consuming meat from a CWD-infected deer population presented with rapid-onset confusion and aggression. His friend, who had also eaten venison from the same deer population, recently died of CJD, raising concerns about a potential link between CWD and human prion disease. Despite aggressive symptomatic treatment of seizures and agitation, the patient’s condition deteriorated and he died within a month of initial presentation. The diagnosis was confirmed postmortem as sporadic CJD with homozygous methionine at codon 129 (sCJDMM1). The patient’s history, including a similar case in his social group, suggests a possible novel animal-to-human transmission of CWD. Based on non-human primate and mouse models, cross-species transmission of CJD is plausible. Due to the challenge of distinguishing sCJDMM1 from CWD without detailed prion protein characterization, it is not possible to definitively rule out CWD in these cases. Although causation remains unproven, this cluster emphasizes the need for further investigation into the potential risks of consuming CWD-infected deer and its implications for public health.
Conclusions:
Clusters of sporadic CJD cases may occur in regions with CWD-confirmed deer populations, hinting at potential cross-species prion transmission. Surveillance and further research are essential to better understand this possible association.
Disclosure: Mr. Trout has nothing to disclose. Dr. Roberts has nothing to disclose. Dr. Tabet has nothing to disclose. Dr. Kotkowski has nothing to disclose. Dr. Horn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cala Trio. The institution of Dr. Horn has received research support from Alzheimer's Association.
https://www.neurology.org/doi/abs/10.1212/WNL.0000000000204407
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update
October 2009 T. Singeltary
14th ICID ISE 114
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
T. Singeltary
Bacliff, TX, USA
Background:
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods:
12 years independent research of available data
Results:
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion:
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
https://web.archive.org/web/20110417201250/http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 Singeltary Journal of Neurology
26 MARCH 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary, retired (medically)
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6
Tracking spongiform encephalopathies in North America, Lancet, Singeltary, August 2003
Tracking spongiform encephalopathies in North America 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
Volume 3, Number 8
01 August 2003
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.
49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.
Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.
Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.
To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.
Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.
Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.
Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.
Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.
Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.
CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
Diagnosis and Reporting of Creutzfeldt-Jakob Disease JAMA 2001 Singeltary on CJD
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
https://jamanetwork.com/journals/jama/article-abstract/1031186
https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
US scientists develop a possible test for BSE
BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999) Cite this as: BMJ 1999;319:1312
Rapid Response: Terry S. Singeltary Sr.
Re: vCJD in the USA * BSE in U.S.
In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease…see;
https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us
Re: vCJD in the USA * BSE in U.S. BMJ 1999;319:1312 Singeltary
https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us
Saturday, June 13, 2009
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009
https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
***> Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024).
OPINION article
Front. Cell. Neurosci., 13 April 2025
Sec. Cellular Neuropathology
Volume 19 - 2025 | https://doi.org/10.3389/fncel.2025.1571662
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
Ilia V. Baskakov, .jpegIlia V. Baskakov1,2*
1Center for Biomedical Engineering and Technology, University of Maryland School of Medicine, Baltimore, MD, United States 2Department of Neurobiology, University of Maryland School of Medicine, Baltimore, MD, United States
Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024). Considering that the mean age of onset for sCJD is 67 years, these early onset cases are exceedingly rare. Both patients were methionine/valine heterozygous at codon 129 of the prion protein gene (PRNP) and were classified as the MV1 subtype of sCJD. Both individuals exhibited an atypical clinical presentation and unusual profile of PrPSc, notably lacking the diglycosylated glycoform. Following clinical onset, disease progression was remarkably slow in both cases, with disease durations of 119 and 39 months in the 15- and 21-year-old individuals, respectively.
Although sCJD in adolescents is very rare, such cases are not unprecedented. Two adolescent cases, in which patients succumbed to the disease at ages 16 and 20, were previously reported in the United Kingdom (Murray et al., 2008). In one of these cases, the diagnosis of sCJD was confirmed based on neuropathological findings, PrPScsubtype analysis, and transmission in mice. Additionally, a 19-year-old patient was identified in Germany among a cohort of 52 sCJD patients aged 50 or younger at symptom onset during 1993–2003 (Boesenberg et al., 2005). The clinical manifestations in younger sCJD patients differ from those in older individuals in terms of clinical signs, disease duration, and neuropathological lesion profiles (Pocchiari et al., 2004; Boesenberg et al., 2005), with a younger age at onset correlating with prolonged survival (Pocchiari et al., 2004). In a cohort of 2,304 sCJD cases identified in Western Europe between 1993 and 2000, two cases with onset between 11 and 20 years exhibited disease durations of 54 and 58 months, respectively (Pocchiari et al., 2004). Valine homozygosity at codon 129 was more frequent in the “young” cohort (patients younger than 50 at symptom onset) compared to the “old” cohort (patients older than 50; Boesenberg et al., 2005). The ratio of type 1 to type 2 PrPSc subtypes, determined based on glycoform ratios and the electrophoretic mobility of proteinase K-resistant core, did not differ between the two groups (Boesenberg et al., 2005).
In the case of the 21-year-old patient identified in the United States (Ahn et al., 2024), the absence of the diglycosylated isoform and the higher molecular weight of the mono- and unglycosylated isoforms, relative to those typically observed in type 1 or type 2 PrPSc subtypes, raise questions about whether the disease phenotype aligns with any known sCJD subtype. The possibility of CJD transmission was ruled out, as the patient had never undergone any medical procedures associated with a risk of prion transmission nor traveled to countries affected by bovine spongiform encephalopathy (BSE; Ahn et al., 2024). Nevertheless, although such an early age of onset is rare for sCJD, it is characteristic of variant CJD (vCJD), which has a median onset age of 28 years.
Unlike sCJD, vCJD is acquired through the consumption of beef or beef products contaminated with BSE, also known as mad cow disease—a fatal prion disorder in cattle (Prusiner, 1997). While prion diseases primarily affect the brain, the lymphoreticular system playing a crucial role in transmission of BSE to humans (Hilton et al., 2004b; Aguzzi et al., 2013). Shortly after exposure, lymphotropic prion strains, including BSE, colonize secondary lymphoid organs (SLOs), where they exploit follicular dendritic cells to replicate and accumulate before spreading to the central nervous system (CNS; Hilton et al., 1998; Brown et al., 1999; McCulloch et al., 2011; Mabbott, 2012; Aguzzi et al., 2013). Similar to BSE, chronic wasting disease (CWD), a prion disease affecting cervids, also exhibits strong lymphotropism (Sigurdson et al., 1999).
CWD, which affects deer, elk, and moose, has been rapidly expanding across Canada and the U.S. As of early 2025, CWD has been detected in 36 U.S. states (USGS, 2025). The disease is highly contagious and primarily transmitted horizontally among cervids. CWD prions are shed in bodily fluids such as urine, saliva, and feces, contributing to persistent environmental contamination, particularly in soil (Tamgüney et al., 2009; Bartelt-Hunt and Bartz, 2013; Henderson et al., 2015, 2017; Davenport et al., 2018; Denkers et al., 2020; Tennant et al., 2020; Hwang et al., 2021; Denkers et al., 2024; Kuznetsova et al., 2024). Prions can be taken up by plants from contaminated soil and accumulate at levels sufficient for transmission to animals (Pritzkow et al., 2015; Carlson et al., 2023). Additionally, ticks have been shown to carry lethal doses of CWD infectivity (Inzalaco et al., 2023). While a substantial portion of the U.S. and Canadian populations is exposed to CWD through environmental contamination, the risk of transmission to humans is considered very low due to a significant species barrier.
The species barrier of CWD transmission to humans has been extensively studied using mouse models expressing human prion protein (PrPC; Kong et al., 2005; Sandberg et al., 2010; Wilson et al., 2012; Race et al., 2019; Hannaoui et al., 2022; Race et al., 2022; Wadsworth et al., 2022). In nearly all studies, humanized mice inoculated with CWD prions showed no clinical or subclinical disease and no detectable prion infectivity, with one notable exception (Hannaoui et al., 2022). In that study, infected humanized mice exhibited atypical clinical signs, prion seeding activity, and transmissible prion infectivity (Hannaoui et al., 2022).
In all previous studies assessing this risk, the intracranial (ic) route was used to administer CWD prions to humanized mice (Kong et al., 2005; Sandberg et al., 2010; Wilson et al., 2012; Race et al., 2019; Hannaoui et al., 2022; Race et al., 2022; Wadsworth et al., 2022). Ic inoculation is the most effective method for transmitting prions both within and across species (Race et al., 2009). However, successful cross-species transmission of lymphotropic prion strains appears to depend on SLOs for adaptation to a new host. Among the aforementioned studies, only one examined PrPSc accumulation in the spleen following CWD transmission to humanized mice (Wilson et al., 2012). When prions, such as BSE and CWD, cross species barriers, SLOs consistently exhibit greater permissiveness to prion replication than the brain (Béringue et al., 2012). Moreover, even after ic inoculation, lymphoreticular tissues exhibit a higher capacity than the brain to sustain and replicate lymphotropic prion strains, particularly at low-dose exposure (Halliez et al., 2014). This may be attributed to differences in glycosylation and sialylation between prions residing in the lymphoreticular system and those in the brain (Srivastava et al., 2015; Wagner et al., 2022). In spleens and lymph nodes, prions exhibit increased sialylation, potentially enhancing their resistance to clearance by innate immune system (Srivastava et al., 2015, 2017). Consequently, SLOs may provide a more favorable environment than the brain for prion adaptation to a new species. Furthermore, prion isolates, including CWD, have been shown to produce divergent disease phenotypes when introduced via ic versus peripheral routes, suggesting that brain and lymphoreticular tissues preferentially support different variants of PrPSc present in natural prion isolates (Béringue et al., 2012; DeFranco et al., 2024). Whether peripheral exposure facilitates more efficient cross-species adaptation of CWD prions remains unclear. Nonetheless, assessing PrPSc accumulation in SLOs following ic inoculation of humanized mice could provide a more sensitive approach for evaluating the potential risk of CWD transmission across species.
Additionally, with one exception (Wilson et al., 2012), all previous studies assessing the zoonotic potential of CWD have employed humanized mice homozygous for either 129MM or 129VV PrP (Kong et al., 2005; Sandberg et al., 2010; Race et al., 2019; Hannaoui et al., 2022; Race et al., 2022; Wadsworth et al., 2022). In the study that employed heterozygous 129MV humanized mice, the risk of transmission was assessed using only one CWD isolate (Wilson et al., 2012). In 129MV hosts, PrPSc structures must accommodate both 129M and 129V PrP molecules, likely resulting in an alternating incorporation of these isoforms. The presence of both 129M and 129V PrPC substrates is expected to boost the conformational diversity of PrPSc variants. Whether the structure of 129MV PrPSc is more compatible with CWD strains than that of 129MM or 129VV PrPSc, and whether the 129MV genotype is more susceptible to CWD prions, remains to be investigated.
With the continuous geographical expansion of CWD into highly populated areas and its increasing prevalence, human exposure—including that of children—to high doses of CWD prions via the environment may become unavoidable. The decomposition of carcasses from free-ranging deer infected with CWD could create environmental hotspots containing high concentrations of prions, posing long-term risks to ecosystems.
The uptake of prions by plants raises the possibility of contamination in the food chain, including dairy products. Evidence from prion research suggests that prions can be present in the mammary glands and milk of sheep incubating scrapie, the prion disease of sheep (Ligios et al., 2005; Lacroux et al., 2008; Maddison et al., 2009). If CWD prions can be adsorbed by the digestive system of cattle without causing clinical disease, they may still be excreted into milk, thereby introducing an unrecognized route of human exposure. The potential for milk contamination in dairy cattle that are not infected but are persistently exposed to CWD prions in contaminated environments warrants investigation.
A number of species, including goats, sheep, swine, rodents, mink, ferrets, raccoons, and possibly wild pigs and cattle are susceptible to CWD (Hamir et al., 2005, 2006; Raymond Gregory et al., 2007; Sigurdson et al., 2008; Heisey et al., 2010; Greenlee et al., 2012; Kurt and Sigurdson, 2016; Moore et al., 2017, 2019, 2022; Soto et al., 2025). Prions are subject to evolution and adaptation (Li et al., 2010; Baskakov, 2014). Upon transmission to new hosts, prion replication in a novel molecular environment enhances the conformational diversity of PrPSc variants, accelerating evolution and generating new strains with altered transmission characteristics (Gonzalez-Montalban et al., 2013; Makarava and Baskakov, 2013; Katorcha et al., 2018). As such, interspecies passage of CWD through different hosts may serve as a breeding ground for novel prion strains to emerge.
To date, more than 10 distinct CWD strains have been identified in deer, elk, moose, and reindeer (Otero et al., 2023; Sun et al., 2023). Assessing the transmissibility of diverse CWD strains is crucial for evaluating the potential risk of transmission to humans. Due to prion protein gene polymorphisms, cross-species transmission of CWD strains among different cervid species can alter strain properties, potentially leading to the emergence of novel variants with modified transmission characteristics (Bian et al., 2019, 2021; Otero et al., 2023). Such adaptations could expand the range of hosts susceptible to CWD.
Given the scale of potential CWD exposure, rare instances of transmission to humans might be expected beyond those directly linked to hunting. Since the clinical presentation of CWD in humans has not been defined, it is challenging to determine whether individuals diagnosed with sporadic sCJD at young ages, such as 15 and 21 years old, may have been infected with CWD. Autopsy of SLOs, including the spleen, lymph nodes, and tonsils, could help differentiate between sporadic and acquired forms of CJD. In sCJD patients, prions are detected in SLOs at a low prevalence (Glatzel et al., 2003), whereas in vCJD cases, linked to consumption of BSE-contaminated products, prions have been found in lymphoreticular tissues at a 100% rate (Hill et al., 1999; Ironside et al., 2002). Similarly, if CWD were transmissible to humans, it is expected that prions would accumulate in SLOs. Therefore, histopathological examination and biochemical analysis of PrPSc in SLOs should be conducted for all young individuals succumbed to CJD, as well as older individuals presenting with atypical clinical or neuropathological features. Additionally, transmission studies in animal models could provide further insights into distinguishing between sCJD and zoonotic forms of CJD.
Lymphotropic prion strains, such as BSE, acquired through cross-species transmission, can persist stably and silently in SLOs for extended periods without neuroinvasion (Peden et al., 2010; Bishop et al., 2013). In fact, SLOs serve as silent reservoirs of prion infection, where prions may remain undetected while posing a potential risk of transmission (Hilton et al., 2004a; Peden et al., 2004; Wroe et al., 2006; Peden et al., 2010; Bishop et al., 2013; Gill et al., 2013). Screening human lymphoreticular tissues in regions with a long history of CWD could provide valuable insights into whether CWD prions are silently harbored within the human population.
In conclusion, the expanding scale of human exposure to the growing CWD epidemic necessitates urgent discussions on safeguarding public health. The implementation of lymphoid tissue autopsies could aid in differentiating between sCJD from CJD acquired via transmission. Furthermore, improved risk assessment for CWD transmission to humans could be achieved by analyzing PrPSc accumulation in both the spleen and brain following ic inoculation of humanized mice along with the use of humanized mouse models with the 129MV genotype.
Author contributions IB: Writing – original draft, Writing – review & editing.
Funding The author(s) declare that financial support was received for the research and/or publication of this article. Financial support for this study was provided by National Institute of Health Grants R01 NS045585 and R01 NS129502 to IB.
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The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.
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References…snip…end
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2025.1571662/full
Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease
Jennifer Zitser, Sven Forner, Katherine Wong, Jin Chengshi, John Neuhaus, Jennifer Martindale, Ben J Raudabaugh, Kendra Benisano, Kelly Goodman-O’Leary, Stacy Metcalf
Brain, awaf077, https://doi.org/10.1093/brain/awaf077 Published: 28 March 2025
Although no neuropsychiatric symptom is pathognomonic for sCJD, certain symptoms might help differentiate sCJD from some other neurodegenerative diseases. Our findings support the inclusion of behavioral symptoms in sCJD diagnostic criteria.
https://academic.oup.com/brain/advance-article-abstract/doi/10.1093/brain/awaf077/8098176?login=false
See further ;
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/neuropsychiatric-symptoms-in-sporadic.html
SUNDAY, MARCH 23, 2025
Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html
https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html
RESTRICTED – POLICY CJD IN ADOLESCENTS (16 year old Vickey Rimmer), FARMERS WITH BSE HERDS, AND FARMERS WIFE with Sporadic CJD
June 2015
https://creutzfeldt-jakob-disease.blogspot.com/2015/06/restricted-policy-cjd-in-adolescents-16.html
https://www.researchgate.net/publication/279942086_RESTRICTED_-_POLICY_CJD_IN_ADOLESCENTS_16_year_old_Vickey_Rimmer_FARMERS_WITH_BSE_HERDS_AND_FARMERS_WIFE_with_Sporadic_CJD
TUESDAY, APRIL 15, 2025
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024).
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2025.1571662/full
https://creutzfeldt-jakob-disease.blogspot.com/2025/04/cases-of-creutzfeldt-jakob-disease-in.html
CASE REPORT article
Front. Cell. Neurosci., 02 January 2025
Sec. Cellular Neuropathology
Volume 18 - 2024 | https://doi.org/10.3389/fncel.2024.1518542
Case report: Atypical young case of MV1 Creutzfeldt-Jakob disease with unusually long survival
Lucie Yeongran Ahn.jpegLucie Yeongran Ahn1Mark L. Cohen,.jpegMark L. Cohen2,3Ignazio Cali,.jpegIgnazio Cali2,3Tia Russell.jpegTia Russell3Jessica Ludwig.jpegJessica Ludwig3Xun Jia.jpegXun Jia3Alberto Bizzi.jpegAlberto Bizzi4Lawrence B. Schonberger.jpegLawrence B. Schonberger5Ryan A. Maddox.jpegRyan A. Maddox5Rohini Paul.jpegRohini Paul6Tania C. Ghazarian.jpegTania C. Ghazarian7Jaspreet Garcha.jpegJaspreet Garcha8Mostafa Hammoudi.jpegMostafa Hammoudi9Brian Stephen Appleby,,, .jpegBrian Stephen Appleby2,3,10,11*
1Medical Scientist Training Program, Case Western Reserve University, Cleveland, OH, United States
2Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, United States
3National Prion Disease Pathology Surveillance Center, Case Western Reserve University, School of Medicine, Cleveland, OH, United States
4Neuroradiology Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Neurologico Carlo Besta, Milan, Italy
5Centers for Disease Control and Prevention (CDC), U.S. Department of Health and Human Services (USDHHS), Atlanta, GA, United States
6Department of Psychiatry, Kaiser Permanente San Jose Medical Center, Graduate Medical Education, San Jose, CA, United States
7Department of Psychiatry, Loma Linda University School of Medicine, Graduate Medical Education, Loma Linda, CA, United States
8Department of Internal Medicine, Community Memorial Health Systems, Ventura, CA, United States 9Department of Neurology, Community Memorial Health Systems, Ventura, CA, United States 10Department of Neurology, Case Western Reserve University, School of Medicine, Cleveland, OH, United States 11Department of Psychiatry, Case Western Reserve University, School of Medicine, Cleveland, OH, United States
Creutzfeldt-Jakob disease (CJD) is a rare, fatal, rapidly progressive neurodegenerative disease resulting from an accumulation of misfolded prion proteins (PrP). CJD affects 1–2 new individuals per million each year, and the sporadic type accounts for 90% of those cases. Though the median age at onset and disease duration vary depending on the subtype of sporadic CJD (sCJD), the disease typically affects middle-aged to elderly individuals with a median survival of 4–6 months. sCJD in younger individuals is extremely rare. Here, we present a 21-year-old female who died with a sporadic prion disease. She presented with psychiatric symptoms followed by a rapidly progressive neurocognitive and motor decline. EEG was negative for periodic sharp wave complexes; however, brain MRI was suggestive of prion disease. The cerebrospinal fluid (CSF) real-time quaking-induced conversion (RT-QuIC) assay was indeterminate. Neuropathologic examination at autopsy revealed severe neuronal loss and gliosis with secondary white matter degeneration but minimal spongiform changes and PrP deposits in the cerebellum and neocortex by immunohistochemistry. Absence of pathogenic mutations and methionine/valine heterozygosity at codon 129 of the prion protein gene (PRNP), atypical type 1 protease-resistant PrP that lacks or shows underrepresentation of the diglycosylated PrP isoform by western blot analysis, and no acquired prion disease risk factors resulted in a final diagnosis of atypical sCJD. Very young onset sCJD often has atypical clinical presentations and disease progression, neuropathological examination results, and/or laboratory test results that may confound diagnosis. It is critical to perform thorough, comprehensive evaluations to make an accurate diagnosis, which includes autopsy confirmation with histology, prion protein typing and prion gene sequencing.
Snip…
Conclusion
In summary, very young sCJD patients with long disease duration may present quite differently than typical sCJD patients. To our knowledge, our case is the second reported case of a very young onset sCJD MV1 subtype with an unusually long disease duration and PrP type. Our case's MRI findings are consistent with what we would expect from an MV1 subtype patient. However, the clinical presentation, disease course, the absence of PSWCs in EEG, and indeterminate RT-QuIC results is not characteristic of sCJD MV1. Ultimately, the neuropathological exam analyses performed at the NPDPSC combined with no known risk factors for acquired prion disease provided confidence that this young patient had sporadic prion disease. An extremely rare, very young onset sCJD with a long disease duration case may present differently from the characteristic clinical presentations of sCJD. Thus, it is critical to comprehensively evaluate clinical findings, but most importantly to verify the presence and type of prion disease in these younger cases though neuropathologic evaluation at autopsy, especially since acquired prion diseases historically occur in young individuals (Rhoads et al., 2020; Manix et al., 2015). Neuropathologic evaluation of young cases in the UK was crucial for identifying variant CJD in those exposed to bovine spongiform encephalopathy (Will et al., 1996). The U.S. is currently dealing with a massive spread of an animal prion disease in the cervid species called chronic wasting disease. As such, it is more important than ever to confirm the presence and type of prion disease is the US, especially in atypical cases of prion disease (Osterholm et al., 2019).
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2024.1518542/full
Research Letter
December 11, 2023
Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020
Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et al John A. Romley, PhD4; John C. Probasco, MD5
Author Affiliations Article Information JAMA Neurol. 2024;81(2):195-197. doi:10.1001/jamaneurol.2023.4678
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
Snip…
Discussion Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing. This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.
https://jamanetwork.com/journals/jamaneurology/fullarticle/2812784
Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023
The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis
Angéline Denouel1 · Jean-Philippe Brandel1,2 · Danielle Seilhean1 · Jean-Louis Laplanche3,4 · Alexis Elbaz5 · Stéphane Haik1,2
Abstract
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.
We included all cases aged 45–89 years old who died with a probable/definite sCJD diagnosis based on the French national surveillance network. We used age-period-cohort (APC) Poisson regression models to study variation in mortality rates by sex, age, period, and time.
A total of 2475 sCJD cases aged 45–89 years were included. Mortality rates increased with age, reached a peak between 75 and 79 years, and decreased thereafter. Mortality rates were higher in women than men at younger ages and lower at older ages. The full APC model with a sex×age interaction provided the best fit to the data, thus in favour of sex, age, period, and cohort effects on mortality rates. In particular, mortality rates increased progressively with successive birth cohorts.
Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.
Keywords Age-period-cohort model · Prion · Temporal trend · Sporadic Creutzfeldt-Jakob disease
snip...
Worldwide, the number of patients with sCJD appears to have progressively increased over time [13]. This increase can be partly explained by increasing life expectancy as well as by better case ascertainment due to improved diagnostic tests and awareness of the disease among clinicians. Indeed, a relationship between surveillance intensity and sCJD incidence has been shown [14]. It cannot be excluded, however, that an actual increase of sCJD cases has occurred, and this hypothesis can be examined using age-period-cohort (APC) models.
snip...
In this paper, we estimated mortality rates from sCJD in France over a 25-year period (1992–2016) based on data from the French national surveillance network.
snip...
The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1).
snip...
Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.
https://link.springer.com/article/10.1007/s10654-023-01004-5
3 cases of the rare CJD in Hood River County, over the last 8 months
PUBLIC SERVICE ANNOUNCEMENTS
For Immediate Release
Date: April 14, 2025
To: All Media
From: Hood River County Health Department
Subject: Update on Creutzfeldt-Jakob Disease (CJD) Investigation
The health department recently reported three cases of the rare brain disorder Creutzfeldt-Jakob Disease (CJD) in Hood River County. Over the last 8 months, there have been 1 confirmed and 2 probable cases. Of these three cases, two people are deceased.
Per state law, the health department investigates and monitors diseases that could be a risk to the public. At this time, there is no identifiable link between these three cases.
The risk of getting CJD is extremely low. It does not spread through the air, touch, social contact, or water. People with questions about their healthcare are encouraged to contact their primary medical team.
This is an active and ongoing investigation in partnership with Oregon Health Authority (OHA) and Centers for Disease Control and Prevention (CDC). The health department will continue to monitor the situation and keep you informed of any risk to public health.
For more information on CJD, please visit CJD Foundation or the CDC’s website.
###
https://www.hoodrivercounty.gov/index.asp?SEC=AF40862A-8B0E-4107-B2B4-956029C71941&DE=11E4744C-DDC6-4DBD-8F95-B86EFBB310E6
Hood River County, Oregon has a population of 23,977 according to the Census Bureau.
https://www.google.com/search?q=hood+river+county+population%3F&rlz=1C9BKJA_enUS1037US1037&oq=hood+river+county+population%3F&gs_lcrp=EgZjaHJvbWUyBggAEEUYOdIBCTE3MDIyajBqN6gCD7ACAeIDBBgBIF8&hl=en-US&sourceid=chrome-mobile&ie=UTF-8
Oregon Prion TSE CJD ???
https://public.tableau.com/app/profile/oregon.public.health.division.acute.and.communicable.disease.pre/viz/WeeklyCommunicableDiseaseReport/ACDPWeeklyReport
https://www.oregon.gov/oha/ph/diseasesconditions/communicabledisease/diseasesurveillancedata/weekly-monthlystatistics/pages/index.aspx
Annual CJD
https://www.oregon.gov/oha/PH/DISEASESCONDITIONS/COMMUNICABLEDISEASE/DISEASESURVEILLANCEDATA/ANNUALREPORTS/Pages/arpt.aspx
https://www.oregon.gov/oha/ph/diseasesconditions/diseasesaz/pages/prion-diseases.aspx
https://www.oregon.gov/oha/PH/DISEASESCONDITIONS/COMMUNICABLEDISEASE/REPORTINGCOMMUNICABLEDISEASE/REPORTINGGUIDELINES/Documents/cjd-prion.pdf
What’s going on with CJD in Maricopa, Arizona? Concerning…
Arizona CJD
Arizona CJD CONFIRMED & PROBABLE CASES 2024* (selected morbidities)
5 cases Maricopa, 1 case Pima
https://www.azdhs.gov/documents/preparedness/epidemiology-disease-control/disease-data-statistics-reports/data-statistics-archive/2024/weekly.pdf
Maricopa is a city in the Gila River Valley in Pinal County, Arizona, United States. With 66,290 residents as of 2022, Maricopa is the largest incorporated municipality in Pinal County.[4]
https://en.m.wikipedia.org/wiki/Maricopa,_Arizona
Illinois CJD Reportable? No, apparently not anymore.
That’s crazy!
***> Creutzfeldt Jakob Disease CJD TSE Prion Cases Increasing March 2025
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html
***> Creutzfeldt Jakob Disease CJD, BSE, CWD, TSE, Prion, December 14, 2024 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html
Transmission of vPSPr in macaque: an open gate to prion-like diseases ?
Emmanuel E Comoya, Jacqueline Mikola, Jérôme Delmottea, Wenquan Zoub,c, Jean-Philippe Deslysa
aInstitut François Jacob, Prion Research group, CEA, Fontenay-aux-Roses, France; bInstitute of Neurology, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi Province, China; cDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
Aims: A novel form of prion disease initially described in 2008 harbours very original features: reported in less than 50 cases worldwide to date, this prionopathy is characterized by the accumulation of abnormal prion protein with variable protease sensitivity depending on the genotype at PRNP codon 129, and subsequently called vPSPr. Their limited resistance, enforced by an incomplete transmissibility in rodent models, question the infectivity of these borderline prion strains. Moreover, their clinical expressions are often evocative of other neurodegenerative diseases (whose transmissibilities are also currently challenged) like Alzheimer’s disease, fronto-temporal dementia or even amyotrophic lateral sclerosis, and concomitant deposition of other prion-like proteins is frequently reported. We aimed to assess the transmissibility of vPSPr cases in cynomolgus macaque, a model reputed as highly relevant of the human situation according to its close phylogeny.
Materials and Methods: Cynomolgus macaques were intracerebrally inoculated with brain samples issued from 1 M/M and 1 M/V vPSPr case respectively, and kept under surveillance throughout their incubation periods. At the onset of clinical signs, extensive histological, immunohistochemical and biochemical analyses were performed according to the techniques previously used on primates in our previous studies.
Results: Whereas the macaque exposed to M/M vPSPr inoculum remains healthy 14 years post-exposure, the macaque exposed to M/V vPSPr inoculum developed unusual neurological and behavioural disturbances after 8.5 years of silent incubation. The expected hallmarks of vPSPr were observed in this animal, including spongiform change and abnormal PrP depositions under different forms. Unexpectedly, we also observed massive Aβ deposits in this mid-aged (13 years) macaque, a unique situation within our cohort of more than 50 over-ten years-old macaques, in which Aβ deposits may occur after 20 years of age but with different features, including co-staining with PrP, than in this animal.
Conclusions: The physiopathological mechanisms underlying the presence of Aβ deposits in both the brain of this animal recipient and its human donor remains to be elucidated. It may be either evocative of a concomitant presence of an age-related, but de facto transmissible, Aβ pathology with vPSPr in the donor patient, or a specific feature of vPSPr. At a time where the transmissibility of prion-like diseases is more than ever under question, this latter hypothesis would place these uncommon prionopathies at the intersection between prion and Alzheimer’s disease, and maybe other neurodegenerative diseases as ALS and synucleinopathies according to the concomitant prion-like depositions described in some vPSPr patients.
Funding: This study has been performed on internal funding of the laboratory.
=====
55. Experimental Transmission of Protease Sensitive Prionopathy (VPSPr) to Nonhuman Primates
Jacqueline Mikola, Wenquan Zouc,b, Jérôme Delmottea, Jean-Philippe Deslysa, Emmanuel Comoya
aInstitut François Jacob, Prion Research group, CEA, Fontenay-aux-Roses, France; bInstitute of Neurology, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, Jiangxi Province, China; cDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
Aim: Transmission is one of the main characteristics of prion diseases. However, VPSPr, which is now presumed to be the sporadic form of the genetic CJD V180I, is incompletely, if any, transmissible in humanized mice, and transmission in bank voles ‘does not replicate the complex VPSPr-PrPd profile’ (Nonno). We strenghtened the panel of experimental transmission of this uncommon prion strain with cynomolgus macaque, considered a relevant model of human prion diseases.
Material and methods: Transmission was performed in two cynomolgus macaques (PrP MM genotype at codon 129). The animal exposed to the brain of a 75 years-old MV patient developed clinical symptoms after 8.5 years of silent incubation. Euthanasia was performed 4 months later for humane reasons. The other animal is still asymptomatic 14 years after exposure to the brain of a MM patient (68 years).
Subsequently, secondary transmission to transgenic mice overexpressing macaque PrP were conducted.
Histological and biochemical studies were performed as previously published.
Results: The known clinical data of the MV patient were limited to executive dysfunction and unsteady gait.
The MV VPSPr-exposed macaque presented several episodes of self-aggression, first focused on his left leg, which extended to the whole hindquarters. Abnormalities of both hind limbs sensitive conduction were recorded.
Among our different analyses, neuropathological examination showed a spongiform change diffuse in the grey matter with a special profile, associated to a reactive astrocytosis and massive neuronal vacuolation, particularly in the rarefied Purkinje cells. Abnormal PrPd consisted of a diffuse background of thin synaptic deposits mixed with mini aggregates (dots) and some small cortical fussy fibrillar plaques, not preserved after proteolysis, and never observed in primates exposed to other prion strains. At the opposite there was no granules or micro-plaques in the cerebellum although they were noted in the donor patient.
Three other facts must be emphasized: i/ the presence of massive amyloid deposits in the neocortex but not in the hippocampus, except the subiculum more or less associated to PrPd. ii/ the negativity of the staining with AT8, AT100, cytoplasmic TDP43. iii/ the inflammatory reaction observed in the lumbar dorsal root ganglia which may be correlated with the peripheral itching of the legs.
Conclusion: Conversely to classical prion transmission in primate, neuropathology exemplified here a tiny specific pattern of PrPd deposits reminiscent of VPSPr in human. Our observations in primate and secondary in mice sustain the transmissibility of (at least MV) vPSPr-related prion strain which extends the field of classical prion diseases.
Funding: This study has been performed on internal funding of the laboratory.
Grant number
Acknowledgement
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
Brain Pathol. 2023 Sep; 33(Suppl 1): e13194.
Published online 2023 Sep 6.
https://doi.org/10.1111/bpa.13194
PMCID: PMC10483180
PMID: 37674376
Special Issue: Abstracts of the 20th International Congress of Neuropathology, Berlin, Germany, September 13–16, 2023
snip...
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
J. Mikol 1, J. Delmotte1, W. Zou2,3, J. P. Deslys1, E. Comoy1 1Commissariat à l'energie Atomique, Institut François Jacob/Prion research Group, Fontenay‐aux‐Roses, France; 2Case Western Reserve University School of Medicine, Department of Pathology, Cleveland, OH, United States; 3Institute of Neurology, Nanchang, China
VPSPr, a novel rare human prion disease described in 2010 is characterized by the accumulation of abnormal PrPd with a limited resistance to proteolysis. It showed uncertain infectivity in transgenic mouse models. Recent publications report VPSPr cases with different unevocative clinical pictures, leading the authors to consider that VPSPr might be underestimated. One of our macaques intracerebrally inoculated with a brain sample derived from a VPSPr patient (Met/Val at codon 129) exhibited after 8.5 years of silent incubation several episodes of self‐aggression first focused on his left leg, which extended to the whole hindquarters, and abnormalities of both hind limbs sensitive conduction. We performed euthanasia 4 months later for humane reasons and examined brain tissues with histological techniques. Pathological examination revealed a spongiform change decreasing from the frontal to the occipital cortex, also present in the thalamus, the basal ganglia, the hippocampus, mild brainstem and spinal cord, accompanied with a massive neuronal vacuolation and reactive astrocytosis. Purkinje cells were rarefied and vacuolated. Abnormal PrPd deposition was present as synaptic deposits mixed with some mini‐aggregates of PrPd diffused in the tissue or packed in small fussy unlimited area of variable intensity, appearing as pre‐plaques. These lesions, never previously observed in primates exposed to other prion strains, were mainly observed in the insula and the frontal cortex but were absent in the thalamus, the basal ganglia and the hippocampus. Furthermore, we observed massive Ab deposits, but not in the hippocampus except the subiculum, not co‐localized with PrPd. In contrast, a two‐times older animal had three‐times less deposits. The original disease phenotype observed in this primate will be discussed and compared to the expressions of VPSPr in humans, and other prion diseases in the macaque model. PS9‐ND‐A186
“Recent publications report VPSPr cases with different unevocative clinical pictures, leading the authors to consider that VPSPr might be underestimated. “
“One of our macaques intracerebrally inoculated with a brain sample derived from a VPSPr patient (Met/Val at codon 129) exhibited after 8.5 years of silent incubation several episodes of self‐aggression first focused on his left leg, which extended to the whole hindquarters, and abnormalities of both hind limbs sensitive conduction.”
Yup, as predicted…that’s why ;
CANADA
Definite and probable CJD, 1998-2023
As of November 30, 2023
Year Sporadic Iatrogenic Genetic vCJD Total
SNIP...
2023, STILL NO MENTION OF VPSPr TSE Prion in Canada statistics...terry
PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;
WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?
THIS problem must be addressed immediately imo.
WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;
QUOTE;
''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''
''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''
end
Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry
TUESDAY, DECEMBER 12, 2023
***> CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023 <***
***> 2023 Professor John Collinge on tackling prion diseases <***
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
There is accumulating evidence also for iatrogenic AD.
Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.
Saturday, January 20, 2024
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
This report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob disease (CJD).
https://www.gov.uk/government/publications/creutzfeldt-jakob-disease-cjd-surveillance-update-2024/creutzfeldt-jakob-disease-cjd-surveillance-update-2024
Iatrogenic Transmissible Spongiform Encephalopathy TSE Prion, CWD, our worst nightmare, what if?
https://itseprion.blogspot.com/
MONDAY, SEPTEMBER 11, 2023
Professor John Collinge on tackling prion diseases
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
Singeltary 2001
Subject: CJD or Alzheimer's or the same ???
Date: Sun, 29 Apr 2001 12:45:28 -0700
From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de
Bovine Spongiform Encephalopathy
Greetings List,
thought some might be interested in this. I have always wondered if CJD and or all TSEs and Alzheimer's could be linked. i have been of the opinion that Alzheimer's is a TSE for a long time, just at the low end of the titre of infectivity scale. i also believe in the accumulation theory. by dose, you could be killed by one sitting, or one injection, or one whatever, depending on the titre of infectivity of that whatever. on the other hand, if the dose is not a lethal dose, over a period of time, the accumulation will become lethal (if consumption continued), and i believe the route/source/titre of infectivity, will be a key roll to the incubation period, and symptoms.
just my opinion...terry
Terry S. Singeltary Sr
