Transmissible Spongiform Encephalopathy

Looming Crisis U. S. Transmissible Spongiform Encephalopathy TSE Prion, in Cattle, Cervid, Sheep, Pigs, and Humans

i must warn you of what TSE Prion Poker Game we find ourselves in, and the looming crisis we face, with regards to Transmissible Spongiform Encephalopathy TSE Prion, in Cattle, Cervid, Sheep, Pigs, and Humans, here in the U.S., not just from the meat, but also from the very environments the meat was grazed on. No Country should import or export TSE Prion. The OIE and USDA et al, made TSE Prion disease a legal trading commodity, when they went from BSE GBR Risk Assessments, and went to BSE MRR, in my honest opinion. i don’t make this up, it’s just science. i am not anti-meat, hunt, gun, i just don’t want to eat TSE Prion disease, they can kill you…

kindest regards, terry

TSE Prion Update USA, 2026

***> cwd, tse, prion, cattle, pigs, sheep, primates, oh my!

Oh snap, now the bacon, CWD, TSE, PrP, the Singeltary Files, Confidential, In Confidence

Confidential, In Confidence, the Singeltary files

IN CONFIDENCE

So it is plausible pigs could be preclinically affected with BSE but since so few are allowed to reach adulthood this has not been recognised through clinical disease. ...

https://web.archive.org/web/20040904150118/www.bseinquiry.gov.uk/files/yb/1990/08/23002001.pdf

CONFIDENTIAL

EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY

While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...

http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf

we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.

http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf

May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...

http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf

3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...

http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf

But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...

http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf

Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....

http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf

***> Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026 Department for Environment, Food and Rural Affairs

Preliminary Outbreak Assessment

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

26 January 2026

Disease report

Chronic wasting disease (CWD) is a fatal neurodegenerative disease of cervids, such as deer, elk, moose and reindeer. It is caused by prions – infectious proteins that cause normal cellular prion proteins to misfold (CIDRAP, 2025). The disease is widespread in captive and free-ranging cervids in North America (Figure 1). For the first time, CWD prions have also been detected in the tissues of wild pigs (Sus scrofa) caught in CWD-affected areas of the USA (Soto et al. 2025). This discovery emerged from a study designed to investigate potential interactions between wild pigs and CWD prions, as wild pigs often coexist with cervids, which can shed prions into the environment. The following assessment discusses the epidemiology of CWD in North America and the detection of CWD prions in wild pigs. It also considers the potential implications for Great Britain.

snip…

Against this background, Soto et al. (2025) investigated potential interactions between wild pigs and CWD prions. They analysed over 300 brain and lymph node samples from 178 wild pigs living across Arkansas and Texas, USA. The animals were captured by the United States Department of Agriculture (USDA) between 2020 and 2021. None of the pigs included in the study were reported to be displaying clinical signs of disease.

Using an ultra-sensitive laboratory method (protein misfolding cyclic amplification (PMCA)), the researchers identified CWD prions in up to 37% of the lymph node samples and 15% of brain samples. The lowest detection rates were in the Texas samples (below 16%), matching the lower CWD prevalence in the state’s cervid population. These findings indicate that wild pigs are naturally exposed to CWD prions in areas where the disease is present (Soto et al., 2025).

snip…

Conclusion

CWD has continued to spread among captive and free-ranging cervids in North America since it was first detected in the 1960s. The finding of CWD prions in wild pigs in the USA suggests they could contribute towards transmission of the disease, influencing its epidemiology, geographic distribution and interspecies spread. However, further research is needed to confirm this. CWD has never been reported in Great Britain and the current risk of CWD prions being introduced into Great Britain’s wild pig or cervid population ranges from very low to negligible.

Readers are reminded to be vigilant for signs of CWD. Information on how to spot the disease can be found here. Suspected cases must be reported immediately to the Defra Rural Services Helpline on 03000 200 301. In Wales, call 0300 303 8268. In Scotland, contact your local Field Services Office. Failure to do so is an offence. We will continue to monitor the situation.

Authors • Lawrence Finn • Dr. Lauren Perrin • John Spiropoulos • Dr. Helen RobertsDepartment for Environment, Food and Rural Affairs

References

snip…see;

https://assets.publishing.service.gov.uk/media/697a3b013c71d838df6bd413/CWD_Prions_in_Cervids_and_Wild_Pigs_in_North_America.pdf

***> In a newly published study in Science Advances, researchers from the University of Calgary and collaborators around the world explored the zoonotic potential of CWD in controlled laboratory settings. While most animals remained asymptomatic, researchers detected low levels of prions in their tissues and transferred samples to other species that developed symptoms of CWD.

“These findings show that even without obvious (clinical signs), infectious prions can still be present and transmissible,” says Dr. Samia Hannaoui, PhD, researcher and assistant professor at the University of Calgary Faculty of Veterinary Medicine (UCVM), and first author on the study.

https://ucalgary.ca/news/new-study-explores-potential-cross-species-spread-chronic-wasting-disease

https://www.science.org/doi/10.1126/sciadv.aeb7613

***> cwd, tse, prion, cattle, pigs, sheep, primates, oh my!

***> Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease

Nathaniel D. Denkers , Clare E. Hoover , Kristen A. Davenport, Davin M. Henderson, Erin E. McNulty, Amy V. Nalls, Candace K. Mathiason, Edward A. Hoover

Published: August 20, 2020

https://doi.org/10.1371/journal.pone.0237410

Abstract

The minimum infectious dose required to induce CWD infection in cervids remains unknown, as does whether peripherally shed prions and/or multiple low dose exposures are important factors in CWD transmission. With the goal of better understand CWD infection in nature, we studied oral exposures of deer to very low doses of CWD prions and also examined whether the frequency of exposure or prion source may influence infection and pathogenesis. We orally inoculated white-tailed deer with either single or multiple divided doses of prions of brain or saliva origin and monitored infection by serial longitudinal tissue biopsies spanning over two years. We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. This was true whether the inoculum was administered as a single bolus or divided as three weekly 100 ng exposures. However, when the 300 ng total dose was apportioned as 10, 30 ng doses delivered over 12 weeks, no infection occurred. While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.

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

Prion Conference 2023

***> 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.”

Strain characterization of chronic wasting disease in bovine-PrP transgenic mice

Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.

Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.

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

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

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

***> cwd to sheep

***> Chronic Wasting Disease CWD vs Scrapie TSE Prion

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

Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.

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

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

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

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

Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies

Location: Virus and Prion Research

***> Title: Differentiation of scrapie from chronic wasting disease in white-tailed deer

Accomplishments

***> 1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.

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

Can Vet J. 2010 Feb;51(2):169–178. Show available content in English French

***> Experimental oral transmission of chronic wasting disease to red deer (Cervus elaphus elaphus): Early detection and late stage distribution of protease-resistant prion protein

Balachandran 1,✉, Noel P Harrington 1, James Algire 1, Andrei Soutyrine 1, Terry R Spraker 1, Martin Jeffrey 1, Lorenzo González 1, Katherine I O’Rourke 1 Author information Copyright and License information PMCID: PMC2808282 PMID: 20436863

Abstract Chronic wasting disease (CWD), an important emerging prion disease of cervids, is readily transmitted by intracerebral or oral inoculation from deer-to-deer and elk-to-elk, suggesting the latter is a natural route of exposure. Studies of host range susceptibility to oral infection, particularly of those species found in habitats where CWD currently exists are imperative. This report describes the experimental transmission of CWD to red deer following oral inoculation with infectious CWD material of elk origin. At 18 to 20 months post-inoculation, mild to moderate neurological signs and weight loss were observed and animals were euthanized and tested using 3 conventional immunological assays. The data indicate that red deer are susceptible to oral challenge and that tissues currently used for CWD diagnosis show strong abnormal prion (PrPCWD) accumulation. Widespread peripheral PrPCWD deposition involves lymphoreticular tissues, endocrine tissues, and cardiac muscle and suggests a potential source of prion infectivity, a means of horizontal transmission and carrier state.

https://pmc.ncbi.nlm.nih.gov/articles/PMC2808282/

***> Experimental Oral Transmission of Chronic Wasting Disease to Reindeer (Rangifer tarandus tarandus)

Gordon B. Mitchell, Christina J. Sigurdson, Katherine I. O’Rourke, James Algire, Noel P. Harrington, Ines Walther, Terry R. Spraker, Aru Balachandran

Published: June 18, 2012

https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0039055

***> Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.

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

***> 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 Tg12mice 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 Tg12mice. 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

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

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

***> 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 tg650mice 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

***> 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 tg650mice 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

***> 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

***> 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://web.archive.org/web/20240531213416/https://www.cdc.gov/chronic-wasting/about/index.html#cdc_disease_basics_prevention-reducing-risk

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.

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

Snip…

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

***> Of note, our data show that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification...

Detection of chronic wasting disease prions in processed meats

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.

"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."

Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.

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

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

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

DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.

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

***> 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...

Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.

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

The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)

Component 6: Transmissible Spongiform Encephalopathies (TSEs)

Problem Statement 6A: Determine pathobiology of prion strains.

The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).

Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa

Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.

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

Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation

Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University

Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.

Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.

Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.

DOI: https://doi.org/10.1080/19336896.2022.2091286

Interpretive Summary:

Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.

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

Highlights

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.

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

Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation

Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin

Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.

A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.

Frontiers in Veterinary Science. 3:78. Interpretive

Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.

Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.

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

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)

https://bse-atypical.blogspot.com/2025/06/the-naturally-occurring-lysine-to.html

*** Grant Agreement number: 222887 ***

*** Project acronym: PRIORITY ***

*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***

Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014

Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es

Project website¡ Error! Marcador no definido. address: www.prionpriority.eu

PRIORITY, PROJECT FINAL REPORT

*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***

snip...

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

see;

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

Scrapie and Atypical Nor-98 Scrapie USA Update

July 31, 2026

Classical Scrapie Confirmed in Oregon Sheep Through Routine Surveillance

Featured Image Post

Routine slaughter testing detected the case. There is no known risk to people, and most Oregon producers are unaffected.

The Oregon Department of Agriculture (ODA), in collaboration with the US Department of Agriculture's (USDA) Animal and Plant Health Inspection Service (APHIS), is announcing the confirmation of classical scrapie in a sheep slaughtered in a facility in Oregon. The animal was tested as part of USDA's ongoing national scrapie surveillance program, which is designed to identify classical scrapie in sheep and goats at slaughter facilities.

Classical scrapie is a fatal, degenerative prion disease of sheep and goats. The disease can be present in apparently healthy animals, making slaughter surveillance critical for early detection. Classical scrapie typically requires several years to produce clinical signs, and affected animals generally live only months after symptom onset.

Upon confirmation, ODA began an epidemiological investigation to identify the flock of origin and determine whether any potentially exposed animals are located within Oregon. This joint response supports the national effort to eradicate classical scrapie from the United States. Before this confirmation, the United States had gone more than five years without a case. The United States was positioned to meet WOAH's 7-year benchmark for self-declared scrapie freedom in January 2028. USDA says the confirmed detection could reset that timeline and may push the earliest possible date for national scrapie-free status to approximately 2033.

APHIS expects minimal impact on trade. Most countries restrict live animal exports only from affected flocks or premises, and products such as wool remain unaffected.

ODA asks Oregon sheep and goat producers to take four steps:

Keep official identification current. Sheep and goats need official ID before they leave the premises where they were born, so animals can be traced if a case is found. Watch for signs. Rubbing or scratching against fences and posts, tremors, an unsteady or high-stepping walk, changes in behavior, and weight loss in an animal that is still eating well. Call your veterinarian right away if you see those signs. Oregon law requires veterinarians to notify ODA by phone as soon as they suspect scrapie, before any lab testing. You can also call ODA's disease reporting line directly at 503-986-4711.

Ask your veterinarian about breeding for scrapie resistance. Some sheep carry genetics that make them highly resistant to classical scrapie, and breeding for those traits protects a flock over time.

For more information about Oregon's scrapie surveillance program, producers may visit ODA's scrapie website at https://oda.direct/scrapie. More information about scrapie and APHIS' eradication program is available at:www.aphis.usda.gov .

Producers or owners who suspect an animal disease should contact their veterinarian to evaluate the animal or herd. Scrapie is a reportable disease in Oregon. Veterinarians and diagnostic laboratories must immediately report suspected cases to ODA's State Veterinarian.

https://oda.direct/scrapie

https://apps.oregon.gov/oregon-newsroom/OR/ODA/Posts/Post/Classical-Scrapie-Confirmed-in-Oregon-Sheep-Through-Routine-Surveillance

USDA Confirms Detection of Classical Scrapie in a Slaughtered Sheep

The U.S. Department of Agriculture’s (USDA) Animal and Plant Health Inspection Service (APHIS) has confirmed classical scrapie in a sheep slaughtered at a facility in Oregon. Final diagnostic results and genotype analysis were completed by the National Veterinary Services Laboratories (NVSL), with confirmatory testing indicating classical scrapie. As required, APHIS has notified the World Organisation for Animal Health (WOAH) of the confirmed detection.

Classical scrapie is a fatal, degenerative prion disease of sheep and goats. Prior to this confirmation, the United States had gone more than 5 years without a case. The United States was positioned to meet WOAH’s 7‑year benchmark for self‑declared scrapie freedom in January 2028. This confirmed detection could reset that timeline and may push the earliest possible date for national scrapie‑free status to approximately 2033.

APHIS and State animal health officials have initiated an epidemiological investigation to determine the flock of origin. The affected animal did not have an ear tag, which may limit traceability and highlights the critical importance of strong animal identification practices nationwide. To support the investigation, NVSL will conduct tissue‑matching and parentage testing on the affected sheep and two additional animals from the same accession.

APHIS expects minimal impact on trade. Most countries restrict live animal exports only from affected flocks or premises, and products such as wool remain unaffected.

USDA encourages producers to reinforce key practices that have advanced scrapie eradication efforts nationwide:

Strict compliance with ear tagging and traceability requirements to ensure exposed animals can be accurately tracked.

Robust surveillance participation, including submission of clinical suspects and routine monitoring.

Use of genetically resistant breeding stock, which significantly reduces disease susceptibility and depopulation needs.

Continued partnership with APHIS and State officials to support investigation and long‑term freedom efforts.

More information about scrapie and APHIS’ eradication program is available at www.aphis.usda.gov.

https://www.aphis.usda.gov/news/program-update/usda-confirms-detection-classical-scrapie-slaughtered-sheep

https://www.aphis.usda.gov/livestock-poultry-disease/sheep-goat/scrapie

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

ARS Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025

snip…

Accomplishments

***> 1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.

2. 02 Showed that gene-targeted mice are capable of reproducing strain specific effects typically limited to natural host species of chronic wasting disease (CWD). CWD is a highly contagious disease of deer, elk, moose, and reindeer found in North America, South Korea, and Scandinavian countries that is caused by misfolded proteins called prions. CWD prions transmit through direct contact between infected animals, or through contaminated soil, grass, or water. All prion diseases exhibit progressive neurodegeneration and ultimately death. Scientists typically study CWD by injecting prions into susceptible animals' brains in lab experiments. Intracranial prion injections are favored because they typically produce shorter incubation periods and higher disease attack rates compared to natural infection. ARS researchers in Ames, Iowa, along with university collaborators showed that this inoculation method can cause the prion strains to change in a way that does not accurately reflect how the disease spreads naturally. They found that using a combination of peripheral inoculation (injection outside the brain) in natural hosts and using novel gene-targeted mice generated in a manner that provides a more natural expression of the inserted prion gene that gives a more accurate picture of how CWD behaves in the real world. The novel mouse model provides an important strategy to precisely assess the zoonotic potential (likelihood of transmission from animals to humans) of CWD and other animal prion diseases using natural routes of transmission. This will impact the tools used and direction of future studies of CWD and other prion diseases allowing more rapid and comprehensive responses to emerging questions aiding both the researchers at the producers they support…end

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

Title: Characterization of classical sheep scrapie in white-tailed deer after experimental oronasal exposure

Author item Greenlee, Justin item MOORE, SARAH - Orise Fellow item Cassmann, Eric item LAMBERT, ZOE - Orise Fellow item Kokemuller, Robyn item Smith, Jodi item Kunkle, Robert item KONG, QINGZHONG - Case Western Reserve University (CWRU) item WEST GREENLEE, HEATHER - Iowa State University

Submitted to: Journal of Infectious Diseases Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/4/2022 Publication Date: 11/8/2022

snip…

Technical Abstract: Scrapie is a prion disease of sheep and goats that is associated with widespread accumulation of abnormal prion protein (PrPSc) in the central nervous and lymphoid tissues. Chronic wasting disease (CWD) is the natural prion disease of cervid species and is similar to scrapie in sheep. The purpose of this study was to determine susceptibility of white-tailed deer (WTD) to the scrapie agent. We inoculated WTD (n=5) by a concurrent oral and intranasal exposure with the scrapie agent from sheep and (n=6) with the scrapie agent from goats. All deer exposed to the agent of scrapie from sheep had evidence of PrPSc accumulation. PrPSc was detected in lymphoid tissues at preclinical time points, and deer necropsied after 28 months post-inoculation had clinical signs, spongiform lesions, and widespread distribution of PrPSc in neural and lymphoid tissues. Western blots done on samples from the brainstem, cerebellum, and lymph nodes of scrapie-infected WTD have a molecular profile similar to CWD and distinct from western blots of samples from the cerebral cortex, retina, or the original sheep scrapie inoculum. WTD are susceptible to the agent of scrapie from sheep and differentiation from CWD may be difficult.

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

Our findings establish that white-tailed deer are susceptible to WTD scrapie and that the presence of WTD scrapie agent in the lymphoreticular system suggests the handling of suspected cases should be consistent with current CWD guidelines because environmental shedding may occur.

snip…

The potential for zoonoses of cervid-derived PrPSc is still not well understood (6,18,45–47); however, interspecies transmission can increase host range and zoonotic potential (48–50). Therefore, to protect herds and the food supply, suspected cases of WTD scrapie should be handled the same as cases of CWD.

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

see;

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

https://transmissiblespongiformencephalopathy.blogspot.com/2026/04/us-report-scrapie-cwd-cattle-sheep-pigs.html

***> USA Report, Scrapie, CWD, BSE, TSE, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026

April 2026

https://fdabse589.blogspot.com/2026/04/usa-report-scrapie-cwd-bse-tse-cattle.html

https://www.researchgate.net/publication/403956772_USA_Report_Scrapie_CWD_BSE_TSE_Cattle_Sheep_Pigs_Cervid_Humans_Zoonotic_2026

Tuesday, June 9, 2026

Hazard identification for the risks to terrestrial animal health from the import of hay and straw, BSE, CWD, Scrapie, TSE, Prion

https://www.gov.uk/government/publications/hazard-identification-for-the-risks-to-terrestrial-animal-health-from-the-import-of-hay-and-straw/hazard-identification-for-the-risks-to-terrestrial-animal-health-from-the-import-of-hay-and-straw

https://defrauktseprp.blogspot.com/2026/06/hazard-identification-for-risks-to.html

Cervid CWD TSE PrP Transmission to Humans, has it already happened and being masked as sporadic CJD, What if? July 2026 Review

https://chronic-wasting-disease.blogspot.com/2026/07/cervid-cwd-tse-prp-transmission-to.html

https://prpsc.proboards.com/thread/224/cervid-transmission-humans-july-review

atypical Nor-98, CH1641, Scrapie, TSE Prion, Update 2026

atypical Nor-98 Scrapie Update 2025 Scrapie typical and atypical USA

FY 2023, 16,646 from sheep and 8,726 from goats.

* There have been 491 NVSL confirmed positive animals (474 classical cases – 471 sheep and 3 goats) and

19 Nor98- like cases since the beginning of RSSS. Figure 3 depicts RSSS collection sites in FY 2023.

https://www.aphis.usda.gov/sites/default/files/scrapie-annual-report.pdf

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)...

https://www.usda.gov/sites/default/files/documents/22-APHIS-2025-ExNotes.pdf

In 2022, APHIS collected samples from more than 23,000 sheep and goats for scrapie testing. Out of the total number of animals tested in 2022, no animals tested positive for classical scrapie and

one sheep tested positive for non-classical scrapie (Nor98-like).

https://www.usda.gov/sites/default/files/documents/23-2024-APHIS.pdf

In FY 2021, APHIS collected samples from more than 30,000 sheep and goats for scrapie testing. Out of the total number of samples processed and reported in FY 2021,

one sheep tested positive for classical scrapie and one sheep tested positive for non-classical scrapie (Nor98-like).

https://www.usda.gov/sites/default/files/documents/23-2023-APHIS.pdf

In FY 2020, APHIS collected samples from 33,839 sheep and goats for scrapie testing. This number represents sample results reported by October 15, 2020 and is expected to slightly increase as the remaining results are processed and reported. No animals tested positive for classical scrapie.

Two sheep tested positive at slaughter for non-classical scrapie (Nor98-like).

https://www.usda.gov/sites/default/files/documents/22APHIS2022Notes.pdf

In FY 2019, APHIS collected samples from 34,730 sheep and goats for scrapie testing, detecting 7 classical scrapie positive animals. Of these animals, five sheep and one goat were from a source flock in Pennsylvania that was found in August 2018, depopulated in October 2018, and tested for scrapie in November 2018. A second goat, which was from an Indiana herd, was sampled at slaughter in June 2019. The source flock completed a cleanup plan and was placed on a 5-year monitoring plan. The source herd of the positive Indiana goat no longer contained any exposed animals and was also placed on a 5-year monitoring plan. A trace-back investigation narrowed the goat’s birth herd to two possible herds. Animals in both herds tested negative for scrapie and were placed on 5-year monitoring plans. There were no classical scrapie cases detected in slaughter sheep in FY 2019.

Also in FY 2019, two sheep tested positive at slaughter for non-classical scrapie (Nor98-like) and were traced back to Colorado flocks.

https://www.usda.gov/sites/default/files/documents/20aphis2021notes.pdf

In FY 2018, APHIS collected samples from 43,625 sheep and goats for scrapie testing, detecting three positive (0.0068%) cases. These figures are based on sample submissions and testing completed by September 30, 2018. FY 2018 values are expected to change when testing is completed for all animals sampled in FY 2018.

In October 2017, a non-classical scrapie case was detected in a sheep from Virginia sampled at slaughter. As a result, the non- classical scrapie affected flock was placed on a 5-year monitoring plan.

https://www.usda.gov/sites/default/files/documents/20aphis2020notes.pdf

In FY 2016, the program identified one flock infected with classical scrapie and one infected with Nor98-like scrapie through slaughter surveillance, and two flocks infected with classical scrapie through on-farm surveillance.

An additional 10 sheep were confirmed with classical scrapie through testing of sheep depopulated from these infected flocks as part of flock clean-up activities conducted in FY 2016.

The Nor98-like scrapie affected flock will be placed on a 5-year monitoring plan.

https://www.usda.gov/sites/default/files/documents/20aphisexnotes2018.pdf

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

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

*** 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

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

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

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

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

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

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

***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.

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

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

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

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

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

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

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

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

PRION 2015 CONFERENCE

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

PRION 2016 TOKYO

Saturday, April 23, 2016

SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X

WS-01: Prion diseases in animals and zoonotic potential

***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efﬁciency comparable to that of cattle BSE. While the efﬁciency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

PRION 2015 CONFERENCE

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

PRION 2016 TOKYO

Saturday, April 23, 2016

SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X

WS-01: Prion diseases in animals and zoonotic potential

***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efﬁciency comparable to that of cattle BSE. While the efﬁciency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

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

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

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

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

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

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

Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014)

Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D 1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy.

(2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy.

Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure.

Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine).

Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased.

Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.

Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAFCOD_UO-P

''Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.''

Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.

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

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

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

Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene

Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur

Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933

ABSTRACT

Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation.

Snip…

Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.

Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene

KEYWORDS:

Atypical BSE Bovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation

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

“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”

USA BSE Testing and Surveillance?

Bottom line, USA is testing so few cows for BSE (<25k tested annually)

https://www.aphis.usda.gov/livestock-poultry-disease/cattle/bse/bse-surveillance-program

BUT, even at those low testing figures, the USA did just confirm another case of BSE just here recently. Feed ban has failed terribly, and CWD is spreading in the USA, at an alarming rate. Recent transmission studies show oral transmission of CWD of Cervid to cattle. Studies also show links of sporadic CJD to BSE, Scrapie, and CWD. It’s a Whole new game of Prion poker now$$$

Wednesday, May 24, 2023

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

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

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

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

SATURDAY, MAY 20, 2023

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

https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html

https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed

MAY 19, 2023

https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse

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

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

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

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

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

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

SATURDAY, AUGUST 29, 2026

Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, CJD, TSE prion Update September 2026

https://transmissiblespongiformencephalopathy.blogspot.com/2026/08/bovine-spongiform-encephalopathy-bse.html

https://prpsc.proboards.com/thread/238/scrapie-prion-update-september-2026

FRIDAY, DECEMBER 22, 2023

***> The Mad Cow That Stole Christmas, 20 Years Later, Singeltary Review

***> The Mad Cow That Stole Christmas, 20 Years Later, What Has Changed, Nothing

***> THE USA has systematically covered up mad cow disease, in my honest opinion, the USA mad cow disease today, is Chronic Wasting Disease CWD TSE Prion disease in Cervid, they can't cover that up.

https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html

MONDAY, JUNE 1, 2026

NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD SURVEILLANCE TABLES OF CASES EXAMINED NPDPSC Last updated March 31st, 2026

https://prionunitusaupdate.blogspot.com/2026/06/national-prion-disease-pathology.html

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025

From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025

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

WEDNESDAY, SEPTEMBER 2, 2026

A new chapter for CWRU’s Prion Center

https://prionunitusaupdate.blogspot.com/2026/09/a-new-chapter-for-cwrus-prion-center.html

https://prpsc.proboards.com/thread/239/new-chapter-cwru-prion-center

2001 Singeltary on CJD, Journal of American Medical Association

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.

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

https://jamanetwork.com/journals/jama/article-abstract/1031186

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

2023

https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html

THURSDAY, SEPTEMBER 24, 2026

Diagnostic Performance Of CSF Protein 14-3-3 For Prion Disease In The Modern Era, Singeltary Review

https://prpsc.proboards.com/thread/245/diagnostic-performance-csf-protein-prion

https://madcowtesting.blogspot.com/2026/09/diagnostic-performance-of-csf-protein.html

Terry S. Singeltary Sr.

Transmissible Spongiform Encephalopathy

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Thursday, September 24, 2026

Looming Crisis U. S. Transmissible Spongiform Encephalopathy TSE Prion, in Cattle, Cervid, Sheep, Pigs, and Humans