Alabama ADAI SUPPORTS CWD GENOMIC TESTING FOR DEER BREEDERS, MORE CWD TSE PRION POKER?
Alabama ADAI SUPPORTS CWD GENOMIC TESTING FOR DEER BREEDERS, MORE CWD TSE PRION POKER?
ADAI SUPPORTS CWD GENOMIC TESTING FOR DEER BREEDERS
February 5, 2026
The United States Department of Agriculture (USDA) awarded the Alabama Department of Agriculture and Industries (ADAI) funding to help the captive cervid herd in Alabama become less susceptible to chronic wasting disease (CWD) by furthering USDA research related to genomically estimated breeding value (GEBV) of deer. To date, research has shown that deer with a GEBV value below -0.056 may be less susceptible to CWD than deer with higher values, and USDA encourages herd management actions to breed towards animals with GEBV values below -0.056. While this award is a renewal, the award for 2026 only allows ADAI to pay for testing of up to ~500 captive deer at a cost of $75 per sample to collect GEBV data that can assist game breeders in herd management plans and collect more data for GEBV research and development. This testing will be performed by the North American Deer Registry (NADR). Approved participants will submit samples to NADR following NADR guidelines, and ADAI will remit payment to NADR for each approved sample ($75/sample). If interested in participating, please complete the information packet (below) and submit to the State Veterinarian’s Office at the email listed on the application.
ADAI will only pay for pre-approved samples. Game breeders wishing to participate must complete the “Request for Captive Game Breeder Whitetail Deer Genetic Testing Assistance” below. Do not send samples to NADR until you receive an approval notification from ADAI.
Request for Captive Game Breeder Whitetail Deer Genetic Testing Assistance
Please complete and return to ADAI for approval no later than February 16, 2026. 2026 CWD Testing Application USDA’s “Genomically Estimated Breeding Value Predictive Genetics and Chronic Wasting Disease” Information Sheet
Updated Genomically Estimated Breeding Value North American Deer Registry: When submitting samples to NADR, please follow all NADR sampling guidelines (https://nadeerregistry.com/). All samples should be submitted in tissue tubes and stored at ambient temperature. NADR also recommends shipping samples with a package tracking number and to avoid the use of letter mailing of samples.
Important Dates:
February 16, 2026: Deadline to submit application to ADAI March 1, 2026: ADAI will begin notifying applicants July 31, 2026: Deadline to submit samples to NADR
https://agi.alabama.gov/animalindustries/2026/02/adai-supports-cwd-genomic-testing-for-deer-breeders/
Alabama ADAI SUPPORTS CWD GENOMIC TESTING FOR DEER BREEDERS, or, are they blindly supporting putting the scientific cart before the cwd horse, thus, risk spreading CWD even further $$$
Greetings Alabama Hunters, Alabama Officials, et al,
Your playing with fire folks…im telling you, your playing with fire 🔥
i don’t make this stuff up.
i have wasted almost 3 decades, every day, studying CWD TSE PrP, and i have seen it in humans ie, my mom, confirmed hvCJD. you don’t want to mess with cwd tse prion.
your probably wondering why someone from Texas is contacting Nebraska officials about cwd.
simply put, i made a promise about 27 years ago to Mom, never forget, and never let them forget, show me the transmission studies.
Back in 97, when Mom was dying from this confirmed hvCJD, transmissible spongiform encephalopathy, which is all CWD is, she did everything Linda Blair did in the movie, The Exorcist, except spin her head 360 degrees. She did levitate in bed jerking so bad, it took three grown adults to hold her down, all the while she was screaming WHY CANT I STOP THIS? you never forget.
Washington Times - Washington,DC,
USA NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent
Washington, DC, Mar. 24 (UPI)
https://www.upi.com/Science_News/2005/03/24/NIH-may-destroy-human-brain-collection/84811111671758/
NIH sends mixed signals on CJD brains
By Steve Mitchell
Medical Correspondent
https://www.upi.com/Science_News/2005/04/07/NIH-sends-mixed-signals-on-CJD-brains/25701112902231/
JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 May 18,2005 Mr. Terry Singeltary,… Bacliff, Texas 77518
Dear Mr. Singeltary:
Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate.
Thank you for taking time to contact me.
Sincerely,
JOHN CORNYN United States Senate JC:djl Enclosure
DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of HealthNational Institute of Neurological Disorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: Email:
May 10, 2005
The Honorable John Cornyn United States Senator Occidental Tower 5005 LBJ Freeway, Suite 1150 Dallas, Texas 75244-6199
Dear Senator Cornyn:
Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.
I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification.)
The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate that they have a compelling research or public health need for such materials. For example, samples have been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National Prion Diseases Pathology Surveillance Center at Case Western Reserve University in Ohio and works with the Centers for Disease Control and Prevention to monitor all cases of CJD in the United States. Dr. Gambetti studies the tissues to learn about the formation, physical and chemical properties, and pathogenic mechanisms of prion proteins, which are believed to be involved in the cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food and Drug Administration, for use in assessing a potential diagnostic test for CJD.
Page 2 - The Honorable John Cornyn
in closing, we know that donating organs and tissue from loved ones is a very difficult and personal choice that must often be made at the most stressful of times. We at the NINDS are grateful to those stalwart family members who make this choice in the selfless hope that it will help others afflicted with CJD. We also know the invaluable contribution such donations make to the advancement of medical science, and we are dedicated to the preservation of all of the tissue samples that can help in our efforts to overcome CJD.
I hope this information is helpful to you in responding to Mr. Singeltary.
Sincerely,
Story C. Landis, Ph.D. Director, National Institute of Neurological Disorder and Stroke
NIH says it will preserve CJD brains
By STEVE MITCHELL
MAY 31, 2005 / 5:26 PM
https://www.upi.com/Science_News/2005/05/31/NIH-says-it-will-preserve-CJD-brains/67711117574761/
Sadly, Apparently, Alabamas run to line their pockets with more cash for monster horns with the Captive deer pens, and with the full backing of Alabama ADAI, the risk to Alabamas Wild deer herds grow even greater, along with the State of Oklahoma, and others, who have also decided to play CWD TSE PRION POKER, where they too decided to risk their wild cervid herds to CWD even further, by releasing GMO supposedly CWD resistant deer into the wild. Evidently, God made them wrong right, and the captive deer industry is going to correct Gods mistakes in making deer and elk. what could go wrong, right $$$
***> i’m extremely concerned for wild Cervid in Alabama, with this farmed GMO cwd cervid experiment in Alabama (and in other States), and i believe CWD is more widespread in Alabama Captive/Breeder than the numbers are showing, here’s why…terry
Question to Google AI;
how many captive deer herds are in the usda certified in alabama ?
Based on available USDA APHIS and Alabama state records up to early 2026, there is no specific, publicly released, and exact, real-time number of USDA-certified captive deer herds in Alabama. However, the following data provides context on the scale of captive operations in the state:
Total Operations: There are over 200 licensed deer breeders in Alabama who provide white-tailed deer to high-fence hunting operations.
USDA Participation: Alabama participates in the voluntary Chronic Wasting Disease (CWD) Herd Certification Program (HCP). While exact "certified" (5-year status) numbers are not broken down by state in recent public, high-level summaries, these 200+ breeders are subject to monitoring, particularly for CWD, by the Alabama Department of Agriculture and Industries (ADAI).
Testing Volume: For 2026, the ADAI planned to fund, via the North American Deer Registry (NADR), testing for up to ~500 captive deer to collect genetic and CWD data.
Recent Developments: In 2025, legislation was passed that impacted the management and testing protocols for Alabama's captive cervids.
The USDA Herd Certification Program is voluntary and requires 5 years of no CWD detection to achieve fully "certified" status.
Question to Google AI;
how many captive deer only, in alabama have been tested for cwd?
***> Based on available Alabama Department of Conservation and Natural Resources (ADCNR) and CWD-INFO.ORG data, there is no specific, publicly reported number of only captive deer tested for CWD in Alabama.
While the state has monitored for CWD in deer populations since 2002, reported testing figures often focus on wild, hunter-harvested deer in management zones.
Positive Cases: As of late 2024, CWD has been confirmed in five hunter-harvested, wild white-tailed deer in northwest Alabama.
Surveillance: The Alabama Department of Conservation and Natural Resources (ADCNR) has been actively monitoring for CWD in both wild and captive (farmed) deer populations.
Testing Scope: Thousands of deer have been tested across the state, particularly since the first case was detected in 2021.
***> No specific, isolated number for exclusively captive, captive-only deer test results is detailed in the provided search results.
end…TSS
EXTREMELY WORRIED!!!
let’s look at what recent science is telling us now, shall we…
“These results indicate that the PRNP polymorphism modulates disease progression but does not prevent infection. Natural isolates also showed biochemical and pathological diversity, demonstrating the existence of diverse prion strains circulating in the environment.”
CWD prion strain variation modeled in the lab and identified in natural cases
SOTO, PAULINA 1Benavente, Maria 2Kramm, Carlos 2Stimming, Tucker 2Bravo-Risi, Francisca 3Pritzkow, Sandra 2Lyon, Adam 2Reed, J Hunter 4Soto, Claudio 2Morales, Rodrigo 5 Vol 1, 2025 - 328841 Abstract Prion 2025
Abstract Introduction: Chronic Wasting Disease (CWD) is a prion disease affecting cervids. It is characterized by distinct prion strains, which can influence transmission rates and disease phenotypes. In white-tailed deer, polymorphism at codon 96 of the PRNP gene (glycine (G) or serine (S)) is associated with differential susceptibility to CWD. The 96SS genotype has been consider “protective”; however, the potential for prion adaptation across deer PrP polymorphic variants remains unclear. Worrisomely, current surveillance strategies do not consider the identification of CWD prion strains, a fact that may contribute to CWD epidemiology.
Methods: We modeled inter-polymorphic prion conversion and demonstrate that prions can efficiently adapt in 96SS hosts with infectious properties like those in classical CWD strains. To investigate this, CWD prions from 96GG and 96SS deer were propagated by PMCA using homologous and heterologous transgenic brain homogenates, resulting in four strain combinations: GG/GG, SS/GG, GG/SS, and SS/SS (donor/recipient). These were characterized biochemically and tested in Tg1536and Tg60 mice. As part of this project, four natural CWD prion isolates identified in screening/diagnostic practices were similarly analyzed.
Results and Discussion: All experimental strains showed biochemical differences, indicating structural divergence. In Tg1536 mice, all strains produced disease with full attack rates. In Tg60mice, few animals developed clinical signs, but all showed PrPSc and vacuolation. These results indicate that the PRNP polymorphism modulates disease progression but does not prevent infection. Natural isolates also showed biochemical and pathological diversity, demonstrating the existence of diverse prion strains circulating in the environment.
PRION 2025 RIO DE JANEIRO
“ These new macaque studies confirmed that scrapie can be transmitted from certain sheep isolates to non-human primate after extended incubation periods (10 years), whereas after passage in Tg110 mice transmission may occur with two-fold shorter incubation periods.”
https://proceedings.science/prion-2025/papers?prod_proceedings_papers%5BrefinementList%5D%5Btrack.title.en%5D%5B0%5D=Animal%20prion%20diseases
https://proceedings.science/prion-2025/papers?prod_proceedings_papers%5BrefinementList%5D%5Bkeywords.en%5D%5B0%5D=Scrapie
https://proceedings.science/prion-2025/papers
https://proceedings.science/prion-2025?prod_proceedings_papers%5BrefinementList%5D%5Bkeywords.en%5D%5B0%5D=PMCA
Oklahoma decides to Play CWD TSE Prion Poker, and no one wins in Prion poker, with a experiment with Oklahomas wild deer herd and release GMO deer, what could go wrong, right? it’s like putting the cart before the horse science. these GMO deer are supposedly to be resistant to CWD, however, no deer has ever been confirmed to be totally resistant to CWD, and in fact, genotypes developed with very long incubation, could therefore, if released into the wild, could help spread cwd even further, exposing even more wild species, and surrounding environments, for even longer periods of time, due to the longer incubation, a terrible potential outcome, one that must be avoided at all cost, imo…terry
SCIENTISTS: RELEASING CAPTIVE-BRED DEER TO FIGHT CWD IN WILD DEER IS UNLIKELY TO WORK
October 1, 2025 By: Lindsay Thomas Jr.
Recently scientists have been exploring the idea that we can fight chronic wasting disease in wild deer by releasing captive-bred, “CWD resistant” deer. Some in the deer farming industry endorsed the idea, and legislators in Oklahoma even authorized a program to begin breeding and releasing deer. Most scientists, however, are urging everyone to pump the brakes.
On July 15, the Theodore Roosevelt Conservation Partnership invited two CWD experts to give a live webinar on the topic, titled “Breeding to Battle CWD: Can Wildlife Evolve Their Way Out of Disease?” To help shed more light on this important topic, this article is a summary of the most important take-home messages for deer hunters that came out of the two presentations. You can also watch the full presentations here.
https://www.trcp.org/chronic-wasting-disease/
PART 1: DR. DEBBIE MCKENZIE
The first speaker was Dr. Debbie McKenzie, an emeritus professor at the University of Alberta’s Centre for Prions and Protein Folding Diseases. Her research focus for the past 35 years has been on prion diseases, specifically CWD.
https://www.ualberta.ca/en/prion-centre/index.html
“Resistance” Needs More Evidence
This entire discussion originated with one study that suggested certain genetic strains of deer – known as the 96SS genotype – could be “resistant” to CWD. That study is a statistical model that suggests 96SS deer test positive for CWD less often than other deer. The study did not challenge live deer with CWD infection. Both speakers suggested they’d welcome the use of Genomically Estimated Breeding Value (GEBV) if it can slow the spread of CWD among captive herds, but they say real-world evidence is lacking so far.
“Though I think there’s some utility with GEBV, I really think we need some challenge experiments so we can demonstrate that these statistical analyses do point to increased resistance,” said Dr. McKenzie.
“Resistant” Deer Still Get CWD But Live Longer
Meanwhile, studies of actual deer, according to Dr. McKenzie, show that 96SS deer are not “resistant” but experience a longer incubation period before dying. “96SS and other polymorphisms are linked to slower disease progression, but they are not resistance genes,” she said. “If those animals are exposed, they will get CWD. It’s just going to take longer from the time they are infected until they are clinically sick.”
This chart from Dr. McKenzie’s presentation shows that some genotypes of deer survive longer than others with CWD but are not “resistant” because they can still acquire the disease. Living Longer With CWD Means Greater Spread
Longer incubation periods with CWD are counterproductive, because even CWD-infected 96SS deer are still shedding prions into the environment and sharing them with other deer. “Shedding is still occurring throughout those longer incubation periods,” said Dr. McKenzie. “If you have double the incubation period, but you’ve only made a 10% reduction in the prion shedding, you’re actually going to end up with more environmental contamination.”
New Strains Might Make “Resistant Deer” Irrelevant
New strains of CWD have already evolved, and more are coming. According to Dr. McKenzie, selecting for 96SS genetics in deer could theoretically lead to reduced susceptibility to some CWD strains. “On the other hand,” she said, “it might mean we’re selecting for susceptibility to a different strain.”
PART 2: DR. SONJA CHRISTENSEN
The second talk by Dr. Sonja Christensen took the science into the real world to look at the likelihood of using genetically resistant deer – assuming they are found to be a real thing – to fight CWD in wild deer populations. Dr. Christensen is an assistant professor at Michigan State University who specializes in wildlife disease ecology, population health, and wildlife management.
http://www.christensen-lab.org/
Wild Deer Are a Different Issue Than Captive Deer
Genetic resistance to CWD could have usefulness in captive deer, where people control which deer do the breeding. “However, I am focused solely on wild deer,” said Dr. Christensen. “Wild populations are inherently messy.”
We Can’t Manage Genetics in Wild Deer
Hunters have mistakenly believed for years that they can change the genetics of future deer through selective harvest, but repeated scientific experiments have failed to show that it’s possible to make a measurable difference – even under very intensive manipulation of deer harvest. The reasons why also explain why releasing a few “CWD resistant” deer in an area would be unlikely to have any impact on wild deer genetics.
https://deerassociation.com/strike-3-for-the-myth-of-the-genetic-cull-buck/
Dr. Christensen listed several of the factors that would complicate any kind of genetic fight against CWD in wild deer. For example, there are over 30 subspecies of whitetails with genetic variations across regions. We lack the ability to control which deer do the breeding. Any genetically modified deer released into the wild might never reproduce due to being killed by predators, other diseases, and other mortality sources beyond our control.
Existing Native Deer Prevent Success
Stocking genetically modified deer could be more successful only if native deer with higher susceptibility to CWD are first removed. Dr. Christensen pointed out that fighting scrapie, a prion disease of sheep that is similar to CWD, required massive culling of sheep to increase resistance, and this was with captive livestock. Obviously, in the wild it would be nearly impossible to identify which deer are more susceptible or remove enough of them to make a difference.
It Would Take a Very, Very Long Time
“In the best case scenario, assuming everything’s working perfectly and we can control all of these factors in a very messy, free-ranging herd, it’s still going to take years and years and years and years to really see this change and pick up on that signal,” said Dr. Christensen.
Why Not Try It Anyway?
Despite all the evidence that stocking “resistant” deer would have no impact on CWD in wild deer populations, Dr. Christensen played devil’s advocate and asked, “Why not try it anyway?” She answered by highlighting the extreme costs and the unintended consequences.
“Captive deer genetics are not like wild deer genetics,” she said. “There might be other traits that are unknowingly entering a population when you release captive deer. Something else might be expressed. Because of the long incubation period, we could unknowingly release CWD-positive deer into an area that doesn’t have the disease. We could be increasing susceptibility to other diseases.”
“The outcome has a lot of uncertainly and cost for wildlife agencies, and that’s a risk to our natural resources that the wildlife agencies are managing in the public trust,” said Dr. Christensen. “Ultimately, that is undermining the North American Model of Wildlife Management and how wildlife management works.”
Other Comments About Release of Captive-Bred Deer to Fight CWD
The CWD Research Consortium, a group of independent researchers from diverse disciplines and institutions currently working on CWD, prepared a document to provide science-based information on the use of selective breeding and release of captive deer for CWD management.
https://www.cwd-research.com/home/selective-breeding-and-release-of-captive-white
The Association of Fish & Wildlife Agencies passed a resolution opposing “the release of any captive cervids into the wild to influence free-ranging cervid population genetics for the purpose of controlling or managing CWD, based on the current best scientific information, and encourages its members in their own jurisdictions to promote and implement the best scientific management practices for CWD.”
https://www.fishwildlife.org/landing/blog/state-fish-wildlife-agency-directors-pass-ten-resolutions-afwas-2024-annual-meeting
The United States Animal Health Association issued a resolution calling on the U.S. Department of Agriculture to conduct a controlled experiment to test the validity of CWD resistant genetics.
https://usaha.org/wp-content/uploads/2024/12/2024-USAHA-Resolutions-21.pdf
Categories: Deer Science Tags: Chronic Wasting Disease, Cwd
About Lindsay Thomas Jr.: Lindsay Thomas Jr. is NDA's Chief Communications Officer. He has been a member of the staff since 2003. Prior to that, Lindsay was an editor at a Georgia hunting and fishing news magazine for nine years. Throughout his career as an editor, he has written and published numerous articles on deer management and hunting. He earned his journalism degree at the University of Georgia.
https://deerassociation.com/scientists-releasing-captive-bred-deer-to-fight-cwd-in-wild-deer-is-unlikely-to-work/
Problem Statement 6B: Reveal genetics of prion disease susceptibility.
Greater frequency of chronic wasting disease in free-ranging elk genetically tolerant to disease progression raises concerns related to prion transmission and strain evolution.
Animal Disease Research Unit, Pullman, Washington
Genetic variations in the prion protein gene of Rocky Mountain elk do not confer complete resistance to fatal infection by chronic wasting disease. However, elk carrying one or two copies of the amino acid leucine (L) variant at position 132 of the prion protein (132L*, where * is either M for methionine or L) survive much longer than 132MM elk. An ARS researcher in Pullman, Washington, in collaboration with researchers at the University of Wyoming, the University of California at Davis, the National Park Service, and the Animal and Plant Health Inspection Service, found a higher frequency of 132L* elk in areas of Wyoming with high infection rates, consistent with the expected positive effect of prolonged survival on reproduction. However, the frequency of chronic wasting disease infection in 132L* elk was also higher than previous estimates. To improve the long-term management of native elk populations, these findings underscore the importance of determining the effects of prolonged infection on disease transmission from 132L* elk and the potential for driving prion strain diversification.
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: Update: CWD genetic resistance project at NADC Author item Cassmann, Eric item Greenlee, Justin Submitted to: North American Deer Farmer Publication Type: Trade Journal Publication Acceptance Date: 11/5/2023 Publication Date: 11/15/2023 Citation: Cassmann, E.D., Greenlee, J.J. 2023.
Update: CWD genetic resistance project at NADC. North American Deer Farmer. P. 83. Interpretive Summary: Technical Abstract: In March of 2020, we began a study to examine the susceptibility of whitetail deer with rare prion protein genotypes to chronic wasting disease (CWD). In the sequence of amino acids that make up the deer prion protein, there are several locations that are variable. These variations are sometimes called polymorphisms. In the data collected from depopulations, whitetail deer with certain prion gene polymorphisms were not positive for CWD. In 2019, Dr. Nick Haley published a paper that showed H95/S96, HH95, and S96/K226 deer from depopulated herds in the US were not CWD positive. Based on the overall low number of deer with these genotypes () we’re unable to determine if they were resistant to CWD or if there were too few deer with these genotypes to be statistically represented in the positive cases. It’s also possible that they could be partially susceptible with longer incubation times than deer with generic (wild type) prion genotypes. Samples gathered at depopulation represent a snapshot of the herd. It is possible these rare genotypes were exposed, but had not yet accumulated abnormal prion protein to a level detectable by the detection methods used. The NADC susceptibility study was initiated to help answer these questions.
We studied deer with polymorphisms at 3 amino acid locations (codons): 95, 96, and 226. Wild type deer are QQ95GG96QQ226. Whitetail deer with wild type prion genotypes were inoculated with CWD and co-housed with other whitetail deer (contact deer) that had rare prion protein genotypes. The genotypes of contact deer included QH95GS96QQ226, QH95GG96QK226, QQ95GS96QQ226, QQ95SS96QQ226, Q95GS96QK226, and QQ95GG96KK226 (bolded text indicates a prion gene polymorphism).
During the first year, we collected feces, saliva, nasal swabs, skin, blood, and rectal biopsies from the inoculated and contact deer to determine if deer are CWD positive and the period of CWD shedding. After the first year, we started collecting rectal biopsies annually on the contact deer, but all other samples are still collected every three months.
Eight out of ten (8/10) inoculated deer developed clinical signs for CWD and tested positive after necropsy (Figure 1). The average time from inoculation to euthanasia of these eight inoculated deer was 23 months.
Two inoculated deer are still on-study; one of these deer has tested positive for CWD on rectal biopsy IHC.
To date, two deer from the contact group have developed CWD clinical signs and tested positive (Figure 2). The positive deer from the contact group had the GS96QK226 and KK226 genotypes.
We have detected CWD prions in rectal biopsies with IHC in three other contact deer as of October 2023. Their prion genotypes are GS96, QH95GS96, and GS96QK226.
As the experiment continues, we hope to answer 2 main questions. (1) Are there any prion protein polymorphisms that make deer resistant to CWD, and (2) what are the CWD shedding dynamics in deer with detectable CWD. One potential outcome of the study would be identifying genotypes with very long incubation periods that, while susceptible to CWD, still could be used to manage CWD.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=410188
Update: CWD genetic resistance project at NADC 1) Are there any prion protein polymorphisms that make deer resistant to CWD, and (2) what are the CWD shedding dynamics in deer with detectable CWD. One potential outcome of the study would be identifying genotypes with very long incubation periods that, while susceptible to CWD, still could be used to manage CWD.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=410188
Another potential and likely outcome of this study, imo, is that genotypes developed with very long incubation, could therefore, if released into the wild, could help spread cwd even further, exposing even more wild species, and surrounding environments, for even longer periods of time, due to the longer incubation, a terrible potential outcome, one that must be avoided at all cost, imo…terry
LEGISLATING CWD TSE Prion, Bills to release Genetically Modified Cervid into the wild, what could go wrong?
“If slower disease progression results in longer-lived, infected deer with longer periods of infectiousness, resistance may lead to increased disease transmission rates, higher prion concentrations in the environment, and increased prevalence, as has been observed in some captive deer herds (Miller et al., 2006; Keane et al., 2008a).”
Genetic susceptibility to chronic wasting disease in free-ranging white-tailed deer: Complement component C1q and Prnp polymorphisms§
Julie A. Blanchong a, *, Dennis M. Heisey b , Kim T. Scribner c , Scot V. Libants d , Chad Johnson e , Judd M. Aiken e , Julia A. Langenberg f , Michael D. Samuel g
snip...
Identifying the genetic basis for heterogeneity in disease susceptibility or progression can improve our understanding of individual variation in disease susceptibility in both free-ranging and captive populations. What this individual variation in disease susceptibility means for the trajectory of disease in a population, however, is not straightforward. For example, the greater, but not complete, resistance to CWD in deer with at least one Serine (S) at amino acid 96 of the Prnp gene appears to be associated with slower progression of disease (e.g., Johnson et al., 2006; Keane et al., 2008a). If slower disease progression results in longer-lived, infected deer with longer periods of infectiousness, resistance may lead to increased disease transmission rates, higher prion concentrations in the environment, and increased prevalence, as has been observed in some captive deer herds (Miller et al., 2006; Keane et al., 2008a). Alternatively, if the slower progression of disease in resistant deer is not associated with longer periods of infectiousness, but might instead indicate a higher dose of PrPCWD is required for infection, transmission rates in the population could decline especially if, as in Wisconsin, deer suffer high rates of mortality from other sources (e.g., hunting). Clearly, determining the relationship between genetic susceptibility to infection, dose requirements, disease progression, and the period of PrPCWD infectiousness are key components for understanding the consequences of CWD to free-ranging populations.
http://web.archive.org/web/20121114223603/http://forest.wisc.edu/files/pdfs/samuel/2009%20blanchong%20et%20al%20genetic%20susceptibility%20chronic%20wasting.pdf
https://dr.lib.iastate.edu/server/api/core/bitstreams/630cd976-0c33-4b0a-bc97-96e2669107d5/content
TEXAS CWD, WHILE IGNORING THE PROBLEM AT HAND, TRYING TO GMO YOUR WAY OUT SAID PROBLEM, TRYING TO LEGISLATING CWD, AND IN DOING SO, POTENTIALLY CREATING A BIGGER PROBLEM, WHAT IF?
WE call this TSE Prion Poker, are you all?
Volume 30, Number 10—October 2024
Research
Temporal Characterization of Prion Shedding in Secreta of White-Tailed Deer in Longitudinal Study of Chronic Wasting Disease, United States
Our findings suggest that deer expressing alternative PRNP polymorphisms might live longer and, although they shed fewer prions throughout CWD course, might over their extended lifespan increase CWD prions in the environment
https://wwwnc.cdc.gov/eid/article/30/10/24-0159_article
Prion protein gene sequence and chronic wasting disease susceptibility in white-tailed deer (Odocoileus virginianus)
Adam L Brandt, Amy C Kelly, Michelle L Green, Paul Shelton, Jan Novakofski & Nohra E Mateus-Pinilla
Pages 449-462 | Received 21 Sep 2015, Accepted 23 Oct 2015, Published online: 21 Dec 2015 https://doi.org/10.1080/19336896.2015.1115179
The presence of aa96S has been associated with slowed disease progression, longer life span among captive deer,Citation26,27 and does not appear to affect the rate at which prions are shed from infected individuals.Citation38 Additionally, CWD infected mule deer have been found to excrete pathogenic prions while asymptomatic.Citation39 This contributes to concerns that wild deer with aa96S may be shedding infectious prions into the environment for longer periods of time than deer lacking the mutation, but are not symptomatic or detectable by immunohistochemical procedures.
https://www.tandfonline.com/doi/full/10.1080/19336896.2015.1115179#d1e354
https://pmc.ncbi.nlm.nih.gov/articles/PMC4964855/pdf/kprn-09-06-1115179.pdf
''There are no known familial or genetic TSEs of animals, although polymorphisms in the PRNP gene of some species (sheep for example) may influence the length of the incubation period and occurrence of disease.''
c) The commonest form of CJD occurs as a sporadic disease, the cause of which is unknown, although genetic factors (particularly the codon 129 polymorphism in the prion protein gene (PRNP)) influence disease susceptibility. The familial forms of human TSEs (see Box 1) appear to have a solely genetic origin and are closely associated with mutations or insertions in the PRNP gene. Most, but not all, of the familial forms of human TSEs have been transmitted experimentally to animals. There are no known familial or genetic TSEs of animals, although polymorphisms in the PRNP gene of some species (sheep for example) may influence the length of the incubation period and occurrence of disease.
https://assets.publishing.service.gov.uk/government/uploads/system/uploads/attachment_data/file/209755/Part_1_-_Introduction.pdf
P-145 Estimating chronic wasting disease resistance in cervids using real time quaking- induced conversion
Nicholas J Haley1, Rachel Rielinqer2, Kristen A Davenport3, W. David Walter4, Katherine I O'Rourke5, Gordon Mitchell6, Juergen A Richt2 1
Our studies demonstrate that in vitro amplification metrics predict in vivo susceptibility, and that alleles with multiple codons, each influencing resistance independently, do not necessarily contribute additively to resistance. Importantly, we found that the white-tailed deer 226K substrate exhibited the slowest amplification rate among those evaluated, suggesting that further investigation of this allele and its resistance in vivo are warranted to determine if absolute resistance to CWD is possible. ***at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified.
PRION 2016 CONFERENCE TOKYO
http://prion2016.org/dl/newsletter_03.pdf
http://chronic-wasting-disease.blogspot.com/2017/04/
***> at present, no PrPC allele conferring absolute resistance in cervids has been identified.
J Gen Virol. 2017 Nov; 98(11): 2882–2892.
Published online 2017 Oct 23. doi: 10.1099/jgv.0.000952
Estimating chronic wasting disease susceptibility in cervids using real-time quaking-induced conversion
Chronic wasting disease (CWD) resistance in cervids is often characterized as decreased prevalence and/or protracted disease progression in individuals with specific alleles; at present, no PrPC allele conferring absolute resistance in cervids has been identified.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5845664/pdf/jgv-98-2882.pdf
APHIS USDA Captive CWD Herds Update by State December 2025 Update
https://chronic-wasting-disease.blogspot.com/2025/12/aphis-usda-captive-cwd-herds-update-by.html
https://prpsc.proboards.com/thread/187/aphis-captive-herds-update-december
USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE 2025-2014 CHRONIC WASTING DISEASE CWD TSE CERVID
https://chronic-wasting-disease.blogspot.com/2025/09/usda-explanatory-notes-animal-and-plant.html
SUNDAY, MAY 04, 2025
Texas Senate Bill 2651 establishment of a pilot program to breed deer resistant to CWD TSE Prion, what could go wrong?
https://chronic-wasting-disease.blogspot.com/2025/05/texas-senate-bill-2651-establishment-of_4.html
FRIDAY, FEBRUARY 21, 2025
LEGISLATING CWD TSE Prion, Bills to release Genetically Modified Cervid into the wild, what could go wrong?
https://chronic-wasting-disease.blogspot.com/2025/02/legislating-cwd-tse-prion-bills-to.html
MONDAY, JUNE 09, 2025
Genetic Approaches and Tools to Prevent, Control, and Eradicate Transmissible Spongiform Encephalopathies 2024 Annual Report ARS Research
https://chronic-wasting-disease.blogspot.com/2025/06/genetic-approaches-and-tools-to-prevent.html
Oklahoma HB3462 Chronic Wasting Disease Genetic Improvement Act and Legislating CWD Science
Bill Summary 2nd Session of the 59th Legislature Bill No.: HB 3462 Version: CS Request No.: 3679 Author: Sen. Green Date: 04/08/2024 Bill Analysis
Greetings to the Great State of Oklahoma!
I must comment on the following please!
HB 3462 creates the Chronic Wasting Disease Genetic Improvement Act. The measure directs the Oklahoma Department of Agriculture, Food, and Forestry to establish a pilot program to enhance the genetic durability of Oklahoma deer against chronic wasting disease no later than November 1, 2024. The program shall require the Department of Wildlife Conservation to collect DNA samples to establish a baseline of average genetic codon markers and genomic breeding values. Participation in the program shall be limited to native white-tailed deer, born and raised in Oklahoma with genetic resistance breeding. Bred deer may be released in 2026, during the months of February and March and through the 15th of April. The Department of Wildlife Conservation may charge a one-time permit fee for citizens purchasing deer. The fee shall not exceed $500.00.
Prepared by: Kalen Taylor
http://webserver1.lsb.state.ok.us/cf_pdf/2023-24%20SUPPORT%20DOCUMENTS/BILLSUM/Senate/HB3462%20CS%20BILLSUM.PDF
Snip…see;
https://chronic-wasting-disease.blogspot.com/2024/05/oklahoma-hb3462-chronic-wasting-disease.html
USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025 and history there from
https://www.researchgate.net/publication/396084947_USDA_National_Scrapie_Program_History_and_Bovine_Spongiform_Encephalopathy_BSE_TSE0AUpdate_2025
ARS Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025
“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.”
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
2025 Annual Report
Objectives
Objective 1: Develop highly sensitive detection tools to determine the distribution of CWD and scrapie prions in natural hosts (sheep, goats, cervids) and their environment.
Objective 2: Investigate the pathobiology of CWD, scrapie prion strains, and atypical TSEs in natural hosts including potential cross species transmission events.
Objective 3: Investigate the genetics of CWD susceptibility and resistance in white-tailed deer.
Objective 4: Evaluate the presence of and determine the appropriate methodology for CWD strain determination.
Approach
Eradication or control of a family of diseases is unlikely or impossible when an understanding of the basic mechanisms and influences on transmission are unknown and for which methods to evaluate disease status are lacking. Scrapie and BSE represent the most thoroughly studied TSEs; however, significant knowledge gaps persist with regard to the atypical variants of these diseases. Further, much of the research emphasis to date on genetics of prion disease has focused on the recipient genotype rather than the source. Since both atypical BSE and atypical scrapie have been suggested to occur spontaneously, eradication of these diseases may not be possible unless we expand our understanding of the disease at both the source and recipient level. A better understanding of the tissue distribution and potential transmission of these atypical isolates is critical to understanding what risk these disease variants may pose to ongoing control and eradication efforts. The European epizootic of BSE is waning and efforts to eradicate scrapie in the U.S. and abroad have progressed but are not complete. In the U.S., chronic wasting disease (CWD) presents the most serious challenge to regulatory efforts. CWD appears to be spreading unchecked in both free-ranging and farmed cervids. Methods for antemortem detection of TSEs in general and CWD in particular are needed to fulfill the goal of eradicating scrapie and controlling CWD. Performing these studies will allow us to address critical knowledge gaps that are relevant to developing measures to restrict further disease expansion beyond current, affected populations. Understanding prion disease persistence in animal populations is challenging due to lack of tools for study and a less than complete understanding of transmission among animals within a flock or herd or in naturally occurring reservoirs. In addition to transmission between hosts of like species, free-ranging cervids may come in contact with numerous other species including cattle, sheep, and other susceptible hosts. Transmission of CWD to other species has been studied but limited with regard to the source genotype used. The four primary objectives are inherently linked. Our focus is on developing tools needed for control and research, and using those tools to advance our understanding the complex disease process with the overall goal of eradication and control of disease in livestock, wildlife of economic importance, and potential wildlife reservoirs.
Progress Report
The goals of the project plan for fiscal year (FY) 2025 consisted of 12 milestones, 11 of which were either fully or substantially met. The only milestone in this plan that was not met was due to insufficient animal availability and space constraints. Previous studies utilizing this space are not complete due to longer than anticipated incubation periods and cannot be initiated until those studies are complete. In work toward addressing
Objective 1, “Develop highly sensitive detection tools to determine the distribution of chronic wasting disease (CWD) and scrapie prions in natural hosts (sheep, goats, cervids) and their environment”, we have worked closely with ARS researchers in Pullman, Washington, Animal and Plant Health Inspection Service (APHIS), and university partners. The tools under development are directly utilized by state diagnostic labs and have been shared with the appropriate end users for evaluation. We have also assessed alternative dyes that have do not induce amyloid formation in the amplification based diagnostic assay known as RT-QuIC. While no increase in sensitivity was observed, differences between strains were found offering an additional means to differentiate strains for some TSEs.
Objective 2, “Investigate the pathobiology of CWD, scrapie prion strains, and atypical TSEs in natural hosts including potential cross species transmission events”, the studies in question have been initiated with the goal of furthering the understanding of these TSEs in agriculturally relevant species including the natural host species and other that may be exposed to these TSEs in an agricultural environment. The studies are ongoing and anticipated to last upwards of 5 year and observation of the animals is ongoing. No anticipated signs of disease or relevant reportable information have been seen nor are they expected until near the onset of clinical signs, but if they are observed they will be reported.
Objective 3, “Investigate the genetics of CWD susceptibility and resistance in white-tailed deer”, consists of two subobjectives:
A) Investigate the susceptibility of white-tailed deer to CWD modeling direct contact exposure with infected deer, and
B) Investigate the susceptibility of white-tailed deer to CWD after direct inoculation.
The first of these has been initiated on schedule while the second has been delayed considerably (greater than 3 years at this point) due to insufficient animal space.Upon completion these two studies will aid in understanding the disease and disease progression.
Objective 4, “Evaluate the presence of and determine the appropriate methodology for CWD strain determination”, is dependent upon obtaining a diverse set of CWD isolates. We are continuing the acquisition of these samples. . Strains are one of the least understood aspects of TSEs as a whole and of importance in understanding the risks of CWD. We have initiated studies that will address the biochemical nature of prion strains and how these strains are maintained in a host which will aid in addressing features and differentiation of strains as additional samples become available.
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
ARS Research Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025 Annual Report
CWD, transmission to, Cattle, Sheep, Pigs, Cervid, oh my!
Price of TSE Prion Poker Goes Up Again…terry
https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/ars-research-elucidating-pathobiology.html
Chronic Wasting Disease CWD vs Scrapie TSE Prion
Volume 30, Number 8—August 2024
Research
Scrapie Versus Chronic Wasting Disease in White-Tailed Deer
Zoe J. Lambert1, Jifeng Bian, Eric D. Cassmann, M. Heather West Greenlee, and Justin J. Greenlee
Author affiliations: Oak Ridge Institute for Science and Education, Oak Ridge, Tennessee, USA (Z.J. Lambert); US Department of Agriculture, Ames, Iowa, USA (Z.J. Lambert, J. Bian, E.D. Cassmann, J.J. Greenlee); Iowa State University, Ames (Z.J. Lambert, M.H. West Greenlee) Suggested citation for this article
Abstract
White-tailed deer are susceptible to scrapie (WTD scrapie) after oronasal inoculation with the classical scrapie agent from sheep. Deer affected by WTD scrapie are difficult to differentiate from deer infected with chronic wasting disease (CWD). To assess the transmissibility of the WTD scrapie agent and tissue phenotypes when further passaged in white-tailed deer, we oronasally inoculated wild-type white-tailed deer with WTD scrapie agent. We found that WTD scrapie and CWD agents were generally similar, although some differences were noted. The greatest differences were seen in bioassays of cervidized mice that exhibited significantly longer survival periods when inoculated with WTD scrapie agent than those inoculated with CWD agent. Our findings establish that white-tailed deer are susceptible to WTD scrapie and that the presence of WTD scrapie agent in the lymphoreticular system suggests the handling of suspected cases should be consistent with current CWD guidelines because environmental shedding may occur.
snip…
The potential for zoonoses of cervid-derived PrPSc is still not well understood (6,18,45–47); however, interspecies transmission can increase host range and zoonotic potential (48–50). Therefore, to protect herds and the food supply, suspected cases of WTD scrapie should be handled the same as cases of CWD.
https://wwwnc.cdc.gov/eid/article/30/8/24-0007_article
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
Author item LAMBERT, ZOE - Oak Ridge Institute For Science And Education (ORISE) item Bian, Jifeng item Cassmann, Eric item WEST GREENLEE, HEATHER - Iowa State University item Greenlee, Justin
Submitted to: Emerging Infectious Diseases Publication Type: Peer Reviewed Journal Publication Acceptance Date: 6/13/2024 Publication Date: N/A Citation: N/A
Interpretive Summary: Prion diseases are a neurodegenerative disease that can spread between animals. They are caused when the normal cellular prion protein misfolds and accumulates in the host’s central nervous system. This change is irreversible and invariably causes neurological disease and death of the host. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in white-tailed deer (WTD), has only been recognized since the 1960s. It has long been suggested that prion disease in deer (chronic wasting disease) was caused by the prion agent from sheep (scrapie). Recently, our lab confirmed that WTD will become infected by scrapie from sheep under conditions that mimic natural exposure. The disease produced in these animals was termed WTD scrapie. This manuscript addresses the next step in disease spread: whether sick WTD can pass WTD scrapie on to other deer. We found that white-tailed deer sick with scrapie can infect other deer under conditions mimicking natural exposure. The work reported in this manuscript demonstrates that CWD is difficult to differentiate from WTD scrapie. Regardless, WTD scrapie prions accumulate in the lymphoreticular system, meaning that environmental contamination is likely through feces, saliva, and other body fluids. Controlling WTD scrapie would require precautions similar to those taken with chronic wasting disease. The presence of WTD scrapie could confound mitigation efforts for chronic wasting disease. This information will be of interest to 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.
Technical Abstract: White-tailed deer (WTD) are susceptible to the scrapie agent from sheep after oronasal inoculation, termed WTD scrapie. However, results from western blotting these brainstems and lymph nodes are difficult to differentiate from WTD infected with chronic wasting disease (CWD). In order to assess the transmissibility of WTD scrapie and tissue phenotypes upon its further passage in WTD, three wildtype WTD (QQ95/GG96) were oronasally inoculated with WTD scrapie. These WTD presented with clinical signs and were euthanized between 21 and 26 months post-inoculation. Enzyme immunoassay (IDEXX) confirmed the presence of misfolded prion protein in the central nervous and lymphoreticular systems of all WTD in the study. Immunohistochemical staining, western blotting, and conformational stabilities were generally similar between the misfolded prion protein of WTD scrapie and CWD, though some differences were noted. Specifically, intraneuronal accumulation of misfolded prion protein was present in retinal ganglion cells of a WTD with WTD scrapie, not CWD. Additionally, epitope mapping revealed that the misfolded prion protein of CWD is slightly longer than that of WTD scrapie. Strong differences were seen in bioassays of cervidized mice, which exhibit significantly longer survival periods when inoculated with WTD scrapie as compared to those inoculated with CWD. Overall, this article establishes that WTD are highly susceptible to the WTD scrapie agent. Though subtle molecular differences exist between the misfolded prion protein of WTD scrapie and CWD, the presence of WTD scrapie in the lymphoreticular system determines that suspected cases be handled consistent with current guidelines for CWD.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=410511
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
Citation: Greenlee, J.J., Moore, S.J., Cassmann, E.D., Lambert, Z.J., Kokemuller, R., Smith, J.D., Kunkle, R.A., Kong, Q., West Greenlee, H.M. 2022. Characterization of classical sheep scrapie in white-tailed deer after experimental oronasal exposure. Journal of Infectious Diseases. 227(12):1386-1395. Article jiac443. https://doi.org/10.1093/infdis/jiac443.
DOI: https://doi.org/10.1093/infdis/jiac443
Interpretive Summary: Chronic Wasting Disease (CWD), a fatal neurodegenerative disease that occurs in farmed and wild cervids (deer and elk) of North America, is a transmissible spongiform encephalopathy (TSE). TSEs are caused by infectious proteins called prions that are resistant to decontamination and environmental degradation. The origin of chronic wasting disease is not known, but it has many similarities to the TSE of sheep called scrapie. It has long been hypothesized that CWD could have arisen through transmission of sheep scrapie to deer. The purpose of this study was to determine if scrapie derived from sheep could be transmitted to white-tailed deer. This study reports that the deer inoculated with sheep scrapie developed clinical signs of TSE and that the abnormal prion protein could be detected in a wide range of neural and lymphoid tissues. These results indicate that deer may be susceptible to sheep scrapie if exposed to the disease in natural or agricultural settings . In addition, several strong similarities between CWD in white-tailed deer and the experimental cases of scrapie in white-tailed deer in this report suggest that it would be difficult to identify scrapie in deer were a case to occur. This information should be considered when developing plans to reduce or eliminate TSEs or advising farmers that wish to keep their deer herds free from prion diseases.
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
ORIGIN OF CHRONIC WASTING DISEASE TSE PRION?COLORADO THE ORIGIN OF CHRONIC WASTING DISEASE CWD TSE PRION?
*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
IN CONFIDENCE, REPORT OF AN UNCONVENTIONAL SLOW VIRUS DISEASE IN ANIMALS IN THE USA 1989
http://webarchive.nationalarchives.gov.uk/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA viewed it as a wildlife problem and consequently not their province!” page 26.
https://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
***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
***> ”Our data suggest that the phenotype of CWD in sheep is indistinguishable from some strains of scrapie in sheep. Given our results, current detection techniques would be unlikely to distinguish CWD in sheep from scrapie in sheep if cross-species transmission occurred naturally.” https://pubmed.ncbi.nlm.nih.gov/34047228/
https://journals.sagepub.com/doi/full/10.1177/10406387211017615
THURSDAY, JANUARY 08, 2026
Confucius Ponders, what about Wild Pigs (Sus scrofa) and CWD TSE Prion, and the Environment, what if?
Confucius Ponders, what about Wild Pigs (Sus scrofa), they can cover some distance rather quickly, what about Wild Pigs (Sus scrofa) digging up the terrain, and as they do it, what if these Wild Pigs (Sus scrofa) were exposed to CWD TSE Prion, and then they go on exposing and saturating the land with CWD TSE Prion, then the soil becomes contaminated with CWD TSE Prion, then what about the plants that grow from that soil for the decades to come, what if???
https://prpsc.proboards.com/thread/190/confucius-ponders-wild-pigs-scrofa
https://chronic-wasting-disease.blogspot.com/2026/01/confucius-ponders-what-about-wild-pigs.html
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026
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.
https://assets.publishing.service.gov.uk/media/697a3b013c71d838df6bd413/CWD_Prions_in_Cervids_and_Wild_Pigs_in_North_America.pdf
Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry
"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."
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.
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
Component 6: Transmissible Spongiform Encephalopathies
Sheep scrapie agent can infect white-tailed deer after oronasal exposure.
The origin of chronic wasting disease (CWD) is not known, but it has many similarities to the sheep prion disease called scrapie. It has long been hypothesized that CWD arose through transmission of sheep scrapie to deer. ARS researchers in Ames, Iowa, conducted research to determine if scrapie derived from sheep could be transmitted to white-tailed deer. The deer inoculated with sheep scrapie developed clinical signs and the abnormal prion protein could be detected in a wide range of tissues. These results indicate that deer may be susceptible to sheep scrapie if exposed to the disease in natural or agricultural settings. In addition, several strong similarities between CWD in white-tailed deer and the experimental cases of scrapie in white-tailed deer suggests that it would be difficult to distinguish scrapie from CWD in deer or identify scrapie if a case occurs. This information should be considered by deer farmers for keeping their herds free from prion diseases.
https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/NP103%20FY2023%20Annual%20Report_Final.pdf
PLoS One. 2020 Aug 20;15(8):e0237410. doi: 10.1371/journal.pone.0237410. eCollection 2020.
Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease
Nathaniel D Denkers 1 , Clare E Hoover 2 , Kristen A Davenport 3 , Davin M Henderson 1 , Erin E McNulty 1 , Amy V Nalls 1 , Candace K Mathiason 1 , Edward A Hoover 1
PMID: 32817706 PMCID: PMC7446902 DOI: 10.1371/journal.pone.0237410
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.
Snip…
Discussion
As CWD expands across North America and Scandinavia, how this disease is transmitted so efficiently remains unclear, given the low concentrations of prions shed in secretions and excretions [13, 14]. The present studies demonstrated that a single oral exposure to as little as 300nmg of CWD-positive brain or equivalent saliva can initiate infection in 100% of exposed white-tailed deer. However, distributing this dose as 10, 30 ng exposures failed to induce infection. Overall, these results suggest that the minimum oral infectious exposure approaches 100 to 300 ng of CWD-positive brain equivalent. These dynamics also invite speculation as to whether potential infection co-factors, such as particle binding [46, 47] or compromises in mucosal integrity may influence infection susceptibility, as suggested from two studies in rodent models [48, 49].
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0237410
PRION 2023 CONTINUED;
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
Prion 2023 Experimental Oronasal Inoculation of the Chronic Wasting Disease Agent into White Tailed Deer
Aims: The purpose of this experiment was to determine whether white-tailed deer (WTD) are susceptible to inoculation of chronic wasting disease (CWD) via oronasal exposure.
Materials and methods: Six male, neutered WTD were oronasally inoculated with brainstem material (10% w/v) from a CWD-positive wild-type WTD. The genotypes of five inoculated deer were Q95/G96 (wild-type). One inoculated deer was homozygous S at codon 96 (96SS). Cervidized (Tg12; M132 elk PrP) mice were inoculated with 1% w/v brainstem homogenate from either a 96GG WTD (n=10) or the 96SS WTD (n=10).
Results: All deer developed characteristic clinical signs of CWD including weight loss, regurgitation, and ataxia. The 96SS individual had a prolonged disease course and incubation period compared to the other deer. Western blots of the brainstem on all deer yielded similar molecular profiles. All deer had widespread lymphoid distribution of PrPCWD and neuropathologic lesions associated with transmissible spongiform encephalopathies. Both groups of mice had a 100% attack rate and developed clinical signs, including loss of body condition, ataxia, and loss of righting reflex. Mice inoculated with material from the 96SS deer had a significantly shorter incubation period than mice inoculated with material from 96GG deer (Welch two sample T-test, P<0.05). Serial dilutions of each inocula suggests that differences in incubation period were not due to a greater concentration of PrPCWD in the 96SS inoculum. Molecular profiles from western blot of brain homogenates from mice appeared similar regardless of inoculum and appear similar to those of deer used for inoculum.
Conclusions: This study characterizes the lesions and clinical course of CWD in WTD inoculated in a similar manner to natural conditions. It supports previous findings that 96SS deer have a prolonged disease course. Further, it describes a first pass of inoculum from a 96SS deer in cervidized mice which shortened the incubation period.
=====end
PRION 2023 CONTINUED;
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
SCRAPIE TSE Prion USA RAPID RESPONSE URGENT UPDATES DECEMBER 25, 2025
https://scrapie-usa.blogspot.com/2025/12/scrapie-tse-prion-usa-rapid-response.html
https://prpsc.proboards.com/thread/186/scrapie-prion-response-urgent-updates
TUESDAY, SEPTEMBER 30, 2025
USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE 2025-2014 CHRONIC WASTING DISEASE CWD TSE CERVID
https://chronic-wasting-disease.blogspot.com/2025/09/usda-explanatory-notes-animal-and-plant.html
TUESDAY, SEPTEMBER 30, 2025
USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025
https://bovineprp.blogspot.com/2025/09/usda-national-scrapie-program-history.html
TUESDAY, SEPTEMBER 30, 2025
USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025
https://scrapie-usa.blogspot.com/2025/09/usda-national-scrapie-program-history.html
***> CWD Action Plan National Program 103 Animal Health 2022-2027 UPDATE JANUARY 2026
https://prpsc.proboards.com/thread/189/action-national-program-animal-health
https://chronic-wasting-disease.blogspot.com/2026/01/cwd-action-plan-national-program-103.html
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
2025 Annual Report
https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/ars-research-elucidating-pathobiology.html
Press release November 3, 2025 Contact: Marianne Gauldin, (334) 242-3469
Mandatory Testing Dates Announced for CWD Management Zone
Under Alabama’s Chronic Wasting Disease (CWD) Management Zone Regulation (220-2-.167), all deer harvested in the High-Risk Zone and the Buffer Zone of the state’s CWD Management Zone (CMZ) must be submitted for CWD testing during specific weekends of the 2025-2026 white-tailed deer season.
The mandatory sampling weekends in the High-Risk Zone of the CMZ are November 22-23, 2025; December 6-7, 2025; and January 17-18, 2026. The mandatory sampling weekends in the Buffer Zone of the CMZ are November 22-23, 2025, and January 17-18, 2026. The High-Risk Zone contains all of Lauderdale and Colbert counties and part of Franklin County, extending north of State Highway 24 from the Alabama/Mississippi state line east to U.S. Highway 43. The Buffer Zone includes the remaining part of Franklin County south of Highway 24 and east of U.S. Highway 43.
Mandatory weekends for the High-Risk Zone and Buffer Zone correspond with peaks in Alabama’s deer harvest, the season’s opening weekend and the rut in northwest Alabama. Outside of those weekends, hunters are encouraged to voluntarily drop off samples for testing at the self-service freezers located in the appropriate CMZ zone. For the remainder of the state outside of the CMZ, hunters are also encouraged to voluntarily drop off samples for testing at self-service freezer locations. Sampling locations throughout the state can be found at www.outdooralabama.com/cwd/cwd-zone-map.
All deer harvested by hunters on certain public land in the CMZ are required to be sampled throughout the season. Those public lands include the Freedom Hills, Lauderdale, and Seven-Mile Island wildlife management areas (WMAs); the Cherokee Physically Disabled Hunting Area; and the Riverton Community Hunting Area.
As an incentive to increase CWD samples submitted for testing on scheduled mandatory testing dates, hunters who harvest a deer from within the CMZ and submit their harvest for CWD testing on the mandatory sampling dates of November 22-23, 2025, and January 17-18, 2026, are eligible to receive a CWD Sampling Permit from ADCNR to harvest one additional antlered deer from within the CMZ for each sample submitted. CWD Sampling Permits are only available at WMA check stations and ADCNR mobile sampling locations. No more than two additional CWD Sampling Permits will be issued per hunter above the season bag limit, combined for the High-Risk and Buffer zones. CWD Sampling Permits are non-transferable.
Carcass restrictions are in place under the CWD regulation that prohibit the transport of harvested deer from the CMZ to areas outside the Buffer or High-Risk zones. Deer harvested within the High-Risk Zone must remain and be disposed of within the High-Risk Zone. Deer harvested within the Buffer Zone must remain and be disposed of within the CMZ. Deboned meat, cleaned skull plates and raw hides with no visible brain or spinal cord tissue may be taken outside of these zones. Transporting deer carcasses out of the management zone can potentially spread CWD to currently unaffected areas.
Hunting license and Game Check requirements apply to all white-tailed deer harvests statewide.
CWD is a contagious neurological disease of white-tailed deer and other deer species. It belongs to a group of diseases known as transmissible spongiform encephalopathies. The disease is caused by a mutated protein called a prion. It is always fatal for white-tailed deer. The first case of CWD in Alabama’s deer herd was detected in Lauderdale County in January 2022.
For more information about CWD in Alabama, visit www.outdooralabama.com/cwd-info.
ADCNR promotes wise stewardship, management and enjoyment of Alabama’s natural resources through four divisions: Marine Resources, State Lands, State Parks, and Wildlife and Freshwater Fisheries. Learn more at www.outdooralabama.com.
###
CWD_Map_Updated_9.28.25.jpg
https://www.outdooralabama.com/cwd-chronic-wasting-disease/cwd-alabama
Alabama CWD Report 12 Cases Confirmed To Date
Alabama Deer Program Report
Courtenay Conring Assistant Chief
I. Harvest/Population Trends
• Alabama hunters reported harvesting 205,913 deer through Game Check during the 2024-2025 hunting season.
• The 2024-25 harvest consisted of 54.7% bucks and 45.3% does.
• Game Check reports were up 1.8% from the 2023- 24 season.
• Game Check compliance for 2023-2024 was estimated at 64.3%. Compliance has not been estimated for 2024-2025.
• 2023-24 harvest estimates generated by the hunter phone survey were:
o 314,496 total deer harvested (49.7% bucks, 50.3% does).
o Up 1.8% from 2022-23 season.
o Up 18.7% from 10-year average.
o 2024-2025 hunter phone survey is in progress.
II. Research
Monitoring Alabama White-tailed Deer Populations to Inform Future Decision Making Working with researchers at Alabama Cooperative Fish and Wildlife Research Unit and Auburn University to develop a modeling, monitoring, and decision analysis framework to inform our understanding of status and trends in WTD abundance and herd composition / population structure and for informing WTD harvest-management decision making at both larger (zone-level) and property-level (e.g., WMA). Information gained from this project will be incorporated into our Statewide Deer Management Plan’s decision support tools, with future phases incorporating impacts of CWD.
III. Regulation Changes/Updates
Nuisance – Deer Depredation Permits and Unantlered Hunting Bag Limits
There has been increased questioning and pressure with Alabama’s legislature, Conservation Advisory Board, and some ag producers regarding issuance of Deer Depredation Permits (DDP). From 2021-2024, all Wildlife Section Districts have had stable to increasing trends of DDP issued and deer permitted to take under the DDP. According to the Alabama Hunter Harvest Annual Reports, statewide harvest has also increased from ~301,000 to 314,000 during the same three-year time frame. In the management zones where depredation data and harvest estimates suggest that deer density is stable or increasing, the daily bag limit of two deer per day remains the same, but hunters can now choose to take one unantlered deer and one antlered deer per day, or two unantlered deer per day. This shift in bag limit structure allows the opportunity for hunters to be a more impactful management option for the ag producers while also increasing hunter satisfaction. Additionally, allowing the harvest of an extra antlerless deer per day could lead to an increase in chronic wasting disease (CWD) sampling opportunity and improve surveillance efforts.
Changes to Game Breeder’s License State Statute (HB509)
Amendments were made to Section 9-11-30, Code of Alabama 1975, to include disease-related language. See Disease Issues/Updates section for details.
IV. Disease Issues / Updates- Olivia Sciandra, Wildlife Health Program Coordinator
• Chronic Wasting Disease New Detection Updates
o During the 2024-2025 season, there was a novel detection of CWD in Colbert County, resulting in the previous CWD management zone (CMZ) expanding to include Franklin County. The CMZ high risk zone now encompasses all of Lauderdale, Colbert, and the northwest corner of Franklin County (north of US-24 and west of US-43). The CMZ
Alabama Deer Program Report Courtenay Conring Assistant Chief
buffer zone is the remainder of Franklin County. After the initial detection in Colbert County, the increased surveillance around the initial CWD-positive yielded no additional detections to date.
• CWD Regulation/Statute Changes
o After the inclusion of Franklin County to the CMZ, there was an update to the 220-2- .167 regulation which allowed feeding/baiting in Franklin County for the remainder of the 24-25 season. In the 25-26 season, feeding and baiting will be suspended in Franklin County. We are working to update the 220-2- .167 regulation so that new counties added to the CMZ mid-season will be allowed to feed and bait for the remainder of that season with the suspension of these activities being initiated in the following season.
o HB509 passed during the 2025 legislative session. Our department will adopt rules in consultation with the State Veterinarian on or before March 1, 2026, to allow for the transfer of cervids from a licensee within a designated disease management zone to another licensee outside of a designated disease management zone under the following circumstances including, but not limited to, that the transferring licensee complies with all postmortem testing programs and:
▪ Double fencing of pens and other standards for pens are followed
▪ A USDA certified live test for CWD is used
▪ Scientifically accepted genomic testing values are satisfied
▪ Or any other test approved by the USDA and recommended by the State Veterinarian is used.
o Additionally, the passage of HB509 will result in the change to Alabama’s CWD Strategic Surveillance and Response Plan which previously required the postmortem testing of 10% of a licensee’s herd if a CWD-positive wild detection occurred within 10-miles of a game breeder facility. ADCNR now does not have the ability to require the killing of a cervid due to disease unless:
▪ The specific disease has been detected in another cervid possessed by the licensee
▪ OR the cervid has been transferred to the licensee from another licensee and is epidemiologically linked to a diseased cervid possessed by the transferring licensee.
• CWD Sampling
o 2024-2025 Statewide Surveillance
▪ 3,170 white-tailed deer sampled statewide (2,891 hunter harvest, 236 roadkill, 26 symptomatic, and 17 “other”.
▪ 1,312 does, 1,857 bucks (1 of unknown sex)
▪ 2,540 adults, 524 yearlings, and 106 fawns
▪ In the CMZ, 1,134 deer were sampled
• 687 of these samples (61%) were obtained during the three mandatory CWD weekends (one each in November, December, and January).
Year of Harvest Date Total
County 2021 2022 2023 2024 2025
Lauderdale 1 1 3 5 1 11
Colbert 0 0 0 1 0 1
Franklin 0 0 0 0 0 0
Total 1 1 3 6 1 12
https://seafwa.org/sites/default/files/public-files/2025-seafwa-cervid-working-group-report.pdf
WEDNESDAY, SEPTEMBER 24, 2025
Alabama CWD Report 12 Cases Confirmed To Date
https://chronic-wasting-disease.blogspot.com/2025/09/alabama-cwd-report-12-cases-confirmed.html
Georgia, Alabama Wildlife Agencies Update CWD Situation In The Two States During Webinar Andrew Curtis | July 28, 2025
Alabama and Georgia are testing hunter-harvested deer for CWD. On July 16, the Georgia-Alabama Land Trust (GALT) hosted a free webinar to discuss updates on Chronic Wasting Disease (CWD) in the two states. The speakers were Olivia Sciandra from the Alabama Department of Conservation and Natural Resources and Kevin Rose, the assistant state deer biologist from the Georgia Department of Natural Resources.
Georgia has had a total of two positive cases of CWD in wild deer near the Berrien-Lanier county line. The first Georgia diagnosis came in a hunter-killed buck in December 2024.
Alabama has confirmed 12 CWD cases since January 2022, all from two adjacent counties in the northwest corner of the state.
Sciandra began the program by reviewing the CWD disease process and then went into what Alabama has been doing to manage the prion disease since its discovery in Alabama back in 2022.
Both states are using similar approaches, but one of the main differences is that Alabama implemented a bait and feed ban in its CWD Management Area (not statewide). Georgia, however, has made no changes to the baiting regulations, but there are four recommendations that Georgia hunters and landowners are encouraged to follow: broadcast feed over at least 500 square feet of ground, avoid feed troughs, gravity feeders or ground piles, move the bait pile periodically, and bait only while hunting.
For those who have followed the Georgia CWD updates since January, not much has changed. Rose reiterated the benefits of Georgia’s being the 36th state to diagnose CWD.
“We have seen what programs work to manage CWD in deer herds in 35 states over the past 50 plus years,” he said.
Georgia DNR has planned for this since 2002, and in 2019, a risk-based surveillance method developed by Cornell University was implemented in Georgia, resulting in the first positive case being detected this past deer season. Rose stated, “Our response plan has evolved over 22 years of monitoring the 35 states that found it before us. CWD is likely here to stay and will require continual monitoring and adaptive management long term.”
Georgia DNR has established a CWD Management Zone that encompasses Lanier and Berrien counties. Many people have asked what will happen if CWD is discovered in another area of Georgia.
“CWD spread is inevitable, and if we detect CWD somewhere else in the state outside the CWD Management Area (CMA), then we will implement cluster sampling in that area like we are currently doing,” Rose explained. “Cluster sampling (harvesting five to seven deer per herd) is the most effective way to manage the spread of disease, and it is intentionally designed to not significantly reduce the population.”
The goal is to manage CWD in a way to not affect hunting, since hunting is such an integral part of our state’s heritage.
This year a Georgia statewide rule was put into effect instructing how to dispose of deer carcasses to prevent the spread of CWD. There are several options listed in the Carcass Disposal Rule: leave the carcass on the property where the deer was killed since that property would already be considered contaminated; use a commercial deer processor; take the carcass to a permitted solid waste landfill or dispose in household garbage, which will end up in the landfills; bury the carcass at least 3 feet below the surface; use a commercial incinerator; or any other approved method listed in the Dead Animal Disposal Act.
All deer harvested in the CWD Management Area are encouraged to be tested. Results from CWD Management Area deer should take two to six weeks, and there will be no charge to the hunter. Deer outside of the CMA that are tested may take months because they are lower priority, and these tests will require payment from the hunter. Test locations will be displayed on an interactive map at georgiawildlife.com/cwd. If a positive result occurs, the hunter will receive a direct phone call and an email before the result is posted on the website dashboard.
Remember, CWD has never been shown to cross over into humans, but the CDC still advises to avoid contaminated meat.
For more information, visit Georgia DNR’s CWD website at georgiawildlife.com/cwd.
https://gon.com/news/georgia-alabama-wildlife-agencies-update-cwd-situation-in-the-two-states-during-webinar
Press release December 23, 2024 Contact: Marianne Gauldin, (334) 242-1814
CWD Detection in Colbert County Expands the State’s CWD Management Zone
A white-tailed deer recently harvested by a hunter in Colbert County in northwest Alabama has been confirmed positive for Chronic Wasting Disease (CWD). The detection of CWD in Colbert County has resulted in an expansion of the state’s CWD Management Zone (CMZ) to include all of Colbert, Lauderdale and Franklin counties in the CMZ.
A portion of Franklin County has been included in the High-Risk Zone (HRZ) within the CMZ. The HRZ portion of Franklin County extends north of State Highway 24 from the Alabama/Mississippi state line east to U.S. Highway 43 (see attached map). The remainder of Franklin County south of State Highway 24 and east of U.S. Highway 43 has been designated as a Buffer Zone within the CMZ.
“I would like to thank our hunters for submitting their harvest samples for testing,” said Chris Blankenship, Commissioner of the Alabama Department of Conservation and Natural Resources (ADCNR). “They are our most important partners in the management of this disease in Alabama.”
Preliminary tests performed at the Alabama Department of Agriculture and Industries (ADAI) discovered the non-negative case in Colbert County. The non-negative result was then confirmed positive by the National Veterinary Services Laboratories in Ames, Iowa. This is the first case of CWD detected in Colbert County and brings the total number of CWD positive detections to nine statewide.
CWD is a contagious neurological disease of white-tailed deer and other deer species. It belongs to a group of diseases known as transmissible spongiform encephalopathies. The disease is caused by a mutated protein called a prion. It is always fatal for deer. The first case of CWD in Alabama’s deer herd was detected in Lauderdale County in January 2022.
During the remainder of deer gun season on privately owned or leased land within the CMZ, the bag limit for unantlered deer has been increased to two per day.
Hunters are required to submit heads for CWD testing from all deer harvested within the CMZ at drop-off freezer locations or at scheduled ADCNR mobile sampling stations during the next mandatory sampling weekend on January 11-12, 2025. The mandatory sampling weekend applies to all of Lauderdale, Colbert and Franklin counties. Hunters are reminded that carcasses and other deer parts harvested within the HRZ must remain in the HRZ. Carcasses or other deer parts cannot be moved outside the CMZ.
Additionally, supplemental wildlife feeding and baiting privileges are not allowed within the CMZ. The supplemental feeding rule does not apply to bird feeders within 100 feet of a building or occupied dwelling or feed inside an active feral hog trap. Supplemental feeding and baiting privileges are allowed outside of the CMZ.
For more information about CWD in Alabama, please visit www.outdooralabama.com/cwd-info.
ADCNR promotes wise stewardship, management and enjoyment of Alabama’s natural resources through four divisions: Marine Resources, State Lands, State Parks and Wildlife and Freshwater Fisheries. Learn more at www.outdooralabama.com.
###
CWD Zone Map 12-23-24.jpg
https://www.outdooralabama.com/articles/cwd-detection-colbert-county-expands-states-cwd-management-zone
*** Alabama CWD TSE Prion 2023
(2020, Alabama, to date, has detected NO cases of CWD TSE Prion...tss)
*** Alabama CWD TSE Prion 2023 TO DATE 5 CASES CONFIRMED
Alabama Two Additional Cases of CWD Confirmed in Northern Lauderdale County
Press release December 15, 2023 Contact: Marianne Gauldin, (334) 242-3469
Alabama Two Additional Cases of CWD Confirmed in Northern Lauderdale County
Two Additional Cases of CWD Confirmed in Northern Lauderdale County The Alabama Department of Conservation and Natural Resources (ADCNR) announces that two additional cases of chronic wasting disease (CWD) in hunter harvested, white-tailed deer have been confirmed in northern Lauderdale County in northwest Alabama. The two additional deer bring Alabama’s total number of confirmed CWD cases to five.
CWD in Alabama’s deer herd was first detected in Lauderdale County in January 2022. After the first case was confirmed, all of Lauderdale and Colbert counties were designated as a CWD Management Zone (CMZ).
So far during the 2023-2024 hunting season, samples have been collected from more than 1,700 white-tailed deer harvested statewide with 420 of those samples collected within the CMZ. One of the positive samples was submitted during the second CMZ mandatory sampling weekend (December 2-3). The other positive sample was voluntarily submitted at a drop-off sampling location by a hunter as part of ADCNR's ongoing CWD monitoring efforts. The next mandatory sampling weekend in the buffer zone of the CMZ is January 6-7, 2024.
“I would like to thank hunters for their continued support by providing a robust number of samples for CWD testing since the disease was first detected in Alabama,” said Chris Blankenship, ADCNR Commissioner. “Hunters are our most important partners in the management of CWD as we move forward with future deer seasons. We also thank the Alabama Department of Agriculture and Industries for their continued partnership with statewide CWD monitoring. Their assistance by testing the samples allows us to better serve our constituents by providing them with timely information on the distribution and extent of CWD in Alabama.”
CWD is a member of the group of diseases called transmissible spongiform encephalopathies (TSEs). Among cervids, CWD is a progressive, fatal disease that commonly results in altered behavior due to microscopic changes of the brain of affected animals. An animal may carry the disease for years without outward indication. In latter stages of the disease, signs may include listlessness, lowering of the head, weight loss, repetitive walking in set patterns and a lack of responsiveness.
It is important that hunters be familiar with Alabama’s CWD regulation and the CWD regulations in other states. To review Alabama’s regulation and the latest information about CWD in the state, visit www.outdooralabama.com/cwd-info.
ADCNR promotes wise stewardship, management and enjoyment of Alabama’s natural resources through four divisions: Marine Resources, State Lands, State Parks, and Wildlife and Freshwater Fisheries. Learn more at www.outdooralabama.com.
###
CMZ map attached (includes locations of positive cases)
CMZ zone map 12-15-23.jpg
https://www.outdooralabama.com/sites/default/files/CMZ%20zone%20map%2012-15-23.jpg
https://www.outdooralabama.com/articles/two-additional-cases-cwd-confirmed-northern-lauderdale-county
https://www.outdooralabama.com/articles/mandatory-cwd-testing-dates-announced-lauderdale-and-colbert-counties
Posted: February 16, 2023
Third Case of CWD Confirmed in Lauderdale County
The Alabama Department of Conservation and Natural Resources (ADCNR) announces that a third case of Chronic Wasting Disease (CWD) in a hunter harvested, white-tailed deer has been confirmed in Lauderdale County in northwest, Alabama. The first two cases of CWD in Alabama’s deer herd were detected in Lauderdale County in early 2022.
Samples were collected from more than 3,500 white-tailed deer harvested statewide with over 1,100 of those samples collected within the CMZ during the 2022-2023 hunting season. More than 98% of all samples collected within the CMZ have been tested by the Alabama Department of Agriculture and Industries and the results have been received by ADCNR. Currently, only one positive has been detected this season. The positive sample was voluntarily submitted by a hunter as part of ADCNR's ongoing CWD monitoring efforts.
https://www.outdooralabama.com/articles/third-case-cwd-confirmed-lauderdale-county
http://www.outdooralabama.com/cwd/latest-cwd-information
https://chronic-wasting-disease.blogspot.com/2023/02/alabama-third-case-of-cwd-confirmed-in.html
https://chronic-wasting-disease.blogspot.com/
FRIDAY, JANUARY 18, 2019
Alabama WFF Ramps Up Chronic Wasting Disease CWD TSE Prion Sampling Effort
https://chronic-wasting-disease.blogspot.com/2019/01/alabama-wff-ramps-up-chronic-wasting.html
THURSDAY, JULY 20, 2017
Alabama Atypical BSE CJD CWD TSE Prion Update
https://bseusa.blogspot.com/2017/07/alabama-atypical-bse-cjd-cwd-tse-prion.html
TUESDAY, MARCH 29, 2016
ALABAMA CHRONIC WASTING DISEASE CWD TSE PRION SURVEILLANCE AND TESTING PROGRAM?
https://chronic-wasting-disease.blogspot.com/2016/03/alabama-chronic-wasting-disease-cwd-tse.html
THURSDAY, NOVEMBER 01, 2012
ALABAMA BIG BUCK PROJECT, A CWD TSE PRION ACCIDENT WAITING TO HAPPEN ALABAMA BIG BUCK PROJECT, A CWD ACCIDENT WAITING TO HAPPEN
http://chronic-wasting-disease.blogspot.com/2012/11/alabama-big-buck-project-cwd-tse-prion.html
***> CWD TSE PrP Environmental Factors
Chronic wasting disease (CWD) prion detection in environmental and biological samples from a taxidermy site and nursing facility, and instruments used in surveillance activities
Available online 9 April 2025
Highlights
• CWD prions were identified in a taxidermy and deer nursing facility.
• Contaminated samples included waters, soils, dermestid beetles, domestic flies and a dumpster.
• Surgical instruments used to collect deer samples can get contaminated with CWD prions.
• Some of the infectious particles are readily released from surgical instruments when washed.
• Our results suggest that taxidermy practices actively contribute in the spreading of CWD.
Snip…
In summary, the information provided in this report demonstrate how anthropogenic activities, specifically taxidermy practices, animal processing, and rehabilitation of CWD susceptible species, may facilitate CWD transmission through the environmental dissemination of CWD prions. This study, along with future research efforts characterizing the overall level of infectivity, provides relevant information on managing CWD and to control its rapid geographic expansion. …
https://www.sciencedirect.com/science/article/abs/pii/S0048969725009544
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
Artificial mineral sites that pre-date endemic chronic wasting disease become prion hotspots
The Ames Research and Educational Center property, centrally located within the CWD zone of southwest Tennessee, contains 49 historical mineral supplementation sites that were decommissioned in 2012. Here, we demonstrate that 32 of the 49 (65%) mineral sites within Ames established prior to the regional CWD outbreak, serve as foci of environmental PrPCWD contamination. Detection of PrPCWD in soils from these artificial mineral sites was dependent on site-specific management efforts. Soil physical properties were very similar across sites and no correlation between PrPCWD detection and soil physical properties was found. 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://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf
Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer
Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.
Prion 2022 Conference abstracts: pushing the boundaries
https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286
Front. Vet. Sci., 14 September 2015 | 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.
journal.frontiersin.org/article/10.3389/fvets.2015.00032/full
SUNDAY, APRIL 06, 2025
Failure to prevent classical scrapie after repeated decontamination of a barn
scrapie-usa.blogspot.com/2025/04/failure-to-prevent-classical-scrapie.html
prpsc.proboards.com/thread/165/failure-prevent-scrapie-repeated-decontamination
"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
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.
web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
***> 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/
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…
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. In fact new data now shows that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification.
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/
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
Chronic wasting disease prions on deer feeders and wildlife visitation to deer feeding areas
First published: 10 February 2025
Snip…
Finally, we swabbed 19 feeders in 2 areas where CWD was newly detected, finding prion contamination on swabs from 4 feeders. We show that deer feeders in free-ranging populations with high CWD prevalence become contaminated with CWD prions quickly, becoming a potential site of exposure of deer to CWD prions. Our results also demonstrate the ability to find evidence of prion contamination on deer feeders, even in areas where CWD is newly detected.
Snip…
We found that supplemental feeding increased the risk of exposure to CWD prions due to contamination of feeders, increased deer visitation, and increased deer-to-deer contact.
The 12-fold increase in deer visitation to feeders compared to mast trees and 2-fold increase compared to food plots demonstrates increased risk for direct disease spread.
https://wildlife.onlinelibrary.wiley.com/doi/10.1002/jwmg.70000
Chronic Wasting Disease in Texas A Real Disease with Proven Impacts
Produced by a coalition of concerned hunters, landowners, & conservationists (last update 1/2025)
storymaps.arcgis.com/stories/b93f528938ac48e9b56dcc79953cbec0
***> Human CWD TSE PrP, what if?
the problem is, to date, there is NO diagnostic criteria set in stone that would confirm a case of human Cwd, like there was with nvCJD (my Mom died from confirmed hvCJD a rare strain of the infamous sporadic CJDs with new strains mounting, sporadic CJD simply means ‘unknown’, IT DOES NOT MEAN 85%+ SPORADIC CJD IS ALL SPONTANEOUS, that’s all iatrogenic CJD is sporadic CJD, until the iatrogenic event is detected, confirmed, traced back, confirmed, put I to the academic domain, and finally, if your lucky, finally published to the media, and finally the public domain.) sorry, I got off course…but let me perfectly clear here, all science to date shows, Human CWD will not look like New Variant Creutzfeldt Jakob disease nvCJD. CWD to humans will look like some variant of sporadic Creutzfeldt Jakob Disease. And here me out very clearly, and this is from the to TSE Prion Gods themselves, old correspondence from way back during my investigations early BSE nvCJD days…2002
“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.”
*** 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”
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
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
-----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, however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41].
snip... full text ;
https://www.vetres.org/articles/vetres/abs/2008/04/v08092/v08092.html
https://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
“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
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 ############
*** 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
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
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
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
Alabama CWD Report 12 Cases Confirmed To Date
Alabama Deer Program Report
Courtenay Conring Assistant Chief
I. Harvest/Population Trends
• Alabama hunters reported harvesting 205,913 deer through Game Check during the 2024-2025 hunting season.
• The 2024-25 harvest consisted of 54.7% bucks and 45.3% does.
• Game Check reports were up 1.8% from the 2023- 24 season.
• Game Check compliance for 2023-2024 was estimated at 64.3%. Compliance has not been estimated for 2024-2025.
• 2023-24 harvest estimates generated by the hunter phone survey were:
o 314,496 total deer harvested (49.7% bucks, 50.3% does).
o Up 1.8% from 2022-23 season.
o Up 18.7% from 10-year average.
o 2024-2025 hunter phone survey is in progress.
II. Research
Monitoring Alabama White-tailed Deer Populations to Inform Future Decision Making Working with researchers at Alabama Cooperative Fish and Wildlife Research Unit and Auburn University to develop a modeling, monitoring, and decision analysis framework to inform our understanding of status and trends in WTD abundance and herd composition / population structure and for informing WTD harvest-management decision making at both larger (zone-level) and property-level (e.g., WMA). Information gained from this project will be incorporated into our Statewide Deer Management Plan’s decision support tools, with future phases incorporating impacts of CWD.
III. Regulation Changes/Updates
Nuisance – Deer Depredation Permits and Unantlered Hunting Bag Limits
There has been increased questioning and pressure with Alabama’s legislature, Conservation Advisory Board, and some ag producers regarding issuance of Deer Depredation Permits (DDP). From 2021-2024, all Wildlife Section Districts have had stable to increasing trends of DDP issued and deer permitted to take under the DDP. According to the Alabama Hunter Harvest Annual Reports, statewide harvest has also increased from ~301,000 to 314,000 during the same three-year time frame. In the management zones where depredation data and harvest estimates suggest that deer density is stable or increasing, the daily bag limit of two deer per day remains the same, but hunters can now choose to take one unantlered deer and one antlered deer per day, or two unantlered deer per day. This shift in bag limit structure allows the opportunity for hunters to be a more impactful management option for the ag producers while also increasing hunter satisfaction. Additionally, allowing the harvest of an extra antlerless deer per day could lead to an increase in chronic wasting disease (CWD) sampling opportunity and improve surveillance efforts.
Changes to Game Breeder’s License State Statute (HB509)
Amendments were made to Section 9-11-30, Code of Alabama 1975, to include disease-related language. See Disease Issues/Updates section for details.
IV. Disease Issues / Updates- Olivia Sciandra, Wildlife Health Program Coordinator
• Chronic Wasting Disease New Detection Updates
o During the 2024-2025 season, there was a novel detection of CWD in Colbert County, resulting in the previous CWD management zone (CMZ) expanding to include Franklin County. The CMZ high risk zone now encompasses all of Lauderdale, Colbert, and the northwest corner of Franklin County (north of US-24 and west of US-43). The CMZ
Alabama Deer Program Report Courtenay Conring Assistant Chief
buffer zone is the remainder of Franklin County. After the initial detection in Colbert County, the increased surveillance around the initial CWD-positive yielded no additional detections to date.
• CWD Regulation/Statute Changes
o After the inclusion of Franklin County to the CMZ, there was an update to the 220-2- .167 regulation which allowed feeding/baiting in Franklin County for the remainder of the 24-25 season. In the 25-26 season, feeding and baiting will be suspended in Franklin County. We are working to update the 220-2- .167 regulation so that new counties added to the CMZ mid-season will be allowed to feed and bait for the remainder of that season with the suspension of these activities being initiated in the following season.
o HB509 passed during the 2025 legislative session. Our department will adopt rules in consultation with the State Veterinarian on or before March 1, 2026, to allow for the transfer of cervids from a licensee within a designated disease management zone to another licensee outside of a designated disease management zone under the following circumstances including, but not limited to, that the transferring licensee complies with all postmortem testing programs and:
▪ Double fencing of pens and other standards for pens are followed
▪ A USDA certified live test for CWD is used
▪ Scientifically accepted genomic testing values are satisfied
▪ Or any other test approved by the USDA and recommended by the State Veterinarian is used.
o Additionally, the passage of HB509 will result in the change to Alabama’s CWD Strategic Surveillance and Response Plan which previously required the postmortem testing of 10% of a licensee’s herd if a CWD-positive wild detection occurred within 10-miles of a game breeder facility. ADCNR now does not have the ability to require the killing of a cervid due to disease unless:
▪ The specific disease has been detected in another cervid possessed by the licensee
▪ OR the cervid has been transferred to the licensee from another licensee and is epidemiologically linked to a diseased cervid possessed by the transferring licensee.
• CWD Sampling
o 2024-2025 Statewide Surveillance
▪ 3,170 white-tailed deer sampled statewide (2,891 hunter harvest, 236 roadkill, 26 symptomatic, and 17 “other”.
▪ 1,312 does, 1,857 bucks (1 of unknown sex)
▪ 2,540 adults, 524 yearlings, and 106 fawns
▪ In the CMZ, 1,134 deer were sampled
• 687 of these samples (61%) were obtained during the three mandatory CWD weekends (one each in November, December, and January).
Year of Harvest Date Total
County 2021 2022 2023 2024 2025
Lauderdale 1 1 3 5 1 11
Colbert 0 0 0 1 0 1
Franklin 0 0 0 0 0 0
Total 1 1 3 6 1 12
https://seafwa.org/sites/default/files/public-files/2025-seafwa-cervid-working-group-report.pdf
WEDNESDAY, SEPTEMBER 24, 2025
Alabama CWD Report 12 Cases Confirmed To Date
https://chronic-wasting-disease.blogspot.com/2025/09/alabama-cwd-report-12-cases-confirmed.html
Georgia, Alabama Wildlife Agencies Update CWD Situation In The Two States During Webinar Andrew Curtis | July 28, 2025
Alabama and Georgia are testing hunter-harvested deer for CWD. On July 16, the Georgia-Alabama Land Trust (GALT) hosted a free webinar to discuss updates on Chronic Wasting Disease (CWD) in the two states. The speakers were Olivia Sciandra from the Alabama Department of Conservation and Natural Resources and Kevin Rose, the assistant state deer biologist from the Georgia Department of Natural Resources.
Georgia has had a total of two positive cases of CWD in wild deer near the Berrien-Lanier county line. The first Georgia diagnosis came in a hunter-killed buck in December 2024.
Alabama has confirmed 12 CWD cases since January 2022, all from two adjacent counties in the northwest corner of the state.
Sciandra began the program by reviewing the CWD disease process and then went into what Alabama has been doing to manage the prion disease since its discovery in Alabama back in 2022.
Both states are using similar approaches, but one of the main differences is that Alabama implemented a bait and feed ban in its CWD Management Area (not statewide). Georgia, however, has made no changes to the baiting regulations, but there are four recommendations that Georgia hunters and landowners are encouraged to follow: broadcast feed over at least 500 square feet of ground, avoid feed troughs, gravity feeders or ground piles, move the bait pile periodically, and bait only while hunting.
For those who have followed the Georgia CWD updates since January, not much has changed. Rose reiterated the benefits of Georgia’s being the 36th state to diagnose CWD.
“We have seen what programs work to manage CWD in deer herds in 35 states over the past 50 plus years,” he said.
Georgia DNR has planned for this since 2002, and in 2019, a risk-based surveillance method developed by Cornell University was implemented in Georgia, resulting in the first positive case being detected this past deer season. Rose stated, “Our response plan has evolved over 22 years of monitoring the 35 states that found it before us. CWD is likely here to stay and will require continual monitoring and adaptive management long term.”
Georgia DNR has established a CWD Management Zone that encompasses Lanier and Berrien counties. Many people have asked what will happen if CWD is discovered in another area of Georgia.
“CWD spread is inevitable, and if we detect CWD somewhere else in the state outside the CWD Management Area (CMA), then we will implement cluster sampling in that area like we are currently doing,” Rose explained. “Cluster sampling (harvesting five to seven deer per herd) is the most effective way to manage the spread of disease, and it is intentionally designed to not significantly reduce the population.”
The goal is to manage CWD in a way to not affect hunting, since hunting is such an integral part of our state’s heritage.
This year a Georgia statewide rule was put into effect instructing how to dispose of deer carcasses to prevent the spread of CWD. There are several options listed in the Carcass Disposal Rule: leave the carcass on the property where the deer was killed since that property would already be considered contaminated; use a commercial deer processor; take the carcass to a permitted solid waste landfill or dispose in household garbage, which will end up in the landfills; bury the carcass at least 3 feet below the surface; use a commercial incinerator; or any other approved method listed in the Dead Animal Disposal Act.
All deer harvested in the CWD Management Area are encouraged to be tested. Results from CWD Management Area deer should take two to six weeks, and there will be no charge to the hunter. Deer outside of the CMA that are tested may take months because they are lower priority, and these tests will require payment from the hunter. Test locations will be displayed on an interactive map at georgiawildlife.com/cwd. If a positive result occurs, the hunter will receive a direct phone call and an email before the result is posted on the website dashboard.
Remember, CWD has never been shown to cross over into humans, but the CDC still advises to avoid contaminated meat.
For more information, visit Georgia DNR’s CWD website at georgiawildlife.com/cwd.
https://gon.com/news/georgia-alabama-wildlife-agencies-update-cwd-situation-in-the-two-states-during-webinar
Press release December 23, 2024 Contact: Marianne Gauldin, (334) 242-1814
CWD Detection in Colbert County Expands the State’s CWD Management Zone
A white-tailed deer recently harvested by a hunter in Colbert County in northwest Alabama has been confirmed positive for Chronic Wasting Disease (CWD). The detection of CWD in Colbert County has resulted in an expansion of the state’s CWD Management Zone (CMZ) to include all of Colbert, Lauderdale and Franklin counties in the CMZ.
A portion of Franklin County has been included in the High-Risk Zone (HRZ) within the CMZ. The HRZ portion of Franklin County extends north of State Highway 24 from the Alabama/Mississippi state line east to U.S. Highway 43 (see attached map). The remainder of Franklin County south of State Highway 24 and east of U.S. Highway 43 has been designated as a Buffer Zone within the CMZ.
“I would like to thank our hunters for submitting their harvest samples for testing,” said Chris Blankenship, Commissioner of the Alabama Department of Conservation and Natural Resources (ADCNR). “They are our most important partners in the management of this disease in Alabama.”
Preliminary tests performed at the Alabama Department of Agriculture and Industries (ADAI) discovered the non-negative case in Colbert County. The non-negative result was then confirmed positive by the National Veterinary Services Laboratories in Ames, Iowa. This is the first case of CWD detected in Colbert County and brings the total number of CWD positive detections to nine statewide.
CWD is a contagious neurological disease of white-tailed deer and other deer species. It belongs to a group of diseases known as transmissible spongiform encephalopathies. The disease is caused by a mutated protein called a prion. It is always fatal for deer. The first case of CWD in Alabama’s deer herd was detected in Lauderdale County in January 2022.
During the remainder of deer gun season on privately owned or leased land within the CMZ, the bag limit for unantlered deer has been increased to two per day.
Hunters are required to submit heads for CWD testing from all deer harvested within the CMZ at drop-off freezer locations or at scheduled ADCNR mobile sampling stations during the next mandatory sampling weekend on January 11-12, 2025. The mandatory sampling weekend applies to all of Lauderdale, Colbert and Franklin counties. Hunters are reminded that carcasses and other deer parts harvested within the HRZ must remain in the HRZ. Carcasses or other deer parts cannot be moved outside the CMZ.
Additionally, supplemental wildlife feeding and baiting privileges are not allowed within the CMZ. The supplemental feeding rule does not apply to bird feeders within 100 feet of a building or occupied dwelling or feed inside an active feral hog trap. Supplemental feeding and baiting privileges are allowed outside of the CMZ.
For more information about CWD in Alabama, please visit www.outdooralabama.com/cwd-info.
ADCNR promotes wise stewardship, management and enjoyment of Alabama’s natural resources through four divisions: Marine Resources, State Lands, State Parks and Wildlife and Freshwater Fisheries. Learn more at www.outdooralabama.com.
###
CWD Zone Map 12-23-24.jpg
https://www.outdooralabama.com/articles/cwd-detection-colbert-county-expands-states-cwd-management-zone
*** Alabama CWD TSE Prion 2023
(2020, Alabama, to date, has detected NO cases of CWD TSE Prion...tss)
*** Alabama CWD TSE Prion 2023 TO DATE 5 CASES CONFIRMED
Alabama Two Additional Cases of CWD Confirmed in Northern Lauderdale County
Press release December 15, 2023 Contact: Marianne Gauldin, (334) 242-3469
Alabama Two Additional Cases of CWD Confirmed in Northern Lauderdale County
Two Additional Cases of CWD Confirmed in Northern Lauderdale County The Alabama Department of Conservation and Natural Resources (ADCNR) announces that two additional cases of chronic wasting disease (CWD) in hunter harvested, white-tailed deer have been confirmed in northern Lauderdale County in northwest Alabama. The two additional deer bring Alabama’s total number of confirmed CWD cases to five.
CWD in Alabama’s deer herd was first detected in Lauderdale County in January 2022. After the first case was confirmed, all of Lauderdale and Colbert counties were designated as a CWD Management Zone (CMZ).
So far during the 2023-2024 hunting season, samples have been collected from more than 1,700 white-tailed deer harvested statewide with 420 of those samples collected within the CMZ. One of the positive samples was submitted during the second CMZ mandatory sampling weekend (December 2-3). The other positive sample was voluntarily submitted at a drop-off sampling location by a hunter as part of ADCNR's ongoing CWD monitoring efforts. The next mandatory sampling weekend in the buffer zone of the CMZ is January 6-7, 2024.
“I would like to thank hunters for their continued support by providing a robust number of samples for CWD testing since the disease was first detected in Alabama,” said Chris Blankenship, ADCNR Commissioner. “Hunters are our most important partners in the management of CWD as we move forward with future deer seasons. We also thank the Alabama Department of Agriculture and Industries for their continued partnership with statewide CWD monitoring. Their assistance by testing the samples allows us to better serve our constituents by providing them with timely information on the distribution and extent of CWD in Alabama.”
CWD is a member of the group of diseases called transmissible spongiform encephalopathies (TSEs). Among cervids, CWD is a progressive, fatal disease that commonly results in altered behavior due to microscopic changes of the brain of affected animals. An animal may carry the disease for years without outward indication. In latter stages of the disease, signs may include listlessness, lowering of the head, weight loss, repetitive walking in set patterns and a lack of responsiveness.
It is important that hunters be familiar with Alabama’s CWD regulation and the CWD regulations in other states. To review Alabama’s regulation and the latest information about CWD in the state, visit www.outdooralabama.com/cwd-info.
ADCNR promotes wise stewardship, management and enjoyment of Alabama’s natural resources through four divisions: Marine Resources, State Lands, State Parks, and Wildlife and Freshwater Fisheries. Learn more at www.outdooralabama.com.
###
CMZ map attached (includes locations of positive cases)
CMZ zone map 12-15-23.jpg
https://www.outdooralabama.com/sites/default/files/CMZ%20zone%20map%2012-15-23.jpg
https://www.outdooralabama.com/articles/two-additional-cases-cwd-confirmed-northern-lauderdale-county
https://www.outdooralabama.com/articles/mandatory-cwd-testing-dates-announced-lauderdale-and-colbert-counties
Posted: February 16, 2023
Third Case of CWD Confirmed in Lauderdale County
The Alabama Department of Conservation and Natural Resources (ADCNR) announces that a third case of Chronic Wasting Disease (CWD) in a hunter harvested, white-tailed deer has been confirmed in Lauderdale County in northwest, Alabama. The first two cases of CWD in Alabama’s deer herd were detected in Lauderdale County in early 2022.
Samples were collected from more than 3,500 white-tailed deer harvested statewide with over 1,100 of those samples collected within the CMZ during the 2022-2023 hunting season. More than 98% of all samples collected within the CMZ have been tested by the Alabama Department of Agriculture and Industries and the results have been received by ADCNR. Currently, only one positive has been detected this season. The positive sample was voluntarily submitted by a hunter as part of ADCNR's ongoing CWD monitoring efforts.
https://www.outdooralabama.com/articles/third-case-cwd-confirmed-lauderdale-county
http://www.outdooralabama.com/cwd/latest-cwd-information
https://chronic-wasting-disease.blogspot.com/2023/02/alabama-third-case-of-cwd-confirmed-in.html
https://chronic-wasting-disease.blogspot.com/
FRIDAY, JANUARY 18, 2019
Alabama WFF Ramps Up Chronic Wasting Disease CWD TSE Prion Sampling Effort
https://chronic-wasting-disease.blogspot.com/2019/01/alabama-wff-ramps-up-chronic-wasting.html
THURSDAY, JULY 20, 2017
Alabama Atypical BSE CJD CWD TSE Prion Update
https://bseusa.blogspot.com/2017/07/alabama-atypical-bse-cjd-cwd-tse-prion.html
TUESDAY, MARCH 29, 2016
ALABAMA CHRONIC WASTING DISEASE CWD TSE PRION SURVEILLANCE AND TESTING PROGRAM?
https://chronic-wasting-disease.blogspot.com/2016/03/alabama-chronic-wasting-disease-cwd-tse.html
THURSDAY, NOVEMBER 01, 2012
ALABAMA BIG BUCK PROJECT, A CWD TSE PRION ACCIDENT WAITING TO HAPPEN ALABAMA BIG BUCK PROJECT, A CWD ACCIDENT WAITING TO HAPPEN
http://chronic-wasting-disease.blogspot.com/2012/11/alabama-big-buck-project-cwd-tse-prion.html
***> CWD TSE PrP Environmental Factors
Chronic wasting disease (CWD) prion detection in environmental and biological samples from a taxidermy site and nursing facility, and instruments used in surveillance activities
Available online 9 April 2025
Highlights
• CWD prions were identified in a taxidermy and deer nursing facility.
• Contaminated samples included waters, soils, dermestid beetles, domestic flies and a dumpster.
• Surgical instruments used to collect deer samples can get contaminated with CWD prions.
• Some of the infectious particles are readily released from surgical instruments when washed.
• Our results suggest that taxidermy practices actively contribute in the spreading of CWD.
Snip…
In summary, the information provided in this report demonstrate how anthropogenic activities, specifically taxidermy practices, animal processing, and rehabilitation of CWD susceptible species, may facilitate CWD transmission through the environmental dissemination of CWD prions. This study, along with future research efforts characterizing the overall level of infectivity, provides relevant information on managing CWD and to control its rapid geographic expansion. …
https://www.sciencedirect.com/science/article/abs/pii/S0048969725009544
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
Artificial mineral sites that pre-date endemic chronic wasting disease become prion hotspots
The Ames Research and Educational Center property, centrally located within the CWD zone of southwest Tennessee, contains 49 historical mineral supplementation sites that were decommissioned in 2012. Here, we demonstrate that 32 of the 49 (65%) mineral sites within Ames established prior to the regional CWD outbreak, serve as foci of environmental PrPCWD contamination. Detection of PrPCWD in soils from these artificial mineral sites was dependent on site-specific management efforts. Soil physical properties were very similar across sites and no correlation between PrPCWD detection and soil physical properties was found. 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://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf
Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer
Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.
Prion 2022 Conference abstracts: pushing the boundaries
https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286
Front. Vet. Sci., 14 September 2015 | 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.
journal.frontiersin.org/article/10.3389/fvets.2015.00032/full
SUNDAY, APRIL 06, 2025
Failure to prevent classical scrapie after repeated decontamination of a barn
scrapie-usa.blogspot.com/2025/04/failure-to-prevent-classical-scrapie.html
prpsc.proboards.com/thread/165/failure-prevent-scrapie-repeated-decontamination
"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
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.
web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
***> 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/
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…
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. In fact new data now shows that exposure to high temperatures used to cook the meat increased the availability of prions for in vitro amplification.
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/
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
Chronic wasting disease prions on deer feeders and wildlife visitation to deer feeding areas
First published: 10 February 2025
Snip…
Finally, we swabbed 19 feeders in 2 areas where CWD was newly detected, finding prion contamination on swabs from 4 feeders. We show that deer feeders in free-ranging populations with high CWD prevalence become contaminated with CWD prions quickly, becoming a potential site of exposure of deer to CWD prions. Our results also demonstrate the ability to find evidence of prion contamination on deer feeders, even in areas where CWD is newly detected.
Snip…
We found that supplemental feeding increased the risk of exposure to CWD prions due to contamination of feeders, increased deer visitation, and increased deer-to-deer contact.
The 12-fold increase in deer visitation to feeders compared to mast trees and 2-fold increase compared to food plots demonstrates increased risk for direct disease spread.
https://wildlife.onlinelibrary.wiley.com/doi/10.1002/jwmg.70000
Chronic Wasting Disease in Texas A Real Disease with Proven Impacts
Produced by a coalition of concerned hunters, landowners, & conservationists (last update 1/2025)
storymaps.arcgis.com/stories/b93f528938ac48e9b56dcc79953cbec0
***> Human CWD TSE PrP, what if?
the problem is, to date, there is NO diagnostic criteria set in stone that would confirm a case of human Cwd, like there was with nvCJD (my Mom died from confirmed hvCJD a rare strain of the infamous sporadic CJDs with new strains mounting, sporadic CJD simply means ‘unknown’, IT DOES NOT MEAN 85%+ SPORADIC CJD IS ALL SPONTANEOUS, that’s all iatrogenic CJD is sporadic CJD, until the iatrogenic event is detected, confirmed, traced back, confirmed, put I to the academic domain, and finally, if your lucky, finally published to the media, and finally the public domain.) sorry, I got off course…but let me perfectly clear here, all science to date shows, Human CWD will not look like New Variant Creutzfeldt Jakob disease nvCJD. CWD to humans will look like some variant of sporadic Creutzfeldt Jakob Disease. And here me out very clearly, and this is from the to TSE Prion Gods themselves, old correspondence from way back during my investigations early BSE nvCJD days…2002
“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.”
*** 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”
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
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
-----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, however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41].
snip... full text ;
https://www.vetres.org/articles/vetres/abs/2008/04/v08092/v08092.html
https://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
“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
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 ############
*** 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
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
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
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
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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
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 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
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.”
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
Mary Van Beusekom, MS Today at 9:15 a.m.
Chronic Wasting Disease BSE CWD zone deer disposal site Lorie Shaull / Flickr cc
Nine months ago, Janie Johnston, 73, left her home in the Chicago suburbs to drive to her doctor's office for routine care. She made it as far as the side of the street opposite the clinic but couldn't figure out how to get there, so she returned home, where she struggled to remember the abbreviation "GPS."
That was the first sign that something was seriously wrong. Soon, the semi-retired geologist couldn't speak in full sentences or feed herself. Within 2 months, the woman who had been reviewing proposals for the National Science Foundation in the weeks leading up to symptom onset was dead of a terrifying neurological disease her family had never heard of: Creutzfeldt-Jakob disease (CJD).
CWD may cause CJD-like disease if it infects people Rather than being genetic or acquired, Johnston's CJD developed when normal prions in her brain spontaneously began misfolding. The abnormal prions accumulated rather than being shed, triggering confusion and fatigue that doctors initially mistook for stroke, meningitis, or alcohol withdrawal. The disease usually occurs in older adults.
Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members. Kristal Enter
While no one is certain, experts think that another always-fatal prion disease—this one currently known to occur only in cervids such as deer, moose, and elk—may behave the same way if it should jump the species barrier and infect people.
Chronic wasting disease (CWD) has been decimating cervid populations throughout North America since it was first diagnosed in a captive Colorado mule deer in 1967. While mitigation measures such as hunting may help slow its spread, it can't be stopped.
This is because cervids are ubiquitous and free ranging, the interval from infection to symptom onset can take years, and prions spread easily from animal to animal and through environmental contamination, which can persist for years.
Johnston's daughter, Kristal Enter, 39, a fundraiser in Boston, is familiar with CWD and its potential implications for human health. "Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members," she told CIDRAP News. "The more we're on top of chronic wasting disease and thinking about it, the better."
But the frightening thing is that, for well over a month during the recent US government shutdown, no one was watching the human disease landscape for CWD, a highly infectious disease with no treatment or cure.
If cases slip by, it will be too late Nine days after the government shutdown began, all four staff members of the Centers for Disease Control and Prevention's (CDC's) National Center for Emerging and Zoonotic Infectious Diseases Prion and Public Health Office were sent home after receiving reduction-in-force (RIF) notices. While the end of the shutdown led all four to be reinstated through at least January, layoffs after that time are possible.
Within the past few months, two other researchers who had been part of the team also had to be let go after their fellowship contracts weren't renewed, per the administration's policy of blocking contract renewals.
The prion unit, which monitors the nation for human prion diseases, is part of the Division of High-Consequence Pathogens and Pathology. It launched in the mid-1990s in response to the outbreak of bovine spongiform encephalopathy (BSE, or "mad cow disease") in UK cattle. BSE prions were inadvertently consumed by people who ate contaminated beef, causing the human form of BSE, variant CJD (vCJD). All infected people—more than 230—died.
The initial goal of the Prion and Public Health Office was to watch for any cases of vCJD in the US population. Since then, its focus has expanded to include advising hospitals on how to prevent and respond to prion contamination of instruments used in neurosurgery (prions are resistant to many usual sterilization methods), as well as working with state health departments on disease surveillance. Unit members also answer questions from the public.
Today, as CWD continues its inexorable march across the landscape, exposing more and more people, the prion unit's priority is conducting surveillance for signs of a CWD species jump into high-risk people such as hunters. Without this expertise, no one will be able to evaluate whether a suspected case of CWD prion transmission to humans is likely from an animal.
The prion unit has launched several epidemiologic studies in collaboration with multiple states to look at whether more hunters are dying of prion diseases than would be expected.
Janie Johnston Janie Johnston / Courtesy of Kristal Enter As an example of the unit's work, last spring, a cluster of CJD cases in Oregon was widely conjectured to be linked to CWD. Such cases require autopsy and an epidemiologic investigation to determine whether CWD was involved and, if so, what kind of public health measures are needed. The prion unit shared ideas and strategy with the Oregon state health department in this investigation, which, thankfully, found no link.
But experts say that without anyone looking for these deviations from normal—particularly given that signs of illness may take years to appear—cases could easily go unnoticed, and it will be too late to implement public health measures that could mitigate some of these consequences.
Lawrence Schonberger, MD, MPH, retired chief of the Prion and Public Health Office, said that, as was the situation with BSE, CWD containment efforts must continue. "Unlike with mad cow disease, however, these efforts to date have not been successful," he said. Surveillance and research "should continue to help people recognize any emerging risk to humans from this agent now and in the future, when this agent's pathogenicity [ability to cause disease] may change."
'Worst time to get rid of such a division' Brian Appleby, MD, is director of the National Prion Disease Pathology Surveillance Center at Case Western University, which conducts diagnostic testing for human prion diseases and conducted Johnston's autopsy. The CDC funds the center, which collaborates with the Prion and Public Health Office on public health efforts and research projects.
When you don't have a neutral party investigating these things or doing neuropathology to confirm or refute those things, you really have no idea what's going on in the public health space. Brian Appleby, MD
If the CDC prion unit were eliminated, "no one would be looking at prion disease," Appleby said. "We wouldn't be able to tell if we have an increase in cases or where they're going or coming from. And when you don't have a neutral party investigating these things or doing neuropathology to confirm or refute those things, you really have no idea what's going on in the public health space."
And with the threat of CWD, "this is probably the worst time to get rid of such a division," he added.
While there have been no stated plans to eliminate the unit, history hints that its continued existence may be in jeopardy. In fact, it was removed from President Donald Trump's budget during both of his administrations, before the House of Representatives and Senate reinstated it, Appleby said.
In the first Trump administration, report language stated that human prion surveillance is redundant because cattle are now screened for BSE, and the National Institutes of Health cover research, said Appleby, who refuted the assertions.
"Part of the reason why we are a safe export country for beef is not just the cattle surveillance for BSE, but the human surveillance for variant CJD," he said, adding that monitoring is a separate function from research. "When we were removed from the president's budget this time around, there was no report language, so we have no signal to know why."
Always 'one step behind' Debbie Yobs, president and executive director of the CJD Foundation, a patient-advocacy organization that works with Appleby's center to provide medical lectures, support groups, and other programs, emphasized the importance of sustained surveillance. "You can't have gaps in monitoring a deadly disease like prion disease," she said.
It's like combining symptoms of Alzheimer's, Parkinson's, and ALS [amyotrophic lateral sclerosis, or Lou Gehrig's disease] and then speeding it all up. Debbie Yobs
CJD is devastating for patients and families, said Yobs, whose husband, Patrick, died at age 45 of the less common, genetic form of the disease. "It's like combining symptoms of Alzheimer's, Parkinson's, and ALS [amyotrophic lateral sclerosis, or Lou Gehrig's disease] and then speeding it all up," she added. "There's no definitive diagnosis except through autopsy."
Indeed, Enter called her family's ordeal "unreal," because although Johnston's case was typical of a sporadic case, CJD affects only about 500 to 600 people in the United States each year, per the CDC. At the same time as the family was grieving, they were trying to learn how hospice staff could best manage Johnston's symptoms, which none of them had dealt with before.
"It becomes incumbent upon the family members to become the experts to guide the care," she said. "What they say about CJD is that every day is another new symptom or new complication to have to try to address. And you always feel like you're one step behind."
https://www.cidrap.umn.edu/chronic-wasting-disease/while-no-one-was-watching-tenuous-status-cdc-prion-unit-risk-cwd-people
“While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists”
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
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
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/10.1212/WNL.0000000000204407
FRIDAY, NOVEMBER 21, 2025
***> While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
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
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 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
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.”
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
Mary Van Beusekom, MS Today at 9:15 a.m.
Chronic Wasting Disease BSE CWD zone deer disposal site Lorie Shaull / Flickr cc
Nine months ago, Janie Johnston, 73, left her home in the Chicago suburbs to drive to her doctor's office for routine care. She made it as far as the side of the street opposite the clinic but couldn't figure out how to get there, so she returned home, where she struggled to remember the abbreviation "GPS."
That was the first sign that something was seriously wrong. Soon, the semi-retired geologist couldn't speak in full sentences or feed herself. Within 2 months, the woman who had been reviewing proposals for the National Science Foundation in the weeks leading up to symptom onset was dead of a terrifying neurological disease her family had never heard of: Creutzfeldt-Jakob disease (CJD).
CWD may cause CJD-like disease if it infects people Rather than being genetic or acquired, Johnston's CJD developed when normal prions in her brain spontaneously began misfolding. The abnormal prions accumulated rather than being shed, triggering confusion and fatigue that doctors initially mistook for stroke, meningitis, or alcohol withdrawal. The disease usually occurs in older adults.
Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members. Kristal Enter
While no one is certain, experts think that another always-fatal prion disease—this one currently known to occur only in cervids such as deer, moose, and elk—may behave the same way if it should jump the species barrier and infect people.
Chronic wasting disease (CWD) has been decimating cervid populations throughout North America since it was first diagnosed in a captive Colorado mule deer in 1967. While mitigation measures such as hunting may help slow its spread, it can't be stopped.
This is because cervids are ubiquitous and free ranging, the interval from infection to symptom onset can take years, and prions spread easily from animal to animal and through environmental contamination, which can persist for years.
Johnston's daughter, Kristal Enter, 39, a fundraiser in Boston, is familiar with CWD and its potential implications for human health. "Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members," she told CIDRAP News. "The more we're on top of chronic wasting disease and thinking about it, the better."
But the frightening thing is that, for well over a month during the recent US government shutdown, no one was watching the human disease landscape for CWD, a highly infectious disease with no treatment or cure.
If cases slip by, it will be too late Nine days after the government shutdown began, all four staff members of the Centers for Disease Control and Prevention's (CDC's) National Center for Emerging and Zoonotic Infectious Diseases Prion and Public Health Office were sent home after receiving reduction-in-force (RIF) notices. While the end of the shutdown led all four to be reinstated through at least January, layoffs after that time are possible.
Within the past few months, two other researchers who had been part of the team also had to be let go after their fellowship contracts weren't renewed, per the administration's policy of blocking contract renewals.
The prion unit, which monitors the nation for human prion diseases, is part of the Division of High-Consequence Pathogens and Pathology. It launched in the mid-1990s in response to the outbreak of bovine spongiform encephalopathy (BSE, or "mad cow disease") in UK cattle. BSE prions were inadvertently consumed by people who ate contaminated beef, causing the human form of BSE, variant CJD (vCJD). All infected people—more than 230—died.
The initial goal of the Prion and Public Health Office was to watch for any cases of vCJD in the US population. Since then, its focus has expanded to include advising hospitals on how to prevent and respond to prion contamination of instruments used in neurosurgery (prions are resistant to many usual sterilization methods), as well as working with state health departments on disease surveillance. Unit members also answer questions from the public.
Today, as CWD continues its inexorable march across the landscape, exposing more and more people, the prion unit's priority is conducting surveillance for signs of a CWD species jump into high-risk people such as hunters. Without this expertise, no one will be able to evaluate whether a suspected case of CWD prion transmission to humans is likely from an animal.
The prion unit has launched several epidemiologic studies in collaboration with multiple states to look at whether more hunters are dying of prion diseases than would be expected.
Janie Johnston Janie Johnston / Courtesy of Kristal Enter As an example of the unit's work, last spring, a cluster of CJD cases in Oregon was widely conjectured to be linked to CWD. Such cases require autopsy and an epidemiologic investigation to determine whether CWD was involved and, if so, what kind of public health measures are needed. The prion unit shared ideas and strategy with the Oregon state health department in this investigation, which, thankfully, found no link.
But experts say that without anyone looking for these deviations from normal—particularly given that signs of illness may take years to appear—cases could easily go unnoticed, and it will be too late to implement public health measures that could mitigate some of these consequences.
Lawrence Schonberger, MD, MPH, retired chief of the Prion and Public Health Office, said that, as was the situation with BSE, CWD containment efforts must continue. "Unlike with mad cow disease, however, these efforts to date have not been successful," he said. Surveillance and research "should continue to help people recognize any emerging risk to humans from this agent now and in the future, when this agent's pathogenicity [ability to cause disease] may change."
'Worst time to get rid of such a division' Brian Appleby, MD, is director of the National Prion Disease Pathology Surveillance Center at Case Western University, which conducts diagnostic testing for human prion diseases and conducted Johnston's autopsy. The CDC funds the center, which collaborates with the Prion and Public Health Office on public health efforts and research projects.
When you don't have a neutral party investigating these things or doing neuropathology to confirm or refute those things, you really have no idea what's going on in the public health space. Brian Appleby, MD
If the CDC prion unit were eliminated, "no one would be looking at prion disease," Appleby said. "We wouldn't be able to tell if we have an increase in cases or where they're going or coming from. And when you don't have a neutral party investigating these things or doing neuropathology to confirm or refute those things, you really have no idea what's going on in the public health space."
And with the threat of CWD, "this is probably the worst time to get rid of such a division," he added.
While there have been no stated plans to eliminate the unit, history hints that its continued existence may be in jeopardy. In fact, it was removed from President Donald Trump's budget during both of his administrations, before the House of Representatives and Senate reinstated it, Appleby said.
In the first Trump administration, report language stated that human prion surveillance is redundant because cattle are now screened for BSE, and the National Institutes of Health cover research, said Appleby, who refuted the assertions.
"Part of the reason why we are a safe export country for beef is not just the cattle surveillance for BSE, but the human surveillance for variant CJD," he said, adding that monitoring is a separate function from research. "When we were removed from the president's budget this time around, there was no report language, so we have no signal to know why."
Always 'one step behind' Debbie Yobs, president and executive director of the CJD Foundation, a patient-advocacy organization that works with Appleby's center to provide medical lectures, support groups, and other programs, emphasized the importance of sustained surveillance. "You can't have gaps in monitoring a deadly disease like prion disease," she said.
It's like combining symptoms of Alzheimer's, Parkinson's, and ALS [amyotrophic lateral sclerosis, or Lou Gehrig's disease] and then speeding it all up. Debbie Yobs
CJD is devastating for patients and families, said Yobs, whose husband, Patrick, died at age 45 of the less common, genetic form of the disease. "It's like combining symptoms of Alzheimer's, Parkinson's, and ALS [amyotrophic lateral sclerosis, or Lou Gehrig's disease] and then speeding it all up," she added. "There's no definitive diagnosis except through autopsy."
Indeed, Enter called her family's ordeal "unreal," because although Johnston's case was typical of a sporadic case, CJD affects only about 500 to 600 people in the United States each year, per the CDC. At the same time as the family was grieving, they were trying to learn how hospice staff could best manage Johnston's symptoms, which none of them had dealt with before.
"It becomes incumbent upon the family members to become the experts to guide the care," she said. "What they say about CJD is that every day is another new symptom or new complication to have to try to address. And you always feel like you're one step behind."
https://www.cidrap.umn.edu/chronic-wasting-disease/while-no-one-was-watching-tenuous-status-cdc-prion-unit-risk-cwd-people
“While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists”
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
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
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/10.1212/WNL.0000000000204407
FRIDAY, NOVEMBER 21, 2025
***> While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
iatrogenic TSE/CWD, my greatest fear, here’s why…
one of the old studies that has always stuck out in my mind, one that the late great Dr. Gibbs, Gajdusek, et al did way back, and to this day is still amazes me...
*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
France issues moratorium on prion research after fatal brain disease strikes two lab workers
By Barbara CasassusJul. 28, 2021 , 4:35 AM
PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.
If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.
The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.
“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.
In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.
A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.
Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.
For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.
In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.
“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission
INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)
Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.
Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)
The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.
In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.
After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.
The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.
The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.
Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”
With reporting by Martin Enserink.
Posted in: EuropeHealthScientific Community
doi:10.1126/science.abl6587
https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
TO THE EDITOR:
We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.
In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.
In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).
Figure 1.
Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.
The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)
There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2
Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.
Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France
M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France
Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France
Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France
Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France
Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France
Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France
Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr
Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.
Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.
5 References
July 2, 2020
N Engl J Med 2020; 383:83-85
DOI: 10.1056/NEJMc2000687
Metrics
https://www.nejm.org/doi/full/10.1056/NEJMc2000687
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
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
WEDNESDAY, OCTOBER 15, 2025
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025
https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
SATURDAY, JANUARY 10, 2026
Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease, a review
https://creutzfeldt-jakob-disease.blogspot.com/2026/01/neuropsychiatric-symptoms-in-sporadic.html
https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review
wasted days and wasted nights…Freddy Fender
Terry S. Singeltary Sr.
one of the old studies that has always stuck out in my mind, one that the late great Dr. Gibbs, Gajdusek, et al did way back, and to this day is still amazes me...
*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
France issues moratorium on prion research after fatal brain disease strikes two lab workers
By Barbara CasassusJul. 28, 2021 , 4:35 AM
PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.
If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.
The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.
“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.
In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.
A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.
Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.
For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.
In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.
“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission
INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)
Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.
Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)
The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.
In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.
After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.
The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.
The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.
Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”
With reporting by Martin Enserink.
Posted in: EuropeHealthScientific Community
doi:10.1126/science.abl6587
https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
TO THE EDITOR:
We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.
In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.
In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).
Figure 1.
Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.
The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)
There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2
Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.
Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France
M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France
Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France
Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France
Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France
Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France
Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France
Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr
Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.
Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.
5 References
July 2, 2020
N Engl J Med 2020; 383:83-85
DOI: 10.1056/NEJMc2000687
Metrics
https://www.nejm.org/doi/full/10.1056/NEJMc2000687
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
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
WEDNESDAY, OCTOBER 15, 2025
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025
https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
SATURDAY, JANUARY 10, 2026
Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease, a review
https://creutzfeldt-jakob-disease.blogspot.com/2026/01/neuropsychiatric-symptoms-in-sporadic.html
https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review
wasted days and wasted nights…Freddy Fender
Terry S. Singeltary Sr.
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