Neuropathologists urge Ottawa to bring back autopsy services for patients suspected of having fatal brain illness
Neuropathologists urge Ottawa to bring back autopsy services for patients suspected of having fatal brain illness
by CANP | Apr 10, 2026 | News
Click here to read the article published in the Globe and Mail in April 2026
Read the article directly with your subscription to the Globe and...
Ottawa to halt funding for tests to detect deaths from Creutzfeldt-Jakob disease
by CANP | Apr 10, 2026 | News
Click here to read the article published in the Globe and Mail in February 2026
Read the article directly with your subscription to the Globe and...
Cessation of Public Health Agency of Canada funding for the CJD Neuropathology Program
by CANP | Mar 25, 2026 | News Dear CANP
Members, Please be informed that the Canadian Association of Neuropathologists has formally sent the attached letter to Dr. Howard Njoo, Chief Medical Officer of Health of Canada, regarding the abrupt cessation of Public Health Agency of Canada funding for...
https://canp.ca/category/news/
https://canp.ca/2026/04/10/neuropathologists-urge-ottawa-to-bring-back-autopsy-services-for-patients-suspected-of-having-fatal-brain-illness/
Neuropathologists urge Ottawa to bring back autopsy services for patients suspected of having fatal brain illness
4/10/26
KELLY GRANT HEALTH REPORTER PUBLISHED YESTERDAY
Neuropathologists across Canada are urging the federal government to revive autopsy services for people suspected of having Creutzfeldt-Jakob disease, the fatal brain illness best known for its links to mad cow disease.
As The Globe and Mail previously reported, the Public Health Agency of Canada abruptly cut off funding in February for CJD autopsies and biopsies performed at a University of Ottawa lab to save money.
The facility had for decades been the main lab accepting brains and tissue samples potentially infected with CJD, a transmissible and deadly neurodegenerative disorder.
Now the Canadian Association of Neuropathologists, which represents specialists who study and diagnose diseases of the nervous system, is asking
https://www.theglobeandmail.com/canada/article-neuropathologists-autopsy-services-creutzfeldt-jakob-disease/#comments
1/6 4/10/26, 10:52 AM
Neuropathologists urge Ottawa to bring back autopsy services for patients suspected of having fatal brain illness - The Globe and …
PHAC to meet with them to find a way to bring the service back.
“Eliminating funding for this centralized program is short-sighted,” the association wrote in a letter obtained by The Globe. “Replicating equivalent infrastructure across provinces and territories would be more expensive and time-consuming. Even if decentralization were preferred, building the required expertise and facilities would likely take years – leaving patients since Feb. 13, 2026, without access to essential diagnostic services.”
New Brunswick doctor renews call for investigation into undiagnosed neurological illness
The neuropathology association sent the letter on March 26 to Howard Njoo, then Canada’s interim chief public health officer, and to the chief medical officers of health for every province and territory.
Veronica Hirsch-Reinshagen, the secretary-treasurer of the Canadian Association of Neuropathologists, was shocked when she learned the autopsy program would be scrapped.
“I didn’t understand why they were doing it,” she said in an interview Thursday from Vancouver, where she is a practising neuropathologist. “What was the rationale behind it?”
Cynthia Hawkins, president of the neuropathology association and a Toronto neuropathologist, said in the same interview that she was most surprised by the fact that PHAC axed the program without talking to neuropathologists about what might replace it.
“It seemed to be a decision that had been made without consulting the neuropathology community, which had been at the front lines for doing this surveillance, certainly at postmortem, for like 30 years,” Dr. Hawkins said. Karine LeBlanc, a spokeswoman for PHAC, said the agency is currently preparing a response to the neuropathologists’ letter. She emphasized that the agency’s other CJD surveillance activities will continue; only the federal funding for brain autopsies and biopsies has been cut.
https://www.theglobeandmail.com/canada/article-neuropathologists-autopsy-services-creutzfeldt-jakob-disease/#comments 2/6
4/10/26, 10:52 AM
Neuropathologists urge Ottawa to bring back autopsy services for patients suspected of having fatal brain illness - The Globe and …
Creutzfeldt-Jakob disease is an incurable disorder caused by infectious misfolded proteins known as prions. CJD is rare, with fewer than 500 cases confirmed in Canada since 2020.
Most are a kind known as sporadic CJD, where normal proteins in the brain misfold into infectious abnormal proteins for no apparent reason. A small number of cases are caused by an inherited genetic mutation.
The least common, but most famous, type of CJD is variant CJD, the type caused by eating beef from cows infected with the animal prion disease bovine spongiform encephalitis – better known as mad cow disease.
PHAC told The Globe in February that there was less need for brain autopsies because modern, reliable lab tests can now detect CJD in patients while they’re still alive.
But the neuropathologists noted in their letter that the tests aren’t foolproof. Brain injury part of a far-reaching shadow crisis amid overdose deaths in B.C. The letter cited a recent case in which a pathologist identified at autopsy a case of inherited CJD in a person who had been “ruled out to have CJD” by earlier lab testing.
The letter also pointed out that inherited cases of CJD frequently go undetected because the disease progresses too rapidly for blood samples to be drawn and sent for genetic testing.
PHAC said in February that the CJD brain autopsy and biopsy program had an annual budget of up to $1.3-million a year.
But Gerard Jansen, the University of Ottawa neuropathologist who ran the CJD autopsy program, told The Globe that the federal government only paid that amount to the lab if the group conducted 100 brain autopsies or biopsies in a year.
The actual number of autopsies was usually about half that, meaning the federal outlay was significantly less than budgeted. Dr. Jansen said in an e-mail
https://www.theglobeandmail.com/canada/article-neuropathologists-autopsy-services-creutzfeldt-jakob-disease/#comments
3/6 4/10/26, 10:52 AM Neuropathologists urge Ottawa to bring back autopsy services for patients suspected of having fatal brain illness - The Globe and …
Thursday that the actual federal contribution barely covered the operating costs of the program, especially in the last four years.
https://canp.ca/wp-content/uploads/2026/04/Neuropathologists-urge-Ottawa-to-bring-back-autopsy-services-for-patients-suspected-of-having-fatal-brain-illness-The-Globe-an.pdf
Terrible, Terrible news, but does not surprise me, with Creutzfeldt Jakob Disease cases rising (this is well documented), sure is a hell of a time to stop reporting it, right, good way to kill any potential link to New Brunswick Neurological Disease outbreak, or any other strange strain, phenotype, of human TSE PrP (i’m thinking Vpspr), strange Vpspr has not yet been documented in Canada, at least on paper. so sad. with chronic wasting disease cwd tse prp spreading across Canada and the USA, atypical and typical BSE, Scrapie, still happening and surveillance there from very questionable, with Mexico still clueless, and human cwd zoonotic disease staring us in the face, if not already happening, and CJD cases rising across the board, this is absolutely not scientific, and incredibly stupid (i’m thinking iatrogenic), that will be a free-for-all in every surgical medical arenas, i guess Canada will go back to SHOOT, SHOVEL, SHUT UP, SCIENCE, (if they ever left it), we all lose then…
MONDAY, FEBRUARY 23, 2026
Canada Ottawa to halt funding for tests to detect deaths from Creutzfeldt-Jakob disease
https://creutzfeldt-jakob-disease.blogspot.com/2026/02/canada-ottawa-to-halt-funding-for-tests.html
https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html
Canada Creutzfeldt Jakob Disease Surveillance Dashboard
https://health-infobase.canada.ca/diseases/cjd/dashboard.html
TUESDAY, APRIL 15, 2025
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024).
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2025.1571662/full
https://creutzfeldt-jakob-disease.blogspot.com/2025/04/cases-of-creutzfeldt-jakob-disease-in.html
CANADA CJD TSE Prion
Creutzfeldt Jacob Disease CJD TSE Prion December 14, 2024 Annual Update
Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report
Definite and probable CJD, 1998-2025
As of August 31, 2025
Year Sporadic Iatrogenic Genetic vCJD Total
1998 22 1 1 0 24
1999 27 2 3 0 32
2000 32 0 3 0 35
2001 27 0 3 0 30
2002 31 0 4 1 36
2003 27 1 1 0 29
2004 42 0 2 0 44
2005 42 0 2 0 44
2006 39 0 5 0 44
2007 35 0 4 0 39
2008 48 0 1 0 49
2009 48 0 5 0 53
2010 35 0 3 0 38
2011 46 0 4 1 51
2012 63 0 1 0 64
2013 50 0 1 0 51
2014 52 0 4 0 56
2015 43 0 9 0 52
2016 61 1 6 0 68
2017 83 0 8 0 91
2018 76 1 6 0 83
2019 76 0 6 0 82
2020 68 0 4 0 72
2021 61 0 5 0 66
2022 93 0 3 0 96
2023 74 0 2. 0 76
2024 70 0 3 0 73
2025 28 0 1 0 29
Total 1,399 6 100 2 1,507
Cases with definite and probable diagnosis to date
Variant CJD (vCJD)
The CJDSS uses current, internationally accepted diagnostic criteria (PDF, 252 KB) to classify definite and probable cases of CJD in Canada.
https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref
Canada, still no documentation of VPSPr on paper…
VPSPr sporadic CJD
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
Brain Pathol. 2023 Sep; 33(Suppl 1): e13194.
Published online 2023 Sep 6.
https://doi.org/10.1111/bpa.13194
PMCID: PMC10483180
PMID: 37674376
Special Issue: Abstracts of the 20th International Congress of Neuropathology, Berlin, Germany, September 13–16, 2023
snip...
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
J. Mikol 1, J. Delmotte1, W. Zou2,3, J. P. Deslys1, E. Comoy1 1Commissariat à l'energie Atomique, Institut François Jacob/Prion research Group, Fontenay‐aux‐Roses, France; 2Case Western Reserve University School of Medicine, Department of Pathology, Cleveland, OH, United States; 3Institute of Neurology, Nanchang, China
VPSPr, a novel rare human prion disease described in 2010 is characterized by the accumulation of abnormal PrPd with a limited resistance to proteolysis. It showed uncertain infectivity in transgenic mouse models. Recent publications report VPSPr cases with different unevocative clinical pictures, leading the authors to consider that VPSPr might be underestimated. One of our macaques intracerebrally inoculated with a brain sample derived from a VPSPr patient (Met/Val at codon 129) exhibited after 8.5 years of silent incubation several episodes of self‐aggression first focused on his left leg, which extended to the whole hindquarters, and abnormalities of both hind limbs sensitive conduction. We performed euthanasia 4 months later for humane reasons and examined brain tissues with histological techniques. Pathological examination revealed a spongiform change decreasing from the frontal to the occipital cortex, also present in the thalamus, the basal ganglia, the hippocampus, mild brainstem and spinal cord, accompanied with a massive neuronal vacuolation and reactive astrocytosis. Purkinje cells were rarefied and vacuolated. Abnormal PrPd deposition was present as synaptic deposits mixed with some mini‐aggregates of PrPd diffused in the tissue or packed in small fussy unlimited area of variable intensity, appearing as pre‐plaques. These lesions, never previously observed in primates exposed to other prion strains, were mainly observed in the insula and the frontal cortex but were absent in the thalamus, the basal ganglia and the hippocampus. Furthermore, we observed massive Ab deposits, but not in the hippocampus except the subiculum, not co‐localized with PrPd. In contrast, a two‐times older animal had three‐times less deposits. The original disease phenotype observed in this primate will be discussed and compared to the expressions of VPSPr in humans, and other prion diseases in the macaque model. PS9‐ND‐A186
https://europepmc.org/article/MED/37674376
“Recent publications report VPSPr cases with different unevocative clinical pictures, leading the authors to consider that VPSPr might be underestimated. “
“One of our macaques intracerebrally inoculated with a brain sample derived from a VPSPr patient (Met/Val at codon 129) exhibited after 8.5 years of silent incubation several episodes of self‐aggression first focused on his left leg, which extended to the whole hindquarters, and abnormalities of both hind limbs sensitive conduction.”
Yup, as predicted…that’s why ;
CANADA
Definite and probable CJD, 1998-2023
As of November 30, 2023
Year Sporadic Iatrogenic Genetic vCJD Total
SNIP...
https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref
2023, STILL NO MENTION OF VPSPr TSE Prion in Canada statistics...terry
FRIDAY, DECEMBER 02, 2022
Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html
FRIDAY, DECEMBER 24, 2021
Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021
https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html
Canada cjd 2020
https://creutzfeldt-jakob-disease.blogspot.com/2020/12/sporadic-creutzfeldt-jakob-disease-scjd.html
https://creutzfeldt-jakob-disease.blogspot.com/2019/08/creutzfeldt-jakob-disease-cjd-tse-prion.html
https://creutzfeldt-jakob-disease.blogspot.com/2018/12/creutzfeldt-jakob-disease-cjd-bse.html
https://creutzfeldt-jakob-disease.blogspot.com/2016/08/creutzfeldt-jakob-disease-usa-2015.html
http://creutzfeldt-jakob-disease.blogspot.com/2015/12/creutzfeldt-jakob-disease-cjd-tse-prion.html
http://creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html
Canada and United States Creutzfeldt Jakob TSE Prion Disease Incidence Rates Increasing
http://creutzfeldt-jakob-disease.blogspot.com/2015/03/canada-and-united-states-creutzfeldt.html
http://creutzfeldt-jakob-disease.blogspot.com/2012/11/sporadic-creutzfeldt-jakob-disease.html
https://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html
https://cjdmadcowbaseoct2007.blogspot.com/2008/01/cjd-human-tse-report-uk-usa-canada-and.html
Canada BSE vs CJD $$$
Commentary: The devastating legacy of Canada's Mad Cow Crisis The Mad Cow Crisis had a lasting impact on Canada's beef industry. Sylvain Charlebois
sylvain.charlebois@dal.ca
I remember well, “Shoot, Shovel, and Shut Up”!
"This all came about through the discovery of a single, isolated case of mad cow disease in one Alberta cow on May 20th. The farmer — I think he was a Louisiana fish farmer who knew nothing about cattle ranching. I guess any self-respecting rancher would have shot, shovelled and shut up, but he didn't do that." — Klein recalls how the mad cow crisis started and rancher Marwyn Peaster's role. The premier was speaking at the Western Governors Association meeting in Big Sky, Mont. September 2004.
"The premier meant that in an ironic or almost a sarcastic way." — Klein spokesman Gordon Turtle.
https://animalhealthreportpriontse.blogspot.com/2021/03/canadas-application-for-negligible-risk.html
OIE-WAHIS CANADA atypical BSE type H Bovine spongiform encephalopathy OIE-WAHIS
https://animalhealthreportpriontse.blogspot.com/2021/
Prion Conference 2009 Book of Abstracts
https://www.food.gov.uk/sites/default/files/media/document/prion2009_bookofabstracts.pdf
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada
Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these countries.
https://www.food.gov.uk/sites/default/files/media/document/prion2009_bookofabstracts.pdf
Cattle with the E211K polymorphism, and gCJD linked to a glutamic acid to lysine substitution at codon 200 (E200K) of PRNP, what if?
https://creutzfeldt-jakob-disease.blogspot.com/2026/01/cattle-with-e211k-polymorphism-and-gcjd.html
Cattle with the E211K vs Humans E200K of PRNP, what if?
https://prpsc.proboards.com/thread/195/cattle-e211k-humans-e200k-prnp
ARS Research Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025 Annual Report
Price of TSE Prion Poker Goes Up Again…terry
https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/ars-research-elucidating-pathobiology.html
TUESDAY, JANUARY 20, 2026
Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases Project Number 5P01AI077774-14 2025
https://chronic-wasting-disease.blogspot.com/2026/01/pathogenesis-transmission-and-detection.html
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
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
https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review
TUESDAY, JULY 13, 2021
Chronic Wasting Disease and the Canadian Agriculture and Agri-food Sectors Current Knowledge Risks and Policy Options
“The science is progressing on the possibility of transmission of CWD to humans through oral transmission, but the complete assessment of this possibility remains to be done.”
https://capi-icpa.ca/wp-content/uploads/2021/07/Chronic-Wasting-Disease_Full-Report_EN.pdf
https://chronic-wasting-disease.blogspot.com/2021/07/chronic-wasting-disease-and-canadian.html
FRIDAY, OCTOBER 31, 2025
***> Captive Cervid and the Economic Burden of Chronic Wasting Disease CWD TSE Prion?
The economic burden of ignoring CWD would be far greater, imo, with time, if no cervid were left, or just a select few, if the environment/property was so exposed and saturated with CWD, that you couldn’t sell it, you couldn’t grow crops because of the soil saturation of the CWD, water tables saturated with CWD, saturation of hay, grains, from crops uptake on said property, cervid meat saturated from Cervid CWD, remember, 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. So, what Do we do, how many humans and animals do we continue to expose, continue to saturate with the CWD TSE Prion, …
https://chronic-wasting-disease.blogspot.com/2025/10/captive-cervid-and-economic-burden-of.html
https://prpsc.proboards.com/thread/183/captive-cervid-economic-burden-prion
TUESDAY, APRIL 07, 2026
APHIS USDA Captive CWD Herds Update by State March 2026
Updated March 2026
https://www.aphis.usda.gov/sites/default/files/status-of-captive-herds.pdf
https://chronic-wasting-disease.blogspot.com/2026/04/aphis-usda-captive-cwd-herds-update-by.html
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
***> 2023 Professor John Collinge on tackling prion diseases <***
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.
https://www.ucl.ac.uk/brain-sciences/research/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases
Front. Public Health, 12 June 2024 Sec. Infectious Diseases: Epidemiology and Prevention Volume 12 - 2024 |
https://doi.org/10.3389/fpubh.2024.1411489
Updated global epidemiology atlas of human prion diseases The top seven countries in PrD cases were the USA (n = 5,156), France (n = 3,276), Germany (n = 3,212), Italy (n = 2,995), China (n = 2,662), the UK (n = 2,521), Spain (n = 1,657), and Canada (n = 1,311).
https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2024.1411489/full
Research Letter December 11, 2023 Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020
Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et al John A. Romley, PhD4; John C. Probasco, MD5 Author Affiliations Article Information JAMA Neurol. 2024;81(2):195-197. doi:10.1001/jamaneurol.2023.4678
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
Snip…
Discussion Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing. This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.
https://jamanetwork.com/journals/jamaneurology/fullarticle/2812784
Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023
The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis
Abstract
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.
snip...
The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1).
snip...
Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.
https://link.springer.com/article/10.1007/s10654-023-01004-5
Thursday that the actual federal contribution barely covered the operating costs of the program, especially in the last four years.
https://canp.ca/wp-content/uploads/2026/04/Neuropathologists-urge-Ottawa-to-bring-back-autopsy-services-for-patients-suspected-of-having-fatal-brain-illness-The-Globe-an.pdf
Terrible, Terrible news, but does not surprise me, with Creutzfeldt Jakob Disease cases rising (this is well documented), sure is a hell of a time to stop reporting it, right, good way to kill any potential link to New Brunswick Neurological Disease outbreak, or any other strange strain, phenotype, of human TSE PrP (i’m thinking Vpspr), strange Vpspr has not yet been documented in Canada, at least on paper. so sad. with chronic wasting disease cwd tse prp spreading across Canada and the USA, atypical and typical BSE, Scrapie, still happening and surveillance there from very questionable, with Mexico still clueless, and human cwd zoonotic disease staring us in the face, if not already happening, and CJD cases rising across the board, this is absolutely not scientific, and incredibly stupid (i’m thinking iatrogenic), that will be a free-for-all in every surgical medical arenas, i guess Canada will go back to SHOOT, SHOVEL, SHUT UP, SCIENCE, (if they ever left it), we all lose then…
MONDAY, FEBRUARY 23, 2026
Canada Ottawa to halt funding for tests to detect deaths from Creutzfeldt-Jakob disease
https://creutzfeldt-jakob-disease.blogspot.com/2026/02/canada-ottawa-to-halt-funding-for-tests.html
https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html
Canada Creutzfeldt Jakob Disease Surveillance Dashboard
https://health-infobase.canada.ca/diseases/cjd/dashboard.html
TUESDAY, APRIL 15, 2025
Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology
Recently, two young individuals, aged 15 and 21, were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) in Canada and the United States, respectively (D'Arcy et al., 2019; Ahn et al., 2024).
https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2025.1571662/full
https://creutzfeldt-jakob-disease.blogspot.com/2025/04/cases-of-creutzfeldt-jakob-disease-in.html
CANADA CJD TSE Prion
Creutzfeldt Jacob Disease CJD TSE Prion December 14, 2024 Annual Update
Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report
Definite and probable CJD, 1998-2025
As of August 31, 2025
Year Sporadic Iatrogenic Genetic vCJD Total
1998 22 1 1 0 24
1999 27 2 3 0 32
2000 32 0 3 0 35
2001 27 0 3 0 30
2002 31 0 4 1 36
2003 27 1 1 0 29
2004 42 0 2 0 44
2005 42 0 2 0 44
2006 39 0 5 0 44
2007 35 0 4 0 39
2008 48 0 1 0 49
2009 48 0 5 0 53
2010 35 0 3 0 38
2011 46 0 4 1 51
2012 63 0 1 0 64
2013 50 0 1 0 51
2014 52 0 4 0 56
2015 43 0 9 0 52
2016 61 1 6 0 68
2017 83 0 8 0 91
2018 76 1 6 0 83
2019 76 0 6 0 82
2020 68 0 4 0 72
2021 61 0 5 0 66
2022 93 0 3 0 96
2023 74 0 2. 0 76
2024 70 0 3 0 73
2025 28 0 1 0 29
Total 1,399 6 100 2 1,507
Cases with definite and probable diagnosis to date
Variant CJD (vCJD)
The CJDSS uses current, internationally accepted diagnostic criteria (PDF, 252 KB) to classify definite and probable cases of CJD in Canada.
https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref
Canada, still no documentation of VPSPr on paper…
VPSPr sporadic CJD
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
Brain Pathol. 2023 Sep; 33(Suppl 1): e13194.
Published online 2023 Sep 6.
https://doi.org/10.1111/bpa.13194
PMCID: PMC10483180
PMID: 37674376
Special Issue: Abstracts of the 20th International Congress of Neuropathology, Berlin, Germany, September 13–16, 2023
snip...
Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)
J. Mikol 1, J. Delmotte1, W. Zou2,3, J. P. Deslys1, E. Comoy1 1Commissariat à l'energie Atomique, Institut François Jacob/Prion research Group, Fontenay‐aux‐Roses, France; 2Case Western Reserve University School of Medicine, Department of Pathology, Cleveland, OH, United States; 3Institute of Neurology, Nanchang, China
VPSPr, a novel rare human prion disease described in 2010 is characterized by the accumulation of abnormal PrPd with a limited resistance to proteolysis. It showed uncertain infectivity in transgenic mouse models. Recent publications report VPSPr cases with different unevocative clinical pictures, leading the authors to consider that VPSPr might be underestimated. One of our macaques intracerebrally inoculated with a brain sample derived from a VPSPr patient (Met/Val at codon 129) exhibited after 8.5 years of silent incubation several episodes of self‐aggression first focused on his left leg, which extended to the whole hindquarters, and abnormalities of both hind limbs sensitive conduction. We performed euthanasia 4 months later for humane reasons and examined brain tissues with histological techniques. Pathological examination revealed a spongiform change decreasing from the frontal to the occipital cortex, also present in the thalamus, the basal ganglia, the hippocampus, mild brainstem and spinal cord, accompanied with a massive neuronal vacuolation and reactive astrocytosis. Purkinje cells were rarefied and vacuolated. Abnormal PrPd deposition was present as synaptic deposits mixed with some mini‐aggregates of PrPd diffused in the tissue or packed in small fussy unlimited area of variable intensity, appearing as pre‐plaques. These lesions, never previously observed in primates exposed to other prion strains, were mainly observed in the insula and the frontal cortex but were absent in the thalamus, the basal ganglia and the hippocampus. Furthermore, we observed massive Ab deposits, but not in the hippocampus except the subiculum, not co‐localized with PrPd. In contrast, a two‐times older animal had three‐times less deposits. The original disease phenotype observed in this primate will be discussed and compared to the expressions of VPSPr in humans, and other prion diseases in the macaque model. PS9‐ND‐A186
https://europepmc.org/article/MED/37674376
“Recent publications report VPSPr cases with different unevocative clinical pictures, leading the authors to consider that VPSPr might be underestimated. “
“One of our macaques intracerebrally inoculated with a brain sample derived from a VPSPr patient (Met/Val at codon 129) exhibited after 8.5 years of silent incubation several episodes of self‐aggression first focused on his left leg, which extended to the whole hindquarters, and abnormalities of both hind limbs sensitive conduction.”
Yup, as predicted…that’s why ;
CANADA
Definite and probable CJD, 1998-2023
As of November 30, 2023
Year Sporadic Iatrogenic Genetic vCJD Total
SNIP...
https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref
2023, STILL NO MENTION OF VPSPr TSE Prion in Canada statistics...terry
FRIDAY, DECEMBER 02, 2022
Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html
FRIDAY, DECEMBER 24, 2021
Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021
https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html
Canada cjd 2020
https://creutzfeldt-jakob-disease.blogspot.com/2020/12/sporadic-creutzfeldt-jakob-disease-scjd.html
https://creutzfeldt-jakob-disease.blogspot.com/2019/08/creutzfeldt-jakob-disease-cjd-tse-prion.html
https://creutzfeldt-jakob-disease.blogspot.com/2018/12/creutzfeldt-jakob-disease-cjd-bse.html
https://creutzfeldt-jakob-disease.blogspot.com/2016/08/creutzfeldt-jakob-disease-usa-2015.html
http://creutzfeldt-jakob-disease.blogspot.com/2015/12/creutzfeldt-jakob-disease-cjd-tse-prion.html
http://creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html
Canada and United States Creutzfeldt Jakob TSE Prion Disease Incidence Rates Increasing
http://creutzfeldt-jakob-disease.blogspot.com/2015/03/canada-and-united-states-creutzfeldt.html
http://creutzfeldt-jakob-disease.blogspot.com/2012/11/sporadic-creutzfeldt-jakob-disease.html
https://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html
https://cjdmadcowbaseoct2007.blogspot.com/2008/01/cjd-human-tse-report-uk-usa-canada-and.html
Canada BSE vs CJD $$$
Commentary: The devastating legacy of Canada's Mad Cow Crisis The Mad Cow Crisis had a lasting impact on Canada's beef industry. Sylvain Charlebois
sylvain.charlebois@dal.ca
I remember well, “Shoot, Shovel, and Shut Up”!
"This all came about through the discovery of a single, isolated case of mad cow disease in one Alberta cow on May 20th. The farmer — I think he was a Louisiana fish farmer who knew nothing about cattle ranching. I guess any self-respecting rancher would have shot, shovelled and shut up, but he didn't do that." — Klein recalls how the mad cow crisis started and rancher Marwyn Peaster's role. The premier was speaking at the Western Governors Association meeting in Big Sky, Mont. September 2004.
"The premier meant that in an ironic or almost a sarcastic way." — Klein spokesman Gordon Turtle.
https://animalhealthreportpriontse.blogspot.com/2021/03/canadas-application-for-negligible-risk.html
OIE-WAHIS CANADA atypical BSE type H Bovine spongiform encephalopathy OIE-WAHIS
https://animalhealthreportpriontse.blogspot.com/2021/
Prion Conference 2009 Book of Abstracts
https://www.food.gov.uk/sites/default/files/media/document/prion2009_bookofabstracts.pdf
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada
Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these countries.
https://www.food.gov.uk/sites/default/files/media/document/prion2009_bookofabstracts.pdf
Cattle with the E211K polymorphism, and gCJD linked to a glutamic acid to lysine substitution at codon 200 (E200K) of PRNP, what if?
https://creutzfeldt-jakob-disease.blogspot.com/2026/01/cattle-with-e211k-polymorphism-and-gcjd.html
Cattle with the E211K vs Humans E200K of PRNP, what if?
https://prpsc.proboards.com/thread/195/cattle-e211k-humans-e200k-prnp
ARS Research Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025 Annual Report
Price of TSE Prion Poker Goes Up Again…terry
https://transmissiblespongiformencephalopathy.blogspot.com/2025/12/ars-research-elucidating-pathobiology.html
TUESDAY, JANUARY 20, 2026
Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases Project Number 5P01AI077774-14 2025
https://chronic-wasting-disease.blogspot.com/2026/01/pathogenesis-transmission-and-detection.html
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
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
https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review
TUESDAY, JULY 13, 2021
Chronic Wasting Disease and the Canadian Agriculture and Agri-food Sectors Current Knowledge Risks and Policy Options
“The science is progressing on the possibility of transmission of CWD to humans through oral transmission, but the complete assessment of this possibility remains to be done.”
https://capi-icpa.ca/wp-content/uploads/2021/07/Chronic-Wasting-Disease_Full-Report_EN.pdf
https://chronic-wasting-disease.blogspot.com/2021/07/chronic-wasting-disease-and-canadian.html
FRIDAY, OCTOBER 31, 2025
***> Captive Cervid and the Economic Burden of Chronic Wasting Disease CWD TSE Prion?
The economic burden of ignoring CWD would be far greater, imo, with time, if no cervid were left, or just a select few, if the environment/property was so exposed and saturated with CWD, that you couldn’t sell it, you couldn’t grow crops because of the soil saturation of the CWD, water tables saturated with CWD, saturation of hay, grains, from crops uptake on said property, cervid meat saturated from Cervid CWD, remember, 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. So, what Do we do, how many humans and animals do we continue to expose, continue to saturate with the CWD TSE Prion, …
https://chronic-wasting-disease.blogspot.com/2025/10/captive-cervid-and-economic-burden-of.html
https://prpsc.proboards.com/thread/183/captive-cervid-economic-burden-prion
TUESDAY, APRIL 07, 2026
APHIS USDA Captive CWD Herds Update by State March 2026
Updated March 2026
https://www.aphis.usda.gov/sites/default/files/status-of-captive-herds.pdf
https://chronic-wasting-disease.blogspot.com/2026/04/aphis-usda-captive-cwd-herds-update-by.html
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
***> 2023 Professor John Collinge on tackling prion diseases <***
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.
https://www.ucl.ac.uk/brain-sciences/research/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases
Front. Public Health, 12 June 2024 Sec. Infectious Diseases: Epidemiology and Prevention Volume 12 - 2024 |
https://doi.org/10.3389/fpubh.2024.1411489
Updated global epidemiology atlas of human prion diseases The top seven countries in PrD cases were the USA (n = 5,156), France (n = 3,276), Germany (n = 3,212), Italy (n = 2,995), China (n = 2,662), the UK (n = 2,521), Spain (n = 1,657), and Canada (n = 1,311).
https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2024.1411489/full
Research Letter December 11, 2023 Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020
Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et al John A. Romley, PhD4; John C. Probasco, MD5 Author Affiliations Article Information JAMA Neurol. 2024;81(2):195-197. doi:10.1001/jamaneurol.2023.4678
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
Snip…
Discussion Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing. This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.
https://jamanetwork.com/journals/jamaneurology/fullarticle/2812784
Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023
The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis
Abstract
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.
snip...
The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1).
snip...
Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.
https://link.springer.com/article/10.1007/s10654-023-01004-5
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
https://jamanetwork.com/journals/jama/article-abstract/1031186
2023
https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
Senator Cornyn helped me long ago when the NIH et al were threatening to destroy our loved ones brains we had donated for research, Senator Cornyn helped us stop that, and we graciously thank him for that. But I don’t think John Cornyn can save us this time, for obvious reasons…
https://www.upi.com/NIH-may-destroy-human-brain-collection/84811111671758/
https://www.upi.com/Groups-seek-to-save-NIH-brain-collection/72961112392131/
https://www.upi.com/NIH-says-it-will-preserve-CJD-brains/67711117574761/
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html
https://www.upi.com/Science_News/2003/12/30/Mad-Cow-Linked-to-thousands-of-CJD-cases/47861072816318/
https://www.upi.com/Health_News/2005/10/21/Questions-linger-in-US-CJD-cases/65761129945790/
THURSDAY, APRIL 03, 2025
Canada, New Brunswick, cases of an undiagnosed neurological illness cases, update
I thought I should give an update on Canada, New Brunswick, cases of an undiagnosed neurological illness cases, that so far, has ruled out any prion disease such as Creutzfeldt-Jakob disease (CJD), per Michael Coulthart, who is the head of the Canadian Creutzfeldt-Jakob Disease Surveillance System. …terry
https://creutzfeldt-jakob-disease.blogspot.com/2025/04/canada-new-brunswick-cases-of.html
iatrogenic Transmissible Spongiform Encephalopathy, Friendly Fire, Pass It Forward, Unforeseen Circumstances
https://itseprion.blogspot.com/
SATURDAY, DECEMBER 18, 2021
CFIA Canada Alberta Laboratory detection of atypical bovine spongiform encephalopathy
https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html
TUESDAY, DECEMBER 21, 2021
OIE-WAHIS CANADA atypical BSE type H Bovine spongiform encephalopathy
https://animalhealthreportpriontse.blogspot.com/2021/12/oie-wahis-canada-atypical-bse-type-h.html
https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html
https://animalhealthreportpriontse.blogspot.com/2021/12/oie-wahis-canada-atypical-bse-type-h.html
YOU can see where the concerns seem to be more for the cattle industry, then surveillance for deadly Human BSE TSE Prion and all it’s variants…terry
BSE testing drags on Canadian cattle sector
Canada has a clean bill of health when it comes to mad cow disease, but we’re still testing like we’re on high alert
By Roy Lewis Reading Time: 3 minutes Published: 2 days ago Livestock
The animal health community continues to debate the need for ongoing BSE testing in Canada, even though the country has not had a case since 2015 and has achieved negligible risk status. The animal health community continues to debate the need for ongoing BSE testing in Canada, even though the country has not had a case since 2015 and has achieved negligible risk status. Photo: File Testing for BSE at slaughterhouses continues, despite the dramatic decline in cases in recent years and the fact that Canada’s last case was in 2015.
I had hoped that requiring meat plants to test specified risk materials such as spinal cords, brains and other nervous-related areas would have been eliminated once the country achieved negligible risk status.
However, the program is still in effect, resulting in higher costs for Canadian plants than for their American counterparts.
WHY IT MATTERS: Continued BSE testing and its extra costs remain a heated topic within the veterinary and agricultural communities.
Some experts argue that rigorous testing protocols should be maintained to prevent any resurgence of the disease, while others believe the risk is now sufficiently low enough to warrant a reduction in testing.
I would definitely agree with the latter.
One thing is certain: BSE left a lasting impact on global food safety policies.
The disease, which first emerged prominently in the 1980s in Great Britain, caused widespread concern and led to the implementation of stringent testing and control measures.
These measures were instrumental in identifying and isolating infected animals, thereby protecting the food supply chain from potential contamination.
Veterinarians were recently told that the BSE program would be changing March 7, but that has since changed. For now, it is the same as usual for the distant future.
The proposed changes would have been to just sample neurological cases that have the same type clinical signs as BSE.
It makes sense to do surveillance on unusual neurological cases, but for now, we are back to the old program.
In Alberta, this means veterinarians conducting complete autopsies on dead, dying, down and diseased cases, and the brain stem sampled and sent to the lab. This costs producers nothing, and in Alberta, they receive some compensation.
I have always said that if there was anything else that the Canadian Food Inspection Agency needed sampled, either for surveillance or research, it could be done by the veterinarians that were essentially certified to do the testing.
I realize that in Alberta it was done by licensed vets certified for collection of the samples, and each province has different protocols.
I do hope that more than 20 years of testing have shown producers the value of the complete postmortem.
Most times, a veterinarian can determine the cause of death from just the gross necropsy, and if not, samples can be taken and sent away.
I realize some cases are chronic, and producers have a good idea what they’re dealing with.
However, especially with sudden deaths and non-specific chronic cases, it is good to find out the positive diagnosis. Is it the start of a herd problem or just an individual case? Are you getting too many peritonitis or thin cows from kidney infection or Johne’s disease? Are you retaining cows until they’re too old and are missing teeth?
Much can be learned from an autopsy, and even if in the future the BSE program is reduced or eliminated, some of these cows and bulls should still be autopsied.
Autopsies are about helping with disease prevention and assessing cattle’s health.
I would have hoped that more than 20 years of the BSE program would have shown the need to monitor the prevalence of other diseases, similar to what the Alberta Veterinary Surveillance Network used to do years ago.
However, I am sure that if something worthy came along, it could be added to the program.
It is good for all of us to see how a co-operative spirit and dedication to testing essentially eradicated BSE in Canada.
It was a prime example of the field-practicing veterinarians working together with the slaughter plants, producers, provincial vets and the CFIA. It was also a win for the Canadian Cattle Association and its provincial counterparts.
The crisis helped unite us much as the threat of tariffs is today.
The CCA is much better off with us all on board working on disease surveillance and being as transparent as we can about the findings. It may be right over the next several months to state what the BSE program has meant to you on your individual farms and ranches.
Today, the news focuses on avian influenza in dairy cattle, foot-and-mouth disease in Europe and bovine tuberculosis in Saskatchewan.
We are stronger if we collectively work on a goal together, and the BSE example has taught us all a lot about how to do just that —“collaborate” for a common end goal.
Roy Lewis Columnist Roy Lewis practised large-animal veterinary medicine for more than 30 years and now works part time as a technical services veterinarian for Merck Animal Health.
https://www.manitobacooperator.ca/livestock/bse-testing-drags-on-canadian-cattle-sector/
CANADA BSE Flying E Ranche Ltd. v. Attorney General of Canada, 2022 ONSC 601
Part XIV - Conclusion
My responses to the Common Issues are as follows:
1. Does section 9 of the Crown Liability and Proceedings Act bar the class members' claim against the government of Canada?
Answer: Yes.
2. Were the defendants negligent and if so, when and how?
Answer: No.
3. Can the amount of compensatory damages, if any, be reasonably determined on an individual basis? If so, how should individual damages be determined?
Answer: As requested, I have not addressed this question.
4. If the answer to question 3 is no, can the amount of compensatory damages, if any, be determined on an aggregate basis? If so, what is the amount of damages and how should they be distributed?
Answer: Had I answered questions 1 and 2 differently, I would have awarded $1.163 billion as compensatory damages to the Class. I have not addressed how damages should be distributed.
It follows that the action is dismissed. Should the parties be unable to agree on costs within 45 days of the release of these Reasons, they may contact my assistant to arrange an appointment to address the procedure for their determination.
Date: 28 January 2022
https://static.albertafarmexpress.ca/wp-content/uploads/2022/03/16140632/Flying-E-Ranch-Ltd.-v.-Attorney-General-of-Canada-Reasons-for-Judgment-PBS-FINAL-2022-Jan-28.pdf
CANADA BSE Flying E Ranche Ltd. v. Attorney General of Canada, 2022 ONSC 601
Date: 28 January 2022
https://static.albertafarmexpress.ca/wp-content/uploads/2022/03/16140632/Flying-E-Ranch-Ltd.-v.-Attorney-General-of-Canada-Reasons-for-Judgment-PBS-FINAL-2022-Jan-28.pdf
Supreme Court denies appeal of BSE class-action lawsuit
OTTAWA — An $8-billion class-action lawsuit on behalf of Canadian cattle producers quietly reached a dead end at the Supreme Court last fall, concluding a legal saga that began 20 years ago in the aftermath of the Bovine Spongiform Encephalopathy (BSE) crisis.
The high court closed the final door on the lawsuit on Oct. 10 when it refused to hear an appeal that, if successful, could have revived the plaintiffs’ multi-billion-dollar case against the federal government. An Ontario Superior Court judge had dismissed the lawsuit three years ago, and the Ontario Court of Appeal denied an appeal of that dismissal on Jan. 31, 2024. Duncan Boswell, senior partner with Gowling WLG in Toronto and lead counsel in the suit, then made the unsuccessful request for an appeal to the Supreme Court.
The class-action lawsuit by Alberta’s Flying E Ranche Ltd. stood to benefit as many as 100,000 Canadian producers in the event of a win. The suit blamed the federal government for a BSE-infected cow that turned up in Alberta in 2003 because, years earlier, Canada negligently allowed importation of cattle from Britain when that country was known to have animals infected with BSE, or mad cow disease. Consequently, the U.S. and other countries ruinously closed their borders to Canadian beef as a result of the Alberta discovery. The legal odyssey dates back to 2005 when the original claims were heard in four provinces before the suit moved exclusively to Ontario court. It was in 2005 that the U.S. allowed young Canadian cattle, under 30 months of age, back into its marketplace. Other countries shut out Canadian beef for much longer.
It was only in 2023 that Japan finally reopened its market fully to Canadian beef.
https://farmersforum.com/supreme-court-denies-appeal-of-bse-class-action-lawsuit/
USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review
USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review of Progress, or not so much, of the attempts to try and eradicate, or at least control the spread of Chronic Wasting Disease CWD TSE PrP. i put that in AI Google, too see what would come up… terry
to AI trials and tribulations and failures of science of CWD Transmissible Spongiform Encephalopathy
AI answer; The scientific pursuit to understand, detect, and control Chronic Wasting Disease (CWD)—a Transmissible Spongiform Encephalopathy (TSE) affecting cervids—has been characterized by significant, long-term challenges, slow progress, and frustrating management failures. CWD is a 100% fatal, infectious, and resilient prion disease that has spread to 24 U.S. states, 3 Canadian provinces, South Korea, and Norway, defying many traditional wildlife management strategies.
Here is an overview of the trials, tribulations, and failures of CWD science:
1. Scientific Tribulations and Challenges Insidious Nature of Prions: Unlike bacteria or viruses, prions are misfolded proteins that are extremely resilient. They resist traditional disinfection, freezing, incineration below 1100°F, radiation, and desiccation. They persist in the environment for years, making containment nearly impossible.
Long Incubation Periods: CWD has a long pre-clinical phase where animals look healthy but shed prions, with incubation ranging from 15 to over 34 months. This makes early detection in wild populations exceptionally difficult.
Environmental Reservoirs: Research has struggled to map how prions bind to soil and minerals, which creates long-lasting "hotspots" of infection.
Diagnostic Limitations: While post-mortem testing of lymph nodes or brain tissue is accurate, it is not practical for massive, live wild populations. Live-animal testing (e.g., rectal biopsy) is less sensitive and cannot effectively monitor the scale of the spread.
2. Scientific Failures and Unresolved Questions Inconclusive Zoonotic Risk: A major failure of scientific communication and research has been the inability to definitively rule out whether CWD can infect humans. While no cases have been reported, laboratory studies on transgenic mice and macaques have yielded mixed results, leading to a "high level of uncertainty".
Failed Containment: Management strategies, including population reduction and, in some cases, eradication of infected herds, have largely failed to halt the spread in the wild. The disease continues to spread geographically and increase in prevalence.
Inconsistent Data: Studies on management, such as culling, have produced conflicting results, with some showing effectiveness and others showing no difference.
3. Management and Policy Failures Captive Herd Industry: A major, ongoing failure is the inability to strictly regulate the captive deer industry, which has acted as a primary driver of long-distance, artificial spread of the disease.
Inconsistent Governance: State wildlife agencies have often failed to apply consistent, science-based regulations, bowing to pressure from the captive deer industry, leading to loopholes in testing and, in some cases, the spread of the disease through the movement of contaminated, farmed deer.
Human Activity Drivers: Failure to curb human-caused concentration of animals, such as baiting and feeding, has worsened the spread.
4. Recent Scientific Advancements (The Path Forward) Ultrasensitive Detection (RT-QuIC): The development of Real-Time Quaking-Induced Conversion (RT-QuIC) and Serial Protein Misfolding Amplification (PMCA) has allowed scientists to detect very low levels of prions, improving the ability to identify prions in soil, water, and even in live animals (e.g., in feces or saliva).
Better Environmental Sampling: Techniques to sample contaminated, environmentally relevant surfaces like metal fences or water sources are now being developed.
The science of CWD has moved from a lack of understanding to a state of high alarm, driven by the realization that this disease is not just a localized issue but a persistent, growing threat to wildlife, ecosystems, and potentially human health. end…TSS
not bad.
please let me elaborate on the dire consequences we face with CWD TSE PrP, if we fail to act swiftly, with sound science, and NOT let the industry continue to legislate junk science, and regulations there from, by trucking CWD to hell and back, no better example is Texas…
USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review
https://www.researchgate.net/publication/400772766_USAHA_129th_Annual_Meeting_CWD_TSE_PrP_2025_and_a_Decade_Review
SATURDAY, APRIL 11, 2026
Chronic Wasting Disease CWD TSE PrP, Cervid, Genetic Manipulation, Unforeseen Circumstances
https://chronic-wasting-disease.blogspot.com/2026/04/chronic-wasting-disease-cwd-tse-prp.html
*** Grant Agreement number: 222887 ***
*** Project acronym: PRIORITY ***
*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***
Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014
Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es
Project website¡ Error! Marcador no definido. address: www.prionpriority.eu
PRIORITY, PROJECT FINAL REPORT
*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***
snip...
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
Block D: Prion epidemiology
Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.
In parallel, studies in sheep have concluded that:
*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes
*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.
*** The combination of data from all our studies leads us to conclude that:
*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).
*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.
snip...
We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:
a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:
• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;
• the uncertainties related to prion epidemiology in animal populations;
• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents; • the intrinsic capacity of prions to cross interspecies transmission barriers; • the lack of sensitive methodology for identifying cross contamination in food.
• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.
The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.
b. Atypical prion agents and surveillance
Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.
*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.
*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.
*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.
*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable
• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)
• to improve the sensitivity of detection assays that are applied in the field towards this type of agent
• to maintain a robust surveillance of both animal and human populations
c. The need for extended research on prions
Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.
Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.
Position of the Priority consortium
Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk
Origins of prion epidemies
Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.
In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.
The human prion problem
The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.
Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.
The animal prion problem
An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.
We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.
The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
SUNDAY, MARCH 8, 2026
Texas Creutzfeldt-Jakob Disease Deaths and Death Rates per Year (2013-2022) More Than Tripled, and case reporting has ceased since then
https://cjdtexas.blogspot.com/2026/03/texas-creutzfeldt-jakob-disease-deaths.html
https://prpsc.proboards.com/thread/209/texas-cases-more-triples-2013
WEDNESDAY, OCTOBER 15, 2025
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025
https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
SATURDAY, JANUARY 10, 2026
Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease, a review
https://creutzfeldt-jakob-disease.blogspot.com/2026/01/neuropsychiatric-symptoms-in-sporadic.html
https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review
SUNDAY, MARCH 23, 2025
Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html
FRIDAY, DECEMBER 13, 2024
Creutzfeldt Jacob Disease CJD, BSE, CWD, TSE Prion, December 14, 2024 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html
Terry S. Singeltary Sr.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
https://jamanetwork.com/journals/jama/article-abstract/1031186
2023
https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html
Senator Cornyn helped me long ago when the NIH et al were threatening to destroy our loved ones brains we had donated for research, Senator Cornyn helped us stop that, and we graciously thank him for that. But I don’t think John Cornyn can save us this time, for obvious reasons…
https://www.upi.com/NIH-may-destroy-human-brain-collection/84811111671758/
https://www.upi.com/Groups-seek-to-save-NIH-brain-collection/72961112392131/
https://www.upi.com/NIH-says-it-will-preserve-CJD-brains/67711117574761/
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html
https://www.upi.com/Science_News/2003/12/30/Mad-Cow-Linked-to-thousands-of-CJD-cases/47861072816318/
https://www.upi.com/Health_News/2005/10/21/Questions-linger-in-US-CJD-cases/65761129945790/
THURSDAY, APRIL 03, 2025
Canada, New Brunswick, cases of an undiagnosed neurological illness cases, update
I thought I should give an update on Canada, New Brunswick, cases of an undiagnosed neurological illness cases, that so far, has ruled out any prion disease such as Creutzfeldt-Jakob disease (CJD), per Michael Coulthart, who is the head of the Canadian Creutzfeldt-Jakob Disease Surveillance System. …terry
https://creutzfeldt-jakob-disease.blogspot.com/2025/04/canada-new-brunswick-cases-of.html
iatrogenic Transmissible Spongiform Encephalopathy, Friendly Fire, Pass It Forward, Unforeseen Circumstances
https://itseprion.blogspot.com/
SATURDAY, DECEMBER 18, 2021
CFIA Canada Alberta Laboratory detection of atypical bovine spongiform encephalopathy
https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html
TUESDAY, DECEMBER 21, 2021
OIE-WAHIS CANADA atypical BSE type H Bovine spongiform encephalopathy
https://animalhealthreportpriontse.blogspot.com/2021/12/oie-wahis-canada-atypical-bse-type-h.html
https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html
https://animalhealthreportpriontse.blogspot.com/2021/12/oie-wahis-canada-atypical-bse-type-h.html
YOU can see where the concerns seem to be more for the cattle industry, then surveillance for deadly Human BSE TSE Prion and all it’s variants…terry
BSE testing drags on Canadian cattle sector
Canada has a clean bill of health when it comes to mad cow disease, but we’re still testing like we’re on high alert
By Roy Lewis Reading Time: 3 minutes Published: 2 days ago Livestock
The animal health community continues to debate the need for ongoing BSE testing in Canada, even though the country has not had a case since 2015 and has achieved negligible risk status. The animal health community continues to debate the need for ongoing BSE testing in Canada, even though the country has not had a case since 2015 and has achieved negligible risk status. Photo: File Testing for BSE at slaughterhouses continues, despite the dramatic decline in cases in recent years and the fact that Canada’s last case was in 2015.
I had hoped that requiring meat plants to test specified risk materials such as spinal cords, brains and other nervous-related areas would have been eliminated once the country achieved negligible risk status.
However, the program is still in effect, resulting in higher costs for Canadian plants than for their American counterparts.
WHY IT MATTERS: Continued BSE testing and its extra costs remain a heated topic within the veterinary and agricultural communities.
Some experts argue that rigorous testing protocols should be maintained to prevent any resurgence of the disease, while others believe the risk is now sufficiently low enough to warrant a reduction in testing.
I would definitely agree with the latter.
One thing is certain: BSE left a lasting impact on global food safety policies.
The disease, which first emerged prominently in the 1980s in Great Britain, caused widespread concern and led to the implementation of stringent testing and control measures.
These measures were instrumental in identifying and isolating infected animals, thereby protecting the food supply chain from potential contamination.
Veterinarians were recently told that the BSE program would be changing March 7, but that has since changed. For now, it is the same as usual for the distant future.
The proposed changes would have been to just sample neurological cases that have the same type clinical signs as BSE.
It makes sense to do surveillance on unusual neurological cases, but for now, we are back to the old program.
In Alberta, this means veterinarians conducting complete autopsies on dead, dying, down and diseased cases, and the brain stem sampled and sent to the lab. This costs producers nothing, and in Alberta, they receive some compensation.
I have always said that if there was anything else that the Canadian Food Inspection Agency needed sampled, either for surveillance or research, it could be done by the veterinarians that were essentially certified to do the testing.
I realize that in Alberta it was done by licensed vets certified for collection of the samples, and each province has different protocols.
I do hope that more than 20 years of testing have shown producers the value of the complete postmortem.
Most times, a veterinarian can determine the cause of death from just the gross necropsy, and if not, samples can be taken and sent away.
I realize some cases are chronic, and producers have a good idea what they’re dealing with.
However, especially with sudden deaths and non-specific chronic cases, it is good to find out the positive diagnosis. Is it the start of a herd problem or just an individual case? Are you getting too many peritonitis or thin cows from kidney infection or Johne’s disease? Are you retaining cows until they’re too old and are missing teeth?
Much can be learned from an autopsy, and even if in the future the BSE program is reduced or eliminated, some of these cows and bulls should still be autopsied.
Autopsies are about helping with disease prevention and assessing cattle’s health.
I would have hoped that more than 20 years of the BSE program would have shown the need to monitor the prevalence of other diseases, similar to what the Alberta Veterinary Surveillance Network used to do years ago.
However, I am sure that if something worthy came along, it could be added to the program.
It is good for all of us to see how a co-operative spirit and dedication to testing essentially eradicated BSE in Canada.
It was a prime example of the field-practicing veterinarians working together with the slaughter plants, producers, provincial vets and the CFIA. It was also a win for the Canadian Cattle Association and its provincial counterparts.
The crisis helped unite us much as the threat of tariffs is today.
The CCA is much better off with us all on board working on disease surveillance and being as transparent as we can about the findings. It may be right over the next several months to state what the BSE program has meant to you on your individual farms and ranches.
Today, the news focuses on avian influenza in dairy cattle, foot-and-mouth disease in Europe and bovine tuberculosis in Saskatchewan.
We are stronger if we collectively work on a goal together, and the BSE example has taught us all a lot about how to do just that —“collaborate” for a common end goal.
Roy Lewis Columnist Roy Lewis practised large-animal veterinary medicine for more than 30 years and now works part time as a technical services veterinarian for Merck Animal Health.
https://www.manitobacooperator.ca/livestock/bse-testing-drags-on-canadian-cattle-sector/
CANADA BSE Flying E Ranche Ltd. v. Attorney General of Canada, 2022 ONSC 601
Part XIV - Conclusion
My responses to the Common Issues are as follows:
1. Does section 9 of the Crown Liability and Proceedings Act bar the class members' claim against the government of Canada?
Answer: Yes.
2. Were the defendants negligent and if so, when and how?
Answer: No.
3. Can the amount of compensatory damages, if any, be reasonably determined on an individual basis? If so, how should individual damages be determined?
Answer: As requested, I have not addressed this question.
4. If the answer to question 3 is no, can the amount of compensatory damages, if any, be determined on an aggregate basis? If so, what is the amount of damages and how should they be distributed?
Answer: Had I answered questions 1 and 2 differently, I would have awarded $1.163 billion as compensatory damages to the Class. I have not addressed how damages should be distributed.
It follows that the action is dismissed. Should the parties be unable to agree on costs within 45 days of the release of these Reasons, they may contact my assistant to arrange an appointment to address the procedure for their determination.
Date: 28 January 2022
https://static.albertafarmexpress.ca/wp-content/uploads/2022/03/16140632/Flying-E-Ranch-Ltd.-v.-Attorney-General-of-Canada-Reasons-for-Judgment-PBS-FINAL-2022-Jan-28.pdf
CANADA BSE Flying E Ranche Ltd. v. Attorney General of Canada, 2022 ONSC 601
Date: 28 January 2022
https://static.albertafarmexpress.ca/wp-content/uploads/2022/03/16140632/Flying-E-Ranch-Ltd.-v.-Attorney-General-of-Canada-Reasons-for-Judgment-PBS-FINAL-2022-Jan-28.pdf
Supreme Court denies appeal of BSE class-action lawsuit
OTTAWA — An $8-billion class-action lawsuit on behalf of Canadian cattle producers quietly reached a dead end at the Supreme Court last fall, concluding a legal saga that began 20 years ago in the aftermath of the Bovine Spongiform Encephalopathy (BSE) crisis.
The high court closed the final door on the lawsuit on Oct. 10 when it refused to hear an appeal that, if successful, could have revived the plaintiffs’ multi-billion-dollar case against the federal government. An Ontario Superior Court judge had dismissed the lawsuit three years ago, and the Ontario Court of Appeal denied an appeal of that dismissal on Jan. 31, 2024. Duncan Boswell, senior partner with Gowling WLG in Toronto and lead counsel in the suit, then made the unsuccessful request for an appeal to the Supreme Court.
The class-action lawsuit by Alberta’s Flying E Ranche Ltd. stood to benefit as many as 100,000 Canadian producers in the event of a win. The suit blamed the federal government for a BSE-infected cow that turned up in Alberta in 2003 because, years earlier, Canada negligently allowed importation of cattle from Britain when that country was known to have animals infected with BSE, or mad cow disease. Consequently, the U.S. and other countries ruinously closed their borders to Canadian beef as a result of the Alberta discovery. The legal odyssey dates back to 2005 when the original claims were heard in four provinces before the suit moved exclusively to Ontario court. It was in 2005 that the U.S. allowed young Canadian cattle, under 30 months of age, back into its marketplace. Other countries shut out Canadian beef for much longer.
It was only in 2023 that Japan finally reopened its market fully to Canadian beef.
https://farmersforum.com/supreme-court-denies-appeal-of-bse-class-action-lawsuit/
USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review
USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review of Progress, or not so much, of the attempts to try and eradicate, or at least control the spread of Chronic Wasting Disease CWD TSE PrP. i put that in AI Google, too see what would come up… terry
to AI trials and tribulations and failures of science of CWD Transmissible Spongiform Encephalopathy
AI answer; The scientific pursuit to understand, detect, and control Chronic Wasting Disease (CWD)—a Transmissible Spongiform Encephalopathy (TSE) affecting cervids—has been characterized by significant, long-term challenges, slow progress, and frustrating management failures. CWD is a 100% fatal, infectious, and resilient prion disease that has spread to 24 U.S. states, 3 Canadian provinces, South Korea, and Norway, defying many traditional wildlife management strategies.
Here is an overview of the trials, tribulations, and failures of CWD science:
1. Scientific Tribulations and Challenges Insidious Nature of Prions: Unlike bacteria or viruses, prions are misfolded proteins that are extremely resilient. They resist traditional disinfection, freezing, incineration below 1100°F, radiation, and desiccation. They persist in the environment for years, making containment nearly impossible.
Long Incubation Periods: CWD has a long pre-clinical phase where animals look healthy but shed prions, with incubation ranging from 15 to over 34 months. This makes early detection in wild populations exceptionally difficult.
Environmental Reservoirs: Research has struggled to map how prions bind to soil and minerals, which creates long-lasting "hotspots" of infection.
Diagnostic Limitations: While post-mortem testing of lymph nodes or brain tissue is accurate, it is not practical for massive, live wild populations. Live-animal testing (e.g., rectal biopsy) is less sensitive and cannot effectively monitor the scale of the spread.
2. Scientific Failures and Unresolved Questions Inconclusive Zoonotic Risk: A major failure of scientific communication and research has been the inability to definitively rule out whether CWD can infect humans. While no cases have been reported, laboratory studies on transgenic mice and macaques have yielded mixed results, leading to a "high level of uncertainty".
Failed Containment: Management strategies, including population reduction and, in some cases, eradication of infected herds, have largely failed to halt the spread in the wild. The disease continues to spread geographically and increase in prevalence.
Inconsistent Data: Studies on management, such as culling, have produced conflicting results, with some showing effectiveness and others showing no difference.
3. Management and Policy Failures Captive Herd Industry: A major, ongoing failure is the inability to strictly regulate the captive deer industry, which has acted as a primary driver of long-distance, artificial spread of the disease.
Inconsistent Governance: State wildlife agencies have often failed to apply consistent, science-based regulations, bowing to pressure from the captive deer industry, leading to loopholes in testing and, in some cases, the spread of the disease through the movement of contaminated, farmed deer.
Human Activity Drivers: Failure to curb human-caused concentration of animals, such as baiting and feeding, has worsened the spread.
4. Recent Scientific Advancements (The Path Forward) Ultrasensitive Detection (RT-QuIC): The development of Real-Time Quaking-Induced Conversion (RT-QuIC) and Serial Protein Misfolding Amplification (PMCA) has allowed scientists to detect very low levels of prions, improving the ability to identify prions in soil, water, and even in live animals (e.g., in feces or saliva).
Better Environmental Sampling: Techniques to sample contaminated, environmentally relevant surfaces like metal fences or water sources are now being developed.
The science of CWD has moved from a lack of understanding to a state of high alarm, driven by the realization that this disease is not just a localized issue but a persistent, growing threat to wildlife, ecosystems, and potentially human health. end…TSS
not bad.
please let me elaborate on the dire consequences we face with CWD TSE PrP, if we fail to act swiftly, with sound science, and NOT let the industry continue to legislate junk science, and regulations there from, by trucking CWD to hell and back, no better example is Texas…
USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review
https://www.researchgate.net/publication/400772766_USAHA_129th_Annual_Meeting_CWD_TSE_PrP_2025_and_a_Decade_Review
SATURDAY, APRIL 11, 2026
Chronic Wasting Disease CWD TSE PrP, Cervid, Genetic Manipulation, Unforeseen Circumstances
https://chronic-wasting-disease.blogspot.com/2026/04/chronic-wasting-disease-cwd-tse-prp.html
*** Grant Agreement number: 222887 ***
*** Project acronym: PRIORITY ***
*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***
Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014
Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es
Project website¡ Error! Marcador no definido. address: www.prionpriority.eu
PRIORITY, PROJECT FINAL REPORT
*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***
snip...
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
Block D: Prion epidemiology
Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.
In parallel, studies in sheep have concluded that:
*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes
*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.
*** The combination of data from all our studies leads us to conclude that:
*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).
*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.
snip...
We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:
a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:
• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;
• the uncertainties related to prion epidemiology in animal populations;
• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents; • the intrinsic capacity of prions to cross interspecies transmission barriers; • the lack of sensitive methodology for identifying cross contamination in food.
• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.
The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.
b. Atypical prion agents and surveillance
Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.
*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.
*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.
*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.
*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable
• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)
• to improve the sensitivity of detection assays that are applied in the field towards this type of agent
• to maintain a robust surveillance of both animal and human populations
c. The need for extended research on prions
Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.
Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.
Position of the Priority consortium
Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk
Origins of prion epidemies
Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.
In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.
The human prion problem
The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.
Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.
The animal prion problem
An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.
We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.
The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
SUNDAY, MARCH 8, 2026
Texas Creutzfeldt-Jakob Disease Deaths and Death Rates per Year (2013-2022) More Than Tripled, and case reporting has ceased since then
https://cjdtexas.blogspot.com/2026/03/texas-creutzfeldt-jakob-disease-deaths.html
https://prpsc.proboards.com/thread/209/texas-cases-more-triples-2013
WEDNESDAY, OCTOBER 15, 2025
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025
https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
https://chronic-wasting-disease.blogspot.com/2025/11/while-no-one-was-watching-tenuous.html
SATURDAY, JANUARY 10, 2026
Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease, a review
https://creutzfeldt-jakob-disease.blogspot.com/2026/01/neuropsychiatric-symptoms-in-sporadic.html
https://prpsc.proboards.com/thread/191/neuropsychiatric-symptoms-sporadic-cjd-review
SUNDAY, MARCH 23, 2025
Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update
https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html
FRIDAY, DECEMBER 13, 2024
Creutzfeldt Jacob Disease CJD, BSE, CWD, TSE Prion, December 14, 2024 Annual Update
https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html
iatrogenic Transmissible Spongiform Encephalopathy (friendly fire)
Terry S. Singeltary Sr.
posted by Terry S. Singeltary Sr. at
11:38 AM
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