Canada Ottawa to halt funding for tests to detect deaths from Creutzfeldt-Jakob disease
Canada Ottawa to halt funding for tests to detect deaths from Creutzfeldt-Jakob disease
Ottawa to halt funding for tests to detect deaths from Creutzfeldt-Jakob disease
KELLY GRANT-HEALTH REPORTER PUBLISHED 9 HOURS AGO
The federal government is scrapping a program that performed autopsies on the brains of people suspected of having Creutzfeldt-Jakob disease, the fatal neurodegenerative disorder that came to public prominence during the mad cow crisis of the late 1990s and early 2000s.
The Public Health Agency of Canada (PHAC) will stop funding the brain autopsies at the end of next month to save money, according to a copy of an e-mail sent to pathology labs across the country on Feb. 13 and obtained by The Globe and Mail.
“As you may be aware, increased fiscal restraint is being experienced across the Government of Canada,” said the message, signed by two senior officials with the country’s Creutzfeldt-Jakob Disease Surveillance System. “After careful consideration, we will be discontinuing financial support related to autopsy services for patients suspected of CJD.”
The budget for the autopsy program fluctuated, depending on the number of brains examined, and ran as high as $1.3-million a year. Fifty autopsies were completed in 2023-24 and 44 in 2024-25, according to Anna Maddison, a spokeswoman for PHAC.
Some doctors and scientists who study prion diseases, including CJD, slammed the decision because they fear it could leave Canada in the dark about possible human cases of chronic wasting disease. CWD is a different prion disease that is spreading among cervids such as deer, elk and moose mainly in Alberta and Saskatchewan.
Canada makes cut to pledge for Global Fund to fight infectious diseases
Chronic wasting disease (CWD) has not been found in humans. But if it were to make the jump from cervids to people, its symptoms could look like the loss of co-ordination and memory typical of CJD.
The best way to know the difference would be to conduct the type of brain autopsy that the federal government just stopped funding, said Joel Watts, a professor of biochemistry at the University of Toronto and the Canada Research Chair in Protein Misfolding Disorders.
“This is something that any scientist would say is a terrible idea,” Dr. Watts said. “It’s one of these cases where I don’t think the government sees the big picture.”
Ms. Maddison of PHAC said there are now reliable lab tests that can be performed on patients suspected of having CJD while they’re still alive. Those advances have lessened the need for confirmatory brain autopsies, she said by e-mail.
However, the experts The Globe spoke to said they were concerned that live tests may not be able to detect a CWD case since one has never been found in humans.
U.S. research funding cuts change landscape for Canadian universities, researchers
As for chronic wasting disease, Ms. Maddison said the expert consensus is that it is “very unlikely” to be transmitted to humans. She added that testing performed at the National Microbiology Laboratory in Winnipeg, “is sensitive for detection of almost all subtypes of CJD, and surveillance for human prion diseases in Canada (including potential novel forms, like CWD) will continue.”
CWD and CJD are both incurable afflictions caused by transmissible misfolded proteins called prions. People who develop CJD can sometimes be mistaken for dementia or Parkinson’s patients as their memories fade and their movements become jerky. The disease is progressive and usually kills in less than a year. There is no treatment.
CJD is rare in Canada. Only 445 cases have been reported since 2020. The vast majority are a type known as sporadic CJD, where normal proteins in the brain misfold into infectious abnormal proteins for no discernable 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 kind caused by eating beef from cows infected with the animal prion disease Bovine Spongiform Encephalitis – better known as mad cow disease.
Variant CJD ceased to be much of an issue after the mad cow crisis, which hit hardest in Britain, was brought under control by rules barring the feeding of meat-and-bone meal to cows.
Gerard Jansen, a neuropathologist at the University of Ottawa, runs the laboratory where the federally funded autopsies were performed.
He learned about the funding cuts on Feb. 13, the same day the PHAC message went out. At the same time, he heard his lab would have to stop accepting brains or brain biopsy samples for the CJD program immediately.
“That basically means that every patient after last Friday is out in the cold, and that includes biopsies,” Dr. Jansen said. His lab is sometimes asked to examine tissue from a brain biopsy to confirm, rule out or subtype a CJD case, he explained.
Dr. Jansen is also worried about the risk, however remote, of missing an early case of CWD in humans. “I think that would be disastrous, frankly.”
Valerie Sim, a neurologist at the University of Alberta who sees patients with prion diseases, echoed those concerns.
https://www.theglobeandmail.com/canada/article-ottawa-halt-funding-tests-detect-deaths-creutzfeldt-jakob-disease/
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…
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
“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
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
“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
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
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 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
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
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
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…
Terry S. Singeltary Sr.USAHA 129th Annual Meeting CWD TSE PrP 2025 and a Decade Review
posted by Terry S. Singeltary Sr. at
4:58 PM
