Saturday, January 28, 2023

Application of real‑time quaking‑induced conversion in Creutzfeldt–Jakob disease surveillance

 Journal of Neurology



ORIGINAL COMMUNICATION

Application of real‑time quaking‑induced conversion in Creutzfeldt–Jakob disease surveillance

Peter Hermann1 · Matthias Schmitz1,2 · Maria Cramm1 · Stefan Goebel1 · Timothy Bunck1 · Julia Schütte‑Schmidt1 · Walter Schulz‑Schaefer3 · Christine Stadelmann4 · Jakob Matschke5 · Markus Glatzel5 · Inga Zerr1,2

Received: 25 November 2022 / Revised: 22 December 2022 / Accepted: 23 December 2022

© The Author(s) 2023

Abstract

Background Evaluation of the application of CSF real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance to investigate test accuracy, influencing factors, and associations with disease incidence.

Methods In a prospective surveillance study, CSF real-time quaking-induced conversion was performed in patients with clinical suspicion of prion disease (2014–2022). Clinically or histochemically characterized patients with sporadic Creutzfeldt– Jakob disease (n=888) and patients with fnal diagnosis of non-prion disease (n=371) were included for accuracy and association studies.

Results The overall test sensitivity for sporadic Creutzfeldt–Jakob disease was 90% and the specificity 99%. Lower sensitivity was associated with early disease stage (p=0.029) and longer survival (p<0.001). The frequency of false positives was significantly higher in patients with inflammatory CNS diseases (3.7%) than in other diagnoses (0.4%, p=0.027). The incidence increased from 1.7 per million person-years (2006–2017) to 2.0 after the test was added to diagnostic the criteria (2018–2021).

Conclusion We validated high diagnostic accuracy of CSF real-time quaking-induced conversion but identified inflammatory brain disease as a potential source of (rare) false-positive results, indicating thorough consideration of this condition in the differential diagnosis of Creutzfeldt–Jakob disease. The surveillance improved after amendment of the diagnostic criteria, whereas the incidence showed no suggestive alterations during the COVID-19 pandemic.

snip...

Development of CJD incidence in Germany (2006– 2021)

The cumulative incidence of sCJD has increased from 1.7 per million person-years 2006–2017 to 2.0 per million person-years in 2018–2021, when diagnostic criteria including RT-QuIC were applied prospectively (Fig. 3). These data also include sCJD cases that were classified without RTQuIC analyses (based on 14-3-3, MRI, and EEG only) as well as autopsy results from cases without available clinical data. In some RT-QuIC-positive cases, no further or no sufficient clinical information was available to the CJD Surveillance group. These cases were indicated as “unclarified” throughout this manuscript. Including these patients resulted in a cumulative incidence of 2.1 per million person-years (2018–2021). We could not observe any suggestive alteration of sCJD incidence in the years of the COVID-19 pandemic 2020 (2.10) and 2021 (2.01) compared to the preceding year 2019 (2.08), the first year in which all cases were systematically classified according to the amended criteria.

snip...

Discussion

CSF RT-QuIC has become the gold standard in the laboratory-based diagnosis of sCJD [29]. It is currently applied as a solitary criterion within the biomarker-set of diagnostic protocols [12] and as a “confirmatory” test after CJD typical results from other biomarker analyses [30]. Here, we present comprehensive data from a well-established surveillance system including all patients with diagnosis of sCJD (n=888) and a highly specific cohort of CJD mimics (n=371).

We observed a sensitivity of 90% for sporadic sCJD, which is comparable to some reports from surveillance centers using 1st generation RT-QuIC [31, 32] and slightly below the sensitivity of a modified protocol called 2nd generation RT-QuIC (IQ), which was introduced in 2015 [33] and showed a sensitivity ranging from 92 to 96% [33–35]. Several factors that may influence the test sensitivity have been proposed in the past. We observed no significant differences between true and false-positive sCJD patients regarding age and sex, in contrast to a previous study that identified false-negative results to be associated with lower age and female sex [19]. We can only speculate on the reasons for this discrepancy, but it could possibly be associated with different test protocols or with the investigated sCJD cohorts. In that study, only autopsy confirmed cases were evaluated and we observed differences of the age and the sex distribution between probable and definite cases in our cohort (Table 2). We observed no difference of the overall test sensitivity between probable and definite patients in our cohort, though. However, we could replicate an association between longer disease duration and test negativity, and showed that false negative RT-QuIC is associated with early disease stage.

We also validated high sensitivity for the most frequent MM/MV1, VV2, and MV2 sCJD subtypes [19, 34, 35], whereas sensitivity seems to be lower in the rare MM2 and VV1 subtypes [19, 34]. In some studies [34], sensitivity was slightly higher than in ours, possibly due to different substrates or test protocols. On the other hand, investigated case numbers of these subtypes were rather low in all studies (around 10 or less in each), which may not allow to draw final conclusions. An explanation for the low sensitivity may be that MM2C and VV1 subtypes show predominant cortical PrPSc pathology in early disease stages [6] and slower disease progression than most other sCJD subtypes, possibly resulting in less amount of PrPSc in the CSF. This would be in line with evaluations in genetic prion diseases that reported low sensitivity in entities with slow disease progression (GSS) or pathology restricted to defined structures (brainstem and thalamus in FFI, cerebellum in GSS) in early disease stages [36].

Regarding the specificity of RT-QuIC, previous analyses showed an excellent accuracy of CSF RT-QuIC of about 99% or higher [12]. Our data from eight years of clinical application indicated a specificity of 99% but for the first time, we evaluated test-related and clinical data in a series of five false-positive cases. Four of them were diagnosed with immune-mediated encephalitis and the rate of false positives was significantly higher than in other diagnostic groups (p=0.027). In addition, single (one of the three) false-positive signal increases were also significantly more frequent among differential diagnoses with inflammatory pathophysiologic background (p=0.001), suggesting a potential causal relationship between encephalitis and false-positive RT-QuIC results. Of course, analytical and pre-analytical factors cannot be excluded. In the literature, only 10 cases of false-positive RT-QuIC or Endpoint-QuIC with clear diagnosis have been reported (see Table 5). They were diagnosed with vascular dementia [32], Alzheimer’s disease [25], mixed dementia [19], and tauopathies [35, 37], immune-mediated encephalitis [20, 35, 38], and amyloid associated vasculitis [39].

The possibility of a higher likelihood of false-positive RT-QuIC among encephalitis patients is an important issue because many inflammatory encephalopathies are highly treatable and may represent the most important clinical mimics of CJD and causes of rapidly progressive dementia [40]. On the other hand, all false-positive patients showed either clinical or CSF characteristics that pointed to other diagnoses than CJD, indicating that consideration of factors such as inflammatory signs in the CSF may improve the specificity of an RT-QuIC-based clinical diagnosis. Total CSF Tau protein may also give additional clues because of better specificity in the discrimination of CJD and acute encephalopathies than 14-3-3, but Tau was not available for the false-positive patients. On the other hand, total-tau may also be extremely elevated in encephalitis [41] due to ongoing severe neuronal damage. More important, none of the patients showed CJD-typical MRI. In our autopsy series, no patient received incorrect ante-mortem diagnosis of CJD based on RT-QuIC positivity. We identified only five false-positive results in eight years of RT-QuIC application in a sum of 4599 patients. However, clinical information was only available for 371 control patients, leading to the reported specificity of 99%.

Further investigations have to validate our findings about false-positive RT-QuIC and investigate potential mechanisms. So far, previous studies have not found association of RT-QuIC efficiency and neuronal damage markers such as total-tau and proteins 14-3-3 in sCJD patients [42]. Total PrP was also not associated with seeding efficiency in sCJD [42] but has not been investigated as a factor for false-positive RT-QuIC, yet. On the other hand, total PrP was not shown to be significantly altered in encephalitis compared to cerebral ischemia or control patients [43]. Another potential reason may be the influence of factors in the CSF that are directly linked to neuro-inflammation. Epileptic activity in encephalitis patients was also discussed as a cause for false-positive results [21]. Our data did not allow the evaluation of the presence of seizures in relation to lumbar puncture in control patients, but patients with primary diagnosis of seizures or status epilepticus caused by idiopathic epilepsy syndromes, or reversible conditions such as alcohol withdrawal showed a low frequency of positive test replicates (one in 30 patients). However, the mechanisms for false-positive results may be related to the CSF. RT-QuIC from other body tissues such as olfactory mucosa [44, 45] are an alternative clinical test and should be investigated in future studies on the specificity of RT-QuIC in neuro-inflammatory diseases. Clarification of the reasons for false-positive PrPSc RT-QuIC reactions may also be highly relevant for the application of other protein amplification assays such as α-Synuclein RT-QuIC. So far, only very few false-positive results have been reported and were associated with Wernicke’s encephalopathy, Alzheimer’s disease, and encephalitis [46, 47].

Although our study provides comprehensive data on clinical experience with RT-QuIC, the study has naturally some limitations. First, our test protocol [24] uses chimeric hamster-sheep recombinant PrP as substrate, whereas most other centers are using hamster recombinant PrP [32, 33]. Some centers showed that IQ-CSF RT-QuIC may have a superior sensitivity for sCJD compared to previous protocols [30, 45, 48]. It remains unclear, whether IQ RT-QuIC underlays the same or similar confounders for PrP seeding as our protocol but international ring trials have shown that RT-QuIC results are highly concordant among different test centers and test protocols [25, 49]. Our protocol has been well established over years and we have achieved a high level of experience to perform the test in a reliable and reproducible way.

Regarding the sensitivity, further histochemical characterization of sCJD was only available in a limited number of cases (n=161), discouraging reliable conclusions on the sensitivity of rare (MM2, VV1) sCJD subtypes. Similarly, Codon 129 was analyzed in a rather small subset of patients (n=114). Lastly, our surveillance data includes a number of uncharacterized cases with suspected prion disease or positive CSF RT-QuIC. In these cases, further clinical data were not available and thus, we cannot exclude additional false-positive or false-negative test results in this group. Our cohort may be prone to an according selection bias. On the other hand, we assume that this bias might rather be less relevant than in retrospective case–control studies or in studies including only autopsy-confirmed cases.

As a secondary outcome, we observed an increased overall sCJD incidence in Germany after inclusion of the test in the clinical diagnostic protocol. In this context, we assume that the increase from 1.7 (2009–2017) to 2.0 (2018–2021) person-years is a result of the improved clinical detection of early sCJD cases (based on positive RT-QuIC) as previously suggested or observed by our group [18] and others [13, 17, 19]. This effect was also described in the context of previous criteria modifications [50]. Another interesting observation was the apparent lack of an alteration of the overall sCJD incidence during the Covid-19 pandemic. Annual numbers of positive RT-QuIC results remained stable between 2019 and 2021 (Fig. 3). Although we cannot exclude influence of viral infections on genesis or course of prion diseases, our surveillance data do not suggest an immediate causal relationship between COVID-19-related factors and CJD on the population level.

Conclusion

Chimeric PrP CSF RT-QuIC is an accurate diagnostic tool for the differential diagnosis of sCJD. If the test is interpreted in the context of a complete diagnostic work-up, it may provide an extremely high level of ante-mortem diagnostic certainty. RT-QuIC negativity combined with absence of CJD-typical results in 14-3-3 analysis and MRI indicates extremely low likelihood of sCJD. The routine application of RT-QuIC improves CJD surveillance and leads to a formal increase of the disease incidence. However, the sensitivity is influenced by disease stage and disease subtype. False positive results may occur and clinicians have to be aware of this possibility. In cases with ambiguous clinical presentation, we recommend consideration of other diagnostics, in particular MRI, and repetitive RT-QuIC analyses from the same sample to exclude the influence of analytical factors. Nonetheless, a consecutive lumbar puncture, at best after therapeutic intervention, may be necessary to detect false positivity based on pre-analytical or disease-related factors. In this context, RT-QuIC from other body tissues such as olfactory mucosa may be an alternative, if available. Inflammatory CNS disease, especially immune-mediated encephalitides, should always be considered as potential clinical and laboratory mimics of CJD. Although general comparability of different RT-QuIC protocols and substrates have been shown, our pilot findings need to be verified through studies using other body tissues and test protocols such as IQ.

Supplementary Information The online version contains supplementary material available at doi.org/10.1007/s00415-022-11549-2 .

Acknowledgements We would like to thank all collaborating health care institutions, hospitals, physicians, patients, and families that provided information for diagnostic classifications. In addition, we acknowledge the Center for Neuropathology and Prion Research at the Ludwig-Maximilian-University in Munich, Germany for performing neuropathological investigations in several cases of suspected prion disease.

Funding Open Access funding enabled and organized by Projekt DEAL. This study was funded by the Robert-Koch-Institute through funds from the Federal Ministry of Health; grant number 1369-341. Data availability All manuscript-related data is available and will be provided upon reasonable request.

Declarations

Conflicts of interest The authors declare that they have no relevant financial or non-financial interests to disclose.


Keywords Creutzfeldt–Jakob disease · Prion · Diagnosis · RT-QuIC · Epidemiology

posted by Terry S. Singeltary Sr. at 10:58 AM

Wednesday, January 25, 2023

Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD

Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD

wow, just wow, i don't know where to start with Canada and CJD and strange neurological conditions mounting. 

seems the New Brunswick strange neurological cases of unknown origin just still will not go away, and CJD cases in Canada keep rising.

Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report Update January 2023

(PLEASE NOTE, ''The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians.'' HAS BEEN USED 'ad nauseam' YEAR, AFTER YEAR, AFTER YEAR, OF RISKING CJD CASES.)

(PLEASE NOTE, OCTOBER OF 2022 THERE WERE 65 CJD CASES REPORTED, TWO MONTHS LATER, DECEMBER 2022, CJD CASES JUMPED TO 154 CASES REPORTED...tss)

(PLEASE NOTE, STILL, CANADA DOES NOT REPORT VPSPr CJD TSE Prion Cases to the public, see links below for this explanation and discussion. tss)

Referrals of suspected CJD reported by CJDSS, 1998-2022 As of December 31, 2022

Year of reporting Number of referrals

1998 43

1999 63

2000 82

2001 101

2002 103

2003 75

2004 90

2005 97

2006 80

2007 101

2008 100

2009 104

2010 76

2011 102

2012 103

2013 99

2014 99

2015 98

2016 117

2017 116

2018 125

2019 142

2020 123

2021 140

2022 154

Total 2533



Canada Definite and probable CJD, 1998-2022 As of October 31, 2022

Year Sporadic Iatrogenic CJD 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 30 0 5 1 36

2003 27 1 1 0 29

2004 42 0 4 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 62 0 1 0 63

2013 50 0 1 0 51

2014 51 0 5 0 56

2015 44 0 8 0 52

2016 57 1 6 0 64

2017 82 0 5 0 87

2018 75 1 5 0 81

2019 76 0 2 0 78

2020 65 0 4 0 69

2021 56 0 3 0 59

2022 64 0 1 0 65

Total 1183 6 83 2 1274

Cases with definite and probable diagnosis to date


PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;

WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?

THIS problem must be addressed immediately imo.

WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;

QUOTE;

''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''

''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''

end



Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry


CANADA CJD 2020

***> CANADA, I find it very odd that Canada has NO recorded or documented cases of Variably Protease-Sensitive Prionopathy (VPSPr)?

CANADA Creutzfeldt-Jakob disease surveillance system (CJDSS) report

Definite and probable CJD, 1998-2020

As of 31 October, 2020

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

1998 22 1 0 1 0 0 24

1999 27 2 2 1 0 0 32

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 1 0 0 44

2005 42 0 1 1 0 0 44

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 35 0 3 0 0 0 38

2011 46 0 3 1 0 1 51

2012 62 0 1 0 0 0 63

2013 50 0 0 0 1 0 51

2014 51 0 4 0 1 0 56

2015 44 0 5 1 2 0 52

2016 57 1 5 1 0 0 64

2017 82 0 2 1 1 0 86

2018 74 1 4 0 1 0 80

2019 76 0 2 0 0 0 78

2020 30 0 2 0 0 0 32

Total 1027 6 45 23 7 2 1110

Cases with definite and probable diagnosis to date.

Gerstmann-Sträussler-Scheinker disease (GSS)

Fatal familial insomnia (FFI)

Variant CJD (vCJD)


creutzfeldt-jakob-disease.blogspot.com/2020/12/sporadic-creutzfeldt-jakob-disease-scjd.html

CANADA

Creutzfeldt-Jakob Disease Surveillance System Report

Definite and probable CJD, 1998-2019

As of 31 July, 2019

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

1998 22 1 0 1 0 0 24

1999 27 2 2 1 0 0 32

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 1 0 0 44

2005 42 0 1 1 0 0 44

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 35 0 3 0 0 0 38

2011 46 0 3 1 0 1 51

2012 62 0 1 0 0 0 63

2013 50 0 0 0 1 0 51

2014 51 0 4 0 1 0 56

2015 44 0 5 1 2 0 52

2016 55 1 5 1 - - 62

2017 78 - 1 1 1 - 81

2018 65 1 3 - 1 - 70

2019 20 0 - - - - 20

Total 926 6 39 23 7 2 1003

Cases with definite & probable diagnosis to date.



***> CANADA CJD 2018

see substantial increase in sporadic cjd in Canada 2017...terry

Definite and probable CJD, 1998-2018

As of 31 October, 2018

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

1998 22 1 0 1 0 0 24

1999 27 2 2 1 0 0 32

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 1 0 0 44

2005 42 0 1 1 0 0 44

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 35 0 3 0 0 0 38

2011 46 0 3 1 0 1 51

2012 62 0 1 0 0 0 63

2013 50 0 0 0 1 0 51

2014 51 0 4 0 1 0 56

2015 44 0 5 1 2 0 52

2016 55 1 5 1 62

2017 77 1 1 1 80

2018 35 1 36

Total 875 5 36 23 7 2 948 





CANADA CJD As of October 31, 2015

Referrals of Suspected CJD Reported by CJDSS, 1997-2015 As of October 31, 2015

Year of Reporting Number of Referrals

1997 4

1998 43

1999 63

2000 82

2001 101

2002 103

2003 75

2004 90

2005 97

2006 80

2007 101

2008 100

2009 104

2010 76

2011 102

2012 103

2013 99

2014 99

2015 80

Total 1602

CJD Deaths Reported by CJDSS, 1994-2015 As of October 31, 2015

Deaths of Definite and Probable CJD

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

Cases with definite & probable diagnosis to date.

1994 2 0 0 1 0 0 3

1995 3 0 0 0 0 0 3

1996 13 0 0 0 0 0 13

1997 16 0 1 1 0 0 18

1998 22 1 0 1 0 0 24

1999 27 2 2 1 0 0 32

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 1 0 0 44

2005 42 0 1 1 0 0 44

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 35 0 3 0 0 0 38

2011 46 0 3 1 0 1 51

2012 62 0 1 0 0 0 63

2013 50 0 0 0 1 0 51

2014 49 0 4 0 1 0 54

2015 23 0 1 0 0 0 24

Total 719 4 27 22 3 2 777

CJD Cases by Province/Territory October 31, 2015

CJD Cases by Province/Territory October 30, 2015

Text Equivalent - CJD Cases by Province/Territory October 31, 2015

Incidence of CJD Deaths Reported by CJDSS in Canada As of October 31, 2015

Year of Death Total CJD Cases Population of Canada Incidence Rate

Cases with definite & probable diagnosis to date.2014 Population Source External Link

1999 32 30,492,106 1.05

2000 35 30,783,969 1.14

2001 30 31,130,030 0.96

2002 36 31,450,443 1.14

2003 29 31,734,851 0.91

2004 44 32,037,434 1.37

2005 44 32,352,233 1.36

2006 44 32,678,986 1.35

2007 39 33,001,076 1.18

2008 49 33,371,810 1.47

2009 53 33,756,714 1.57

2010 38 34,131,451 1.11

2011 51 34,472,304 1.48

2012 63 34,880,248 1.81

2013 51 35,289,003 1.45

2014 54 35,675,834 1.51

2015 24 35,702,707 0.81

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats-eng.php 


P.179: Sporadic Creutzfeldt-Jakob disease in Canada

Zheng Wang,1 Gerard Jansen,1,2 Stacy Sabourin,1 Rolande D’Amour,1 Tim Connolly,1 Jennifer Kruse,1 David J Knox,3 Neil R Cashman,4 and Michael B Coulthart1 1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public Health Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa Hospital; Ottawa, ON Canada; 3National Microbiology Laboratory; Public Health Agency of Canada; Winnipeg, MB Canada; 4Brain Research Centre; University of British Columbia; Vancouver, BC Canada

Background. Sporadic Creutzfeldt-Jakob Disease (sCJD) is a fatal, transmissible neurodegenerative disease. Systematic surveillance has repeatedly shown annual mortality in the range 1 to 2 per million population, has elucidated key characteristics of sCJD, and led to recognition of a new form of CJD, variant CJD (vCJD), which is associated with BSE. In 1998, Canada launched comprehensive national CJD surveillance to assess the characteristics of CJD in Canada, identify any acquired cases of CJD (such as vCJD, of which 2 imported cases have been identified in Canada to date), and mitigate public health risks. This study describes the epidemiology of sCJD in Canada from 1998 to 2012.

Methods. Case ascertainment was based on internationally accepted criteria. Demographic and medical information were collected by standardized questionnaire and medical chart review. Poisson regression and descriptive analysis were employed.

Results. A total of 563 sCJD deaths (definite: 462, probable: 101) in Canadian residents were registered from 1998 to 2012. Average annual sCJD mortality was 1.2 per million population, increasing gradually from 0.9 in 1999 to 1.7 in 2012 (P = 0.0004). All provinces saw average annual mortalities ranging from 1.0 to 1.6 (P = 0.25), except three territories where population is small (~25,000 to ~45,000) and no cases were identified. sCJD occurred at similar rates in males (1.1) and females (1.2) (P = 0.21). sCJD was rare under 50 years of age with only 11 cases identified (2.7%). Mortality increased after 50 and peaked at 7.4 per million in the 70–74 age group. Median age at death was 69 and median duration of illness was 4 months. Genotype at codon 129 (N = 358) revealed that the MM subgroup accounted for 223 (62%, median age at death: 69, duration: 4), the MV subgroup was 82 (23%, median age at death: 68, duration: 9), and the VV subgroup was 53 (23%, median age at death: 66, duration: 5). Results of molecular typing (Parchi Scheme) for 256 cases are; MM1: 140, MM2: 11, MV1: 28, MV2: 18, VV1: 5, VV2: 25, Mixture: 29.

Conclusion. Characteristics of sCJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be partly attributed to increased awareness of CJD among Canadian clinicians. These findings support the conclusion that Canadian CJD surveillance system is sufficiently sensitive to accurately characterize the epidemiology of sCJD in Canada, and to detect potential additional cases of acquired CJD such as vCJD or human chronic wasting disease.

Conclusion. Characteristics of sCJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be partly attributed to increased awareness of CJD among Canadian clinicians. These findings support the conclusion that Canadian CJD surveillance system is sufficiently sensitive to accurately characterize the epidemiology of sCJD in Canada, and to detect potential additional cases of acquired CJD such as vCJD or human chronic wasting disease.

HD.18: Creutzfeldt-Jakob disease reporting in Canada

Zheng Wang,1 Gerard H. Jansen,1, 2 Elina Olsen,1 Rolande D’Amour,1 Stacy Sabourin,1 Tim Connolly,1 Jennifer Kruse,1 Neil Cashman3 and Michael Coulthart1 1Public Health Agency of Canada; Ottawa, ON CA; 2Department of Pathology; Ottawa Hospital; Ottawa, ON CA; 3Brain Research Centre; University of British Columbia; Vancouver, BC CA

Background. To deal with risks of infectious transmission of Creutzfeldt-Jakob disease (CJD), in 1998 the Government of Canada launched a prospective national CJD surveillance system (CJDSS). In 2000, CJD became nationally notifiable in Canada, and since then all Canadian Provinces and Territories (P/Ts) have made CJD reportable. It has been recognized that the CJDSS registers more cases of CJD than are reported to P/T Ministries of Health (PTMH). Because the CJDSS may not legally share personal information with PTMH, in 2008 the CJDSS began to systematically discuss the issue of CJD reporting with referring health care professionals (HCP). The present study was undertaken to estimate any changes in P/T CJD reporting from 2008, and to identify possible areas for further improvement.

Materials and Methods. P/T CJD data were retrieved from the Public Health Agency of Canada’s National Notifiable Disease System (NNDS) database, and compared with CJDSS data. CJDSS intake sheets were examined, to determine if the case had been reported to the PTMH at the time of notification.

Results. From 2005 to 2010, NNDS received complete data on CJD from 5 P/Ts. During the same period, 134 cases of CJD (probable or neuropathologically confirmed) were reported by the 5 P/Ts while 210 CJD deaths (probable or definite) were recorded in the CJDSS from the same 5 P/Ts. Between 2008 and 2010 there was an increase of ~48% in P/T CJD reports compared with the period 2005–2007. In contrast, the CJDSS registered only ~12% more CJD deaths between 2008 and 2010 compared with 2005–2007, supporting an interpretation of improved P/T reporting. Examination of intake sheets from 172 notifications that were made to the CJDSS from the same 5 P/Ts between 2008 and 2010 revealed that 30 were known to have been reported to PTMH at the time of referring (24 were CJD, 5 were non-CJD, and 1 was unclassifiable). 142 were not reported or had unknown reporting status. Reasons cited by HCPs for not reporting included (1) uncertainty of the CJD diagnosis; (2) uncertainty regarding responsibility for reporting; (3) lack of awareness that CJD is reportable; and (4) uncertainty regarding when or how to report.

Conclusion. The considerable increase of CJD reports in P/Ts since 2008 occurred concurrently with efforts of the CJDSS to engage HCPs on the issue of CJD reporting requirements. P/T CJD reports may include non-CJD cases. Inter-jurisdiction collaboration is underway to further improve CJD reporting.



Canada CJD 2015



2012

CANADA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss

PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD) in Canada is also on a steady increase.

please see ;

> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.

CJD Deaths Reported by CJDSS1, 1994-20122

As of May 31, 2012

Deaths of Definite and Probable CJD

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

1994 2 0 0 1 0 0 3

1995 3 0 0 0 0 0 3

1996 13 0 0 0 0 0 13

1997 16 0 1 1 0 0 18

1998 22 1 0 1 0 0 24

1999 26 2 2 1 0 0 31

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 0 0 0 43

2005 42 0 0 2 0 0 44

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 34 0 3 0 0 0 37

2011 37 0 2 1 0 1 41

2012 1 0 0 0 0 0 1

Total 525 4 19 22 1 2 573

1. CJDSS began in 1998

2. Data before 1998 are retrospective and partial, data from 1998 to 2008 are complete, and data for 2009 - 2012 are provisional

3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.

CJD Deaths Reported by CJDSS1, 1994-20122

As of May 31, 2012



SEE DECEMBER 2012 CANADA


UNITED STATES

USA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss

National Prion Disease Pathology Surveillance Center

Cases Examined1

(May 18, 2012)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 50 32 28 4 0 0

1997 114 68 59 9 0 0

1998 88 52 44 7 1 0

1999 123 74 65 8 1 0

2000 145 103 89 14 0 0

2001 210 120 110 10 0 0

2002 248 149 125 22 2 0

2003 266 168 137 31 0 0

2004 326 187 164 22 0 13

2005 344 194 157 36 1 0

2006 382 196 166 28 0 24

2007 377 213 185 28 0 0

2008 396 232 206 26 0 0

2009 423 256 212 43 1 0

2010 413 257 216 41 0 0

2011 410 257 213 43 0 0

2012 153 82 51 15 0 0

TOTAL 44685 26406 2227 387 6 3

1 Listed based on the year of death or, if not available, on year of referral;

2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;

3 Disease acquired in the United Kingdom;

4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 14 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 17 (16 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded. The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).

Rev 5/18/2012


> 6 Includes

> 17 (16 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded.

> The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).

WELL, it seems the USA mad cow strains in humans classified as type determination pending tdpCJD, VPSPr, sFFI, and sCJD) have steadily increased over the years, and the same old song and dance continues with sporadic CJD cases $$$



VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $

OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles

Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA

Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.

Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.

Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.

In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.

Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.

The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.



Wednesday, March 28, 2012

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $


SEE DECEMBER 2012 CANADA
 
CANADA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss

PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD) in Canada is also on a steady increase.

 please see ;

> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.

CJD Deaths Reported by CJDSS1, 1994-20122

As of Oct 31, 2012

Deaths of Definite and Probable CJD

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

1994 2 0 0 1 0 0 3

1995 3 0 0 0 0 0 3

1996 13 0 0 0 0 0 13

1997 16 0 1 1 0 0 18

1998 22 1 0 1 0 0 24

1999 27 2 2 1 0 0 32

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 1 0 0 44

2005 41 0 1 1 0 0 43

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 35 0 3 0 0 0 38

2011 41 0 2 1 0 1 45

2012 20 0 0 0 0 0 20

Total 549 4 20 22 1 2 598

1. CJDSS began in 1998

2. Data before 1998 are retrospective and partial, data from 1998 to 2008 are complete, and data for 2009 - 2012 are provisional

3. Final classification of 48 cases from 2009, 2010, 2011 and 2012 is pending.


snip...see full text ;


CJD Deaths Reported by CJDSS1, 1994-20122

As of May 31, 2012

Deaths of Definite and Probable CJD

Year Sporadic Iatrogenic Familial GSS FFI vCJD Total

1994 2 0 0 1 0 0 3

1995 3 0 0 0 0 0 3

1996 13 0 0 0 0 0 13

1997 16 0 1 1 0 0 18

1998 22 1 0 1 0 0 24

1999 26 2 2 1 0 0 31

2000 32 0 0 3 0 0 35

2001 27 0 2 1 0 0 30

2002 31 0 2 2 0 1 36

2003 27 1 1 0 0 0 29

2004 42 0 1 0 0 0 43

2005 42 0 0 2 0 0 44

2006 39 0 1 3 1 0 44

2007 35 0 0 4 0 0 39

2008 48 0 1 0 0 0 49

2009 48 0 3 2 0 0 53

2010 34 0 3 0 0 0 37

2011 37 0 2 1 0 1 41

2012 1 0 0 0 0 0 1

Total 525 4 19 22 1 2 573

1. CJDSS began in 1998

2. Data before 1998 are retrospective and partial, data from 1998 to 2008 are complete, and data for 2009 - 2012 are provisional

3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.

CJD Deaths Reported by CJDSS1, 1994-20122

As of May 31, 2012




USA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss

CANADA CJD 2011

Risk.49: Creutzfeldt-Jakob Disease in Canada, 1998–2009

Zheng Wang,1,† Gerard Jansen,1, 2 Elina Olsen,1 Stacy Sabourin,1 Rolande D’Amour,1 Tim Connolly,1 Jennifer Kruse,1 Neil Cashman3 and Michael Coulthart1

1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public Health Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa Hospital; Ottawa, ON Canada; 3Brain Research Centre; University of British Columbia; Vancouver, BC Canada†Presenting author; Email: zheng.wang@phac-aspc.gc.ca

Background. Creutzfeldt-Jakob Disease (CJD) is a fatal, transmissible neurodegenerative disease with sporadic, genetic and acquired forms. In 1998, Canada launched comprehensive national CJD surveillance to assess the characteristics of CJD in Canada and its risks to the health of Canadians. This study describes the broad characteristics of CJD in Canada from 1998–2009.

Methods. Case ascertainment was based on internationally accepted criteria. Demographic information and risk-factor data were collected by standardized questionnaire and medical chart review. Poisson regression, descriptive analysis, and case investigation were employed.

Results. A total of 453 CJD deaths in Canadian residents were registered from 1998–2009. Four hundred and fifteen (92%) were sporadic (sCJD), 33 (7%) were genetic and five (1%) were acquired. Average annual sCJD mortality was 1.1 per million population, increasing gradually from 0.9 in 1999 to 1.4 in 2009 (P = 0.27). All provinces saw average annual mortalities ranging from 1.0 to 1.5 (P = 0.85), except three territories where population is small (~25,000 to ~45,000), sCJD occurred equally in both genders at 1.1. sCJD was rare under 50 years of age with only 11 cases identified (2.7%). Mortality increased after 50 and peaked at 8 per million in the 70–74 age group. Median age at death was 69 and median duration of illness was 4 months. Genetic TSE accounted for 33 deaths: 19 were GSS (P102L: 5, D202N: 2, P105T: 2, Q217R:1, A117V: 1, unknown mutation: 8); 13 were familial CJD (E200K: 9, D178N: 2, V203I: 1, V189I:1); one was FFI (D178N). Median age for genetic TSE was 59 and median duration of illness was 27 months. For the five acquired cases of CJD, four were associated with dura mater procedures (3 Lyodura, 1 Tutoplast) and were identified from 1998–2003 in patients aged 14–59. Investigation indicated the infections possibly occurred from 1981–1992 with incubation times from 10–16 years. One biochemically and neuropathologically confirmed variant CJD death occurred in 2002 in a person under 40 years old, likely acquired overseas.

Discussion and Conclusion. Characteristics of CJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians. The finding of four dura matter associated CJD cases and one imported vCJD case in Canada demonstrate risks to Canadians from acquired CJD exist. Continued surveillance for iatrogenic risks and novel forms of CJD is warranted.



CANADA CJD UPDATE 2011

CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011

3. Final classification of 49 cases from 2009, 2010, 2011 is pending.

snip...



Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html

CANADA CJD 2007

Creutzfeldt-Jakob Disease Surveillance System (CJD-SS)

Referrals of Suspected CJD Reported by CJD-SS(1), 1997-2007(2)

Year of Reporting Numbers of Referrals

1997 4 

1998 43 

1999 63 

2000 82 

2001 101 

2002 103 

2003 75 

2004 89 

2005 97 

2006 78 

2007 88

Total 823

1CJD-SS began in April 1998 2Data before April 1998 are retrospective and partial, data from 1999 to 2005 are complete, and data for 2006 and 2007 are provisional As of December 1, 2007




A simple look at sporadic CJD statistics in EU countries with BSE will reveal this;

CANADA 2 IN 94 COMPARED TO 30 IN 2002

FRANCE 35 IN 93 COMPARED TO 102 IN 2003

GERMANY 21 IN 93 COMPARED TO 112 IN 2003

ITALY 27 IN 93 TO COMPARED TO 75 IN 2003

UK 37 IN 93 COMPARED TO 74 IN 2003

USA (UNKNOWN...TSS)




snip...

IF we look at sporadic incidence of CJD in UK from 1993 to 2003, the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to show an increase to me? I do not understand the statement ;

However, in the period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of U.K. sporadic CJD deaths (52).

IF we go further and look at some of the other documented BSE countries, you will the increase of sporadic CJD there as well ;

Canada from 2 to 25

France from 35 to 108

Germany 21+ to 96

Italy 27 to 76





Government officials misled the public about the “mysterious” New Brunswick neurological disease, and we have the documents to prove it Here are the 10 biggest takeaways after pouring over thousands of pages of internal government documents obtained through access to information

JANUARY 23, 2023

ON THE MONDAY SHOW BY SARAH LAWRYNUIK


WEDNESDAY, OCTOBER 27, 2021 

New Brunswick October 27, 2021 - Health minister availability Report on cluster of unknown neurological disorders update 




WEDNESDAY, NOVEMBER 30, 2022 

USDA Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, Testing and Surveillance 2022 A Review of History 


FRIDAY, DECEMBER 02, 2022 

Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update


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

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


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

Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France 

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). 

Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. 

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

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

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

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

We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. 

=============== 

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

=============== 

***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. 

============== 

PRION 2015 CONFERENCE


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

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

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


PRION 2016 TOKYO

Saturday, April 23, 2016

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

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

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

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


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

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

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

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


TUESDAY, DECEMBER 21, 2021 

OIE-WAHIS CANADA atypical BSE type H Bovine spongiform encephalopathy


SATURDAY, DECEMBER 18, 2021 

CFIA Canada Alberta Laboratory detection of atypical bovine spongiform encephalopathy


I remember another famous quote;

Alberta Premier Ralph Klein has taken aim at the owner of the province's infamous mad cow, saying a "self-respecting" rancher would not have taken the animal to slaughter but instead would have simply "shot, shovelled and shut up."


OIE ATYPICAL BSE 2019

the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided. The Group acknowledged the challenges in demonstrating the zoonotic transmission of atypical strains of BSE in natural exposure scenarios. Overall, the Group was of the opinion that, at this stage, it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that may be different between atypical strains.

4. Definitions of meat-and-bone meal (MBM) and greaves

snip...

REFERENCES

SNIP...END SEE FULL TEXT;


As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.


TUESDAY, SEPTEMBER 07, 2021
Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom


BSE in North America

Through August 2018, BSE surveillance has identified 26 cases in North America: 

6 BSE cases in the United States and 20 in Canada. 

Of the 6 cases identified in the United States, one was born in Canada; of the 20 cases identified in Canada, one was imported from the United Kingdom (see graph below).


BSE Cases Identified in Canadian-born Cattle

Update: February 12, 2015 a New Case of BSE Detected in Canada

The Canadian Food Inspection Agency (CFIA) external icon announced the confirmation of another bovine spongiform encephalopathy (BSE) in a beef cow from Alberta born in March 2009. See the CFIAexternal icon notice.

Based on the known or most likely year of birth, an average of 1.4 cases of BSE occurred among the group of animals born each year in Canada from 1991 through 2004. The highest reported number of cases by birth year in a single year, 3 BSE cases, occurred in 2000, 2001 and 2002. The most recently reported case extends the period of BSE transmission in Canada through at least the early half of 2009.



MONDAY, NOVEMBER 30, 2015 

Report on the Investigation of the Nineteenth Case of Bovine Spongiform Encephalopathy (BSE) in Canada November 2015


FRIDAY, FEBRUARY 20, 2015 

A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015)
 

SATURDAY, FEBRUARY 14, 2015 

Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta 

Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta The Canadian Food Inspection Agency (CFIA) has confirmed bovine spongiform encephalopathy (BSE) in a beef cow from Alberta. No part of the animal's carcass entered the human food or animal feed systems.

The Government of Canada is committed to protecting human and animal health and takes the management of BSE very seriously. Immediately upon confirmation of this case, the CFIA launched an investigation and is working closely with provincial and industry partners.

BSE is a progressive, fatal neurological disease in cattle. Canada's last confirmed BSE case was reported in 2011. This latest case was detected through the national BSE surveillance program, which continues to play an important role in Canada's strategy to manage BSE.

As part of the investigation, the CFIA is seeking to confirm the age of the animal, its history and how it became infected. The investigation will focus in on the feed supplied to this animal during the first year of its life. The Agency will also trace out all animals of equivalent risk. Equivalent risk animals will be ordered destroyed and tested for BSE.

Canada remains a "controlled BSE risk" country, as recognized by the World Organisation for Animal Health (OIE). Accordingly, this case should not affect current exports of Canadian cattle or beef.

The case will be reported to the OIE, in line with Canada's international obligations and our commitment to transparency. It will be reported on the CFIA website, as part of the Agency's monthly reportable diseases update.


Transcript - Briefing (February 13, 2015) Date/Date: February 13, 2015 4:00 p.m.

Location/Endroit: Teleconference, Ottawa, Ontario

Principal(s)/Principaux:

Denis Schryburt, Media Relations Officer, Canadian Food Inspection Agency Paul Mayers, Vice-President, Policy and Programs, CFIA Dr. Martine Dubuc, Vice-President, Science, CFIA, and Delegate for Canada for the World Organization for Animal Health Nathalie Durand, Agriculture and Agri-Food Canada Subject/Sujet: The Canadian Food Inspection Agency Holds a Technical Briefing to Provide More Information on a BSE (Bovine Spongiform Encephalopathy) Find in Alberta.

Operator: Good afternoon, ladies and gentlemen. Bonjour, mesdames et messieurs. Welcome to the Canadian Food Inspection Agency's technical briefing on Bovine Spongiform Encephalopathy in Alberta. Bienvenue à la séance d'information technique sur le cas d'Encéphalopathie spongiforme bovine en Alberta. I would like to turn the meeting over to the technical briefing operator, Mr. Denis Schryburt. J'aimerais maintenant céder la parole au modérateur de cette séance, M. Denis Schryburt. À vous la parole, M. Schryburt. Please go ahead, sir.

Denis Schryburt: Thank you very much. Good afternoon and thank you for joining us today. My name is Denis Schryburt, Media Relations Officer at the Canadian Food Inspection Agency, and I'll be moderating today's technical briefing. I will begin by introducing our speakers who will make a short statement in both official languages and then open it up to the media for questions.

Our first speaker is Paul Mayers, Vice-President, Policy and Programs, followed by Dr. Martine Dubuc, Vice-President, Science, and Delegate for Canada for the World Organization for Animal Health.

Bonjour et merci de vous joindre à nous aujourd'hui. Mon nom est Denis Schryburt, agent des Relations avec les médias à l'Agence canadienne d'inspection des aliments, et j'animerais la séance d'information technique aujourd'hui. Je vais débuter par présenter nos porte-parole qui feront une brève déclaration dans les deux langues officielles et ensuite répondre à vos questions.

Notre premier porte-parole est Paul Mayers, vice-président, Politique et Programmes, suivi par Martine Dubuc, vice-présidente, science, et la délégué pour Canada pour l'Organisation mondiale de la santé animale.

I will now invite Paul Mayers to make a brief statement in English. Mr. Mayers.

Paul Mayers: Thank you, Denis. Good afternoon, everyone, and thank you for calling in today. We'd like to provide some information today on a developing animal health situation. The Canadian Food Inspection Agency has confirmed Bovine Spongiform Encephalopathy, also known as BSE, in a beef cow from Alberta.

First of all, no part of the animal's carcass entered the human food or animal feed system. Canada's suite of internationally recognized safeguards effectively protects the safety of food and animal feed. There is no risk to food safety.

The Government of Canada is committed to protecting human and animal health and takes the management of BSE very seriously. Immediately upon confirmation of this case, the CFIA launched an investigation and is working closely with provincial and industry partners. This investigation will follow the well-developed procedures we've employed in response to previous BSE cases.

Canada's last confirmed BSE case was reported in 2011. This latest case in Alberta was detected through the National BSE Surveillance Program, which is a program that continues to play an important part in Canada's strategy to manage BSE. The fact that we continue to see very high levels of producer participation in the surveillance program underscores the commitment present throughout the cattle and beef sectors to responsibly manage BSE.

The detection of a small number of additional BSE cases is not unexpected in the context of the 30,000 samples we take annually, as Canada continues our ongoing management of this disease.

As has been our practice for CFIA investigations of BSE cases, the Agency is seeking to confirm the age of the animal, its history and how it may have become infected. We're also working to trace out all animals of equivalent risk such as the animals that may have been exposed to the same feed as the infected animal in the first year of its life. Equivalent risk animals will be ordered destroyed, and they will be tested for BSE.

The CFIA will notify the World Organization for Animal Health, also known as the OIE, in line with Canada's international obligations and our commitment to transparency.

This finding should not affect Canada's status as a controlled BSE risk country as recognized by the OIE. Canada continues to effectively manage BSE through a series of integrated safeguards designed to protect both human and animal health. These include prohibiting risk materials from entering the human food and animal feed chains and testing cattle for BSE.

Again, the CFIA is strongly committed to protecting animal health. Our investigation is underway, and we are mobilizing all necessary resources to address this situation.

Thank you.

Denis Schryburt: Thank you, Mr. Mayers. Et maintenant j'invite Martine Dubuc à faire une déclaration en français. Mme Dubuc, s'il-vous-plaît.

Dr Martine Dubuc: Merci. Bonjour à tous, et merci de vous être joints à la téléconférence aujourd'hui. Nous aimerions vous donner aujourd'hui des renseignements sur une situation de santé animale en évolution.

L'Agence canadienne d'inspection des aliments a confirmé un cas d'Encéphalopathie spongiforme bovine, aussi connu sous le nom d'ESB, chez une vache de boucherie provenant de l'Alberta.

 
snip... 

-30-


Timeline of Events: Bovine Spongiform Encephalopathy – Alberta – February 2015

February 13

The Canadian Food Inspection Agency (CFIA) holds a technical briefing related to the Bovine Spongiform Encephalopathy (BSE) positive case found in Alberta.

February 12

The CFIA notifies key trading partners of the new finding and posts the information on its website.

February 11

The CFIA confirms BSE in one beef cow in Alberta.

The CFIA continue to gather information on the animal's herd of origin and to trace the suspect animal's offspring.

February 10

The CFIA gather preliminary information on the suspect animal's herd of origin.

February 9

The CFIA receives a tissue sample from the affected animal and begins confirmatory testing at its laboratory in Lethbridge.

CFIA inspectors follow up at the farm, obtain additional samples, discuss next steps with producer and begin the investigation.

February 7

The province of Alberta reports a non-negative test for BSE to the CFIA.


Friday, February 20, 2015

A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015)



Saturday, February 14, 2015

Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta


Current as of: 2015-01-31

Sheep flocks and/or goat herds confirmed to be infected with classical scrapie in Canada in 2015 Date confirmed Location Animal type infected January 5 Ontario Goat

http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/2015/scrapie-2015-/eng/1423162035296/1423162036030

http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/scrapie/eng/1329723409732/1329723572482

Tuesday, February 10, 2015

Alberta Canada First case of chronic wasting disease found in farm elk since 2002


Tuesday, May 21, 2013

Canada, USA, Bad feed, mad cows: Why we know three BSE cases had a common origin and why the SSS policy is in full force $$$


Thursday, January 17, 2013

Canada, U.S. agree on animal-disease measures to protect trade, while reducing human and animal health protection


Sunday, December 2, 2012

CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’


Tuesday, October 2, 2012

Canadian veterinarian fined after approving banned BSE high risk cattle for export to U.S.A.


Monday, April 23, 2012

BOVINE SPONGIFORM ENCEPHALOPATHY BSE CJD TSE PRION DISEASE UPDATE CANADA 2012


Thursday, February 10, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31



Wednesday, August 11, 2010

REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA

 
Thursday, August 19, 2010

REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA


Published Date: 2010-03-11 19:00:03 

Subject: PRO/AH/EDR> BSE, bovine - Canada: (AB) Archive Number: 20100311.0792

BSE, BOVINE - CANADA: (ALBERTA) 

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A ProMED-mail post <http://www.promedmail.org> ProMED-mail is a program of the International Society for Infectious Diseases <http://www.isid.org>

[1] Date: 25 Feb 2010 Source: Canadian Food Inspection Agency [edited]


Bovine spongiform encephalopathy (BSE) cases have been confirmed in Canada in 2010. BSE is a reportable disease under the "Health of Animals Regulations." This means that all suspected cases must be reported to the CFIA. The following table lists individual animals confirmed to be infected with BSE in Canada in 2010, updated 28 Feb 2010:

Date confirmed: 25 Feb 2010 Location: Alberta Animal type infected: Beef cow Age of animal: 72 months

-- Communicated by: Terry S. Singeltary Sr. <flounder9@verizon.net>

****** [2] Date: 11 Mar 2010 Source: Meat Trade News [edited]

 <http://www.meattradenewsdaily.co.uk/news/100310/canada___case_of_bse_mad_cow_disease_in__year_old_cow.aspx> 

The Badger has learned a new case of BSE was discovered 2 weeks ago, but the public was not informed as part of the government's new communication strategy. The decision not to announce new cases of BSE was made in August 2009, and the public was informed by the Canadian Food Inspection Agency (CFIA) online.

"The CFIA is committed to providing all stakeholders, including the general public, media and trading partners, with timely information about disease detections in farmed animals. As such, we have revised how we report online for disease detections in farmed animals to provide a more comprehensive view of Canada's animal health status. All confirmed cases of federally reportable diseases in farmed animals will be centrally located on our website. This information will be updated monthly," explained CFIA spokesperson Jenn Gearey.

The new communication strategy means journalists will not be notified when any new cases of BSE are discovered.

The latest finding of BSE -- Canada's 17th domestic case -- was announced to industry stakeholders, such as processors, on 25 Feb 2010, but not to the media or general public. And while the CFIA claims its reportable diseases page will be updated monthly, no new information has been posted since 31 Jan 2010.

The infection was detected through the national surveillance program in a 6-year-old black angus cow in the same general area of Alberta, home to most of Canada's BSE activity.

The last case discovered in Canada was in May 2009, the only occurrence that year. In 2008, there were 4 incidents; in 2007, there were 3, and in 2006, there were 5 cases of BSE.

Canada's international risk status has not been affected by the latest case.

-- Communicated by: Terry S. Singeltary Sr. <flounder9@verizon.net>

****** [3] Date: 10 Mar 2010 Source: Reuters Canada [edited] <http://ca.reuters.com/article/domesticNews/idCATRE6295A420100310> 

Canada has confirmed its 17th case of mad cow disease, a finding that will delay any upgrade to its international risk status by one year, a top industry official said on Wednesday [10 Mar 2010].

The animal was born in February 2004, making it Canada's latest-born case of bovine spongiform encephalopathy (BSE). The new case pushes back the earliest date for an upgrade to Canada's controlled risk status from the World Organization for Animal Health (OIE) to 2016, said Ted Haney, president of the Canada Beef Export Federation.

A country cannot apply to upgrade to negligible status sooner than 11 years after the latest-born case of BSE. The process then takes about one year.

Canada, along with many other countries with controlled risk status from the OIE, can ship beef as long as it meets conditions such as disease surveillance.

The infected animal, which has been slaughtered, has not affected trade, Haney said.

The 2003 discovery of the 1st case of mad cow disease on a Canadian farm caused many countries to halt imports of Canadian beef. Most markets have since reopened, but the cattle industry remains in a slump due to other factors such as a strong Canadian dollar.

Mad cow disease is believed to be spread when cattle eat protein rendered from the brains and spines of infected cattle or sheep. Canada banned that practice in 1997.

The Canadian Food Inspection Agency tightened feed rules further in 2007 and said the moves should help eliminate the disease nationally within a decade, although the agency cautioned it still expected to discover the occasional new case.

CFIA spokeswoman Julie LePage confirmed the 17th case but could not provide details of the new case.

The CFIA notified cattle industry officials of the new case late last month [February 2010] but did not issue a news release, Haney said.

[Byline: Rod Nickel]

-- Communicated by: ProMED-mail <promed@promedmail.org>

[While it may be CFIA's decision on how to notify the public, it may not be in the best interest as far as public relations are concerned. Also, the Reuters article does not make much sense. How can the OIE status change be delayed to 2016 if it is 11 years from the last case? From the CFIA website [article [1] in this posting], it appears the animal was confirmed positive on 25 Feb 2010. Thus, 11 years would be 2021. - Mod.TG] See Also 2009 ---- BSE, bovine - Canada (AB) 20090517.1841 BSE, bovine, 2008 - Canada: (AB, BC) CFIA reports 20090417.1459 2008 ---- BSE, bovine - Canada (04): (BC) 20081119.3648 BSE, bovine - Canada (03): (AB) 20080819.2580 BSE, bovine - Canada (02): (BC) 20080623.1941 BSE, bovine - Canada (AB) 20080226.0786 2007 ---- BSE, bovine - Canada (AB) (03) 20071218.4076 BSE, bovine - Canada (BC) 20070502.1430 BSE, bovine - Canada (AB) (02) 20070308.0813 BSE, bovine - Canada (AB) 20070208.0499 2006 ---- BSE, bovine - Canada (AB)(06) 20061227.3621 BSE, bovine - Canada (AB)(05) 20060825.2413 BSE, bovine - Canada (AB)(04) 20060823.2384 ...................................................tg/msp/lm

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Increased Atypical Scrapie Detections

Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.


Published Date: 2008-08-19 11:00:29 Subject: PRO/AH/EDR> BSE, bovine - Canada (03): (AB) Archive Number: 20080819.2580

BSE, BOVINE - CANADA (03): (ALBERTA) 

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A ProMED-mail post <http://www.promedmail.org> ProMED-mail is a program of the International Society for Infectious Diseases <http://www.isid.org>

[1] Date: Fri 15 Aug 2008 Source: Canadian Food Inspection Agency (CFIA) [edited]


BSE [bovine spongiform encephalopathy] case confirmed in Alberta

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The Canadian Food Inspection Agency (CFIA) has confirmed bovine spongiform encephalopathy (BSE) in a 6-year-old beef cow from Alberta. No part of the animal's carcass entered the human food or animal feed systems.

The animal's birth farm has been identified, and an investigation is underway. The CFIA is tracing the animal's herdmates at the time of birth and examining possible sources of infection. The age and location of the infected animal are consistent with previous cases detected in Canada.

This case was detected through the national BSE surveillance program, which has been highly successful in demonstrating the low level of BSE in Canada. The program continues to play an important role in Canada's strategy to manage BSE.

Canada remains a Controlled Risk country for BSE, as recognized by the World Organisation for Animal Health (OIE). Accordingly, this case should not affect exports of Canadian cattle or beef.

For information: Canadian Food Inspection Agency Media relations: 613-228-6682

-- Communicated by: Terry S Singeltary Sr <flounder9@verizon.net>

****** [2] Date: Sat 16 Aug 2008 Source: Montana News Station, Associated Press (AP) report [edited]

 <http://www.montanasnewsstation.com/Global/story.asp?S=8851685> 

New mad cow case found in Canada 

--------------------------------

A new case of mad cow disease was confirmed in Canada, its 14th case since 2003.

Government inspectors say no part of the animal entered the human food system. The Canadian Food Inspection Agency (CFIA) says the disease was found in a 6-year-old beef cow. The agency did not say where the cow was born. The agency says it is tracing other cattle in the herd and is trying to determine how the cow became infected with the disease. They say the new case should not affect exports of Canadian cattle or beef.

Mad cow disease causes spongy holes in the brain. In people, a rare but fatal form of the disease has been linked to eating infected tissue from cows.

The inspection agency has said a ban on using animal materials in feed products has virtually eliminated the spread of BSE in Canada, but it said a small number of mad cow cases are still expected to surface.

-- Communicated by: ProMED-mail Rapporteur Brent Barrett

****** [3] Date: Sat 16 Aug 2008 Source: The Edmonton Journal [edited] <http://www.canada.com/edmontonjournal/news/story.html?id=e1dd935b-43dc-417a-a76e-6376990ba413>

Another mad cow case confirmed 

------------------------------

A 6-year-old beef cow was confirmed Friday [15 Aug 2008] as the 13th case of mad cow disease in Alberta, the Canadian Food Inspection Agency said.

It is the 14th confirmed case of bovine spongiform encephalopathy, or BSE, in Canada.

"At this point, it is too early to say how it could have been infected," said Natalie Bragg, a veterinary program specialist with the food inspection agency.

The cow was born, raised, and died on the same farm in northern Alberta, Bragg said. The agency does not release specific locations of infected animals. The animal was euthanized after it became sick. A sample from the animal was tested twice and confirmed as carrying BSE on Friday [15 Aug 2008].

The agency said no traces of BSE made it into either human or animal food supplies. Bragg said the investigation is now focused on the feed on the farm, including how it was transported and stored. The animal was born after a 1997 ban on feed containing cattle or other ruminant parts was introduced.

The agency is also tracking all other animals that were born within a year, and on the same farm, as the dead cow.

Last month [July 2008], it was revealed that Alberta plans to test 50 per cent fewer cattle for BSE by stopping targeted tests of elderly bovines or those without proper documentation. The step was taken because animals that are 9 years old or older are far less likely to contract the diseases, the Alberta government said. Between 2004 and mid-2006, 54 per cent of cattle tested in the province were 9 years or older. 2 of those cows tested positive.

Alberta tests up to 30 000 cattle a year, roughly half of the national BSE monitoring.

Cattle industry officials downplayed the latest BSE discovery, saying it should have little impact on their business, including international beef exports. "So far there has been no major reaction and no markets have closed, and that's because we have kept our international clients educated," said Cam Daniels of Canada Beef Export Federation.

Daniels regularly travels to Japan, China, Mexico, Macau, the Middle East, and other places to talk to importers and distributors. "They understand clearly the control measures we have in place, and they know we're expecting more cases because we are working diligently to find them," he said.

While everyone looks forward to the time when mad cow is eradicated in Canada, some overseas clients view the climbing number of cases as positive right now because it means our surveillance programs are working, said Alberta Beef Producers spokeswoman Lori Creech. "They prefer we find them, rather than in the words of Ralph Klein, 'Shoot, shovel and shut up,' " she said.

"We are very transparent and open as a country. This latest case doesn't affect our status in the world at all because it's not unexpected that they would find another animal with BSE."

The World Organization for Animal Health (OIE) lists Canada as a controlled risk country for BSE.

[Byline: Ryan Cormier and Keith Gerein]

-- Communicated by: ProMED-mail <promed@promedmail.org>

[Although Canada is proposing a decrease of its surveillance their system is clearly working. The system may be expensive, but it appears to be a model for others. - Mod.TG

Alberta can be located on the HealthMap/ProMED-mail interactive map of Canada at <http://healthmap.org/promed?v=55.4,-101.9,4>. - CopyEd.MJ] See Also BSE, bovine - Canada (02): (BC) 20080623.1941 BSE, bovine - Canada (AB) 20080226.0786 2007 ---- BSE, bovine - Canada (AB) (03) 20071218.4076 BSE, bovine - Canada (BC) 20070502.1430 BSE, bovine - Canada (AB) (02) 20070308.0813 BSE, bovine - Canada (AB) 20070208.0499 ...................................tg/mj/dk

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CANADA BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION DISEASE


MONDAY, JANUARY 09, 2023 CANADA 

Canada Unstable funding threatens ‘zombie deer’ CWD TSE PRION research in the Prairies 


FRIDAY, DECEMBER 09, 2022 

Manitoba, Canada PROVINCE ADVISES ADDITIONAL CASES OF CHRONIC WASTING DISEASE DETECTED


THURSDAY, OCTOBER 06, 2022 

CANADA, Alberta, Québec, Manitoba, Saskatchewan, CWD UPDATE 2022 


USA BSE

Notice of Request To Renew an Approved Information Collection: Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission

Singeltary further comments in attachment;

Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment

https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf

Sunday, January 10, 2021 
APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission June 17, 2019

APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission

Greetings APHIS et al, 

I would kindly like to comment on APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], and my comments are as follows, with the latest peer review and transmission studies as references of evidence.

THE OIE/USDA BSE Minimal Risk Region MRR is nothing more than free pass to import and export the Transmissible Spongiform Encephalopathy TSE Prion disease. December 2003, when the USDA et al lost it's supposedly 'GOLD CARD' ie BSE FREE STATUS (that was based on nothing more than not looking and not finding BSE), once the USA lost it's gold card BSE Free status, the USDA OIE et al worked hard and fast to change the BSE Geographical Risk Statuses i.e. the BSE GBR's, and replaced it with the BSE MRR policy, the legal tool to trade mad cow type disease TSE Prion Globally. The USA is doing just what the UK did, when they shipped mad cow disease around the world, except with the BSE MRR policy, it's now legal. 

Also, the whole concept of the BSE MRR policy is based on a false pretense, that atypical BSE is not transmissible, and that only typical c-BSE is transmissible via feed. This notion that atypical BSE TSE Prion is an old age cow disease that is not infectious is absolutely false, there is NO science to show this, and on the contrary, we now know that atypical BSE will transmit by ORAL ROUTES, but even much more concerning now, recent science has shown that Chronic Wasting Disease CWD TSE Prion in deer and elk which is rampant with no stopping is sight in the USA, and Scrapie TSE Prion in sheep and goat, will transmit to PIGS by oral routes, this is our worst nightmare, showing even more risk factors for the USA FDA PART 589 TSE PRION FEED ban. 

The FDA PART 589 TSE PRION FEED ban has failed terribly bad, and is still failing, since August 1997. there is tonnage and tonnage of banned potential mad cow feed that went into commerce, and still is, with one decade, 10 YEARS, post August 1997 FDA PART 589 TSE PRION FEED ban, 2007, with 10,000,000 POUNDS, with REASON, Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. you can see all these feed ban warning letters and tonnage of mad cow feed in commerce, year after year, that is not accessible on the internet anymore like it use to be, you can see history of the FDA failure August 1997 FDA PART 589 TSE PRION FEED ban here, but remember this, we have a new outbreak of TSE Prion disease in a new livestock species, the camel, and this too is very worrisome.

WITH the OIE and the USDA et al weakening the global TSE prion surveillance, by not classifying the atypical Scrapie as TSE Prion disease, and the notion that they want to do the same thing with typical scrapie and atypical BSE, it's just not scientific.

WE MUST abolish the BSE MRR policy, go back to the BSE GBR risk assessments by country, and enhance them to include all strains of TSE Prion disease in all species. With Chronic Wasting CWD TSE Prion disease spreading in Europe, now including, Norway, Finland, Sweden, also in Korea, Canada and the USA, and the TSE Prion in Camels, the fact the the USA is feeding potentially CWD, Scrapie, BSE, typical and atypical, to other animals, and shipping both this feed and or live animals or even grains around the globe, potentially exposed or infected with the TSE Prion. this APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], under it's present definition, does NOT show the true risk of the TSE Prion in any country. as i said, it's nothing more than a legal tool to trade the TSE Prion around the globe, nothing but ink on paper.

AS long as the BSE MRR policy stays in effect, TSE Prion disease will continued to be bought and sold as food for both humans and animals around the globe, and the future ramifications from friendly fire there from, i.e. iatrogenic exposure and transmission there from from all of the above, should not be underestimated. ...


APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission

Comment from Singeltary Sr., Terry

Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022



Suppressed peer review of Harvard study October 31, 2002
October 31, 2002

Review of the Evaluation of the Potential for Bovine Spongiform Encephalopathy in the United States Conducted by the Harvard Center for Risk Analysis, Harvard School of Public Health and Center for Computational Epidemiology, College of Veterinary Medicine, Tuskegee University

Final Report 

Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Update; Notice of Availability and Technical Meeting 

Owens, Julie

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Monday, July 24, 2006 1:09 PM

To: FSIS RegulationsComments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)


Response to Public Comments on the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update,

October 31, 2005

INTRODUCTION

The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website: http://www.fsis.usda.gov/Science/Risk_Assessments/index.asp). Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, RCALF USA, Linda A Detwiler, and Terry S. Singeltary. This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind: 


03-025IFA

03-025IFA-2

Terry S. Singeltary

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Thursday, September 08, 2005 6:17 PM

To: fsis.regulationscomments@fsis.usda.gov

Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle


ONE final comment tonight, i just cannot take anymore. well, ill just let the facts speak for themselves, no need to even comment ;

Section 2. Testing Protocols and Quality Assurance Controls

In November 2004, USDA announced that its rapid screening test, Bio-Rad Enzyme Linked Immunosorbent Assay (ELISA), produced an inconclusive BSE test result as part of its enhanced BSE surveillance program. The ELISA rapid screening test performed at a BSE contract laboratory produced three high positive reactive results.40 As required,41 the contract laboratory forwarded the inconclusive sample to the APHIS National Veterinary Services Laboratories (NVSL) for confirmatory testing. NVSL repeated the ELISA testing and again produced three high positive reactive results.42 In accordance with its established protocol, NVSL ran its confirmatory test, an immunohistochemistry (IHC) test, which was interpreted as negative for BSE. In addition, NVSL performed a histological43 examination of the tissue and did not detect lesions44 consistent with BSE.

Faced with conflicting results, NVSL scientists recommended additional testing to resolve the discrepancy but APHIS headquarters officials concluded no further testing was necessary because testing protocols were followed. In our discussions with APHIS officials, they justified their decision not to do additional testing because the IHC is internationally recognized as the "gold standard." Also, they believed that conducting additional tests would undermine confidence in USDA’s established testing protocols.

full text 130 pages ;


PDF]Freas, William TSS SUBMISSION
File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ...

http://web.archive.org/web/20170301223601/https://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf

Terry S. Singeltary Sr.

posted by Terry S. Singeltary Sr. at 12:13 PM