The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2019
European Food Safety Authority (EFSA)
First published: 17 November 2020 https://doi.org/10.2903/j.efsa.2020.6303
Requestor: European Commission
Question number: EFSA‐Q‐2019‐00003
Acknowledgements: EFSA wishes to thank for the support provided to this scientific output to the EFSA staff members: Katrin Bote, Maria Francesca Iulietto, Alexandra Papanikolaou, Luca Pasinato, Angel Ortiz Pelaez, Valentina Rizzi, Alban Shajah and Anca Stoicescu; to the OIE staff member Paula Caceres Soto, and to the EFSA contractor: Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta (Unit BEAR – Biostatistica Epidemiologia e Analisi del Rischio and staff: Giuseppe Ru and Francesco Ingravalle).
Approved: 16 October 2020
Abstract
This report presents the results of surveillance on transmissible spongiform encephalopathies (TSE) in cattle, sheep, goats, cervids and other species, and genotyping in sheep, carried out in 2019 by 28 Member States (MS), and by Iceland, Montenegro, North Macedonia, Norway, Serbia and Switzerland (non‐MS). In total, 1,150,388 cattle were tested by MS, a 2.7% decrease from 2018 and 44,557 by the six non‐MS. Six cases of H‐BSE were reported by France (4) and Spain (2), and 1 L‐BSE by Poland. The number of H‐ BSE cases was the largest reported per year including the youngest ever case (5.5 years of age). In total, 338,098 sheep and 143,529 goats were tested in the EU, an increase of 3.9% in both species compared with 2018. In sheep, 17 inconclusive cases by two MS and 997 cases of scrapie were reported: 911 classical (97 index cases (IC), one of ARR/ARR genotype and 98.7% with genotypes of susceptible groups) by seven MS, 86 atypical (AS) (80 IC) by 11 MS. Thirty‐one ovine scrapie cases were reported by Iceland and Norway. Random genotyping was only reported by eight MS: Cyprus excluded, 15.7% of genotyped sheep carried genotypes of susceptible groups. In goats, three inconclusive cases by two MS and 390 cases of scrapie were reported: 379 classical (24 IC) by six MS, 11 atypical (10 IC) by six MS. The heterogeneous enforcement of a 3‐year surveillance programme for chronic wasting disease (CWD) in six MS (Estonia, Finland, Latvia, Lithuania, Poland and Sweden) resulted in the testing of 7,980 cervids and confirmation of three CWD cases in wild moose in Sweden. Other seven MS tested 2,732 cervids with no positive results. Norway tested 30,147 cervids in 2019, with two new moose cases. In total, 122 animals from four other species reported by three MS TSE tested negative.
Summary
This report of the European Food Safety Authority (EFSA) presents the detailed results of surveillance activities on animal transmissible spongiform encephalopathies (TSE) carried out during 2019 in the European Union (EU) and in six non‐Member States (non‐MS), i.e. Iceland, Montenegro, North Macedonia, Norway, Serbia and Switzerland, as well as genotyping data in sheep. TSE monitoring data for cattle, sheep, goats, cervids and species other than domestic ruminants are reported by country according to Regulation (EC) 999/2001 (the TSE Regulation) and consist of testing data as well as case data.
Surveillance data were mainly submitted through the EFSA TSE data reporting tool. Six MS submitted data directly as eXtensible Markup Language (XML) files by using their own system for the automatic upload of data into the EFSA Data Collection Framework (DCF) whereas the rest of the reporting countries submitted XML files via the EFSA TSE data collection tool. The electronically submitted data were extracted from the EFSA database and further processed and validated by EFSA to summarise the information and to elaborate the summary tables presented in the current EU summary report (EUSR).
A descriptive summary of the reported data is provided at reporting country level or aggregated at EU level for all reporting countries. When possible, descriptions and calculations were stratified according to the available variables, such as surveillance target group (healthy slaughtered animals, animals culled under bovine spongiform encephalopathy (BSE)/TSE control and eradication measures, etc.) or surveillance type (passive vs. active), country, sampling year (since 2001 for bovine animals and 2002 for small ruminants), case type (i.e. classical BSE (C‐BSE), atypical BSE (H‐BSE or L‐BSE), classical scrapie (CS) or atypical scrapie (AS)), flock/herd status (infected/non‐infected), index case and age class.
In total, 1,150,388 cattle were tested in 2019 in the EU. BSE testing was concentrated in the group of risk animals (emergency slaughtered animals (ES), animals with clinical signs at ante mortem inspection (AM) and fallen stock (FS)) with over 83% of all cattle tested in the EU, with FS being the largest contributor with 918,182 cattle tested in 2019 (92.4% of all cattle in the risk group). An additional 44,557 cattle were tested by four of the six non‐MS, with no cases reported. Serbia (the main contributor with 20,110 cattle tested) and Montenegro and reported mostly cattle from the HS target group.
Seven atypical cases of BSE were confirmed in 2019 by: Spain (2 H‐BSE), France (4 H‐BSE) and Poland (1 L‐BSE), all born between 2001 and 2008 except one H‐type case of 5.5 years in Spain. From an epidemiological point of view, the highlights in the BSE caseload of the reporting year are: the number of H‐type BSE cases was the largest reported in a single year, equal to that of 2009 (6), (six cases per million tested) and the report by Spain of a H‐type case of 5.5 years, the youngest atypical BSE ever reported since the TSE data are collected and published. An additional H‐BSE case was reported by Brazil.
In total, 481,627 small ruminants were tested in 2019 in the EU: 338,098 sheep (3.9% increase from 2018) and 143,529 goats (3.9% increase). In addition, 23,473 sheep and 217 goats were tested by four and three of the six non‐MS, respectively.
In sheep, 997 scrapie cases were reported in the EU in 2019, 641 more case than in 2018. A total 31 cases of scrapie in sheep were reported by two (Iceland and Norway) of the four non‐MS that tested in total 23,473 sheep, namely, Iceland, North Macedonia, Norway and Serbia. Classical scrapie (CS) was reported by seven MS and one non‐MS: Cyprus, Greece, Italy, Portugal, Romania, Spain, the United Kingdom and Iceland, whereas atypical scrapie (AS) was reported by 11 MS and one non‐MS: Finland, France, Germany, Hungary, Ireland, Italy, Poland, Portugal, Slovakia, Spain, the United Kingdom and Norway. An additional total of 17 cases in sheep were reported as inconclusive by Spain (5) and Italy (12) (See Table 31), which are not included in the total scrapie caseloads for these two countries.
Most of the ovine cases (91.7%) were reported by four countries, namely Greece, Italy, Romania and Spain, as it was the case in the previous year. In total, 911 ovine cases in the EU were CS (91.4%) and 86 were AS (8.6%). Among the non‐MS, 21 CS cases were reported by Iceland and 10 AS cases by Norway. In sheep, 17.8% (177) of all cases in the EU reported in 2019 were index cases, with a much higher proportion in AS cases (80/86: 93%) compared to CS cases (97/911: 10.6%). In total, 98.7% of the CS cases in sheep reported in 2019 with known genotypes belonged to animals holding genotypes of the susceptible groups (NSP3, NSP3O, NSP4 or NSP5). One CS case was reported by Spain in a sheep carrying the ARR/ARR genotype, a very rare occurrence.
In goats, 390 scrapie cases were reported in the EU in 2019, which is a reduction of 25.4% (–133) compared with 2018 when 523 cases were reported. Out of 390 caprine scrapie cases, 379 were CS (97.2%, with Cyprus accounting for 81.3% of these) and 11 were AS. This reduction is mainly due to the decrease in the number of cases in goats in two reporting countries: Cyprus (from 382 to 309), Spain (from 91 to 37). CS was reported by six MS, namely Cyprus, Greece, Hungary, Italy, Spain and the United Kingdom. AS was also reported by six MS: Cyprus, France, Italy, Poland, Portugal and Spain. The 217 goats tested by three non‐MS (Iceland, North Macedonia and Norway) were all negative. An additional total of three cases in goats were reported as inconclusive by Spain (1) and Italy (2) (See Table 33), which are not included in the total scrapie caseloads for these two countries.
In goats, only 8.7% of all cases reported in the EU in 2019 were index cases, lower than in 2018 (9.6%), with a higher proportion in AS (10/11: 90.9%) than in CS (24/379: 6.3%). Currently, CS is still the most frequently reported type of scrapie in the EU in both species. Focusing on the last 10 years (2010–2019), in sheep, there has been a significant average decrease of, respectively, 5% per year in CS and 4% per year in AS. In goats, there was no detectable trend for both CS and AS.
The long‐term trends of CS in terms of cases per 10,000 tests in both species, the situation in 2019 confirmed the 10‐year statistically significant decreasing trend in sheep and no detectable trend in goats, respectively. With regard to the long‐term trends of AS, there was also a 10‐year statistically significant decreasing trend in sheep and no detectable trend in goats.
In 2019, the genotyping activity from random samples of the national EU sheep populations was carried out by eight MS: Belgium, Cyprus (where genotyping is conducted systematically in the breeding sheep population), France, Greece, Italy, Latvia, the Netherlands and Poland. After excluding Cyprus, 15.7% of the randomly genotyped sheep still carried genotypes of the susceptible groups, lower than the 19.2% in 2018. The percentage of susceptible sheep rose to 45.5% in Greece and 29.9% in Italy, two of the countries with the highest caseload in 2019, whereas the percentage was 10.3% in the other five MS.
The enforcement of a 3‐year surveillance programme for CWD in six MS – Estonia, Finland, Latvia, Lithuania, Poland and Sweden – resulted in the testing of 7,980 cervids and the confirmation of three cases of CWD in wild moose by Sweden. The implementation of the mandatory surveillance in the six MS is quite heterogeneous in terms of design (number and characteristics of the declared primary sampling units, PSU), number of cervids tested in general and per PSU and distribution of testing by species and target groups. The targeting of mostly hunted/slaughtered fit for human consumption (HSHC) animals observed in the first year of implementation was increased in 2019 up to 60.5% of all tested cervids, consolidating a situation in which the sensitivity of the surveillance system is lower than expected. Other seven MS – Austria, Hungary, Italy, Romania, Slovenia, Spain and the United Kingdom – tested 2,732 cervids, all with negative results and with Romania accounting for 77,7% of all cervids tested by them. Norway continued its intensified testing programme in wild and captive cervids and tested 30,147 animals in 2019, mostly semi‐domesticated reindeer (42.9%), followed by wild moose (19.7%) and red deer (17.2%), leading to the detection of two moose cases. Additionally, Iceland reported testing of 114 cervids, with no cases.
A total of 122 animals of other species were TSE tested by Finland (114), Estonia (6) and Spain (2): 12 raccoon dogs (Nyctereutes procyonoides), 66 American minks (Neovison vison), 42 foxes (genus Vulpes) and 2 chamois (Rupicapra rupicapra). None of them tested positive.
1 Introduction
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1.2.4 TSE surveillance in cervids and other species
By Commission Regulation (EU) 2017/19728 requirements for a 3‐year surveillance programme for chronic wasting disease (CWD) in cervids in Estonia, Finland, Latvia, Lithuania, Poland and Sweden were established. Annexes I and III of Regulation (EC) No 999/2001 are amended in accordance with the Annex to this Regulation, which describes the surveillance programme.
The Member States which have a wild and/or farmed and/or semi‐domesticated population of moose and/or reindeer (Estonia, Finland, Latvia, Lithuania, Poland and Sweden) shall carry out a 3‐year monitoring programme for CWD in cervids, from 1 January 2018 to 31 December 2020. The TSE tests performed for the purpose of this monitoring programme shall take place between 1 January 2018 and 31 December 2020; however, the collection of samples for the purpose of the monitoring programme may start in 2017. The 3‐year monitoring programme for CWD in cervids is described in detail in Annex III, chapter A, Part III of the TSE Regulation. The other MS may carry out monitoring for CWD in cervids on a voluntary basis.
1.3 Testing protocols
The testing protocol for BSE surveillance in bovine animals is described in pages 8 and 9 of the 2016 EUSR on TSE (EFSA, 2017). The testing protocol for TSE surveillance in small ruminants is described in pages 13 and 14 of the 2016 EUSR on TSE (EFSA, 2017).
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3 Assessment
3.1 BSE surveillance in bovine animals
About 118.8 million bovine animals have been tested for BSE in EU since 2001. In 2019, there was a 2.7% reduction in the number of tested bovine animals in the EU, from 1,181,934 in 2018 to 1,150,388 in 2019, due to a reduction in the HS target group: from 177,536 in 2018 to 156,229 in 2019 and the slight reduction in the number of tested animals in the FS from 932,049 in 2018 to 918,182 in 2019, despite a 7.3% increase of animals tested in the ES group (from 64,262 in 2018 to 68,969 in 2019). Romania and Bulgaria continue being the main contributors to the HS testing group with 137.463 (88%) tested cattle.
The six non‐MS (Iceland, Montenegro, Norway, North Macedonia, Serbia and Switzerland) tested 44,557 cattle in 2019; Serbia, a new reporting country, was the main contributor with 20,110 cattle tested. The two new reporting countries, Montenegro and Serbia, reported mostly cattle tested in the HS target group and Serbia 30 extra cattle as clinical suspects.
There was a decrease of 1% in the number of animals tested in the risk group (ES+AM+FS), from 1,003,707 in 2018 to 993,332 in 2019. Similar to the previous year, cattle in the risk group accounted for over 86.4% of all tested cattle in the EU and cattle tested in the FS target group accounted for 92.4% of all risk cattle tested.
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The distribution of the number of bovine animals tested for BSE by age group, surveillance target group and reporting country in 2019 can be found in the following link https://doi.org/10.5281/zenodo.4087794 distributed as follows:
Table 6: Number of bovine animals tested by age group in the EU MS and non‐MS reporting countries in 2019.
Table 7: Number of bovine animals in the risk group (animals with clinical signs at ante‐mortem, emergency slaughtered and fallen stock), by age group, tested in EU MS and non‐MS reporting countries in 2019.
Table 8: Number of tested healthy slaughtered bovine animals by age group in EU MS and in non‐MS reporting countries in 2019.
Table 9: Number of BSE suspected bovine animals, by age group, tested in EU MS and in non‐MS-reporting countries in 2019.
Table 10: Number of bovine animals culled under BSE eradication measures, by age group, tested in EU MS and in non‐MS-reporting countries in 2019.
In the EU, seven BSE cases were reported in 2019, all atypical BSE submitted to the FS testing group: six H‐type (two by Spain and four by France) and one L‐type by Poland. Table 11 reports the main clinical and epidemiological data of the positive cases. One additional H‐type case was reported outside Europe, by Brazil in a 17‐year‐old beef cow found dead during ante‐mortem inspection at abattoir. The animal was born and raised on a full‐cycle beef farm on extensive grazing.10
The number of H‐type BSE cases in the EU was the largest reported in a single year (6), equal to the number reported in 2009. As a result, the proportion of cases per million tests raised to 6 for H‐type BSE in 2019 (Figure 1). The number of L‐type BSE was in line with the number of cases reported in the last few years. Most of the atypical cases reported in 2019 were detected in beef cattle older than 11 years, except one of the two H‐type BSE cases reported by Spain, an unusually young dairy animal (5.5 years). In fact, it is the youngest atypical BSE case ever reported since the TSE data are collected and published.
Based on 115 atypical BSE cases with known age of, the average age at detection was 11.85 years (range: 5.5–18.5 years) and tested in the FS target group that accounts, as mentioned above, for most of the tested animals.
The number of BSE cases by reporting country, type and year (up to 2019, with a focus on the last 5 years) is shown in Tables 12–14.
Time series analysis carried out over the last 10‐year period (period 2010–2019) shows a significant decreasing trend in the occurrence of C‐BSE (annual RR = 0.59 i.e. an annual decrease of 41% in the proportion of cases per tested animals; p < 0.0001), whereas no significant trend for the two atypical BSE forms was found (H‐BSE:1.05 p = 0.43; L‐BSE: 1.05 p = 0.47).
Maps showing the geographical distribution of the cumulative number of cases and the cumulative proportion of cases per million tests of C‐BSE cases born after the total (reinforced) feed ban (BARB), H‐BSE and L‐BSE for the period 2001–2019 are shown in Appendix B.
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Table 12. Total number of reported BSE cases (classical‐BSE + atypical H‐BSE + atypical L‐BSE) in reporting countries and worldwide by year (up to 2019) and country
BRA: Brazil; CAN: Canada; ISR: Israel; JPN: Japan; LI: Liechtenstein; NO: Norway; CH: Switzerland; USA: The United States of America. BSE: bovine spongiform encephalopathy; H‐BSE: H‐type BSE; L‐BSE: L‐type BSE.
a Included imported cases: CAN one case in 1993; Denmark one case in 1992; France one case in 1999; Germany one case in 1992, three cases in 1994, two cases in 1997; Ireland five cases in 1989, one case in 1990, two cases in 1991 and 1992, one case in 1994 and one case in 1995; Italy two cases in 1994, 2001 and 2002; Portugal one case in 1990, 1991, 1992, 2000 and 2004 and three cases in 1993; Slovenia one case in 2004; Switzerland one case in 2012; USA one case in 2003.
b NL: The number of classical scrapie for NL should be updated in the database for 2001 from 18 cases to 19 cases. Therefore, the total of the Netherlands for the column up to 2013 is 88 instead of 87.
c Gavier‐Widen et al. (2008). Each cell reports the total number of BSE cases (C‐BSE + H‐BSE + L‐BSE). Grey‐shaded cells indicate the year(s) and Member State(s) where at least one BARB case was detected (EFSA, 2017). EU countries without BSE cases are not included. Source: data regarding non‐EU cases and cases in EU Member States for the period 1987–2002 were made available by the European Commission (European Commission, 2016). Data were retrieved from the EU TSE Database and the OIE website (http://www.oie.int/wahis).
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3.2 TSE surveillance in small ruminants
Since 2002, nearly 10 million small ruminants have been tested as part of the official EU TSE surveillance in the EU. In 2019, 481,627 small ruminants were tested by the 28 MS: 338,098 sheep (70.2%) and 143,529 goats (29.8%), which represents a 3.9% increase (18,113) in the number of tested small ruminants in the EU, compared with that of 2018.
In four of the six non‐MS reporting countries (Iceland, North Macedonia, Norway, Serbia), a total of 23,690 small ruminants were tested: 23,473 sheep (99.1%) and 217 goats (0.9%), an increase of 1,740 from 2018, mostly due to the increase in testing by Norway and Iceland, and not by the addition of the two new reporting countries (only Serbia tested 165 sheep slaughtered for human consumption). Montenegro and Switzerland did not report data on small ruminants.
In sheep, the increase in the total tested in the EU was 3.9% (338,098 tested in 2019 compared with 325,386 in 2018), due to the increase of testing in both TSE‐infected flocks with a 9.4% increase in 2019 (from 37,662 in 2018 to 41,197 in 2019), and non TSE‐infected flocks with a 3% increase in 2019 (from 285,897 in 2018 to 294,527 in 2019).
In goats, there was also a 3.9% increase in the tested animals in the EU (143,529 in 2019 compared with 138,128 in 2018), due to the increase of testing in both TSE‐infected herds with a 5.2% increase in 2019 (from to 12,375 in 2018 to 13,013 in 2019) and non‐TSE‐infected herds with a 3.3% increase in 2019 (from 125,658 in 2018 to 129,810 in 2019).
The numbers of sheep and goats tested for TSE by reporting country, surveillance target group and flock/herd status in 2019 are summarised in Tables 19 and 20, respectively. Taking into account the number of samples tested in the SHC and NSHC target groups and those required according to the TSE Regulation (Table 4), 22 MS fulfilled the requirements for sheep testing. In goat surveillance, 23 MS fulfilled the requirements for goat testing.
The pattern of testing in 2019 in sheep by country and flock status was very similar to that of 2018. In 2019, for each sheep tested in a TSE‐infected flock in the EU, there were 7.1 sheep tested in non‐TSE‐infected flocks, similar to 2018 and lower than in 2017 with nearly 11 tested in non‐TSE‐infected flocks. The increase in the overall testing of sheep in 2019 is mostly due to the higher level of testing in three reporting countries: Spain, Italy and Poland, with an overall increase of 4,776, 4,161 and 2,711 sheep tested, respectively.
The pattern of testing in 2019 in goats by country and flock status was very similar to that of 2018. In 2019, for each goat tested in a TSE‐infected herd in the EU, there were 10 goats tested in non‐TSE infected herds, similar to 2018 and much lower than in 2017 with nearly 31 goats tested in non‐TSE‐infected herds in 2017. The increase in the overall testing of goats in 2019 is mostly due to the higher level of testing in Italy, with an overall increase of 4,796 goats tested.
Table 19. Number of sheep tested for TSE by reporting country, surveillance target group and flock status in 2019 in the EU and other reporting countries
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In total, 997 scrapie cases in sheep were reported in the EU in 2019, 64 (6.9%) more than in 2018. They were reported by 14 MS, four less than in 2018. Austria, Denmark and Sweden, that reported AS in 2018, and Bulgaria, that reported CS in 2018, did not report scrapie cases in 2019. In addition, 31 scrapie cases in sheep were reported by two (Iceland and Norway) of the six non‐MS reporting countries.
CS was reported by seven different MS and one non‐MS: Cyprus, Greece, Spain, Italy, Portugal, Romania, the United Kingdom and Iceland, whereas AS was reported by 11 MS and one non‐MS: Finland, France, Germany, Hungary, Ireland, Italy, Poland, Portugal, Slovakia, Spain, the United Kingdom and Norway. Most of the ovine cases in the EU (91.7%) were reported by four countries, namely Greece, Italy, Romania and Spain, as it was the case in the previous years.
In total, 911 sheep scrapie cases in the EU in 2019 were CS cases (91.4%) and 86 were AS cases (8.6%). Among the non‐EU reporting countries, 21 CS cases were reported by Iceland and 10 AS cases by Norway. Table 21 shows the number of scrapie cases in sheep by reporting country, case type, index case status and surveillance target group in 2019. The geographical distribution of AS and CS in 2019 in sheep is shown in Appendix C. An additional total of 17 cases in sheep were reported as inconclusive by Spain (5) and Italy (12) (See Table 31), which are not included in the total scrapie caseloads for these two countries.
In sheep, 17.8% (177) of all cases in the EU reported in 2019 were index cases, a reduction of 12.8% from the previous year (203 cases), with a much higher proportion in AS cases (80/86: 93%) than in CS cases (97/911: 10.6%), reflecting the within‐flock spread of CS. Using the absolute number of index cases as a proxy for the flock‐level incidence in sheep and comparing 2018 with 2019, there was nearly no difference in the number of CS index cases (from 98 in 2018 to 97 in 2019, –1%) and a decrease in the number of AS index cases (from 105 to 80, –25.2%).
In total, 390 scrapie cases in goats were reported in the EU in 2019 by nine MS (two more than in 2018), with a 25.4% reduction (–133) compared with 2018 when 523 cases were reported. This change is due mainly to the decrease in the number of cases in goats in two reporting countries: Cyprus (from 382 to 309), Spain (from 91 to 37). Cyprus, Italy and Spain reported both CS and AS. Greece, Hungary and the United Kingdom reported only CS cases whereas France, Poland and Portugal reported only AS cases. As mentioned previously, most of the CS cases were reported by Cyprus with a slight increase in the contribution of this country to the CS caseload in 2019 (from 73.7% in 2018 to 81.3% in 2019). The three non‐MS that reported tested goats did not report any scrapie cases.
In total, 379 caprine cases in the EU in 2019 were CS cases (97.2%) and 11 were AS (7.8%). Table 13 shows the number of scrapie cases in goats by reporting country, case type, index case status and surveillance target group in 2019. The geographical distribution of AS and CS in 2019 in goats is shown in Appendix C. An additional total of three cases in goats were reported as inconclusive by Spain (1) and Italy (2) (See Table 33), which are not included in the total scrapie caseloads for these two countries.
In goats, 8.7% (34) of all cases reported in the EU in 2019 were index cases, lower than the 9.6% (50) in 2018, with a higher proportion in AS (10/11: 90.9%) than in CS (24/379: 6.3%). Cyprus and Italy accounted for 61.8% of all index cases in goats. Using the absolute number of index cases in goats as a proxy for the herd‐level incidence in goats and comparing 2019 with 2018, there was a decrease in the number of CS index cases (from 44 to 24, –45.5%) and an increase in the number of AS index cases (from 6 to 10).
In general, considering the total number of cases by type and without restricting the calculation to index cases only, CS is still the most frequently reported type of scrapie in the EU in both the species. In 2019, the CS/AS ratio was 10.6:1 in sheep (higher than in 2018: 7.3:1) and 34.4:1 in goats (lower than in 2018: 86.1:1). If, for goats, Cyprus is excluded, the CS/AS ratio in goats was 7.1:1 in 2019, higher than in 2018 (27.2:1).
Table 21. Number of scrapie cases in sheep by country, case type, index case status, surveillance target group in 2019 in the EU and other reporting countries
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Focusing on the last 10 years, the evolution in the number of scrapie cases detected at EU level is shown for each species and by case type in Figure 2. After the 2006 peak in the number of reported scrapie cases in sheep with 2,596 CS cases (when the number of tests also peaked), CS cases have decreased from 1,444 in 2011 to 554 in 2016. An increase was observed in 2017 (839 CS and unknown cases), and a similar order of magnitude was observed in 2018 (820 cases). A new increase to 911 is evident in 2019, mainly due to CS cases from TSE‐infected flocks reported by Greece, Italy and Spain.
Greece, Spain and Italy increased the overall number of CS scrapie cases by nearly 55.1%, 11.8% and 14%, respectively, compared to the previous year. This trend was likely associated with the increase in the testing of TSE‐infected flocks. In Romania, despite testing in both years around seven thousand sheep in TSE‐infected flocks, the total number of CS cases decreased from 203 in 2018 to 141 in 2019 (–30.5%). The number of index cases of CS in the four most affected reporting countries was 94, very similar to that in 2018, 92.
In goats, the decreasing trend in the absolute number of CS cases is clear. The evolution is mainly affected by one single MS (Cyprus), where the number of detected cases has consistently declined since the peak in 2013 when 1,678 cases were reported by Cyprus and 1,799 in total including all other MS. Since then, the total number of CS cases has decreased consistently to 308 in 2019. Spain has also observed a substantial reduction in the number of CS in goats from 89 in 2018 to 35 in 2019, with only two index CS cases. Greece also reported fewer CS cases whereas the total number in Italy increased from 15 to 26, although the number of index cases remained stable.
Based on the 20,364 cases of scrapie with known type, species and age between 2009 and 2019, in sheep, the average age of AS cases (85.2 months) is significantly higher (p < 0.001) than that of CS cases (51.1 months). Similarly, in goats, the average age of AS cases (87.6 months) is significantly higher (p < 0.001) than that of CS cases (52.4 months). When comparing sheep with goats, there is no significant difference in the average age for AS (87.6 months in goats and 85.2 months in sheep) (p = 0.54), with the average age of CS cases in sheep (51.1 months) being very similar to that of goats (52.4 months) although statistically different (p < 0.05).
Tables 23 and 24 show the cases of CS and AS, respectively, in sheep for the period 2002–2019, with a focus on the last 5 years. Tables 25 and 26 show the cases of CS and AS, respectively, in goats for the period 2002–2019, with a focus on the last 5 years.
In sheep, in 2019, the number of index cases of CS and AS per 10,000 tests carried out by target group at EU level was: 1) for CS: 3.3 in NSHC, 2.2 in SHC and 714.3 in SU; 2) for AS: 3.4 in NSHC and 1.7 in SHC.
In goats, in 2019, the number of index cases of CS and AS per 10,000 tests carried out by target group at EU level was: (1) for CS: 1.9 in NSHC and 1.8 in SHC; (2) for AS: 0.9 in NSHC and 0.7 in SHC.
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Table 36 shows the genotypes of sheep scrapie cases in 2019 in the EU and other reporting countries.
In total, 855 (98.7%) of the 866 cases of CS in sheep with known genotype reported in the EU in 2019 (93.8% of the total CS caseload) were from the susceptible genotype groups (NSP3, NSP3O, NSP4 and NSP5). This is very similar to previous years in which over 93% of all CS cases with known genotypes were of the susceptible groups. In non‐MS reporting countries, all CS cases reported by Iceland were of the susceptible genotype groups. It is important to highlight the reporting of a CS case by Spain in a sheep holding the ARR/ARR genotype (NSP1), a very rare occurrence.
For AS, the same genotype groups (NSP3, NSP3O, NSP4 and NSP5) accounted for 45.2% of all cases with known genotype (38.4% of the total AS caseload), very similar to 2018. Figure 4 shows the frequency distribution of genotypes of sheep scrapie cases by case type, year and NSP group in the period 2010–2019 in the reporting countries.
Table 37 shows the genotypes obtained in 2019 from the random samples of tested sheep in the reporting countries. The number of MS that did not report any random genotypes is 20, as in 2018, following the changes in the legislation that entered into force in 2018. The eight MS that conducted the genotype to a random sample of sheep were Belgium, Cyprus (where genotyping is conducted systematically in the breeding sheep population), France, Greece, Italy, Latvia, the Netherlands and Poland. One of the six non‐MS reporting country, Iceland, also reported random genotype results. In the subset of EU MS that carried out the activity in 2019, excluding data from Cyprus, 15.7% of the sheep population (with known genotype) is susceptible to CS (NSP3, NSP3O, NSP4 and NSP5), lower than the 19.2% in 2018 and the 26.5% in 2017. This percentage rose to 45.5% in Greece and 29.9% in Italy, two of the countries with the highest caseload in 2019, whereas it was 10.3% in the other five MS.
Considering the past 10 years of random sampling and excluding Cyprus, the proportion of sheep in the resistant genotype group (NSP1; black colour in the bars of Figure 5) shifted from 25.5% of the total number of genotyped sheep in 2010 (in which 24 MS contributed) to 55.7% in 2019 (on which seven MS contributed).
Table 36. Distribution of genotypes of confirmed scrapie cases in sheep in 2019 by reporting country and National Scrapie Plan (NSP) group
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3.3 TSE surveillance in cervids
In 2019, 10,712 cervids were tested for TSE in the EU by 13 MS. Six of them were subject to mandatory surveillance (hereinafter referred to as the MS6) (Section 1.2.4): Estonia, Finland, Latvia, Lithuania, Poland and Sweden increased over 50% the number of cervids tested compared to the previous year from 5,110 to 7,980 (74.5% of the total). The seven MS not subject to mandatory surveillance that contribute to the monitoring were Austria, Hungary, Italy, Romania, Slovenia, Spain and the United Kingdom, reporting 2,732 tested cervids (25.5% of the total). However, Austria, Slovenia and the United Kingdom only reported one, one and two tested cervids, respectively.
Out of the 7,980 tested cervids by the MS6, 5,142 (64.4%) were wild animals, mostly roe deer (2,684), followed by moose (1,502) and red deer (807). Among the 2,838 (35.6%) captive, farmed or semi‐domesticated cervids tested by the MS6, 2,565 (90.4%) were semi‐domesticated reindeer reported by Sweden and Finland.
During the second year of mandatory surveillance in MS6, three cases of CWD in wild moose were reported by Sweden. The number of cervids tested and positive in 2019 by species, management system and reporting country are displayed in Table 38. The description of the CWD cases in 2019 is shown in Table 41.
In terms of testing at PSU11 level, the number and proportion of PSU for wild/semi‐domesticated and farmed/captive cervids declared and tested by the MS6 is shown in Table 39. There is also large variability in the proportion of PSU from which samples have been collected, which is determined by the number of PSU included in the sampling programme. E.g. Estonia sampled 9 (60%) of the ‘PSU (wild)’ and 1 ‘PSU (captive/semi‐domesticated)’ (6.7%). Poland sampled cervids from 100% and 18.8% of the 16 (wild) and 16 (captive/farmed), respectively. Sweden tested cervids from 49 out of the 50 PSU for wild (98%) and 34 of the 160 for farmed and semidomesticated cervids (21.3%). Finland sampled from 42% of the wild PSU and 90.7% of the semi‐domesticated PSU. Latvia declared overall 100 PSU but submitted samples from 180 different PSU codes. Lithuania did not submit PSU codes for the tested cervids. Median, minimum and maximum number of cervids tested in the different types of PSU and countries of the MS6 are also displayed in Table 39.
The most common target group tested by the MS6 was the ‘Hunted/slaughtered fit for human consumption’ (HSHC) that accounted for 4,832 (60.5% of all tested cervids). Within this category, Sweden reported 1,833 semi‐domesticated reindeer tested. All the rest were risk animals in the different target groups: road/predator killed (RK): 1,988 (24.9%); fallen/culled (FC): 973 (12.2%); clinical suspect animals (SUS): 60 (0.75%); hunted/slaughtered not fit for human consumption (HSNHC): 127 (1.6%). There is a large variability between MS6 countries in the proportion of cervids tested in the HSHC, ranging from 3% tested by Finland to 93% by Latvia or 85.9% by Sweden. The numbers of tested cervids by reporting country, management system and target group in 2019 are displayed in Table 40.
Among the non‐MS6, Romania accounted for 77.7% of all tested cervids (2,124), followed by Italy with 551. The majority of the tested cervids were roe deer (79.9%), followed by red deer (16.2%). All tested cervids by the non‐MS6 resulted negative.
Norway continued its intensified testing programme in wild and captive cervids and tested 30,147 animals in 2019, mostly semi‐domesticated reindeer (42.9%), followed by wild moose (19.7%) and red deer (19%) mostly from the hunted/slaughtered fit for human consumption target group, leading to the detection of two cases in moose (Table 41). Iceland also reported 114 reindeer tested in 2019, all negative.
Table 38. Number of cervids tested in the reporting countries in 2019 by management system, species and country(a)
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3.4 Other species
Three MS, Estonia, Finland and Spain reported results on samples tested for TSE in species other than cattle, domestic sheep and goats, and cervids. In total, 122 samples were collected and tested from the following species: raccoon dog (Nyctereutes procyonoides), American mink (Neovison vison), fox (genus Vulpes) and chamois (Rupicapra rupicapra). None of them tested positive (Table 42).
Table 42. Numbers of animals in species other than cattle, sheep, goats and cervids tested for TSE in reporting countries in 2019
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TSE: Transmissible spongiform encephalopathies.
4 Conclusions
As part of the BSE surveillance system in cattle in the EU, over 1.1 million cattle were tested in 2019, 2.7% less than in the previous year. The testing throughput combined with a risk‐based strategy (86.4% of all tests were targeting risk animals) contributed to maximise the sensitivity of the BSE surveillance system considering the EU as a single epidemiological unit. Seven atypical cases of BSE (one L‐BSE and six H‐BSE cases) were confirmed in 2019 by three reporting countries: France (four H‐BSE), Spain (two H‐BSE) and Poland (one L‐BSE). A total 44,557 cattle were tested by four of the six non‐MS reporting countries, with no cases reported. In particular, in the two new reporting countries, Montenegro only tested cattle in the target group slaughter for human consumption and in Serbia, 85.7% cattle were tested in the same target group.
From an epidemiological point of view, the highlights in the BSE caseload of the reporting year are the number of H‐type BSE cases was the largest reported in a single year, equal to that of 2009 (6) (six cases per million tested) and the report by Spain of a H‐type case of 5.5 years, the youngest atypical BSE ever reported since the TSE data are collected and published. An additional H‐BSE case was reported by Brazil.
A total of 481,627 small ruminants were tested in 2019 in the EU, as part of the TSE surveillance system, leading to an overall testing of about 10 million tests since 2002. Twenty‐two MS complied with the EU monitoring requirements in sheep and 23 MS in goats. Compared with 2018, there was a decrease in the detection of the scrapie cases (CS and AS) in non‐infected sheep flocks (from 203 to 177) despite the 3% increase in the level of testing in non‐infected flocks. This may be consistent with a decrease in the overall incidence of the disease. In the same period, there was a similar decrease in the detection of the scrapie cases (CS and AS) in non‐infected goat herds (from 50 to 34), despite the 3.3% increase in the testing in non TSE‐infected herds, due to the low number of index cases reported by Cyprus (8). Out of the two new reporting countries, only Serbia tested small ruminants: 165 sheep slaughtered for human consumption without any case detected.
For CS in sheep, compared with 2018, the increase in the testing activity has resulted in an increase in the EU caseload but not in the proportion of cases per 10,000 tested animals or in the number of index cases, which were reduced again in 2019. Overall, the three countries with the largest number of CS cases, namely Greece, Italy and Spain, have reported more CS cases compared to 2018 but similar number of index cases and increased testing in TSE‐infected flocks, where most of the CS cases are detected. Romania reported 30% fewer number of cases with the same level of testing than in 2018. From a geographical point of view, the disease is reported by a minority of the MS: seven in 2019, same number as in 2018 although some countries differ. As in 2018, only a very small proportion (1.2%) of the CS caseload is from MS other than Greece, Spain, Italy and Romania.
In goats, in total, 390 scrapie cases in goats were reported in the EU in 2019 by nine MS (two more than in 2018), with a 25.4% reduction (–133) compared with 2018 when 523 cases were reported, mainly due to the improved situation in Cyprus and Spain.
When looking at the long‐term trends of CS in terms of cases per 10,000 tests in both species, the situation in 2019 confirmed the 10‐year statistically significant decreasing trend in sheep and no detectable trend in goats, respectively, as estimated through modelling of the available epidemiological data. The disadvantage of the control of the disease in goat herds, due to the lack of both breeding for resistance programmes and genetic‐based culling and restocking of infected herds, may explain to a certain extent this long‐term trend. However, forthcoming amendments in the TSE regulation per Regulation (EU) 772/2020 will allow the management of outbreaks in goat herds with caprine animals carrying at least one of the following alleles: K222, D146 and S146.
For AS in sheep, compared with 2018, the above‐described testing activity resulted in a decrease in the caseload and in the proportion of cases per 10,000 tested animals, a decrease in the number of index cases and the number of MS reporting cases, despite the overall increase in testing. The opposite occurred in goats where the increase in the testing activity compared with 2018 resulted in the increase of the AS caseload, in the proportion of cases per 10,000 tested animals and in the number of index cases. These changes could be due to the annual variability.
With regard to the long‐term trends of AS, there was also a 10‐year statistically significant decreasing trend in sheep and no detectable trend in goats.
The genotyping data collected in 2019 from ovine CS cases consistently confirm the association between the occurrence of the disease and the susceptible genotypes (NSP3, NSP3O, NSP4 or NSP5), with 98.7% of the cases with known genotypes carrying them. The 2019 genotyping data from random samples of the EU sheep population (after excluding Cyprus) did show an improvement compared with the previous years with an average 15.7% of the genotyped sheep carrying genotypes of the susceptible group, from 19.2% in 2018. Some caution is needed in interpreting this result as it could reflect the implementation of the amendment of the TSE Regulation with the subsequent reduction in the number of contributing MS. A CS case in a sheep holding the ARR/ARR genotype (NSP1) was reported by Spain.
The enforcement of the 3‐year surveillance programme for CWD in six MS – Estonia, Finland, Latvia, Lithuania, Poland and Sweden – resulted in 2019 in an increase of over 50% the number of cervids tested compared to the previous year: from 5,110 to the testing of 7,980 cervids and the confirmation of three cases of CWD by Sweden.
The surveillance was complemented by the additional 2,732 cervids tested by other six MS (77.7% of them tested by Romania) with no additional cases detected. The implementation of the mandatory surveillance in the six MS is quite heterogeneous in terms of the design (number and characteristics of the declared PSU), the number of cervids tested in general and per PSU in particular and the distribution of testing by species and target groups. The targeting of mostly hunted/slaughtered fit for human consumption (HSHC) animals observed in the first year of implementation was further increased in 2019 up to 60.5% of all tested cervids, consolidating a situation in which the sensitivity of the surveillance system is lower than expected, following the proposed surveillance system by EFSA in 2017 (EFSA BIOHAZ Panel, 2017b). No conclusions can be drawn about the presence of the disease in other MS and about the prevalence in the affected MS.
Norway continued its intensified testing programme in wild and captive cervids and tested 30,147 cervids in 2019, leading to the detection of two moose cases. At the end of the reporting year and since 2016, Norway had confirmed in total 19 cases in reindeer, 6 in moose and 1 in red deer. Iceland also reported 114 tested cervids in 2019, all negative.
Notes
snip...see full report;
TUESDAY, NOVEMBER 17, 2020
Finland Transmissible Spongiform Encephalopathy TSE was found in a killed moose in Laukas
THURSDAY, OCTOBER 29, 2020
OIE ICELAND, SCRAPIE
FRIDAY, OCTOBER 09, 2020
Norway Regulatory process VKM order for CWD TSE Prion after discovery on the Hardangervidda in 2020
MONDAY, SEPTEMBER 14, 2020
Norway Chronic Wasting Disease (CWD) identified in a wild reindeer at Hardanger Plateau
FRIDAY, SEPTEMBER 11, 2020
Norway Skrantesjuke CWD TSE Prion detected on reindeer buck from Hardangervidda
WEDNESDAY, APRIL 01, 2020
Norway Chronic Wasting Disease CWD TSE Prion Skrantesjuke 2 Positive Moose for 2019
MONDAY, NOVEMBER 18, 2019
Norway Chronic Wasting Disease CWD TSE Prion Detected in Sixth Moose
WEDNESDAY, MARCH 06, 2019
Norway The Madness Continues in Nordfjella Chronic Wasting Disease CWD TSE Prion
THURSDAY, FEBRUARY 14, 2019
Norway Eradication of Chronic Wasting Disease is not completed
WEDNESDAY, MAY 29, 2019
Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures USDA HERE'S YOUR SIGN!
WEDNESDAY, OCTOBER 28, 2020
EFSA Annual report of the Scientific Network on BSE-TSE 2020 Singeltary Submission
2.3.2. New evidence on the zoonotic potential of atypical BSE and atypical scrapie prion strains
2.3.2. New evidence on the zoonotic potential of atypical BSE and atypical scrapie prion strains
Olivier Andreoletti, INRA Research Director, Institut National de la Recherche Agronomique (INRA) – École Nationale Vétérinaire de Toulouse (ENVT), invited speaker, presented the results of two recently published scientific articles of interest, of which he is co-author: ‘Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice’ (MarinMoreno et al., 2020) and ‘The emergence of classical BSE from atypical/Nor98 scrapie’ (Huor et al., 2019).
In the first experimental study, H-type and L-type BSE were inoculated into transgenic mice expressing all three genotypes of the human PRNP at codon 129 and into adapted into ARQ and VRQ transgenic sheep mice. The results showed the alterations of the capacities to cross the human barrier species (mouse model) and emergence of sporadic CJD agents in Hu PrP expressing mice: type 2 sCJD in homozygous TgVal129 VRQ-passaged L-BSE, and type 1 sCJD in homozygous TgVal 129 and TgMet129 VRQ-passaged H-BSE.
SUNDAY, OCTOBER 11, 2020
Bovine adapted transmissible mink encephalopathy is similar to L-BSE after passage through sheep with the VRQ/VRQ genotype but not VRQ/ARQ
THURSDAY, SEPTEMBER 24, 2020
The emergence of classical BSE from atypical/ Nor98 scrapie
FRIDAY, OCTOBER 23, 2020
Scrapie TSE Prion Zoonosis Zoonotic, what if?
Atypical scrapie has been transmitted experimentally to AHQ sheep by the intracranial145 and oral146 routes. An increased risk of atypical scrapie has also been identified in sheep with the AF141RQ haplotype.137 Atypical scrapie does experimentally transmit to sheep with the AL141RQ haplotype but with very long incubation periods without clinical signs.123 Furthermore, sheep with the ARR haplotype, which confers resistance to classical scrapie and is the cornerstone of genotype-based eradication programs, do not appear to be protected against developing atypical scrapie.41,137
Atypical scrapie has also been reported in goats,103,142 where the molecular profile on western blot is similar to atypical scrapie in sheep, but the distribution of lesions within the brain is more rostral (thalamus and midbrain) than atypical scrapie of sheep.142 Similar to sheep with atypical scrapie, histidine substitution at PRNP codon 154 is a risk factor for atypical scrapie in goats,32 and PrPSc has not been demonstrated in the lymphoid tissues of affected goats.142
end...see;
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,?? +Author Affiliations
*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway
***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)
Abstract
Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice.
*** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
OR
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
OR
*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.
OR here;
*** 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.
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.
Fri, Nov 13, 2020 10:50 am
Subject: CWD TSE PRION, SCRAPIE, BSE, AND PORCINE, PIGS, WILD BOAR, ZOONOTIC ZOONOSIS RISK FACTORS AND POTENTIALS
CJD FOUNDATION VIRTUAL CONFERENCE CJD Foundation Research Grant Recipient Reports Panel 2 Nov 3, 2020
zoonotic potential of PMCA-adapted CWD PrP 96SS inoculum
4 different CWD strains, and these 4 strains have different potential to induce any folding of the human prion protein.
***> PIGS, WILD BOAR, CWD <***
***> POPULATIONS OF WILD BOARS IN THE UNITED STATES INCREASING SUPSTANTUALLY AND IN MANY AREAS WE CAN SEE A HIGH DENSITY OF WILD BOARS AND HIGH INCIDENT OF CHRONIC WASTING DISEASE
HYPOTHOSIS AND SPECIFIC AIMS
HYPOTHOSIS
BSE, SCRAPIE, AND CWD, EXPOSED DOMESTIC PIGS ACCUMULATE DIFFERENT QUANTITIES AND STRAINS OF PRIONS IN PERIPHERAL TISSUES, EACH ONE OF THEM WITH PARTICULAR ZOONOTIC POTENTIALS
Final Report – CJD Foundation Grant Program A.
Project Title: Systematic evaluation of the zoonotic potential of different CWD isolates. Principal Investigator: Rodrigo Morales, PhD.
Systematic evaluation of the zoonotic potential of different CWD isolates. Rodrigo Morales, PhD Assistant Professor Protein Misfolding Disorders lab Mitchell Center for Alzheimer’s disease and Related Brain Disorders Department of Neurology University of Texas Health Science Center at Houston Washington DC. July 14th, 2018
Conclusions and Future Directions • We have developed a highly sensitive and specific CWD-PMCA platform to be used as a diagnostic tool. • Current PMCA set up allow us to mimic relevant prion inter-species transmission events. • Polymorphic changes at position 96 of the prion protein apparently alter strain properties and, consequently, the zoonotic potential of CWD isolates. • Inter-species and inter-polymorphic PrPC → PrPSc conversions further increase the spectrum of CWD isolates possibly present in nature. • CWD prions generated in 96SS PrPC substrate apparently have greater inter-species transmission potentials. • Future experiments will explore the zoonotic potential of CWD prions along different adaptation scenarios, including inter-species and inter-polymorphic.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research
Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease
Author item MOORE, SARAH - Orise Fellow item Kunkle, Robert item KONDRU, NAVEEN - Iowa State University item MANNE, SIREESHA - Iowa State University item SMITH, JODI - Iowa State University item KANTHASAMY, ANUMANTHA - Iowa State University item WEST GREENLEE, M - Iowa State University item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 3/15/2017 Publication Date: N/A Citation: N/A Interpretive Summary:
Technical Abstract: Aims: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of cervids. We previously demonstrated that disease-associated prion protein (PrPSc) can be detected in the brain and retina from pigs challenged intracranially or orally with the CWD agent. In that study, neurological signs consistent with prion disease were observed only in one pig: an intracranially challenged pig that was euthanized at 64 months post-challenge. The purpose of this study was to use an antigen-capture immunoassay (EIA) and real-time quaking-induced conversion (QuIC) to determine whether PrPSc is present in lymphoid tissues from pigs challenged with the CWD agent.
Methods: At two months of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 month challenge groups). The remaining pigs (>6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). The retropharyngeal lymph node (RPLN) was screened for the presence of PrPSc by EIA and immunohistochemistry (IHC). The RPLN, palatine tonsil, and mesenteric lymph node (MLN) from 6-7 pigs per challenge group were also tested using EIA and QuIC.
Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).
Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.
Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP
Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.
Interpretive Summary:
Technical Abstract: We have previously shown that the chronic wasting disease (CWD) agent from white-tailed deer can be transmitted to domestic pigs via intracranial or oral inoculation although with low attack rates and restricted PrPSc accumulation. The objective of this study was to assess the potential for cross-species transmission of pig-passaged CWD using bioassay in transgenic mice. Transgenic mice expressing human (Tg40), bovine (TgBovXV) or porcine (Tg002) PRNP were inoculated intracranially with 1% brain homogenate from a pig that had been intracranially inoculated with a pool of CWD from white-tailed deer. This pig developed neurological clinical signs, was euthanized at 64 months post-inoculation, and PrPSc was detected in the brain. Mice were monitored daily for clinical signs of disease until the end of the study. Mice were considered positive if PrPSc was detected in the brain using an enzyme immunoassay (EIA). In transgenic mice expressing porcine prion protein the average incubation period was 167 days post-inoculation (dpi) and 3/27 mice were EIA positive (attack rate = 11%). All 3 mice were found dead and clinical signs were not noted prior to death. One transgenic mouse expressing bovine prion protein was euthanized due to excessive scratching at 617 dpi and 2 mice culled at the end of the study at 700 dpi were EIA positive resulting in an overall attack rate of 3/16 (19%). None of the transgenic mice expressing human prion protein that died or were euthanized up to 769 dpi were EIA positive and at study end point at 800 dpi 2 mice had positive EIA results (overall attack rate = 2/20 = 10%). The EIA optical density (OD) readings for all positive mice were at the lower end of the reference range (positive mice range, OD = 0.266-0.438; test positive reference range, OD = 0.250-4.000). To the authors’ knowledge, cervid-derived CWD isolates have not been successfully transmitted to transgenic mice expressing human prion protein. The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.
cwd scrapie pigs oral routes
***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***
>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***
***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).
***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.
Experimental transmission of the chronic wasting disease agent to swine after oral or intracranial inoculation
S. Jo Moore1,2, M. Heather West Greenlee3, Naveen Kondru3, Sireesha Manne3, Jodi D. Smith1, Robert A. Kunkle1, Anumantha Kanthasamy3 and Justin J. Greenlee1* + Author Affiliations
1Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, Ames, Iowa, United States of America 2Oak Ridge Institute for Science and Education, Oak Ridge, Tennessee, United States of America 3Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, Iowa, United States of America
ABSTRACT
Chronic wasting disease (CWD) is a naturally occurring, fatal neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Crossbred piglets were assigned to one of three groups: intracranially inoculated (n=20), orally inoculated (n=19), or non-inoculated (n=9). At approximately the age at which commercial pigs reach market weight, half of the pigs in each group were culled (‘market weight’ groups). The remaining pigs (‘aged’ groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by western blotting (WB), antigen-capture immunoassay (EIA), immunohistochemistry (IHC) and in vitro real-time quaking induced conversion (RT-QuIC). Brain samples from selected pigs were also bioassayed in mice expressing porcine prion protein. Four intracranially inoculated aged pigs and one orally inoculated aged pig were positive by EIA, IHC and/or WB. Using RT-QuIC, PrPSc was detected in lymphoid and/or brain tissue from one or more pigs in each inoculated group. Bioassay was positive in 4 out of 5 pigs assayed. This study demonstrates that pigs can support low-level amplification of CWD prions, although the species barrier to CWD infection is relatively high. However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.
IMPORTANCE We challenged domestic swine with the chronic wasting disease agent by inoculation directly into the brain (intracranially) or by oral gavage (orally). Disease-associated prion protein (PrPSc) was detected in brain and lymphoid tissues from intracranially and orally inoculated pigs as early as 8 months of age (6 months post-inoculation). Only one pig developed clinical neurologic signs suggestive of prion disease. The amount of PrPSc in the brains and lymphoid tissues of positive pigs was small, especially in orally inoculated pigs. Regardless, positive results in orally inoculated pigs suggest that it may be possible for swine to serve as a reservoir for prion disease under natural conditions.
FOOTNOTES
↵*Corresponding author: Email: justin.greenlee@ars.usda.gov This is a work of the U.S. Government and is not subject to copyright protection in the United States. Foreign copyrights may apply.
----Original Message-----
From: Terry Singeltary
To: Tracy.A.Nichols
Sent: Fri, Mar 30, 2018 12:51 pm
Subject: Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification Program Standards Singeltary Submission March 30, 2018
Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification Program Standards Singeltary Submission March 30, 2018
Greetings APHIS, USDA, Dr. Tracy Nichols, et al,
I wish to kindly submit my comments on the Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification Program Standards please. i have submitted online and sent a hard copy to Dr. Nichols via email. i know that my concern may not be the same concern as others, but ramifications from cwd tse prion can be long lasting, and science is still emerging. however, the science today warrants immediate and further actions be taken. my comments, with reference materials, are as follows, and will be formatted in such a way, i will address issues by numbers 1-10, and under each one of my comments by each number, i will reference my comments with science to back up what i am stating/asking...thank you kindly, terry
snip...see full text;
WEDNESDAY, NOVEMBER 4, 2020
CWD TSE PRION, SCRAPIE, BSE, AND PORCINE, PIGS, WILD BOAR, ZOONOTIC ZOONOSIS RISK FACTORS AND POTENTIALS
MONDAY, NOVEMBER 16, 2020
North America coyotes or pumas can serve as a vehicle for prions contributing to the spread of the infectious agent in the environment
SUNDAY, OCTOBER 4, 2020
Cattle Meat and Offal Imported from the United States of America, Canada and Ireland to Japan (Prions) Food Safety Commission of Japan
TUESDAY, SEPTEMBER 29, 2020
ISO's Updated 22442 Animal Tissue Standards — What Changed? TSE Prion!
''Why is USDA "only" testing 25,000 samples a year?
USDA's surveillance strategy is to focus on the targeted populations where we are most likely to find disease if it is present. This is the most effective way to meet both OIE and our domestic surveillance standards. After completing our enhanced surveillance in 2006 and confirming that our BSE prevalence was very low, an evaluation of the program showed that reducing the number of samples collected to 40,000 samples per year from these targeted, high risk populations would allow us to continue to exceed these standards. In fact, the sampling was ten times greater than OIE standards. A subsequent evaluation of the program in 2016 using data collected over the past 10 years showed that the surveillance standards could still be met with a further reduction in the number of samples collected by renderers and 3D/4D establishments which have a very low OIE point value because the medical history of these animals is usually unknown. Therefore, in 2016, the number of samples to be tested was reduced to 25,000 where it remains today.''
Introduction
USDA conducts surveillance for Bovine spongiform encephalopathy (BSE), referred to as "mad cow disease", in cattle to determine if, and at what level, the disease is present in the U.S. cattle population. Our surveillance program allows us to assess any change in the BSE disease status of U.S. cattle, and identify any rise in BSE prevalence in this country. Identifying any changes in the prevalence of disease allows us to match our preventive measures - feed ban for animal health, and specified risk material removal for public health - to the level of disease in U.S. cattle.
It is the longstanding system of interlocking safeguards, including the removal of specified risk materials - or the parts of an animal that would contain BSE - at slaughter and the FDA's ruminant-to-ruminant feed ban that protect public and animal health from BSE.
It is the longstanding system of interlocking safeguards, including the removal of specified risk materials - or the parts of an animal that would contain BSE - at slaughter and the FDA's ruminant-to-ruminant feed ban that protect public and animal health from BSE.
Why did USDA decrease the number of samples per year in 2006?
After the first confirmation of BSE in an animal in Washington State in December 2003, USDA evaluated its BSE surveillance efforts in light of that finding. We determined that we needed to immediately conduct a major surveillance effort to help determine the prevalence of BSE in the United States. Our goal over a 12-18 month period was to obtain as many samples as possible from the segments of the cattle population where we were most likely to find BSE if it was present. This population of cattle was exhibiting some signs of disease. We conducted this enhanced surveillance effort from June 2004 - August 2006. In that time, we collected a total of 787,711 samples and estimated the prevalence of BSE in the United States to be between 4-7 infected animals in a population of 42 million adult cattle. We consequently modified our surveillance efforts based on this prevalence estimate to a level we can monitor for any potential changes, should they occur. Our statistical analysis indicated that collecting approximately 40,000 samples per year from the targeted cattle population would enable us to conduct this monitoring.
Why is USDA "only" testing 25,000 samples a year?
USDA's surveillance strategy is to focus on the targeted populations where we are most likely to find disease if it is present. This is the most effective way to meet both OIE and our domestic surveillance standards. After completing our enhanced surveillance in 2006 and confirming that our BSE prevalence was very low, an evaluation of the program showed that reducing the number of samples collected to 40,000 samples per year from these targeted, high risk populations would allow us to continue to exceed these standards. In fact, the sampling was ten times greater than OIE standards. A subsequent evaluation of the program in 2016 using data collected over the past 10 years showed that the surveillance standards could still be met with a further reduction in the number of samples collected by renderers and 3D/4D establishments which have a very low OIE point value because the medical history of these animals is usually unknown. Therefore, in 2016, the number of samples to be tested was reduced to 25,000 where it remains today.
How can USDA find every case of BSE in the United States when you are only testing 25,000 animals?
The goal of our BSE surveillance program, even under the enhanced program, has never been to detect every case of BSE. Our goal is determine whether the disease exists at very low levels in the U.S. cattle population, and we do this by testing those animals most likely to have BSE. It is the longstanding system of interlocking safeguards, including the removal of specified risk materials - or the parts of an animal that would contain BSE - at slaughter and the FDA's ruminant-to- ruminant feed ban that protect public and animal health from BSE.
Why didn’t USDA continue to test animals at the enhanced surveillance level?
USDA's 2004-2006 enhanced surveillance program was initiated in response to the first detection of BSE in the United States and was designed to detect the overall prevalence of the disease in this country. This required a very intensive effort and it allowed us to estimate extremely low prevalence levels of disease. Once that prevalence level was determined, USDA modified its testing levels to monitor any changes in the level of disease. Our current testing of approximately 25,000 targeted animals a year allows USDA to detect BSE at the very low level of less than 1 case per million adult cattle, assess any change in the BSE status of U.S. cattle, and identify any rise in BSE prevalence in this country.
Is USDA's surveillance program a food safety or public health measure?
The primary, and most effective, food safety or public health measure is the removal of specified risk materials (SRMs) - or the parts of an animal that would contain BSE - from every animal at slaughter. USDA's BSE surveillance program is not a food safety measure; it is an animal health monitoring measure. However, it does support existing public health and food safety measures. By allowing us to monitor the level of disease in the US cattle population, we can help determine the appropriate level of public health and animal health measures required, and whether they should be increased or decreased.
Why doesn't USDA test every animal at slaughter?
There is currently no test to detect the disease in a live animal. BSE is confirmed by taking samples from the brain of an animal and testing to see if the infectious agent - the abnormal form of the prion protein - is present. The earliest point at which current tests can accurately detect BSE is 2 to 3 months before the animal begins to show symptoms, and the time between initial infection and the appearance of symptoms is about 5 years. Therefore, there is a long period of time during which current tests would not be able to detect the disease in an infected animal.
Since most cattle are slaughtered in the United States at a young age, they are in that period where tests would not be able to detect the disease if present. Testing all slaughter cattle for BSE could produce an exceedingly high rate of false negative test results and offer misleading assurances of the presence or absence of disease.
Simply put, the most effective way to detect BSE is not to test all animals, which could lead to false security, but to test those animals most likely to have the disease, which is the basis of USDA's current program.
Since most cattle are slaughtered in the United States at a young age, they are in that period where tests would not be able to detect the disease if present. Testing all slaughter cattle for BSE could produce an exceedingly high rate of false negative test results and offer misleading assurances of the presence or absence of disease.
Simply put, the most effective way to detect BSE is not to test all animals, which could lead to false security, but to test those animals most likely to have the disease, which is the basis of USDA's current program.
What animals are USDA testing in the surveillance program? These are random samples at slaughter, aren't they?
No. USDA's BSE surveillance program is specifically targeted to the population most likely to have the disease, if it is present. This population is NOT clinically healthy animals that would be presented for slaughter. Rather, it includes animals that have some type of abnormality, such as central nervous system signs; non-ambulatory, or a "downer"; emaciated; or died for unknown reasons. Because these animals would not pass the required ante-mortem inspection requirements at slaughter for human consumption, we collect the majority of our samples at facilities other than slaughter facilities - at rendering or salvage facilities, on-farm, at veterinary clinics or veterinary diagnostic laboratories. With this targeted approach, we can monitor the presence of disease in the US cattle population in a much more efficient and meaningful way. The key to surveillance is to look where the disease is going to occur.
Key Points: BSE Ongoing Surveillance Plan
Note: This Fact Sheet is based on the USDA Animal and Plant Health Inspection Service (APHIS) Bovine Spongiform Encephalopathy (BSE) Ongoing Surveillance Plan, July 20, 2006. To learn more, read the complete BSE Ongoing Surveillance Plan (PDF, 187 KB).
KEY POINTS
In addition to a stringent feed ban imposed by the Food and Drug Administration in 1997 as well as the removal of all specified risk material which could harbor BSE, USDA has a strong surveillance program in place to detect signs of BSE in cattle in the United States. In fact, we test for BSE at levels greater than World Animal Health Organization standards. The program samples approximately 25,000 animals each year and targets cattle populations where the disease is most likely to be found. The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including emaciation or injury, and dead cattle, as well as non-ambulatory animals. Samples from the targeted population are taken at farms, veterinary diagnostic laboratories, public health laboratories, slaughter facilities, veterinary clinics, and livestock markets.
USDA's National Veterinary Services Laboratories (NVSL) in Ames, IA, along with contracted veterinary diagnostic laboratories, use rapid screening tests as the initial screening method on all samples. Any inconclusive samples undergo further testing and analysis at NVSL.
NOT A FOOD SAFETY TEST
BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.
The BSE surveillance program is not for the purposes of determining food safety. Rather, it is an animal health surveillance program. USDA's BSE surveillance program allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population and provides assurances to consumers and our international trading partners that the interlocking system of safeguards in place to prevent BSE are working..
The safety of the U.S. food supply from BSE is assured by the removal of specified risk materials - those tissues known to be infective in an affected animal - from all human food. These requirements have been in place since 2004.
The BSE surveillance program is not for the purposes of determining food safety. Rather, it is an animal health surveillance program. USDA's BSE surveillance program allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population and provides assurances to consumers and our international trading partners that the interlocking system of safeguards in place to prevent BSE are working..
The safety of the U.S. food supply from BSE is assured by the removal of specified risk materials - those tissues known to be infective in an affected animal - from all human food. These requirements have been in place since 2004.
ONGOING BSE SURVEILLANCE PROGRAM SUMMARY
USDA's BSE surveillance program samples approximately 25,000 animals each year and targets cattle populations where the disease is most likely to be found. The statistically valid surveillance level of 25,000 is consistent with science-based internationally accepted standards. This level allows USDA to detect BSE at the very low level of less than 1 case per million adult cattle, assess any change in the BSE status of U.S. cattle, and identify any rise in BSE prevalence in this country.
The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including emaciation or injury, and dead cattle, as well as nonambulatory animals. Samples from the targeted population are taken at farms, veterinary diagnostic laboratories, public health laboratories, slaughter facilities, veterinary clinics, and livestock markets.
USDA's National Veterinary Services Laboratories (NVSL) in Ames, IA, along with contracted veterinary diagnostic laboratories, will continue to use rapid screening tests as the initial screening method on all samples. Any inconclusive samples will be sent to NVSL for further testing and analysis. USDA's surveillance program uses OIE's weighted surveillance points system, which was adopted in May 2005 and reflects international scientific consensus that the best BSE surveillance programs focus on obtaining quality samples from targeted subpopulations rather than looking at the entire adult cattle population.
The number of points a sample receives correlates directly to an animal's clinical presentation at the time of sampling. The highest point values are assigned to those samples from animals with classic clinical signs of the disease. The lowest point values correspond to clinically normal animals tested at routine slaughter.
The goal of this weighted approach is to ensure that countries sample those cattle populations where the disease is most likely to be found. This system is not different from USDA's previous BSE surveillance approach, it is simply a different method for evaluating surveillance programs. Both approaches target those cattle populations where BSE is most likely to be found. The OIE is simply assigning point values to different categories of animals.
USDA has been targeting these subpopulations since BSE surveillance was initiated in 1990, and will continue to do so under the OIE weighted approach. Under the OIE guidelines, points compiled over a period of 7 consecutive years are used as evidence of adequate surveillance. At the current ongoing level of surveillance, the United States will far exceed OIE guidelines under the point system.
The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including emaciation or injury, and dead cattle, as well as nonambulatory animals. Samples from the targeted population are taken at farms, veterinary diagnostic laboratories, public health laboratories, slaughter facilities, veterinary clinics, and livestock markets.
USDA's National Veterinary Services Laboratories (NVSL) in Ames, IA, along with contracted veterinary diagnostic laboratories, will continue to use rapid screening tests as the initial screening method on all samples. Any inconclusive samples will be sent to NVSL for further testing and analysis. USDA's surveillance program uses OIE's weighted surveillance points system, which was adopted in May 2005 and reflects international scientific consensus that the best BSE surveillance programs focus on obtaining quality samples from targeted subpopulations rather than looking at the entire adult cattle population.
The number of points a sample receives correlates directly to an animal's clinical presentation at the time of sampling. The highest point values are assigned to those samples from animals with classic clinical signs of the disease. The lowest point values correspond to clinically normal animals tested at routine slaughter.
The goal of this weighted approach is to ensure that countries sample those cattle populations where the disease is most likely to be found. This system is not different from USDA's previous BSE surveillance approach, it is simply a different method for evaluating surveillance programs. Both approaches target those cattle populations where BSE is most likely to be found. The OIE is simply assigning point values to different categories of animals.
USDA has been targeting these subpopulations since BSE surveillance was initiated in 1990, and will continue to do so under the OIE weighted approach. Under the OIE guidelines, points compiled over a period of 7 consecutive years are used as evidence of adequate surveillance. At the current ongoing level of surveillance, the United States will far exceed OIE guidelines under the point system.
Current Monthly Test Results
APHIS reports ongoing surveillance test totals monthly.
Collection Month | FY2020 | Number of Valid Samples | OIE Points |
|---|---|---|---|
| October | 2019 | 1,152 | 23,238 |
| November | 2019 | 1,569 | 36,255 |
| December | 2019 | 1,531 | 19,100 |
| January | 2020 | 2,340 | 29,204 |
| February | 2020 | 3,293 | 47,333 |
| March | 2020 | 3,115 | 27,953 |
| April | 2020 | 2,194 | 13,334 |
| May | 2020 | 1,660 | 21,163 |
| June | 2020 | 1,294 | 25,063 |
| July | 2020 | 128 | 5,966 |
Total as of July 1, 2020 | 18,276 | 248,609 | |
Beginning in Fiscal Year 2016, the BSE ongoing surveillance program will sample approximately 25,000 animals each year. Under the program, USDA will continue to collect samples from a variety of sites and from the cattle populations where the disease is most likely to be detected.
***> P.108: Successful oral challenge of adult cattle with classical BSE
Sandor Dudas1,*, Kristina Santiago-Mateo1, Tammy Pickles1, Catherine Graham2, and Stefanie Czub1 1Canadian Food Inspection Agency; NCAD Lethbridge; Lethbridge, Alberta, Canada; 2Nova Scotia Department of Agriculture; Pathology Laboratory; Truro, Nova Scotia, Canada
Classical Bovine spongiform encephalopathy (C-type BSE) is a feed- and food-borne fatal neurological disease which can be orally transmitted to cattle and humans. Due to the presence of contaminated milk replacer, it is generally assumed that cattle become infected early in life as calves and then succumb to disease as adults. Here we challenged three 14 months old cattle per-orally with 100 grams of C-type BSE brain to investigate age-related susceptibility or resistance. During incubation, the animals were sampled monthly for blood and feces and subjected to standardized testing to identify changes related to neurological disease. At 53 months post exposure, progressive signs of central nervous system disease were observed in these 3 animals, and they were euthanized. Two of the C-BSE animals tested strongly positive using standard BSE rapid tests, however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.. Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.
***Our study demonstrates susceptibility of adult cattle to oral transmission of classical BSE.
We are further examining explanations for the unusual disease presentation in the third challenged animal.
P98 The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge
Greenlee JJ (1), Moore SJ (1), and West Greenlee MH (2) (1) United States Department of Agriculture, Agricultural Research Service, National Animal Disease Center, Virus and Prion Research Unit, Ames, IA, United States (2) Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, United States.
In 2006, a case of H-type bovine spongiform encephalopathy (BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K).
The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease.
Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route.
The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure.
Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with either H-type BSE from the 2004 US Htype BSE case (n=3) or from the 2006 US H-type case associated with the E211K polymorphism (n=4) using 10% w/v brain homogenates.
Cattle were observed daily throughout the course of the experiment for the development of clinical signs.
At approximately 50 months post-inoculation, one steer (EK211 inoculated with E211K associated H-BSE) developed clinical signs including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and was euthanized.
Enzyme immunoassay confirmed that abundant misfolded protein was present in the brainstem, and immunohistochemistry demonstrated PrPSc throughout the brain.
Western blot analysis of brain tissue from the clinically affected steer was consistent with the E211K H-type BSE inoculum.
With the experiment currently at 55 months post-inoculation, no other cattle in this study have developed clinical signs suggestive of prion disease. This study demonstrates that the H-type BSE agent is transmissible by the oronasal route.
These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
WEDNESDAY, AUGUST 15, 2018
***> The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge
MONDAY, JANUARY 09, 2017
Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle
CDC Volume 23, Number 2—February 2017
*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
Detection of PrPBSE and prion infectivity in the ileal Peyer’s patch of young calves as early as 2 months after oral challenge with classical bovine spongiform encephalopathy
Ivett Ackermann1 , Anne Balkema‑Buschmann1 , Reiner Ulrich2 , Kerstin Tauscher2 , James C. Shawulu1 , Markus Keller1 , Olanrewaju I. Fatola1 , Paul Brown3 and Martin H. Groschup1*
Abstract
In classical bovine spongiform encephalopathy (C-BSE), an orally acquired prion disease of cattle, the ileal Peyer’s patch (IPP) represents the main entry port for the BSE agent. In earlier C-BSE pathogenesis studies, cattle at 4–6 months of age were orally challenged, while there are strong indications that the risk of infection is highest in young animals. In the present study, unweaned calves aged 4–6 weeks were orally challenged to determine the earli‑ est time point at which newly formed PrPBSE and BSE infectivity are detectable in the IPP. For this purpose, calves were culled 1 week as well as 2, 4, 6 and 8 months post-infection (mpi) and IPPs were examined for BSE infectivity using a bovine PrP transgenic mouse bioassay, and for PrPBSE by immunohistochemistry (IHC) and protein misfolding cyclic amplifcation (PMCA) assays. For the frst time, BSE prions were detected in the IPP as early as 2 mpi by transgenic mouse bioassay and PMCA and 4 mpi by IHC in the follicular dendritic cells (FDCs) of the IPP follicles. These data indi‑ cate that BSE prions propagate in the IPP of unweaned calves within 2 months of oral uptake of the agent.
In summary, our study demonstrates for the frst time PrPBSE (by PMCA) and prion infectivity (by mouse bioassay) in the ileal Peyer’s patch (IPP) of young calves as early as 2 months after infection. From 4 mpi nearly all calves showed PrPBSE positive IPP follicles (by IHC), even with PrPBSE accumulation detectable in FDCs in some animals. Finally, our results confrm the IPP as the early port of entry for the BSE agent and a site of initial propagation of PrPBSE and infectivity during the early pathogenesis of the disease. Terefore, our study supports the recommendation to remove the last four metres of the small intestine (distal ileum) at slaughter, as designated by current legal requirements for countries with a controlled BSE risk status, as an essential measure for consumer and public health protection.
PLOS ONE Journal
IBNC Tauopathy or TSE Prion disease, it appears, no one is sure
Terry S. Singeltary Sr., 03 Jul 2015 at 16:53 GMT
***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.
***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.
*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***
http://www.plosone.org/annotation/listThread.action?root=86610
***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.
*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***
http://www.plosone.org/annotation/listThread.action?root=86610
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
P98 The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge
Greenlee JJ (1), Moore SJ (1), and West Greenlee MH (2) (1) United States Department of Agriculture, Agricultural Research Service, National Animal Disease Center, Virus and Prion Research Unit, Ames, IA, United States (2) Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, United States.
reading up on this study from Prion 2018 Conference, very important findings ;
***> This study demonstrates that the H-type BSE agent is transmissible by the oronasal route.
***> These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
PRION 2018 CONFERENCE ABSTRACT
WEDNESDAY, OCTOBER 24, 2018
Experimental Infection of Cattle With a Novel Prion Derived From Atypical H-Type Bovine Spongiform Encephalopathy
let's take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow.
This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$
ALABAMA MAD COW g-h-BSEalabama
In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine–human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks.
Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA
NATURE|Vol 457|26 February 2009
> Epidemiological investigations conducted by USDA personnel failed to reveal any evidence of a feed source contaminated with TSE material fed to this animal
LMAO!
BANNED MAD COW FEED IN COMMERCE IN ALABAMA
Date: September 6, 2006 at 7:58 am PST PRODUCT
a) EVSRC Custom dairy feed, Recall # V-130-6;
b) Performance Chick Starter, Recall # V-131-6;
c) Performance Quail Grower, Recall # V-132-6;
d) Performance Pheasant Finisher, Recall # V-133-6.
CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.
REASON
Dairy and poultry feeds were possibly contaminated with ruminant based protein.
VOLUME OF PRODUCT IN COMMERCE 477.72 tons
DISTRIBUTION AL
______________________________
PRODUCT Bulk custom dairy pre-mixes,
Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete. REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.
VOLUME OF PRODUCT IN COMMERCE 350 tons
DISTRIBUTION AL and MS
______________________________
PRODUCT
a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb.. bags, Recall # V-121-6;
b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags, Recall # V-122-6;
c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall # V-123-6;
d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6;
e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6;
f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6;
g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6
CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006. Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing.
REASON Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags
DISTRIBUTION AL, GA, MS, and TN
END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006
###
Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS Products manufactured from 02/01/2005 until 06/06/2006
Date: August 6, 2006 at 6:16 pm PST PRODUCT
a) CO-OP 32% Sinking Catfish, Recall # V-100-6;
b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6;
c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;
d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6;
g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6;
h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6;
i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6;
j) CO-OP LAYING CRUMBLES, Recall # V-109-6;
k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6;
l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;
m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE
Product manufactured from 02/01/2005 until 06/06/2006
RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.
REASON Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE 125 tons
DISTRIBUTION AL and FL
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II
______________________________
PRODUCT
a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6;
b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6;
c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6;
d) Feather Meal, Recall # V-082-6 CODE
a) Bulk
b) None
c) Bulk
d) Bulk
RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.
REASON
Possible contamination of animal feeds with ruminent derived meat and bone meal.
VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons
DISTRIBUTION Nationwide
END OF ENFORCEMENT REPORT FOR July 12, 2006
###
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II
___________________________________
PRODUCT
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007
CODE
Cattle feed delivered between 01/12/2007 and 01/26/2007
RECALLING FIRM/MANUFACTURER
Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing.
REASON
Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE
42,090 lbs.
DISTRIBUTION
WI
___________________________________
PRODUCT
Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007
CODE
The firm does not utilize a code - only shipping documentation with commodity and weights identified.
RECALLING FIRM/MANUFACTURER
Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.
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.
VOLUME OF PRODUCT IN COMMERCE
9,997,976 lbs.
DISTRIBUTION
ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
***> Wednesday, January 23, 2019
***> CFIA SFCR Guidance on Specified risk material (SRM) came into force on January 15, 2019 <***
In the USA, USDA et al sometimes serves SRM’s up as appetizers or horderves.
Thursday, November 28, 2013
Department of Justice Former Suppliers of Beef to National School Lunch Program Settle Allegations of Improper Practices and Mistreating Cows
seems USDA NSLP et al thought that it would be alright, to feed our children all across the USA, via the NSLP, DEAD STOCK DOWNER COWS, the most high risk cattle for mad cow type disease, and other dangerous pathogens, and they did this for 4 years, that was documented, then hid what they did by having a recall, one of the largest recalls ever, and they made this recall and masked the reason for the recall due to animal abuse (I do not condone animal abuse), not for the reason of the potential for these animals to have mad cow BSE type disease (or other dangerous and deadly pathogens). these TSE prion disease can lay dormant for 5, 10, 20 years, or longer, WHO WILL WATCH OUR CHILDREN FOR THE NEXT 5 DECADES FOR CJD ???
Saturday, September 21, 2013
Westland/Hallmark: 2008 Beef Recall A Case Study by The Food Industry Center January 2010 THE FLIM-FLAM REPORT
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ???
this recall was not for the welfare of the animals. ...tss you can check and see here ; (link now dead, does not work...tss)
try this link ;
Sunday, November 13, 2011
*** California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock
Wednesday, March 2, 2016
RANCHO He did not know that they were placing healthy cow heads next to suspect carcasses BSE TSE Prion
Sunday, June 14, 2015
Larry’s Custom Meats Inc. Recalls Beef Tongue Products That May Contain Specified Risk Materials BSE TSE Prion
Thursday, June 12, 2014
Missouri Firm Recalls Ribeye and Carcass Products That May Contain Specified Risk Materials 4,012 pounds of fresh beef products because the dorsal root ganglia may not have been completely removed
Saturday, November 10, 2012
Wisconsin Firm Recalls Beef Tongues That May Contain Specified Risk Materials Nov 9, 2012 WI Firm Recalls Beef Tongues
Saturday, July 23, 2011
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE
Sunday, October 18, 2009
Wisconsin Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, October 17, 2009
Thursday, October 15, 2009
Nebraska Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, Oct 15, 2009
Thursday, June 26, 2008
Texas Firm Recalls Cattle Heads That Contain Prohibited Materials
Tuesday, July 1, 2008
Missouri Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs
Friday, August 8, 2008
Texas Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs 941,271 pounds with tonsils not completely removed
Saturday, April 5, 2008
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS
Wednesday, April 30, 2008
Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings
Wednesday, April 30, 2008
Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings
Friday, October 15, 2010
BSE infectivity in the absence of detectable PrPSc accumulation in the tongue and nasal mucosa of terminally diseased cattle
SPECIFIED RISK MATERIALS SRMs
USDA BSE TSE PRION SURVEILLANCE, FEED, TESTING, SRM FIREWALLS...LMAO!
THE USDA FDA TRIPLE MAD COW DISEASE FIREWALL, WERE NOTHING MORE THAN INK ON PAPER !
infamous august 4, 1997 BSE TSE prion mad cow feed ban, part of usda fda et al TRIPLE MAD COW FIREWALL, 10 YEARS AFTER ;
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II PRODUCT Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007 CODE Cattle feed delivered between 01/12/2007 and 01/26/2007 RECALLING FIRM/MANUFACTURER Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing. REASON Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI
___________________________________
PRODUCT Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007 CODE The firm does not utilize a code - only shipping documentation with commodity and weights identified. RECALLING FIRM/MANUFACTURER Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007.
Firm initiated recall is complete. 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.
VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
16 years post mad cow feed ban August 1997
2013
Sunday, December 15, 2013
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
17 years post mad cow feed ban August 1997
Tuesday, December 23, 2014
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION
*** Monday, October 26, 2015 ***
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE October 2015 ***
Thursday, July 24, 2014
*** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigations
SEE MORE;
Tuesday, September 10, 2019
THURSDAY, JULY 20, 2017
USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200
WEDNESDAY, APRIL 24, 2019
***> USDA Announces Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019 <***
MONDAY, JANUARY 09, 2017
Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle
CDC Volume 23, Number 2—February 2017
*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
TUESDAY, AUGUST 28, 2018
USDA finds BSE infection in Florida cow 08/28/18 6:43 PM
http://animalhealthreportpriontse..blogspot.com/2018/08/usda-finds-bse-infection-in-florida-cow.html
WEDNESDAY, AUGUST 29, 2018
USDA Announces Atypical Bovine Spongiform Encephalopathy Detection USDA 08/29/2018 10:00 AM EDT
WEDNESDAY, AUGUST 29, 2018
Transmissible Spongiform Encephalopathy TSE Prion Atypical BSE Confirmed Florida Update USA August 28, 2018
H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism:
clinical and pathologic features in wild-type and E211K cattle following intracranial inoculation
H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism: clinical and pathologic features in wild-type and E211K cattle following intracranial inoculation
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism: clinical and pathologic features in wild-type and E211K cattle following intracranial inoculation
Authors
item Moore, Sarah - item West Greenlee, Mary - item Smith, Jodi item Nicholson, Eric item Vrentas, Catherine item Greenlee, Justin
Submitted to: Prion
Publication Type: Abstract Only
Publication Acceptance Date: August 12, 2015
Publication Date: May 25, 2015
Citation: Moore, S.J., West Greenlee, M.H., Smith, J., Nicholson, E., Vrentas, C., Greenlee, J. 2015. H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism: clinical and pathologic features in wild-type and E211K cattle following intracranial inoculation. Prion 2015. p. S5.
Technical Abstract: In 2006 an H-type bovine spongiform encephalopathy (BSE) case was reported in an animal with an unusual polymorphism (E211K) in the prion protein gene. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele are predisposed to rapid onset of H-type BSE when exposed. The purpose of this study was to investigate the phenotype of this BSE strain in wild-type (E211E) and E211K heterozygous cattle. One calf carrying the wild-type allele and one E211K calf were inoculated intracranially with H-type BSE brain homogenate from the US 2006 case that also carried one K211 allelle. In addition, one wild-type calf and one E211K calf were inoculated intracranially with brain homogenate from a US 2003 classical BSE case. All animals succumbed to clinical disease. Survival times for E211K H-type BSE inoculated catttle (10 and 18 months) were shorter than the classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Animals challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K H-type BSE and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K H-type BSE remains stable when transmitted to cattle without the E211K polymorphism, and exhibits a number of features that differ from classical BSE in both wild-type and E211K cattle.
*** All animals succumbed to clinical disease. Survival times for E211K H-type BSE inoculated catttle (10 and 18 months) were shorter than the classical BSE inoculated cattle (both 26 months). ***
-------- Original Message --------
Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD
Date: Thu, 28 Nov 2002 10:23:43 -0000
From: "Asante, Emmanuel A" e.asante@ic.ac.uk
To: "'flounder@wt.net'" flounder@wt.net
Dear Terry,
I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention.
Thank you for your interest in the paper.
In respect of your first question, the simple answer is, ***yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any further sub-classification in respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc.
I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes..
Emmanuel Asante
< >
____________________________________
Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac..uk (until 9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now)
_________end...TSS___________________
***Our study demonstrates susceptibility of adult cattle to oral transmission of classical BSE. ***
***our findings suggest that possible transmission risk of H-type BSE to sheep and human. ***
P.86: Estimating the risk of transmission of BSE and scrapie to ruminants and humans by protein misfolding cyclic amplification
Morikazu Imamura, Naoko Tabeta, Yoshifumi Iwamaru, and Yuichi Murayama National Institute of Animal Health; Tsukuba, Japan
To assess the risk of the transmission of ruminant prions to ruminants and humans at the molecular level, we investigated the ability of abnormal prion protein (PrPSc) of typical and atypical BSEs (L-type and H-type) and typical scrapie to convert normal prion protein (PrPC) from bovine, ovine, and human to proteinase K-resistant PrPSc-like form (PrPres) using serial protein misfolding cyclic amplification (PMCA).
Six rounds of serial PMCA was performed using 10% brain homogenates from transgenic mice expressing bovine, ovine or human PrPC in combination with PrPSc seed from typical and atypical BSE- or typical scrapie-infected brain homogenates from native host species. In the conventional PMCA, the conversion of PrPC to PrPres was observed only when the species of PrPC source and PrPSc seed matched. However, in the PMCA with supplements (digitonin, synthetic polyA and heparin), both bovine and ovine PrPC were converted by PrPSc from all tested prion strains. On the other hand, human PrPC was converted by PrPSc from typical and H-type BSE in this PMCA condition.
Although these results were not compatible with the previous reports describing the lack of transmissibility of H-type BSE to ovine and human transgenic mice, ***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.
================
Full text Singeltary et al PLOS
THURSDAY, JULY 20, 2017
USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200
LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow.
This new prionopathy in humans?
the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......
wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow,
WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ???
there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$
ALABAMA MAD COW g-h-BSEalabama
In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America.
This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD.
This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene.
We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail:maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
THURSDAY, SEPTEMBER 26, 2019
Veterinary Biologics Guideline 3.32E: Guideline for minimising the risk of introducing transmissible spongiform encephalopathy prions and other infectious agents through veterinary biologics
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001
Date: Tue, 9 Jan 2001 16:49:00 -0800
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
snip...
[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.
[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?
[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]
[host Richard] could you repeat the question?
[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?
[not sure whom ask this] what group are you with?
[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.
[not sure who is speaking] could you please disconnect Mr. Singeltary
[TSS] you are not going to answer my question?
[not sure whom speaking] NO
snip...see full archive and more of this;
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.
*** It also suggests a similar cause or source for atypical BSE in these countries. ***
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. ***
see page 176 of 201 pages...tss
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;
2020
MONDAY, JULY 6, 2020
Guidance for reporting 2020 surveillance data on Transmissible Spongiform Encephalopathies (TSE)
SUNDAY, OCTOBER 4, 2020
Cattle Meat and Offal Imported from the United States of America, Canada and Ireland to Japan (Prions) Food Safety Commission of Japan
THURSDAY, AUGUST 20, 2020
Why is USDA "only" BSE TSE Prion testing 25,000 samples a year?
ZOONOSIS OF SCRAPIE TSE PRION
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.
==============
***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
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion... Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.
To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.
These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.
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.
***> why do we not want to do TSE transmission studies on chimpanzees $
5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man.
***> I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough.
***> Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
snip...
R. BRADLEY
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.
***> 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. <***
Transmission of scrapie prions to primate after an extended silent incubation period
Emmanuel E. Comoy, Jacqueline Mikol, Sophie Luccantoni-Freire, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Valérie Durand, Capucine Dehen, Olivier Andreoletti, Cristina Casalone, Juergen A. Richt, Justin J. Greenlee, Thierry Baron, Sylvie L. Benestad, Paul Brown & Jean-Philippe Deslys Scientific Reports volume 5, Article number: 11573 (2015) | Download Citation
Abstract
Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period, with features similar to those reported for human cases of sporadic CJD. Scrapie is thus actually transmissible to primates with incubation periods compatible with their life expectancy, although fourfold longer than BSE. Long-term experimental transmission studies are necessary to better assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98 scrapie.
SNIP...
Discussion We describe the transmission of spongiform encephalopathy in a non-human primate inoculated 10 years earlier with a strain of sheep c-scrapie. Because of this extended incubation period in a facility in which other prion diseases are under study, we are obliged to consider two alternative possibilities that might explain its occurrence. We first considered the possibility of a sporadic origin (like CJD in humans). Such an event is extremely improbable because the inoculated animal was 14 years old when the clinical signs appeared, i.e. about 40% through the expected natural lifetime of this species, compared to a peak age incidence of 60–65 years in human sporadic CJD, or about 80% through their expected lifetimes. 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.
The second possibility is a laboratory cross-contamination. Three facts make this possibility equally unlikely. First, handling of specimens in our laboratory is performed with fastidious attention to the avoidance of any such cross-contamination. Second, no laboratory cross-contamination has ever been documented in other primate laboratories, including the NIH, even between infected and uninfected animals housed in the same or adjacent cages with daily intimate contact (P. Brown, personal communication). Third, the cerebral lesion profile is different from all the other prion diseases we have studied in this model19, with a correlation between cerebellar lesions (massive spongiform change of Purkinje cells, intense PrPres staining and reactive gliosis26) and ataxia. The iron deposits present in the globus pallidus are a non specific finding that have been reported previously in neurodegenerative diseases and aging27. Conversely, the thalamic lesion was reminiscent of a metabolic disease due to thiamine deficiency28 but blood thiamine levels were within normal limits (data not shown). The preferential distribution of spongiform change in cortex associated with a limited distribution in the brainstem is reminiscent of the lesion profile in MM2c and VV1 sCJD patients29, but interspecies comparison of lesion profiles should be interpreted with caution. It is of note that the same classical scrapie isolate induced TSE in C57Bl/6 mice with similar incubation periods and lesional profiles as a sample derived from a MM1 sCJD patient30.
We are therefore confident that the illness in this cynomolgus macaque represents a true transmission of a sheep c-scrapie isolate directly to an old-world monkey, which taxonomically resides in the primate subdivision (parvorder of catarrhini) that includes humans. With an homology of its PrP protein with humans of 96.4%31, cynomolgus macaque constitutes a highly relevant model for assessing zoonotic risk of prion diseases. Since our initial aim was to show the absence of transmission of scrapie to macaques in the worst-case scenario, we obtained materials from a flock of naturally-infected sheep, affecting animals with different genotypes32. This c-scrapie isolate exhibited complete transmission in ARQ/ARQ sheep (332 ± 56 days) and Tg338 transgenic mice expressing ovine VRQ/VRQ prion protein (220 ± 5 days) (O.. Andreoletti, personal communication). From the standpoint of zoonotic risk, it is important to note that sheep with c-scrapie (including the isolate used in our study) have demonstrable infectivity throughout their lymphoreticular system early in the incubation period of the disease (3 months-old for all the lymphoid organs, and as early as 2 months-old in gut-associated lymph nodes)33. In addition, scrapie infectivity has been identified in blood34, milk35 and skeletal muscle36 from asymptomatic but scrapie infected small ruminants which implies a potential dietary exposure for consumers.
Two earlier studies have reported the occurrence of clinical TSE in cynomolgus macaques after exposures to scrapie isolates. In the first study, the “Compton” scrapie isolate (derived from an English sheep) and serially propagated for 9 passages in goats did not transmit TSE in cynomolgus macaque, rhesus macaque or chimpanzee within 7 years following intracerebral challenge1; conversely, after 8 supplementary passages in conventional mice, this “Compton” isolate induced TSE in a cynomolgus macaque 5 years after intracerebral challenge, but rhesus macaques and chimpanzee remained asymptomatic 8.5 years post-exposure8. However, multiple successive passages that are classically used to select laboratory-adapted prion strains can significantly modify the initial properties of a scrapie isolate, thus questioning the relevance of zoonotic potential for the initial sheep-derived isolate. The same isolate had also induced disease into squirrel monkeys (new-world monkey)9. A second historical observation reported that a cynomolgus macaque developed TSE 6 years post-inoculation with brain homogenate from a scrapie-infected Suffolk ewe (derived from USA), whereas a rhesus macaque and a chimpanzee exposed to the same inoculum remained healthy 9 years post-exposure1. This inoculum also induced TSE in squirrel monkeys after 4 passages in mice. Other scrapie transmission attempts in macaque failed but had more shorter periods of observation in comparison to the current study. Further, it is possible that there are differences in the zoonotic potential of different scrapie strains.
The most striking observation in our study is the extended incubation period of scrapie in the macaque model, which has several implications. Firstly, our observations constitute experimental evidence in favor of the zoonotic potential of c-scrapie, at least for this isolate that has been extensively studied32,33,34,35,36. The cross-species zoonotic ability of this isolate should be confirmed by performing duplicate intracerebral exposures and assessing the transmissibility by the oral route (a successful transmission of prion strains through the intracerebral route may not necessarily indicate the potential for oral transmission37). However, such confirmatory experiments may require more than one decade, which is hardly compatible with current general management and support of scientific projects; thus this study should be rather considered as a case report.
Secondly, transmission of c-BSE to primates occurred within 8 years post exposure for the lowest doses able to transmit the disease (the survival period after inoculation is inversely proportional to the initial amount of infectious inoculum). The occurrence of scrapie 10 years after exposure to a high dose (25 mg) of scrapie-infected sheep brain suggests that the macaque has a higher species barrier for sheep c-scrapie than c-BSE, although it is notable that previous studies based on in vitro conversion of PrP suggested that BSE and scrapie prions would have a similar conversion potential for human PrP38.
Thirdly, prion diseases typically have longer incubation periods after oral exposure than after intracerebral inoculations: since humans can develop Kuru 47 years after oral exposure39, an incubation time of several decades after oral exposure to scrapie would therefore be expected, leading the disease to occur in older adults, i.e. the peak age for cases considered to be sporadic disease, and making a distinction between scrapie-associated and truly sporadic disease extremely difficult to appreciate.
Fourthly, epidemiologic evidence is necessary to confirm the zoonotic potential of an animal disease suggested by experimental studies. A relatively short incubation period and a peculiar epidemiological situation (e.g., all the first vCJD cases occurring in the country with the most important ongoing c-BSE epizootic) led to a high degree of suspicion that c-BSE was the cause of vCJD. Sporadic CJD are considered spontaneous diseases with an almost stable and constant worldwide prevalence (0.5–2 cases per million inhabitants per year), and previous epidemiological studies were unable to draw a link between sCJD and classical scrapie6,7,40,41, even though external causes were hypothesized to explain the occurrence of some sCJD clusters42,43,44. However, extended incubation periods exceeding several decades would impair the predictive values of epidemiological surveillance for prion diseases, already weakened by a limited prevalence of prion diseases and the multiplicity of isolates gathered under the phenotypes of “scrapie” and “sporadic CJD”.
Fifthly, considering this 10 year-long incubation period, together with both laboratory and epidemiological evidence of decade or longer intervals between infection and clinical onset of disease, no premature conclusions should be drawn from negative transmission studies in cynomolgus macaques with less than a decade of observation, as in the aforementioned historical transmission studies of scrapie to primates1,8,9. Our observations and those of others45,46 to date are unable to provide definitive evidence regarding the zoonotic potential of CWD, atypical/Nor98 scrapie or H-type BSE. The extended incubation period of the scrapie-affected macaque in the current study also underscores the limitations of rodent models expressing human PrP for assessing the zoonotic potential of some prion diseases since their lifespan remains limited to approximately two years21,47,48. This point is illustrated by the fact that the recently reported transmission of scrapie to humanized mice was not associated with clinical signs for up to 750 days and occurred in an extreme minority of mice with only a marginal increase in attack rate upon second passage13. The low attack rate in these studies is certainly linked to the limited lifespan of mice compared to the very long periods of observation necessary to demonstrate the development of scrapie. Alternatively, one could estimate that a successful second passage is the result of strain adaptation to the species barrier, thus poorly relevant of the real zoonotic potential of the original scrapie isolate of sheep origin49. The development of scrapie in this primate after an incubation period compatible with its lifespan complements the study conducted in transgenic (humanized) mice; taken together these studies suggest that some isolates of sheep scrapie can promote misfolding of the human prion protein and that scrapie can develop within the lifespan of some primate species.
In addition to previous studies on scrapie transmission to primate1,8,9 and the recently published study on transgenic humanized mice13, our results constitute new evidence for recommending that the potential risk of scrapie for human health should not be dismissed. Indeed, human PrP transgenic mice and primates are the most relevant models for investigating the human transmission barrier. To what extent such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown.. During the past decades, many protective measures have been successfully implemented to protect cattle from the spread of c-BSE, and some of these measures have been extended to sheep and goats to protect from scrapie according to the principle of precaution. Since cases of c-BSE have greatly reduced in number, those protective measures are currently being challenged and relaxed in the absence of other known zoonotic animal prion disease. We recommend that risk managers should be aware of the long term potential risk to human health of at least certain scrapie isolates, notably for lymphotropic strains like the classical scrapie strain used in the current study. Relatively high amounts of infectivity in peripheral lymphoid organs in animals infected with these strains could lead to contamination of food products produced for human consumption. Efforts should also be maintained to further assess the zoonotic potential of other animal prion strains in long-term studies, notably lymphotropic strains with high prevalence like CWD, which is spreading across North America, and atypical/Nor98 scrapie (Nor98)50 that was first detected in the past two decades and now represents approximately half of all reported cases of prion diseases in small ruminants worldwide, including territories previously considered as scrapie free... 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.
Friday, December 14, 2012
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012
snip.....
In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.
Animals considered at high risk for CWD include:
1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and
2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.
Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.
The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.
Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.
There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.
snip.....
36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).
The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).
Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.
snip.....
The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).
snip.....
In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.
snip.....
In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible... For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.
snip.....
Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.
snip.....
TUESDAY, OCTOBER 29, 2019
America BSE 589.2001 FEED REGULATIONS, BSE SURVEILLANCE, BSE TESTING, and CJD TSE Prion
the feds just released this statement and you should read this very carefully about the mad cow feed ban that never was, and still isn't, and why this is so important, since USDA APHIS ARS Scientist recent transmitted Chronic Wasting Disease CWD TSE Prion, BY ORAL ROUTES, to PIGS AND SHEEP. this is terrible news, and proves the mad cow feed ban never worked, especially since it really never existed;
ponder this; ***> Adriano Aguzzi...''We even showed that a prion AEROSOL will infect 100% of mice within 10 seconds of exposure''
SUNDAY, SEPTEMBER 1, 2019
FDA Reports on VFD Compliance
Before and after the current Veterinary Feed Directive (VFD) rules took full effect in January, 2017, the FDA focused primarily on education and outreach to help feed mills, veterinarians and producers understand and comply with the requirements. Since then, FDA has gradually increased the number of VFD inspections and initiated enforcement actions when necessary.
***> cattle, pigs, sheep, cwd, tse, prion, oh my!
***> In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006).
Sheep and cattle may be exposed to CWD via common grazing areas with affected deer but so far, appear to be poorly susceptible to mule deer CWD (Sigurdson, 2008). In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). It is not known how susceptible humans are to CWD but given that the prion can be present in muscle, it is likely that humans have been exposed to the agent via consumption of venison (Sigurdson, 2008). Initial experimental research suggests that human susceptibility to CWD is low and there may be a robust species barrier for CWD transmission to humans (Sigurdson, 2008), however the risk appetite for a public health threat may still find this level unacceptable.
TUESDAY, APRIL 18, 2017
*** EXTREME USA FDA PART 589 TSE PRION FEED LOOP HOLE STILL EXIST, AND PRICE OF POKER GOES UP ***
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
Date: Fri, 18 Oct 2002 23:12:22 +0100
From: Steve Dealler
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
To: BSE-L@ References: <3daf5023 .4080804="" wt.net="">
Dear Terry,
An excellent piece of review as this literature is desparately difficult to get back from Government sites.
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!
Steve Dealler ===============
BSE Inquiry Steve Dealler
Management In Confidence
BSE: Private Submission of Bovine Brain Dealler
snip...see full text;
MONDAY, FEBRUARY 25, 2019
***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019
MONDAY, DECEMBER 16, 2019
Chronic Wasting Disease CWD TSE Prion aka mad cow type disease in cervid Zoonosis Update
***> ''In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
What if?
> However, to date, no CWD infections have been reported in people.
key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
Chronic Wasting Disease CWD TSE Prion aka mad deer disease zoonosis
We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence;
(3) Reliable essays can be established to detect CWD infection in humans; and
(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.
ZOONOTIC CHRONIC WASTING DISEASE CWD TSE PRION UPDATE
Prion 2017 Conference
First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1
University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen
This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves.
Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice.
At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation.
PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS
PRION 2018 CONFERENCE
Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice
Hermann M. Schatzl, Samia Hannaoui, Yo-Ching Cheng, Sabine Gilch (Calgary Prion Research Unit, University of Calgary, Calgary, Canada) Michael Beekes (RKI Berlin), Walter Schulz-Schaeffer (University of Homburg/Saar, Germany), Christiane Stahl-Hennig (German Primate Center) & Stefanie Czub (CFIA Lethbridge).
To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years.
After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were detected in spinal cord and brain of some euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and pre-clinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles.
Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate.
The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.
Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP.
Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP.
The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD..
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***
READING OVER THE PRION 2018 ABSTRACT BOOK, LOOKS LIKE THEY FOUND THAT from this study ;
P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States
Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1) (1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA..
SEEMS THAT THEY FOUND Highly endemic states had a higher rate of prion disease mortality compared to non-CWD
states.
states.
AND ANOTHER STUDY;
P172 Peripheral Neuropathy in Patients with Prion Disease
Wang H(1), Cohen M(1), Appleby BS(1,2) (1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio..
IN THIS STUDY, THERE WERE autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017,
AND
included 104 patients. SEEMS THEY FOUND THAT The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%),
AND
THAT The Majority of cases were male (60%), AND half of them had exposure to wild game.
snip…
see more on Prion 2017 Macaque study from Prion 2017 Conference and other updated science on cwd tse prion zoonosis below…terry
PRION 2019 ABSTRACTS
1. Interspecies transmission of the chronic wasting disease agent
Justin Greenlee
Virus and Prion Research Unit, National Animal Disease Center, USDA Agriculture Research Service
ABSTRACT
The presentation will summarize the results of various studies conducted at our research center that assess the transmissibility of the chronic wasting disease (CWD) agent to cattle, pigs, raccoons, goats, and sheep. This will include specifics of the relative attack rates, clinical signs, and microscopic lesions with emphasis on how to differentiate cross-species transmission of the CWD agent from the prion diseases that naturally occur in hosts such as cattle or sheep. Briefly, the relative difficulty of transmitting the CWD agent to sheep and goats will be contrasted with the relative ease of transmitting the scrapie agent to white-tailed deer.
53. Evaluation of the inter-species transmission potential of different CWD isolates
Rodrigo Moralesa, Carlos Kramma,b, Paulina Sotoa, Adam Lyona, Sandra Pritzkowa, Claudio Sotoa
aMitchell Center for Alzheimer’s disease and Related Brain Disorders, Dept. of Neurology, McGovern School of Medicine University of Texas Health Science Center at Houston, TX, USA; bFacultad de Medicina, Universidad de los Andes, Santiago, Chile
ABSTRACT
Chronic Wasting Disease (CWD) has reached epidemic proportions in North America and has been identified in South Korea and Northern Europe. CWD-susceptible cervid species are known to share habitats with humans and other animals entering the human food chain. At present, the potential of CWD to infect humans and other animal species is not completely clear. The exploration of this issue acquires further complexity considering the differences in the prion protein sequence due to species-specific variations and polymorphic changes within species. While several species of cervids are naturally affected by CWD, white-tailed deer (WTD) is perhaps the most relevant due to its extensive use in hunting and as a source of food. Evaluation of inter-species prion infections using animals or mouse models is costly and time consuming. We and others have shown that the Protein Misfolding Cyclic Amplification (PMCA) technology reproduces, in an accelerated and inexpensive manner, the inter-species transmission of prions while preserving the strain features of the input PrPSc. In this work, we tested the potential of different WTD-derived CWD isolates to transmit to humans and other animal species relevant for human consumption using PMCA. For these experiments, CWD isolates homozygous for the most common WTD-PrP polymorphic changes (G96S) were used (96SS variant obtained from a pre-symptomatic prion infected WTD). Briefly, 96GG and 96SS CWD prions were adapted in homologous or heterologous substrate by PMCA through several (15) rounds. End products, as well as intermediates across the process, were tested for their inter-species transmission potentials. A similar process was followed to assess seed-templated misfolding of ovine, porcine, and bovine PrPC. Our results show differences on the inter-species transmission potentials of the four adapted materials generated (PrPC/PrPSc polymorphic combinations), being the homologous combinations of seed/substrate the ones with the greater apparent zoonotic potential. Surprisingly, 96SS prions adapted in homologous substrate were the ones showing the easiest potential to template PrPC misfolding from other animal species. In summary, our results show that a plethora of different CWD isolates, each comprising different potentials for inter-species transmission, may exist in the environment. These experiments may help to clarify an uncertain and potentially worrisome public health issue. Additional research in this area may be useful to advise on the design of regulations intended to stop the spread of CWD and predict unwanted zoonotic events.
56. Understanding chronic wasting disease spread potential for at-risk species
Catherine I. Cullingham, Anh Dao, Debbie McKenzie and David W. Coltman
Department of Biological Sciences, University of Alberta, Edmonton AB, Canada
CONTACT Catherine I. Cullingham cathy.cullingham@ualberta.ca
ABSTRACT
Genetic variation can be linked to susceptibility or resistance to a disease, and this information can help to better understand spread-risk in a population. Wildlife disease incidence is increasing, and this is resulting in negative impacts on the economy, biodiversity, and in some instances, human health. If we can find genetic variation that helps to inform which individuals are susceptible, then we can use this information on at-risk populations to better manage negative consequences. Chronic wasting disease, a fatal, transmissible spongiform encephalopathy of cervids (both wild and captive), continues to spread geographically, which has resulted in an increasing host-range. The disease agent (PrPCWD) is a misfolded conformer of native cellular protein (PrPC). In Canada, the disease is endemic in Alberta and Saskatchewan, infecting primarily mule deer and white-tail deer, with a smaller impact on elk and moose populations. As the extent of the endemic area continues to expand, additional species will be exposed to this disease, including bison, bighorn sheep, mountain goat, and pronghorn antelope. To better understand the potential spread-risk among these species, we reviewed the current literature on species that have been orally exposed to CWD to identify susceptible and resistant species. We then compared the amino acid polymorphisms of PrPC among these species to determine whether any sites were linked to susceptibility or resistance to CWD infection. We sequenced the entire PrP coding region in 578 individuals across at-risk populations to evaluate their potential susceptibility. Three amino acid sites (97, 170, and 174; human numbering) were significantly associated with susceptibility, but these were not fully discriminating. All but one species among the resistant group shared the same haplotype, and the same for the susceptible species. For the at-risk species, bison had the resistant haplotype, while bighorn sheep and mountain goats were closely associated with the resistant type. Pronghorn antelope and a newly identified haplotype in moose differed from the susceptible haplotype, but were still closely associated with it. These data suggest pronghorn antelope will be susceptible to CWD while bison are likely to be resistant. Based on this data, recommendations can be made regarding species to be monitored for possible CWD infection.
KEYWORDS: Chronic wasting disease; Prnp; wildlife disease; population genetics; ungulates
Thursday, May 23, 2019
Prion 2019 Emerging Concepts CWD, BSE, SCRAPIE, CJD, SCIENTIFIC PROGRAM Schedule and Abstracts
see full Prion 2019 Conference Abstracts
THURSDAY, OCTOBER 04, 2018
Cervid to human prion transmission 5R01NS088604-04 Update
snip…full text;
SATURDAY, FEBRUARY 09, 2019
Experts: Yes, chronic wasting disease in deer is a public health issue — for people
SATURDAY, FEBRUARY 23, 2019
Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019
TUESDAY, NOVEMBER 04, 2014
Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011
Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "
Transmission Studies
Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS
resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.
snip....
Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿
Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations
In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.
*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From: Sent: Sunday, September 29, 2002 10:15 AM
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
> However, to date, no CWD infections have been reported in people.
sporadic, spontaneous CJD, 85%+ of all human TSE, just not just happen. never in scientific literature has this been proven.
if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;
sporadic = 54,983 hits https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic
spontaneous = 325,650 hits https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous
key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
FRIDAY, JULY 26, 2019
Chronic Wasting Disease in Cervids: Implications for Prion Transmission to Humans and Other Animal Species
TUESDAY, JANUARY 21, 2020
***> 2004 European Commission Chronic wasting disease AND TISSUES THAT MIGHT CARRY A RISK FOR HUMAN FOOD AND ANIMAL FEED CHAINS REPORT UPDATED 2020
***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***
***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<***
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***
***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<***
***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
SATURDAY, OCTOBER 24, 2020
Texas Kimble County Farm Chronic Wasting Disease CWD TSE Prion Approximate Herd Prevalence 12%
SUNDAY, AUGUST 30, 2020
Texas CWD TSE Prion 3 More Documented, 185 Cases To Date
SATURDAY, AUGUST 08, 2020
TAHC Chapter 40, Chronic Wasting Disease Singeltary Comment Submission August 8, 2020
FRIDAY, AUGUST 7, 2020
National List of Reportable Animal Diseases (NLRAD) proposed rule CWD, Scrapie, BSE, TSE, Prion Disease Singeltary Submission Docket APHIS-2017-0002
SATURDAY, OCTOBER 10, 2020
Utah As of October 7, 2020, 118 mule deer and two elk have tested positive for CWD TSE Prion
FRIDAY, OCTOBER 09, 2020
Maryland detects additional 28 positives from last year's CWD TSE Prion sampling, total stands at 80 confirmed cases to date
THURSDAY, SEPTEMBER 24, 2020
ARKANSAS CHRONIC WASTING DISEASE CWD TSE PRION UPDATE 845 Cases Positive To Date
FRIDAY, JANUARY 24, 2020
Arkansas Chronic Wasting Disease CWD TSE Prion FY2020 211 Positive Cases as of January 17, 2020
SUNDAY, JANUARY 05, 2020
Arkansas Chronic Wasting Disease CWD TSE Prion 2019 to 2020 Totals As Of December 3, 2019 399 Confirmed with more pending results
SUNDAY, SEPTEMBER 20, 2020
Wisconsin Sinks Further Into the Abyss With CWD TSE Prion 2020
THURSDAY, OCTOBER 08, 2020
Wyoming Chronic wasting disease 2020 surveillance and monitoring
FRIDAY, SEPTEMBER 18, 2020
CWD found in new deer and elk hunt areas in northeast Wyoming
TUESDAY, SEPTEMBER 22, 2020
Michigan CWD TSE Prion 189 Positive To Date UPDATE September 2020
SATURDAY, JANUARY 04, 2020
Mississippi CWD TOTALS JUST ABOUT DOUBLE Since October 1, 2019 To Date Statewide Total is 37 Confirmed
WEDNESDAY, MAY 06, 2020
Missouri 46 new cases Chronic Wasting Disease found, total to date at 162 documented CWD
SUNDAY, JANUARY 19, 2020
Missouri CWD TSE Prion 2019-2020 SAMPLING RESULTS TO DATE 25 Positive
THURSDAY, JANUARY 02, 2020
Missouri MDC officially reports more than 20 new cases of Chronic Wasting Disease CWD TSE Prion
WEDNESDAY, OCTOBER 21, 2020
Montana 18 deer test positive for chronic wasting disease CWD TSE Prion
CWD positives from across the state, no new areas
TUESDAY, MAY 19, 2020
Montana White-tailed deer in Gallatin County suspected positive for CWD
FRIDAY, FEBRUARY 07, 2020
Montana 142 animals tested positive for CWD thus far during 2019/20 sampling
FRIDAY, JANUARY 17, 2020
Montana Moose Tests Positive for Chronic Wasting Disease CWD TSE PRION in Libby Area
Montana Fish, Wildlife & Parks 2019 CWD Surveillance Hunter Test Results CWD TSE PRION LOOKS LIKE 136 POSITIVE SO FAR, count them up...
WEDNESDAY, DECEMBER 25, 2019
Montana 16 more deer positive for CWD first time positive hunting district 705 in southeast
WEDNESDAY, MARCH 25, 2020
Michigan CWD TSE Prion Total Suspect Positive Deer Moves Up To 188 with total deer tested 80,687 to date
THURSDAY, JANUARY 30, 2020
Michigan CWD TSE Prion Total Suspect Positive Deer Jumps To 181 to date
MONDAY, JANUARY 27, 2020
updated
Michigan CWD TSE Prion MDARD 3 positive white-tailed deer from a Newaygo County deer farm depopulation and quarantine efforts update?
SUNDAY, DECEMBER 22, 2019
Illinois CWD TSE Prion 90 CWD-positive deer with 826 confirmed positive Total positives through June 30, 2019
TUESDAY, FEBRUARY 11, 2020
Missouri MDC 2019-2020 SAMPLING RESULTS CWD TSE PRION TO DATE 28 Positive
SUNDAY, APRIL 12, 2020
PENNSYLVANIA REVISED CWD RESPONSE PLAN DRAFT AVAILABLE FOR REVIEW
WEDNESDAY, MARCH 04, 2020
Politicians State Rep. David Maloney, R-Berks Helping to Spread Chronic Wasting Disease CWD TSE Prion
FRIDAY, OCTOBER 16, 2020
Minnesota CWD TSE Prion confirmed in Houston County farmed deer herd
SATURDAY, MARCH 14, 2020
Minnesota 4 More Farmed Deer and 1 wild positive for CWD TSE Prion
FRIDAY, FEBRUARY 28, 2020
Virginia DGIF say 21 new cases of CWD TSE Prion confirmed in white-tailed deer in northwest Virginia throughout 2019
TUESDAY, MARCH 03, 2020
North Dakota Eight deer taken during the 2019 deer gun season tested positive for chronic wasting disease CWD TSE Prion
TUESDAY, FEBRUARY 11, 2020
South Dakota Chronic Wasting Disease CWD TSE Prion Detected in New Areas
MONDAY, FEBRUARY 10, 2020
Iowa CWD TSE Prion 2019/20 (confirmed or suspect) 43 cases to date Wild Cervid
SATURDAY, FEBRUARY 01, 2020
Colorado confirmed CWD TSE Prion in 24 game management units in the state where it previously hadn’t been found
WEDNESDAY, JANUARY 29, 2020
Utah CWD TSE Prion Since July 1, 2019, the DWR confirmed 16 positive deer statewide Six of those, including Coal, were in the La Sal Unit, 59 test pending
TUESDAY, JANUARY 28, 2020
Mississippi MDWFP North MS CWD Management Zone Since October 2019, 25 CWD-positive deer have been detected from this zone
SATURDAY, JANUARY 25, 2020
Tennessee 2019-20 deer season 462 CWD TSE Prion Confirmed To Date
Fri, Jan 24, 2020 2:29 pm
Wyoming Game & Fish Discovers CWD-Positive Mule Deer in Pinedale, Discourages Feeding of Wildlife
''As of September 2019, CWD has been identified in 31 of 37 (84%) Wyoming mule deer herds, nine of 36 (25%) elk herds, and generally wherever white-tailed deer occur. Increasing prevalence and distribution of CWD has the potential to cause widespread and long-term negative impacts to Wyoming’s cervid populations. Prevalence of this disease in chronically infected Wyoming deer herds has exceeded 40%, with one elk herd exhibiting nearly 15% prevalence.''
''for the first time, there is clear evidence that CWD is adversely affecting the overall health and viability of some herds.''
MONDAY, FEBRUARY 03, 2020
Montana Chronic Wasting Disease CWD TSE Prion in Eastern Part of State Game Farm Elk
TUESDAY, JANUARY 07, 2020
Oklahoma Farmed Elk Lincoln County CWD Depopulation 3 Positive Elk with 1 Additional Dead Trace Out Confirmed Positive
THURSDAY, JULY 30, 2020
Ohio Deer Summary 2019 - 2020 CWD TSE Prion 24 Confirmed To Date All Captive Cervid
WEDNESDAY, JANUARY 29, 2020
Pennsylvania CWD TSE Prion 2019-20 hunting seasons as of January 14, 148 of the samples had tested positive for CWD in Wild Deer
WEDNESDAY, JANUARY 29, 2020
Pennsylvania CWD TSE Prion 2019-20 hunting seasons as of January 14, 148 of the samples had tested positive for CWD in Wild Deer
SUNDAY, DECEMBER 22, 2019
Pennsylvania Steady Climb of CWD TSE Prion Confirms 250 Positive To Date In Wild Cervid As At September 12, 2019
Pennsylvania Captive Cervid Industry Total CWD TSE Prion ??? anyone's guess...
SUNDAY, OCTOBER 11, 2020
Michigan Chronic Wasting Disease CWD TSE Prion increases to 191 positive to date
TUESDAY, SEPTEMBER 22, 2020
Michigan CWD TSE Prion 189 Positive To Date UPDATE September 2020
MONDAY, JANUARY 27, 2020
Michigan CWD TSE Prion MDARD 3 positive white-tailed deer from a Newaygo County deer farm depopulation and quarantine efforts update?
TUESDAY, JANUARY 07, 2020
Michigan Total CWD TSE Prion Positive Suspect-Positive Deer Jump To 174 confirmed to date
TUESDAY, JANUARY 14, 2020
Michigan MDARD has confirmed chronic wasting disease (CWD) in 3 white-tailed deer from a Newaygo County deer farm
SATURDAY, JANUARY 25, 2020
Tennessee 2019-20 deer season 462 CWD TSE Prion Confirmed To Date
FRIDAY, JANUARY 24, 2020
Wyoming Game & Fish Discovers CWD-Positive Mule Deer in Pinedale, Discourages Feeding of Wildlife
''As of September 2019, CWD has been identified in 31 of 37 (84%) Wyoming mule deer herds, nine of 36 (25%) elk herds, and generally wherever white-tailed deer occur. Increasing prevalence and distribution of CWD has the potential to cause widespread and long-term negative impacts to Wyoming’s cervid populations. Prevalence of this disease in chronically infected Wyoming deer herds has exceeded 40%, with one elk herd exhibiting nearly 15% prevalence.''
''for the first time, there is clear evidence that CWD is adversely affecting the overall health and viability of some herds.''
THURSDAY, SEPTEMBER 24, 2020
ARKANSAS CHRONIC WASTING DISEASE CWD TSE PRION UPDATE 845 Cases Positive To Date
FRIDAY, JANUARY 24, 2020
Arkansas Chronic Wasting Disease CWD TSE Prion FY2020 211 Positive Cases as of January 17, 2020
SUNDAY, JANUARY 05, 2020
Arkansas Chronic Wasting Disease CWD TSE Prion 2019 to 2020 Totals As Of December 3, 2019 399 Confirmed with more pending results
WEDNESDAY, JANUARY 29, 2020
Utah CWD TSE Prion Since July 1, 2019, the DWR confirmed 16 positive deer statewide Six of those, including Coal, were in the La Sal Unit, 59 test pending
FRIDAY, JANUARY 17, 2020
North Dakota 11 Positive Chronic Wasting Disease CWD TSE Prion detected since Sept 1, 2019
TUESDAY, JANUARY 21, 2020
Minnesota CWD update test results from deer harvested in the 2019 hunting season and the special hunts have returned 27 wild deer tested positive for CWD all from the southeast DMZ
FRIDAY, JANUARY 10, 2020
Minnesota Investigation leads to additional CWD positive deer on Pine County farm
THURSDAY, JANUARY 23, 2020
Canadian Food Inspection Agency (CFIA) has updated the following chapter of the Accredited Veterinarian's Manual: Chapter 13 Chronic Wasting Disease Herd Certification Programs
TUESDAY, JANUARY 21, 2020
2004 European Commission Chronic wasting disease AND TISSUES THAT MIGHT CARRY A RISK FOR HUMAN FOOD AND ANIMAL FEED CHAINS REPORT UPDATED 2020
SUNDAY, FEBRUARY 09, 2020
Management of chronic wasting disease in ranched elk: conclusions from a longitudinal three-year study
Although the herd owners were presented with additional management directives, including culling of CWD positive bulls and those animals positive by an amplification assay (RT-QuIC), they were not implemented due to concern regarding its potential impact on hunting revenue.
FRIDAY, DECEMBER 06, 2019
Estimating relative CWD susceptibility and disease progression in farmed white-tailed deer with rare PRNP alleles
THURSDAY, DECEMBER 19, 2019
TSE surveillance statistics exotic species and domestic cats Update December 2019
TUESDAY, AUGUST 18, 2020
Sheep Scrapie, Bovine BSE, Cervid CWD, ZOONOSIS, TSE Prion Roundup August 18, 2020
Mad Camel Disease CPD TSE Prion dromedary camels (Camelus dromedarius) is spreading
In 2018 prion disease was detected in camels at an abattoir in Algeria for the first time.
Prion disease has recently been confirmed in three dromedary camels (Camelus dromedarius) from an Algerian slaughterhouse (Babelhadj et al., 2018) after clinical signs compatible with those of TSEs in other species were observed ante mortem. Disease associated pathological changes or prion protein were found in brain by Western blotting, histology, immunohistochemistry (IHC) and paraffin-embedded tissue blot; PrPSc was also detected in the lymph nodes of the one camel tested by IHC.
Information gathered from breeders and slaughterhouse personnel suggests that similar clinical signs had been observed since the 1980s (Babelhadj et al., 2018). Subsequently, the disease has also been reported in a single case of a 12 year old dromedary camel from the region of Tataouine, Tunisia (Agrimi, 2019; OIE bulletin 2019).
There are many knowledge gaps about the biological characteristics of this new TSE, termed camel prion disease (CPD). Detection of infection in lymph nodes of one animal suggests extra-neural pathogenesis and, therefore, potential transmission of CPD between animals similar to that of classical scrapie and CWD. Such transmission of CPD could be facilitated over long distances by the traditional nomadic herding practices of dromedaries and the trade patterns between Algeria and other countries in North Africa and the Middle East (Bouslikhane, 2015). In light of the devastation caused by BSE, and its subsequent zoonotic transmission, CPD was used here to assess the probability of entry of a novel prion disease agent into the UK via livestock and livestock products. The approach used was to assess the aggregated probability, using the number of imports per year to avoid potential under-estimation as has previously been described (Kelly et al., 2018). Of note, the zoonotic potential of the disease is unknown and this assessment is of the probability of introduction of the CPD agent into the UK only, not of any onward transmission to humans or animals.
snip...
3. Results
3.1. Risk assessment
3.1.1. Probability camel is infected with camel prion disease in exporting country (p1)
Detection of abnormal neurological signs since the 1980s within a restricted geographical area of Algeria suggests that the expansion of CPD to other areas (and countries) may be restricted or that the disease can remain largely undiagnosed. According to a recent presentation of the Mediterranean Animal Health Network, the disease was also reported in Tunisia and the incidence in the initial region of Algeria was described as ‘rapidly and progressively increasing’ (Agrimi, 2019). It is, therefore, possible that movement of camels has allowed infected animals to enter other countries. Asides from the legal trade of camels, approximately 268 million people in Africa practice some form of pastoralism (Luizza, 2017). For example, over 95% of cross-border trade within the Horn of Africa is unofficial and carried out by nomadic pastoralists trading livestock. Given that disease was first noticed in the 1980s and the nomadic way of life in this area, exporting countries were therefore considered as those making up the regions of North Africa and the Middle East for the purpose of this assessment.
Twenty of 937 camels in 2015 and 51 of 1,322 in 2016 showed neurologic signs at slaughter giving an overall estimated apparent prevalence of 3.1% in dromedaries brought for slaughter (Babelhadj et al., 2018). In the absence of further information including confirmatory testing, an assumption was made that the prevalence of CPD in live camels in the regions of interest was high with high uncertainty because of the lack of testing data from countries other than Algeria and in only 3 camels in Algeria itself.
see full report;
Assessing the aggregated probability of entry of a novel prion disease agent into the United Kingdom
Monday, September 14, 2020
Assessing the aggregated probability of entry of a novel prion disease agent into the United Kingdom
Tuesday, September 15, 2020
Mad Camel Disease CPD TSE Prion dromedary camels (Camelus dromedarius) is spreading
CDC
New Outbreak of TSE Prion in NEW LIVESTOCK SPECIES
Mad Camel Disease
Volume 24, Number 6—June 2018 Research
Prion Disease in Dromedary Camels, Algeria Abstract
Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE). After the BSE epidemic, and the associated human infections, began in 1996 in the United Kingdom, general concerns have been raised about animal prions. We detected a prion disease in dromedary camels (Camelus dromedarius) in Algeria. Symptoms suggesting prion disease occurred in 3.1% of dromedaries brought for slaughter to the Ouargla abattoir in 2015–2016. We confirmed diagnosis by detecting pathognomonic neurodegeneration and disease-specific prion protein (PrPSc) in brain tissues from 3 symptomatic animals. Prion detection in lymphoid tissues is suggestive of the infectious nature of the disease. PrPSc biochemical characterization showed differences with BSE and scrapie. Our identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of the potential risks to human and animal health.
SNIP...
The possibility that dromedaries acquired the disease from eating prion-contaminated waste needs to be considered.
Tracing the origin of prion diseases is challenging. In the case of CPD, the traditional extensive and nomadic herding practices of dromedaries represent a formidable factor for accelerating the spread of the disease at long distances, making the path of its diffusion difficult to determine. Finally, the major import flows of live animals to Algeria from Niger, Mali, and Mauritania (27) should be investigated to trace the possible origin of CPD from other countries.
Camels are a vital animal species for millions of persons globally. The world camel population has a yearly growth rate of 2.1% (28). In 2014, the population was estimated at ≈28 million animals, but this number is probably underestimated.. Approximately 88% of camels are found in Africa, especially eastern Africa, and 12% are found in Asia. Official data reported 350,000 dromedaries in Algeria in 2014 (28).
On the basis of phenotypic traits and sociogeographic criteria, several dromedary populations have been suggested to exist in Algeria (29). However, recent genetic studies in Algeria and Egypt point to a weak differentiation of the dromedary population as a consequence of historical use as a cross-continental beast of burden along trans-Saharan caravan routes, coupled with traditional extensive/nomadic herding practices (30).
Such genetic homogeneity also might be reflected in PRNP. Studies on PRNP variability in camels are therefore warranted to explore the existence of genotypes resistant to CPD, which could represent an important tool for CPD management as it was for breeding programs for scrapie eradication in sheep. In the past 10 years, the camel farming system has changed rapidly, with increasing setup of periurban dairy farms and dairy plants and diversification of camel products and market penetration (13). This evolution requires improved health standards for infectious diseases and, in light of CPD, for prion diseases.
The emergence of another prion disease in an animal species of crucial importance for millions of persons worldwide makes it necessary to assess the risk for humans and develop evidence-based policies to control and limit the spread of the disease in animals and minimize human exposure. The implementation of a surveillance system for prion diseases would be a first step to enable disease control and minimize human and animal exposure. Finally, the diagnostic capacity of prion diseases needs to be improved in all countries in Africa where dromedaries are part of the domestic livestock.
***> IMPORTS AND EXPORTS <***
***SEE MASSIVE AMOUNTS OF BANNED ANIMAL PROTEIN AKA MAD COW FEED IN COMMERCE USA DECADES AFTER POST BAN ***
WEDNESDAY, JUNE 10, 2020
Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice
Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice
Atypical BSE prions showed a modification in their zoonotic ability after adaptation to sheep-PrP producing agents able to infect TgMet129 and TgVal129, bearing features that make them indistinguishable of sporadic Creutzfeldt-Jakob disease prions.
our results clearly indicate that atypical BSE adaptation to an ovine-PrP sequence could modify the prion agent to potentially infect humans, showing strain features indistinguishable from those of classic sCJD prions, even though they might or might not be different agents.
However, the expanding range of TSE agents displaying the capacity to transmit in human-PrP–expressing hosts warrants the continuation of the ban on meat and bone meal recycling and underscores the ongoing need for active surveillance
A REVIEW of facts and science on scrapie zoonosis potential/likelihood and the USA incredible failure of the BSE 589.2001 FEED REGULATIONS (another colossal failure, and proven to be a sham)
1st up BSE 589.2001 FEED REGULATIONS
MONDAY, NOVEMBER 02, 2020
Successful transmission of the chronic wasting disease (CWD) agent to white-tailed deer by intravenous blood transfusion
***> There have been 6 cases of BSE identified in the United States. The following information provides descriptions of these 6 cases:
2018- Florida
WEDNESDAY, APRIL 24, 2019
***> USDA Announces FLORIDA Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019 <***
2017 – Alabama
TUESDAY, JULY 18, 2017
***> USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama
THURSDAY, JULY 20, 2017
***> USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589..200
2012 - California
2012 ATYPICAL L-TYPE BASE BSE TSE PRION CALIFORNIA ‘confirmed’ Saturday, August 4, 2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012
Summary Report BSE 2012
Executive Summary
Saturday, August 4, 2012
Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation
in the url that follows, I have posted
SRM breaches first, as late as 2011.
then
MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until 2007, when they ceased posting them.
then,
MAD COW SURVEILLANCE BREACHES.
Friday, May 18, 2012
Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States Friday May 18, 2012
2012 ATYPICAL L-TYPE BASE BSE TSE PRION CALIFORNIA ‘confirmed’ Saturday, August 4, 2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
SUNDAY, NOVEMBER 13, 2011
California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock
2006 - Alabama
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
THURSDAY, JULY 20, 2017
USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200
TUESDAY, JULY 18, 2017
USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama
WEDNESDAY, JULY 19, 2017
OIE REPORT Bovine spongiform encephalopathy United States of America
Date: February 4, 2004 at 10:53 am PST
Alabama man dies of Creutzfeldt-Jakob disease, Alabama does not have to report CJD
The Associated Press
A DeKalb County resident who died last year was diagnosed with a form of a rare illness sometimes linked to mad cow disease, but it was unclear how he got the infection.
Doctors determined that a man who died in November while under hospice care suffered from Creutzfeldt-Jakob disease, Coroner Tom Wilson said Tuesday. The disease was listed as the cause of death on the death certificate, he said.
Health officials said the illness kills a few people each year in Alabama, but there has never been any sign of a link with mad cow disease, which has drawn wide attention since the Dec. 23 announcement that a cow in Washington state had tested positive for it.
Wilson declined further comment and referred questions to New Beacon Hospice, which cared for the victim in DeKalb County. Mary Colley of New Beacon refused comment, citing patient confidentiality laws.
Officials with the DeKalb County Public Health Department and the area health office covering northeast Alabama said they were unaware of the case. Doctors are not required to report cases of Creutzfeldt-Jakob to the state.
WHNT-TV of Huntsville reported that the man was diagnosed with sporadic Creutzfeldt-Jakob disease, a designation given cases where the source of the infection was unknown.
Researchers believe there is a connection between mad cow disease, or bovine spongiform encephalopathy, and a variation of Creutzfeldt-Jakob disease, a fatal disorder that causes rapid dementia and loss of muscle control.
Sharon Thompson, a nurse with the epidemiology office of the Alabama Department of Public Health in Montgomery, said four to five people die each year in Alabama of Creutzfeldt-Jakob disease.
None of those deaths have been from the variant of the disease sometimes linked to mad cow disease, she said. "There are cases of it that occur naturally," said Thompson.
About one person in 1 million died of Creutzfeldt-Jakob annually in the United States from 1979 through 1994, according to statistics from the Centers for Disease Control and Prevention in Atlanta.
ALABAMA CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE REPORTING
***> cjd tse prion disease is not reportable apparently <***
2005 - Texas
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
THURSDAY, OCTOBER 22, 2015
Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened
HOW TO COVER UP MAD COW DISEASE IN TEXAS
FRIDAY, DECEMBER 14, 2018
MAD COW USA FLASHBACK FRIDAY DECEMBER 14, 2018
2003 - Washington State
WEDNESDAY, OCTOBER 21, 2020
Human Prion Disease Surveillance in Washington State, 2006-2017
MONDAY, OCTOBER 05, 2020
USA, UK, JAPAN, CJD TSE PRION STATISTICS UPDATE OCTOBER 2020
SATURDAY, SEPTEMBER 26, 2020
A nationwide trend analysis in the incidence and mortality of Creutzfeldt–Jakob disease in Japan between 2005 and 2014 with increasing trends of incidence and mortality
snip...
Overall, the AAPCs of age-adjusted CJD-associated mortality rates rose significantly over the study period (3.2%; 95% confidence interval [CI] 1.4–5.1%). The AAPC of the age-adjusted incidence rates also increased (overall 6.4%; 95% CI 4.7–8.1%). The CJD-associated increases in the mortality and incidence rates were especially prominent among adults over the age of 70 years. Given this trend in aging of population, the disease burden of CJD will continue to increase in severity. Our findings thus recommend that policymakers be aware of the importance of CJD and focus on preparing to address the increasing prevalence of dementia.
snip...
Volume 26, Number 8—August 2020
Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons
THURSDAY, JULY 02, 2020
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA Diagnosis and Reporting of Creutzfeldt-Jakob Disease
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.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
Terry S. Singeltary Sr.
